clinical cards 3/19/12

Smoking, Chronic bladder irritation (nephrolithiasis, UTI, etc), chemical exposures (cytoxan, amino biphenyl, naphthylamines), prior pelvic irradiation, Schistosoma haematobium infection (SCC only)

removal of bladder, distal ureters, pelvic peritoneum, prostate, seminal vesicles, uterus, fallopian tubes, ovaries, and ant vaginal wall
+ urinary diversion
+ bilateral LND

supine, with EMPTY BLADDER

indications for adj therapy (intravesical therapy): G2-3, T1, Tis, multifocal or residual dz

N1 single LN in true pelvis (obturator, hypogastric, ext iliac or presacral)
N2 mult LNs in true pelvis
N3 mets to common iliac

hydronephrosis
multifocality CIS
persistently + margin on TURBT
non-TCC histology
inappropriate bladder capacity and function
inability to tolerate chemo
trigone involvement
stage IV dz
pT3b-T4 (relative)

gemcitabine and cisplatin (alt MVAC, taxane, premetrexed)

~70% (Shipley data)

whole pelvis (bladder + LNs) to 40-55 Gy --> tumor boost to 64-66 Gy

70%

~15%

T0-1: 5%
T2: 18%
T3a: 26% --> T3b: 46%
T4: 42%

BCG (bacillus Calmette-Guerin): form of immunotherapy

5%

chemo x 2-3 +/- RT is administered --> restage (w/ cysto and CT); if resectable, pt should proceed to cystectomy; if not, consolidation chemo +/- RT

Y, randomized trials (below), results favor the surgery arms. Therefore consider preop RT to convert unresectable tumors

Danish National Cancer Group (Sell A et al.)

MDA RCT (Miller et al.)

8%; bone, lungs, or liver

38% w/ MVAC (Grossman et al. NEJM 2003)

All of the following:
1. completely resected
2. Ta
3. grade 1
4. no residual on urine cytology

short-term: urinary frequency/urgency, dysuria, UTIs,bladder spasms, loose stools, RT dermatitis, fatigue
long-term: hematuria, urinary frequency/urgency, dysuria, hematochezia, erectile dysfunction, increased risk of fracture, 2nd malignancies

IV ( I, II, III follow T1, 2, 3 (4a)

blacks and women have poorer prognosis

yes. Mult RCTs shown SURVIVAL benefit to neoadj chemo prior to radical cystectomy; metaanalysis ~ 5%

agents should be selected tailored to those histologies

overall ~50%
0: 98%
I: 88%
II: 63%
III: 46%
IV: 15%

Conventional Fields:
anterior field
Superior border: L5-S1 (s2-s3)
Inferior border: bottom of obturator foramen
lateral border: 1.5 cm of pelvic brim
block femoral heads
lateral field
Sup and Inf border same
Anterior border: 1.5-2 cm anterior to bladder
Posterior border: 2.5 cm posterior to the bladder
block anal canal and tissue above symphysis

Cystoscopy
TURBT w/ bimanual exam under anesthesia
urine cytology, evaluation of upper tract (IVP, renal u/s w/ retrograde pyelogram, CT urography, ureteroscopy, or MRI urogram) especially in setting of +cytology and negative cystoscopy
CT, MRI, bone scan (if elevated alk phos)

1 tumor grade
2 depth of invasion
3 stage
4 histologic subtype

vermilionectomy w/ adv of mucosal flap ("lip shave"); excision from vermilion to orbicularis mm

80% arise from lower gingiva

anterior: incisors
posterior: vertebral spinous processes
superior: 1.5 cm above dorsum of tongue
inferior: thyroid notch

lesion involves < 30% and T1 lesion and lesion does not involve the oral commissure (WLE w/ 0.5 cm margin)

tongue, FOM that are midline

+margin, +ECE, and/or PNI (based on Bernier and cooper)

> 2 cm, large lesions (> 50%), upper lip, or if lesion involves oral commissure

oral tongue can go to level IV

5% of oral tongue present w/ bilateral neck dz; if n1-n2 then 30%

lung > bone > liver

LDR: 60-70 Gy (40-60 cGy/hr); HDR: 60 Gy (5 Gy BID x 12 fx)

N1: single, ipsi < 3 cm
N2a: single, ipsi 3-6
N2b: mult, ipsi 3-6
N2c: bilat, < 6 cm
N3: > 6 cm

asymptomatic red/raised lesions, ill-fitting dentures, bleeding mass, pain, dysphagia (2/2 tongue fixation), trismus (pterygoids are involved), and otalgia

oral tongue

6 MV w/ 30 degree wedges (heels ant)

IVA - T4a or N2; IVB - T4b or N3; IVC - M1

I and II

20-30%

RT preferred: no bone erosion or if lesion extends to ant tonsillar pillar, soft palate, or buccal mucosa; Surgery preferred if bone erosion --> PORT

10-14 days before starting

H&P w/ palpation, direct endoscopy +bx, CBC, CMP, CT/MRI H&N, and CT or PET/CT (chest)

CN XII (hypoglossal) - right is affected

lateral undersurface

PORT: 54 - 66 Gy (+margins); definitive: 54 -> 70 Gy to gross dz (general given opposed lats but now IMRT (T3-T4)

tumor thickness > 3 mm, grade III Dz, + LVSI, and recurrent lesion

IA - submental; IB - submandibular

erythroplakia (30% progression) and leukoplakia (10%)

midline primaries, ant tongue tumors, and ipsilateral LNs

lip - 5%; oral tongue - 20%; rest 10-20%

level V - posterior triangle; VI - paratracheal/prelaryngeal

1 cm

surgery preferred ; if there is soft palate or RMT can consider definitive RT

auricotemporal nerve (branch of V3)

circumvallate papillae

tobacco (smoked or chewed), betel nut consumption, alcohol, poor oral hygiene, and vitamin A deficiency

N2a+, ECE, no neck dissection in high-risk patients, and depth of invasion > 3 mm

Most: Lip (45%); Least: hard palate (5%)

level II: skull base to hyoid; level III: hyoid to bottom of cricoid; level IV: cricoid to clavicles

spare the sup oral cavity/hard palate and to surround lateral oral tongue lesion w/ other mucosa to minimize the buildup effect on the lateral surfaces

must include entire hemimandible (from the mental foramen to the temporomandibular joint)

hard/soft palate and circumvallate papillae

90% arise from the lower lip

sup: 1-1.5 cm above dorsum of tongue or 2 cm above tumor
inf: thyroid notch
post: spinous process
ant: 2 cm ant to tumor

30%

hypotension and nausea

lip commissure, buccal mucosa, and RMT w/ tonsillar pillar involvement (because of better cosmesis with RT)

midline prelaryngeal level VI node (often involved in thyroid cancer)

largnoscopy q3 mos; imaging for signs and symptoms, TSH, speech/hearing/dental, and smoking cess

ant 2/3rd - CN VII ; post 1/3 - CN IX

< 70 Gy

+ or close (<2 mm) margin, depth of invasion >2 mm, PNI, T3-4 Dz

6 weeks

motor: CN XII; sensory: CN V

minor salivary gland tumors (adenoid cystic, mucoepidermoid, adenoCA)

opens at the ant FOM and drains the submandibular gland

6-8 wks (12-15 wks if eval by PET/CT)

base of tongue, posterior pharyngeal wall, tonsils, and soft palate

neck mass

30-50%

no, extremely rare

sore throat, dysphagia, otalgia, neck mass, hot potato voice

sore throat, trismus, otalgia, neck mass

leukoplakia, sorethroat, trismus/perforation, phonation defect w/ advanced lesions

pain/odynophagia, bleeding

oral tongue: CN V (auriculotemporal branch) -> preauricular area; BOT: CN IX (Jacobson nerve) -> tympanic cavity; CN X (arnold nerve) -> postauricular area

ant to hard palat2 and 2n molar inf (or 2 cm beyond tumor)

SCC and NHL

mean dose to either < 26 Gy

better laryngeal sparing with split-field IMRT techniques; drawback - may have to junction RT dose through involved nodes

if weight loss > 10% over 3 mos

V50 < 30%

mean < 50 Gy

ant tonsillar pillar (palatoglossus mm); post tonsillar pillar (palatopharyngeal mm); inf glossotonsillar sulcus

70% and 30%

p16 staining (E7 inactivates Rb which upregulates p16)

persistent nodal dz - documented by FNA, CT (4-6 wks), or PET/CT (10-12 wks)

~30%

advantage: conformality; disadvantage: nonstandard doses/fx are required

50% locally; 50% distally

inferior: thyroid notch; posterior: behind spinous process

ant: oral tongue/circumvallate papillae
superior: hard palate/ soft palate junction
inferior: valleculae
posterior: pharyngeal wall
lateral: tonsil

40-80%

upper jugulodigastric (level II)

+margin, ≥ 2 LNs, + ECE, PNI, or LVSI

nonsmoker and nondrinkers

genioglossus (ant mandible to tongue), styloglossus (styloid process to tongue), palatoglossus (palate to tongue; also forms ant tonsillar pillar), hyoglossus (hyoid bone to tongue)

nonmutated p53 (nonsmokers and nondrinker); p53 generally predicts poor response to txt

HPV 16

smoking, alcohol, HPV infection, betel nut consumption

better

above mastoid tip, floor of sphenoid sinus (ant), external auditory meatus

increasing while other's are decreasing

prior RT, NF-2, and HRT in women

angioblastic

for incidental/asymptomatic and stable lesions

12-16 Gy to 50% IDL at tumor margin

cavernous sinus, petroclival region, post saggital sinus, and optic nerve

roughly 10-15 %; grade II 6%; grade III 4%; grade I 90%

4 years

older patients and calcifications

20 years

75%

grade I - benign, grade II - atypical, grade III - anaplastic/malignant

15-20% (2nd only to gliomas); most common benign primary tumor

psammoma bodies and calcifications

goldsmith et al. showed improved PFS with doses > 52 Gy

12 --> 3 years

grade II- atypical, clear, choroid; grade III - anaplastic, rhabdoid, papillary

merlin; meningiomas, bilateral neuromas/epndymomas and juvenile subcapsular cataracts

8 Gy

headache

60%; chloroma, lymphoma, and sarcoidosis

98% (worse in men and if RT dose < 12 Gy)

high grade, young age, chromosome alterations, poor performance status, STR

late in life (70s) and females (2:1)

54 Gy for benign and 60 Gy for malignant tumors

10%, 40%

recurrent disease, STR, atypical, anaplastic, or brain invasion

Simpson grade (I/GTR - IV/STR); likelihood of LR

optic nerve sheath and cavernous sinus

definitive RT alone, cordectomy, partial laryngectomy, surgery + CRT (+ margin, ECE)

T1: 1 subsite (epiglottis, aryepiglottic folds, false cords, arytenoid cartilage)
T2: 1 adjacent subsite or outside supraglottis (BOT, vallecula, pyriform sinus) w/o fixation of larynx
T3: cord fixation and/or invasion of postcricoid area or pre-epiglottic space
T4a (resectable): through thyroid cartilage, trachea, soft tissue neck, deep/intrinsic mm of tongue, thyroid, esophagus
T4b: invasion of prevertebral space, mediastinum, carotid

stage III or IV (>80%)

TVCs, the FVCs, and superior subglottis

T1-3N+: induction chemo -> RT or surgery depending on response
T4: total laryngectomy/largoesophgectomy (w/ CRT for +margin, +ECE)

pyriform sinus & post pharynx: M > F post cricoid: F > M

5 yr OS was same (55%); 5 yr largnectomy free survival WORSE FOR RT ALONE (35%) vs chemo+RT (con and sequ 45%); LRC also WORSE FOR RT ALONE (50%) and SEQUENTIAL (55%) versus chemo-RT (70%) --> MAKING CHEMO-RT STANDARD

T1: limited to TVCs w/ normal mobility (a - 1 cord; b - both cords);
T2: extends to supra- or subglottis with impaired vocal cord mobility;
T3: fixed vocal cords;
T4a-b: same

75% overall have nodal involvement

level II, III, and V and the retropharyngeal nodes

T1: limited to subglottis; T2: extension of vocal cords, with normal or impaired mobility; T3: limited to larynx with vocal cord fixation; T4a-b: same as others

neck mass, sore throat, hoarseness, and otalgia (CN X involvement/arnold nerve)

T1/2 - 40% ; T3/4 - 60%

stage I: T1N0; stage II: T2N0; stage III: T3N0 or T1-3N1; IVA: T4a or N2; IVB: T4b or N3; IVC: M1

pyriform sinus (75), post pharyngeal wall (20), and postcricoid (5)

removal of thyroid and cricoid cartilage, epiglottis, and strap mm w/ reconstruction of the pharynx as well as a permanent tracheostomy

caudal tilt technique (both couch tilted away from the gantry by 10 degrees and gantry angulated 10 degrees above horizontal plane) or IMRT

supraglottic laryngectomy, transoral laser resection, or definitive RT

75 -> 85 -> 95

20% of all H&N (most common; 12K)

if stoma not being boosted: low anterior neck field will cover and isocenter can be above stoma; if stoma to be boosted: iso set below stoma

3%; 25% despite treatment develop mets

OS 10%(45 -> 55); LRC 13% (34 -> 47)

supraglottic: II-IV; glottic: no drainage; subglottic: pretrachea and delphian (level VI)

spans from C4-C6/hyoid bone to the inferior edge of the cricoid cartilage

20-50% (40%)

56.25 for CIS and 63 Gy for T1 lesions at 2.25 Gy/fx

60

LC: 85%; OS 90%

ant/post commissures and the true vocal cords

postcricoid extension/involvement

55% unilateral nodal dz; 15% bilateral nodal disease

speech impairment, dysphagia (1/4th); xerostomia

70 Gy at 2 Gry fx or 65.25 Gy at 2.25Gy fractions

0.5 cm below the TVCs down to the 1st tracheal ring

European trials' patients: more + margins (28 vs 18), worse tumor differentiation, more HPX (20 vs 10), and more patients who started RT 6 weeks later

RT (75 vs 30)

supraglottic, glottic, and subglottic

opposed laterals: sup - base of skull ; ant - 1 cm flash; post - bethind spinous process; inf - shoulders ;
low ant neck: sup match with lat fiels, laterally at 2/3rd of clavicular length, inf 1 cm below clavicle (include stoma)

T1: < 2cm or 1 subsite ; T2: 2-4 cm or > 1 subsite ; T3: > 4 cm or fixation of hemilarynx ; T4a: invades thyroid/cricoid cartilage, hyoid bone, thyroid gland, esophagus, or central soft tissue ; T4b: invades prevertebral fascia, carotid aa, or mediastinal structures

epiglottis (suprahyoid and infrahyoid), aryepiglottic folds, arytenoids, and false vocal cords

mucosal stripping and close followup

total laryngectomy +/- neck dissection

hoarseness, odynophagia/sore throat, otalgia (via the arnold nerve, CN X), aspiration/choking, and neck mass

smoking, alcohol, betel nut consumption, nutritional deficiency (vit C, Fe deficiency), prior H&N cancer

1. emergency tracheostomy 2. subglottic extension 3. ant soft tissue extension

glottic 70%; supraglottic 30%; subglottic 1%

postcricoid area , post pharyngeal wall, pyriform sinus

FVCs, epiglottis, and aryepiglottic folds

T1: 70 Gy/2 Gy fx; T2: hyperfractionated 76.8 in 1.2 Gy BID or concomitant boost tech to 72 Gy (54 Gy /1.8 Gy fx to area of subclinical dz in last 12 days additional 1.5 Gy dose to gross disease to 72 Gy)

for T1 generally wedges used; can remove wedges to create "therapeutic" hot spot at area of disease

20%

cord stripping/laser excision or definitive RT

acute: hoarseness, sore throat, odynophagia, skin irritation ; late: laryngeal edema, glottic stenosis, xerostomia, L'hermitte syndrome, myelitis, laryngeal necrosis

hyoid: C3 and TVCs at C5-6

node of rouviere

smoking, alcohol, and voice abuse

5 x5 laterals - sup: thyroid notch ; inf: cricoid cartilage; post - ant edge of vertebral body; ant - 1 cm flash of skin

AFP, BHCG, and LDH (CMP)

following orchicetomy 3 options: 1. observation 2. RPLND 3. BEP x 2 cycles (stage IB only)

NSGCTs are younger (27 yrs) when compared to SGCTs (36) and mixed (33)

30%

diagnosed via a radical inguinal orchiectomy (do not bx a testicular mass)

CXR should be performed; testicular US is considered optional

teratoma w/ malignant transformation histologically resembles a somatic cancer, such as an adenocarcinoma or sarcoma

24-36 hrs

palliation of metastatic dz; no established role for RT in the primary txt of NSGCT

30% risk of relapse

CT chest should be ordered in pt with a positive CT abd/pelvis or abnl CXR

choriocarcinoma

considered and treated as an NSGCT pt

germ cell tumor is most common

randomized 382 pts w/ stage I NSGCT to RPLND vs BEP x 1 cycle; 2yr RFS was 92% w/ surgery vs 99% w/ BEP. BEP superior to RPLND

retrograde ejaculation resulting in infertility; nerve-sparing techniques can preserve ejaculation in 95% of cases

testicular/retroperitoneal primary tumor ; no nonpulmonary visceral mets ; AFP < 1000 ; B-HCG < 5000 ; LDH < 1.5 the upper limit of nl

2 cycles of BEP chemo

BEP x 3 cycles or cisplatin/etoposide (EP) x 4 cycles

mature or immature depending on whether it contains adult-type differentiated cell types (mature) or partial somatic differentiation similar to that found in a fetus (immature)

more than doubled

no major impact on the clinical outcome

seminomatous or nonseminomatous

1. mediastinal primary tumor ; 2. nonpulmonary visceral mets ; 3. AFP > 10,000 ; 4. BHCG > 50,000 5. LDH > 10

yolk sac tumors

prior personal hx of GCT; postive family hx; cryptochidism; testicular dysgenesis; klinefelter syndrome

40%

5-7 days (a FULL WEEK p)

CT abd/pelvis

yolk sac tumros

it is not different

chemo; BEP x 3 cycles or EP x 4 cycles

5-10%

embryonal cell carcinoma, choriocarcinoma, yolk sac tumor, teratoma, mixed

Cowden and Gardner

duodenum > jejunum > ileum

at 40 yrs old or 10 yrs prior to earliest cancer dx in the family; every 1-5 years

every 1-2 years

N1 - 1-3 LNs ; N2 >/= 4 LNs

yes

liver (then lung)

APC; associated with familial adenomatous polyposis

colonoscopy (q10yrs), fecal occult blood test (q1yr) and sigmoidoscopy (q5yrs), or doubt contrast barium enema (q5yrs)

hematochezia

ulcerative colitis

CBC, Chem 7, LFTs, and CEA

63

CEA should return to reference range 4-6 weeks following surgery; slow rise suggest LR while rapid suggest metastatic disease

I: T1-2 N0 M0
IIA: T3 N0 M0 IIB: T4a IIC: T4b
IIIA: T1-2N1 or T1N2a IIIB: T3-4aN1 T2-3N2a T1-2N2b IIIIC: T4a N2a T3-4a N2b T4bN1-2
IVA: M1a IVB: M1b

M1A - single organ or site ; M1b - mets > 1 organ/site or deposits in peritoneum

7 th decade of life

calcium, vit D, and folic acid supplementation lower risk of colorectal cancer; Aspirin/NSAIDs lower risk of developing colorectal polyps

depth of invasion

5%

S3 at region of peritoneal reflection; 15 cm long

rich in fat and low in fiber and antioxidants

T1: submucosa
T2: muscularis propria
T3: invades muscularis propria into pericolorectal tissues
T4: a - penetrates to surface of visceral peritoneum b - adheres to other organs or structures

Dukes A: stage I Dukes B: stage II Dukes C: any nodal involv (stage III)

familial adenomatous polyposis (FAP) and HNPCC (aka Lynch syndrome)

hMLH1 or hMSH2 which regulate mismatch repair

k-ras

villous adenomas

h&P, CEA q3-6 mos for 2 years then q 6mos for 5 years; CT C/A/P q1year for 3 years; colonoscopy at 1 year, then 3 years, then q5years

yes

80%

6-8 weeks (to allow for downstaging)

improved DFS with neoadjuvant CRT 65 vs 53; no diff in LR or OS; pCR was 15%

3 field: PA field - superior at L5-S1; inferior at bottom of ischial tuberosity/3 cm below tumor volume; lateral - 1.5 cm from pelvic inlet; Lateral field - posterior at behind entire bony sacrum; anterior behind pubic syphisis for T3 (in front if T4); sup-inf same as PA

many RCTs (MRC3, NSABP R-01) that investigated adj RT alone showing improvement in LC only but no improvement in OS, DFS

preop RT (5Gy x 5 fx) then TME surgery versus TME surgery and chemo-RT if + margin; found decrease in LF (11 to 4%) with preop RT and increase in DFS (72 -> 78); no diff in OS

swedish rectal cancer trial compared neoadj RT (25 Gy in 5 fx) to surgery alone and found significant improvement in OS (30 -> 38) and LR(26 -> 9); big critique is lack of TME and high recurrence rate for surgery alone arm (26%)

OS benefit (30 -> 38)and improved LR rates (22-> 13%) with preop RT; lower SBO rates (11 -> 5)

no; TME does not offset the benefit of neoadj RT based on Dutch TME study (peeters et al.) which compared TME alone to neoadj RT -> TME. There was no OS benefit but decreased LR rate (11 -> 6) with addition of neoadj RT

predicts for inferior LC, DM, and OS rates

5 Gy x 5 fx = 25 Gy; 1 week

tumor bed (+2-5 cm margin) and presacral/internal iliac nodes

poster border on the lateral fields usually stays the same (behind the bony sacrum) for the cone down portion; given the high rate of LR in this region

locoregional

close/+margin or as an additional boost (esp w/ T4tumors or recurrent tumors); typical dose is 10-15 Gy

T1-2N0: upfront surgery +/- adj CRT; T3-T4 or N+ neoadj CRT -> surgery and adj 5FU/leuc or FOLFOX

4-6 weeks

~25%; with modern (TME) techniques LR 11% (decreased further with neoadj chemo-RT to 5%

INT 0130 trial which compared adj chemotherapy to adjuvant chemoRT in colon cancer; found no difference in OS or LC w/ addition of RT; study was underpowered to show a diff btw groups

continous is associated w/ greater GI toxicity; bolus with greater hematologic toxicity

GITSG and Intergroup/NCCTG

CT simulation in the prone position, on a belly board, and with a full bladder (optional placement of anal/vaginal markers and/or rectal contrast)

median bowel movement frequency, fecal incontinence, and impaired social life

5FU (225 mg/m2) concurrently via continuous infusion (shown improved 4 yr OS when compared to bolus)

high risk T1 lesions (poorly diff; margin < 3 mm; > 3 cm in size; LVSI); T2 after local excision; All T3-T4 or N+ cancer after LAR or APR

4-5 cycles of bolus 5FU (at 4 weeks)

18% of pts will be overtreated with neoadj CRT (because of EUS suggesting T3-4 or N+ disease) which is derived from german trial's postop arm and found that on path 18 % actually had T1-2 disease and did not require CRT

6-18 mos; diarrhea (bloody), colicky abd pain, and n/v; less common - SBO, fistulas, bowel perforation, and severe bleeding; bowel malabsorption; damage to the ileum (impair resoprtion of vitamin B12 and bile acid)

only 54% of pts in adjuvant arm received full RT dose (versus 92% in neoadjuvant arm)

local excision (transanal excision), abdominoperineal resection (APR), or low anterior resection

benefit of neoadjuvant chemoRT w/ regards to LR (13 -> 6); fewer toxicities; and better sphincter preservation (19 -> 39)

EORTC; no benefit w/ OS, DFS, or LC

external iliac nodes (because of bladder, vaginal or uterine invol)

fixed T4 colon cancer or w/ close/+margin 45-50.4 Gy

8%

45 Gy

YES

reduces rate of positive radial margin and improves LC

T1, < 3 cm, < 3 mm depth, involves < 1/3rd rectal circumference, N0 by EUS or MRI, low-grade, no LVSI………..reliable patient

SBO

39%

LVSI,
age > 60,
grade,
deep myometrial invasion,
tumor size,
lower uterine segment involvement,
anemia,
poor karnofsky performance status

combination oral contraceptives and physical activity

observiation vs pelvic RT
no lymphadenctomy
LRR decreased from 14 -> 5; no difference in 10 yr OS
75% of LRs were in the vaginal vault;

- gross PA disease
- positive pelvic LN
- gross adnexal mass
- more than 1/3rd myometrial involvement

25% of patients have these features

30 Gy LDR and 5Gy x 3 fx (15 Gy) at 0.5 cm depth

pelvic RT followed by VB (chemo for Grade 3)

superior L4-5
inferior bottom of obturator foramen
lateral 1.5-2 cm lat to pelvic brim
anterior front of symphisis
posterior split sacrum at s3

adj chemo +/- RT should be given

limited to endometrium or less than 1/2 of myometrium

limited to greater than 1/2 of myometrium

invades stromal cervix but no extension beyond uterus (endocervical cervix DOES NOT COUNT)

involves serosa and/or adnexa by direct extension

vaginal involvement or parametrial involvement

invades bladder mucosa (bullous edema is not sufficient) and/or bowel mucosa

removal of uterus and 1-2 cm of vaginal cuff;
wide excision of parametrial and paravaginal tissues (including median one half of cardinal and uterosacral ligaments)
ligation of uterine artery at ureter

grade depends on the GLANDULAR component
G1: < 5 % nonsquamous solid growth pattern
G2: 6-50% nonsquamous solid growht pattern
G3: >50% nonsquamous solid growth pattern

favored chemotherapy 75 -> 82%

vertical incision
peritoneal washing/cytology (controversial)
exploration of all peritoneal surfaces with biopsy any lesion
TAH/BSO
uterus bivalved in OR
omental biopsy
sampling of pelvic LNs

exogenous unopposed estrogen, endogenous estrogen (obesity, functional ovarian tumors, late menopause, nulliparity, chronic anovulation/polycystic ovarian syndrome), tamoxifen, advancing age (75% postmenopausal), hereditary (HNPCC), family hx, hypertension?

endometrium, myometrium, and serosa

occurs at 2-3 Gy

consider pelvic RT to 45 Gy --> intracavitary RT boost using 2 tandem intrauterine applicators to 6.3 Gy x 3 fx prescribed to 2 cm depth (serosal surface); If pelvic RT is contraindicated, consider definitive intracavitary RT alone

parametrial, internal and external iliac, obturator, common iliac, presacral

para-aortic nodes (fundus)

none (except for grade 3 - consider VC)

chemo (CAP) vs pelvic RT;
no difference in PFS, OS, or toxicity
in high-intermediate risk patients (stage I, age > 70 or grade 3; stage II or +cytology) there was an increase in progression free survival (66 -> 84)

type I: endometrioid, 70-80%, estrogen related
type II: nonendometrioid, typically papillary serous or clear cell, high grade, not estrogen related, aggressive clinical course

all patients got VB then randomized to observation vs pelvic RT;
pelvic RT decreased LC (7 -> 2) but more DM (5 vs 10); no diff in OS
in subset of pts with invasion of 1/2 or more and G3 (improved OS by 10% and improved LR 5->20%)

if endometrial Bx is nondiagnostic

surgical stage I-II, positive cytology or positive pelvic LNs; randomized to pelvic EBRT (+/- VB) + / - chemotherapy (variety of regimes allowed)
5 yr PFS favored the RT + chemo arm (75 -> 82)

5-20%

95% sensitivity; 85% specificity

proximal: 120 Gy
distal: 100 Gy (98 Gy)

80%

papillary serous, clear cell, and pure squamous

randomized patients to observation vs pelvic RT (patients had a pelvic and PA node sampling) with improvement of LR 12 -> 3%

uterus and small rim of vaginal cuff

in cases of distal vaginal involvement

- resection of uterus and upper vagina
- dissection of paravaginal and parametrial tissues to pelvic sidewall
- ligation of uterine aa at its origin at int iliac
- dissection of pelvic LNs

endometrium involv (all grades): < 5%
inner or middle 1/3rd (all grades): 5-10%
outer 3rd: G1 - 10%, G2 - 20%, G3 - 30%

33 % of patients with positive pelvic nodes have PA nodes

~1% have skip mets to PA nodes

endeometriod carcinoma: proximal 3-5 cm of vagina
CCC, UPSC, and stage IIIB: entire vaginal canal

prescribe to 0.5 cm beyond the vaginal mucosa

occurs at 5-10 Gy

within 1 week

WART versus chemothrapy (AC); chemo improved overall survival (42 -> 55); PFS (38 -> 50); increased toxicity

I: 7
II: 15%
III: 30%
IVA: 40%

transformation zone (dynamic area between the original and present squamoculumnar junction)

GOG 123: stage IB2 cervical cancer pts were randomized to preop RT vs chemo-RT -> adj simple hysterectomy

benefit of chemo for pCR 41 -> 52 and OS 74 -> 83

~22% of HGSIL progress to invasive cancer; ASCUS (< 1%) and LGSIL (~5%)

abnl vaginal bleeding, postcoital bleeding, and abnl vaginal d/c

> 99%

pts with tumors < 2 cm and only 1+ node positive did not benefit from CRT compared to RT alone

mucinous, endometrioid, clear cell, and serous

all patients with HGSIL should undergo colposcopsy with biopsy

shorter txt time
preservation of ovarian function
possibly better sexual functioning after treatement
no 2nd malignancy risk

+LVSI, > 4 cm size, > 1/3rd stromal invasion ; GOG 92 stage IB cervical cancer patients who underwent surgery and had negative nodes but > 1 adverse features; RT decreased recurrence 21 -> 14% (46%) and OS trend 71 -> 80% (NS)

repeat PAP 6 months later (w/ colposcopy if repeat is abnormal); it can resolve ~40% of the time

stage IA (brachy LDR ~70 Gy or HDR 7 Gy x 5-6 fx) w/ LC of 97%

bladder: 5 mm behind pos surface of foley balloon (filled with 7 cc of contrast)
rectum: 5 mm behind the post vaginal wall btw the ovoids at the inf point of the last intrauterine tandem source
vaginal point: lat edge of the ovoids on AP film and mid ovoid on lat film

Landoni et al. Looked at IB and IIA randomized to class III hysterectomy (radical hysterectomy) versus RT;
OS (83) and DFS (74) were equal; 64% of surgery pts received adj RT;
grade 2-3 morbidity was higher in surgery arm 28 vs 12%

stage IA1; (IA2 or higher should get radical hysterectomy w/ pelvic LND)

47

70 % SCC vs 25% adenocarcinoma

5 cm lateral from the midline at same level of point A (2 cm above the external cervical os);

represents the obturator nodes; usually represented by 1/3rd of point A

all cervical cancer removed with margin but the internal os is left behind and stitched close (w/ small meatus for menses);

procedure allows future pregnancy, deliverved via c-section;

reserved for stage IA1 as well as select IA2 and IB1 disease

I: 15%
II: 30%
III: 50%
IVA: 60%

IA: microscopic disease < 5mm DOI and < 7 mm horizontal spread (1 - < 3 mm depth; 2 - 3-5 mm depth)
IB: visible tumor (1 < 4 cm; 2 > 4 cm)
IIA: upper vagina/beyond uterus
IIB: parametria invasion

IIIA: lower 3rd of vagina
IIIB: pelvic side wall involvement

IVA: invades mucosa of bladder or rectum (must be biopsy proven); (does not count bullous edema)

depth of invasion, FIGO stage, and LVSI

subtypes 6 and 11

1. patients with PA disease 2. pts w/ positive pelvic nodes who are not receiving chemotherapy

RTOG 7920 (Rotman et al.) pelvic RT verus EFRT alone; addition of PA field improved 10 yr OS (44 -> 55) w/o improvement in LC or DM; increased toxicity 4 -> 8

RTOG 90-01 (Eifel et al.) pelvic chemo-RT versus EFRT (included advanced cervix and + pelvic LN patients)
chemo RT improved OS (41-> 67), DFS (46-61), LRF and DM

atypical squamous cells of unknown significance; repeat pap in 6 months then colposcopy if abnormal again

no OS and trend to improved LR benefit;

questions answered in GOG 71(Keys et al) trial; patients with > 4 cm tumors randomized to RT alone vs RT + adj hysterectomy
no diff in OS or severe toxicity; trend to improved LR (RT 26 vs surgery 14)

option is to give upfront CRT and assess for response at 2 months; if residual disease present then can salvage

LDR: 0.4 - 0.8 Gy/hr
HDR: 12 Gy /hr

corpus, parametria, and vagina

CXR, IVP, and cystoscopy, proctoscopy, and hysteroscopy

uterine perforation (< 3%), vaginal laceration (< 1 %), DVT (<1%)

point A is 2 cm above the external cervical os and 2 cm lateral to the central canal/tandem; corresponds to the paracervical triangle where the uterine vessels cross the ureter

< 3mm: 1%
3-5 mm: 5%
5-10 mm: 15%
10-20 mm: 25%

+/- close margin, LN mets, microscopic parametrial invasion

> 70%

~70% reduction

pruritis, desquamation, hemorrhoids, colitis, cystitis, vaginitis/ulceration, candidiasis, and nausea

when there is lower 1/3rd vaginal involvement

stage IA: 65-75 Gy
stage IB-IIB: 75-85 Gy
stage III-IVA: 85-90 Gy

rectal 75 Gy; bladder 80 Gy; max vaginal point 120 Gy

extent of dissection; class II dissected to just medial of ureters with removal of parametrial tissues:

class III is dissection to pelvic sidewall + LND with removal of upper 1/2 of vagina (only vaginal cuff in class II)

ovarian failure 5-10 Gy; sterility 2-3 Gy

lateral: external iliac nodes
posterior: common iliac and lat sacral nodes
post-lat: internal iliac nodes

early onset of sexual activity
multiple partners
exposure to high risk partners
hx of STD
smoking
parity (high)
prolonged use of oral contraceptives

vaginal stenosis, ureteral stricture, fistula, SBO, and femoral head fractures

tumor stage

colposcopy -> conization, LEEP, laser, cryotherapy, or simple hysterectomy

I: 80
II: 65
III: 40
IV: 15

NONE, no RT indicated in these special sites

Heyn et al. Found vincristine and actinomycin D improved OS after surgery (compared to observation)

1. evaluate regional nodes
2. include scar and drains
3. spare strip of skin or portion of joint/epiphysis

scalp, cheek, parotid, oral cavity, oropharynx, and larynx

> 10 yo is worse than < 10 yo

prostate, paratesticular, bladder, nasopharynx, extremities

subarachnoid space involvement w/ skull base erosion, CN palsy, intracranial extension

no; answered in IRSIII

distal spermatic duct

NO RT for all embryonal group I patients; 36 Gy for alveolar group I (unfav histology)

hyperdiploid better (embryonal) than diploid (alveolar)

2-6 years (embryonal) and 15-19 (alveolar)

70%

H&N (PM > orbit > non-PM) > GU > Ext/trunk

orbit and parameningeal sites

t(2:13) (70%), t(1:13) (20%)

PAX3 or PAX7 w/ FKHR

mesenchymal stem cells

botryoid > spindle cell > embryonal > alveolar > undifferentiated (95 - 85 - 65 - 55 - 40)

local failure; LN positivity biggest predictor

myoD

15%; prostate, trunk and extremities

low risk (embryonal + fav site + any group)

low risk

intermediate risk

intermediate risk (unfav histology, any site, group 1-III

if R0-1 - 41.4 Gy; if gross disease 50.4 Gy; RT never omitted for node + disease

embryonal (classic, spindle cell, and botryoid), alveolar, pleomorphic, and undifferentiated

MRI brain, CSF cytology (neuroaxial MRI if +)

genetic: beckwith-wiedermann syndrome, li fraumeni, and NF-1

environmental: parental marijuana/cocaine use and prior RT

H&N: embryonal
GU: botryoid
ext/trunk: alveolar

bone > BM > lung

prostate (40%)

generally: max safe resection (or bx alone) -> chemo +/- RT (timing depends of risk groupings)

veno-occlusive disease of the liver

VAC - vincristine, actinomycin, cytoxan
IE - ifosfamide, etoposide

orbit, non-PM H&N, non-prostate/bladder GU, biliary (BHOG)

T1 (confined to anatomic site of origin): a ≤ 5 cm; b > 5 cm
T2 (ext or fixed to adjacent tissue): a ≤ 5 cm; b > 5 cm
N1: regional node involvement

most (50%) present with group III disease

15 Gy (in 1.5 Gy / fx)

I: R0 resectin, localized disease
II: R1 resection and/or resected +LN
III: R2 (both primary and +LN) or biopsy only
IV: distant mets

stage I: favorable site (any T, any N)
stage II: unfavorable site ( < 5 cm, N0)
stage III: unfavorable site (> 5 cm, and/or N1)
stage IV: M1

MMNNOOPP
middle ear
mastoid
nasal cavity
nasopharynx
infratemporal fossa
pterygopalatine fossa
paranasal sinus
parapharyngeal space

infants: botryoid; young children: embryonal; adolescents: alveolar; adults: pleomorphic

pancreatic cancer, including N staging (N0-N1); managed with similar approaches

like hepatocellular carcinoma (surgical resection vs local ablation therapy)

35-50%

radical CCY and regional nodal dissection (porta hepatis, gastrohepatic ligament, and retroduodenal nodes)

T2

T3 (perforates serosa and/or liver and/or 1 adjacent organ/structure

T4

same as primary liver tumor

30%

radical re-resection of gallbladder bed (2 cm margin), regional nodal dissection, and resection of port site

intrahepatic, extrahepatic and hilar (klatskin tumor)

50%; liver and peritoneal involvement

primary sclerosing cholangitis
ulcerative colitis

CEA and CA 19-9

MRCP

jaundice and abdominal pain

T3 (T1 - confined to bile duct; T2 invades beyond bile duct; T4-celiac axis or SMA)

45%; cystic (pericholedocal -> retropancreaticoduodenal), hilar, pericholedochal, and celiac

mucin-producing adenoCA

papillary adenocarcinoma

extrahepatic (intrahepatic due poorly -> contraindicated)

cholelithiasis (via chronic inflammation)

staging laparoscopy (to r/o peritoneal dissemination)

mass greater than 1/3rd of the intrathoracic diameter at T5-6 on upright PA film

mass > 5 cm or mass > 10 cm

follicular lymphoma t(14;18) -> Bcl-2

CLL -> t(14,19), trisomy 12, chromosome 13 deletion

MALT lymphoma -> t(11:18)

painless adenopathy; 30% of B symptoms;

waxing and waning adenopathy suggests indolent form of NHL

tumor bulk may cause intestinal obstruction, urinary tract obstruction, or nerve compression

coronary artery disease, hypothyroidism, and 2nd malignancies

cbc, cmp, ldh, beta 2 microglobulin, serum protein electrophoresis, HIV, hep B (may reactivate w/ rituximab tx), hep C

BM biopsy; LP in CNS symptoms, testicular, paranasal sinus, or immunodeficiency

typically involves more nodes at presentation, more likely to be extranodal, more likely to spread in noncontigous fashion, and has prognosis more affected by subtype

FL is mix of centrocytes (small cells) and centroblasts (large, non cleaved cells); grade correlates to density of centroblasts

SLL - same as CLL but w/ predominant manifestion in spleen, liver, or nodes (vs peripheral blood or BM)

transformation of SLL/CLL to Diffuse large b cell lymphoma; occurs in 5% of cases

fever (38 C or 100.4), > 10% weight loss in 6 mos, or drenching night sweats

35% are indolent; follicular lymphoma > SLL > MALT lymphoma

low grade: follicular (g1-2), CLL, MALT, mycosis fungoides

intermediate grade: follicular (g3), mantle cell, DLBCL, NK Tcell, peripheral T cell, anaplastic large cell

high-grade: burkitt, lymphoblastic

NO (5 randomized studies)

CHOP-R
cyclophosphamide
hydroxydaunomycin (adriamycin)
oncovin (vincristine)
prednisone

APLES: age (> 60), Performance (ECOG > 2), LDH, extranodal group, stages (III-IV)

HASEL

Hgb < 12
Age > 60
stage
Extranodal sites (>5)
LDH

palliation of symptoms

consolidation after 3-4 cycles of RCHOP in fav dz

partial response to chemo

patients with bulky disease

CHOP x 8 cycles versus CHOP x 3 cycles + IFRT
initial report showed benefit OS (10%) eventually disappeared (curves crossed)

locregional RT to 30-36 Gy (can observe or ? Combined modality)

R CHOP x 6-8 cycles; IFRT considered for initially bulky sites

high-dose chemo + stem cell transplant

7th decade

cigarette smoking, phenacetin exposure, heavy metal exposure

lung > bone > regional LNs

T3a
T3b: extends into vena cava below diaphragm
T3c: extends into vena cava above diaphragm or invades wall of vena cava

T4

T1: ≤ 7 cm (a - ≤ 4 cm; b - 4-7 cm)
T2: > 7 cm (a - 7-10; b - > 10 cm)

incidence has been increasing; Men > women

3-5 years after nephrectomy

clear cell

3%

6%

clear cell, chromophilic, chromophobic, collecting duct

people endemic to the Balkan nations have a 50-100 fold increased incidenc of RCC

gerota fascia -> perirenal fat -> adrenal gland (perirenal fat sup to the kidney) -> renal capsule -> renal cortex

sorafenib associated with hand-food syndrome;

sunitive: mucositis

III (N1); IV (T4)

hematuria, flank pain, and a palpable mass

(fever, night sweats, and weight loss)

grade, TNM stage, DNA ploidy, genetic RCC syndromes

angiomyolipomas, fibromas, lipomas, lymphangiomas, oncocytomas, hemangiomas

hypercalcemia, hepatic impairment, hypertension

VHL tumor suppression on 3p25

TNM stage, performance status, furhman grade

3%; von hippel lindau, birt-hogg-dube syndrome, tuberous sclerosis, hereditary papillary RCC, HRCC

brain mets (20-40%)

pineoblastoma and RB (any PNET)

choroid plexus

CN IV exits on the dorsal side of the brain

25% low grade vs 75% high grade

optic glioma and JPA

bilateral acoustic neuroma, spinal ependymoma

subependymal giant cell astrocytoma, retinal hamartoma

hemangioblastoma

meningioma

medulloblastoma

medulloblastoma, glioblastoma

pineoblastoma

CNS lymphoma

MEN 1

CN VI palsy (no abduction of the lateral rectus)

meningioma, (also chloroma, lymphoma, and sarcoidosis

in children at L3-4 and adults L1-2; the thecal sac ends at S2-3 in both

stylomastoid foramen

EGFR and PDGFR

Wernicke's area - left temporal lobe at the post end of the lateral sulcus

middle meningeal artery and vein;

V3

CD 133 - thought to be precursors for astrocytomas

ionizing RT

Glioma (40%) > meningioma (20%)

CNs III, IV, VI, and V1

31 spinal nerves (8 cervical, 12 thoracic, 5 lumbar, 5 sacral and 1 coccygeal)

meningioma and pituitary tumors (uncommon)

broca motor area (dominant/left frontal lobe)

radionecrosis, leukoencephalopathy, retinopathy, cataracts, endocrine deficits, memorly loss, learning deficits, and hearing loss

genetic mutation is chromosome 17; neurofibromin; predisposes to optic/intracranial gliomas

CNs III, IV, V1, and V2 and internal carotid aa

schwannomas

yok sac tumors

internal auditory meatus

medulloblastomas (and other blastomas - except astroblastoma/glioblastoma multiforme), CNS lymphoma, choroid plexus carcinomas, germ cell tumors, and mets

alopecia, dermatitis, fatigue, transient worsening of neurologic symptoms, n/v, otitis externa, seizures, and edema

CNs IX-XI

children: juvenile pilocytic astrocytoma
adults: glioblastoma

V2

AMEN

nuclear Atypia
cellularity and Mitosis
Endothelial proliferation
Necrosis

prolactinoma (30%) > GH (25%) > ACTH (15%)

microadenoma (< 1 cm)

macroadenoma (> 1 cm)
picoadenoma (<0.3 cm)

adults: pituitary adenoma

children: craniopharyngioma

medically inoperable
persistence of hormone defect after surgery
macroadenoma with STR or decompression
recurrent tumor after surgery

bitemporal hemianopsia, HA, and oculomotor deficits

months to years for hormone normalization after RT for pituitary tumros

45-50.4 Gy; 54 Gy

diabetes insipidus (6%) -> hyponatremia and CSF leak

MEN 1 (3 P's - pituitary, parathyroid, pancreas); 11q13 mutant

MEN2

Growth Hormone > 10 (not suppressed by glucose)

elevated IGF-1

the cells of nonfunctional tumors are chromophobic

anterior: GH, prolactin, FSH, LH, ACTH, TSH

poster: oxytocin, ADH

75% of tumors are functional
25% of tumors are nonfunctional

high preop GH and somatomedin C levels, tumors > 1 cm, and extrasellar extension

with conventional RT, 50-54 Gy

ACTH -secreting adenoma in patients with a hx of adrenalectomy (patient can develop hyperpigmentation of skin due to alpha-melancytestimulating hormone)

size > 2 cm
high preop prolactin level
increasing age
longer duration of amenorrhea

GH: acromegaly, gigantism

ACTH: cushing disease

95%; better for microadenomas

RT: > 90%

sella turcica

< 25 ng/mL normal level of prolactin after RT

galactorrhea, amenorrhea, decreased libido, and infertility (PL > 25)

ketoconazole (best)
cyproheptadine (inhibits ACTH secretion)
mitotane (decreased cortisol synthesis)
RU-486 (block glucocorticoid receptor)
metyrapone

pituitary lesion is > 3 cm and/or lesion is < 2 mm from the chiasm

high cortisol not suppressed by low-dose dexamethasone and normal or increased ACTH

initially 80%; but decreases to 40%

calcifications and amyloid deposits

with GK: less homogenous dose, more precise setup, and less normal tissue treated

transsphenoidal microsurgery: microadenomas, decompression, debulking of large tumors, reducing hyperfunctioning tumors

frontal craniotomy: large tumors with cavernous sinus invasion or frontal/temporal lobe

somatostatin, octreotide, pegvisomant (GH receptor antagonist)

microadenomas; benefit in better control of hormone secretions

anterior: rathke pouch

posterior: extension of the 3rd ventricle

GH (best) > ACTH > prolactinoma

TSH-secreting tumors (risk factors: thyroid ablation, hashimoto thyroiditis, prior RT/surgery)

hypopituitarism

40-45 Gy; GH levels first, then LH/FSH then TSH/ACTH

needs to be D/C b/c of lower RT sensitivity with concurrent medical treatment

monitoring IGF-1 levels

males/elderly: nonfunctioning or GH

females: prolactin and ACTH

macroadenomas

synaptophysin, chromogranin, and hormone specific stains

tumor, craniopharyngioma, meningioma, glioma, suprasellar germ cell, mets, and benign lesions (cyst, aneurysm, empty sella syndrome)

elevated cortisol due to a variety of causes (adrenal prod'n, exogenous use); patients have low ACTH, unlike in Cushing disease)

Growth hormone

functioning (50-54 Gy FST, and 20 Gy SRS) vs non functioning (45-50 Gy FST, and 15 Gy SRS)

basophilic (FLAT): fsh, lh, acth, tsh
acidophilic: prolactin, gh

RT+axillary LND: 15-40%
RT and SLN Bx: 10%
SCLV increases to 15-20%
PAB increases to 40%

number of LNs (axilla) and breast tumor size

limited edema of skin or "peau d'orange"
ulceration of skin
fixation of tumor to chest wall
axillary LNs > 2.5 cm
fixation of axillary LN

twice the risk for inflamm breast cancer

neoadjuvant chemo -> MRM -> PMRT +/- add'l chemo/hormones/herceptin
OS (5 yrs): 40%

LRF was high
CMF chemo was used
RT fields were en face photons for IM nodal coverage (though no excessive cardiac deaths)

inadequate surgical txt of axilla -> median of 7 nodes removed
excess of LF occurring in the axilla (44% in CMF arm)
outdated use of CMF chemo

total mastectomy + axillary LN sampling (median of 7 nodes removed); significantly less than most US centers (that aim for > 10)

levels I-II; only if suspcious nodes present does level III carried out

tamoxifen x 1yr +/- PMRT
LR 35 -> 8
OS 34 -> 45

stage III disease (T3N1, N2-3, or T4
Inflamm breast cancer
some include T3N0 (stage IIB)

no diff in OS or DFS

27% conversion rate

yes

administered q2 weeks versus q3weeks

3%

pectoralis muscle

premenopausal women w/ positive axillarly LNs s/p total mastecotmy+ALND randomized to CMF chemo +/- RT (5field)

≥ 4 LNs and suggested for T3 tumors with axillary LN disease

10% (4-13%)

rapid onset (< 3 mos)
generalize induration (often w/o associated mass)
diffuse skin erythema affecting more than 2/3rd of the breast
warmth/tenderness

cancer cells in dermal lymphatics (not necessary for diagnosis)

axillary LNs not dissected; only breast tissue with overlying skin removed

resection of all breast parenchyma, a large portion of breast skin, and major and minor pectoral mm en bloc with axillary LNs

+ LVSI
high grade
younger age
ECE ≥ 2 mm
≤ 10 LN examined
≥ 20% LN +
larger tumor size (T2 or > 4 cm)
close margins

(10 yr LRR > 15%)

yes

skin sparing mastectomy with placement of tissue expanders done prior to RT; tissue expander deflated during RT and re-expanded afterward;

breast reconstruction should be done 6-12 mos after RT

YES. Intergroup trial C9741
AC x 4 -> taxol x 4 given q3wks vs q2wks.
DFS improved from 75 -> 82
OS was 0.69 in favor q2wk schedule
severe neutropenia less frequent with dose-dense schedule

relative ratio of 1.25 based on EBCTCG

improves response rates, DFS, and OS

5% (4.6% baed on SEER)

core biopsy and wire localization for the biopsy bed (in case patient has CR to chemo)

30%

after cycle 1 of CMF and 3-5 weeks postoperatively

OS benefit (45->54) and LRR benefit 32 -> 9
survival benefit seen in all patients

adj RT improved LRR before DM 39 -> 13
DFS 31 -> 48
OS 37 -> 47

there is a 25% chance for a negative axilla upon resection

T4a: local extension but technically resectable
T4b: unresectable disease

increasing

right lobe, left lobe, isthmus, pyramidal lobe

papillary (10 yr OS 93 vs 85)

lobectomy+isthmusectomy
near-total thyroidectomy
total thyroidectomy

thyroid bed, bilateral necks, and upper mediastinal nodes

major: age, tumor size
minor: histology, local tumor extension, LN status

RT exposure to the H&N as a child ; no increased risk if exposure after age 20

papillary > follicular > hurthle cell

T1 < 2 cm (a - < 1, b 1-2)
T2: 2-4 limited to thyroid
T4: > 4 cm

N1a: mets to pre/paratracheal nodes, prelaryngeal nodes (level VI)
N1b: mets to cervical neck (levels I-V), upper mediastinal nodes

PET/CT

thryoid cancer

adenomas

suspected or proven residual normal thyroid tissue or residual tumor

thryoid hormone withdrawal or by using recombinant TSH

definitive surgery and EBRT for palliation

MEN 2a and MEN 2b

recurrent laryngeal nerve

t4b/extrathryoid extension but not thyroid confined or metastatic dz

epithelial follicular cells: remove iodide from the blood to form T3 and T4
parafollicular cells: produce calcitonin

patients with none of the following risk factors: age > 45, tumor > 4 cm, aggressive histology variant, prior hx of RT, DM, N+, local extension, and + margins

papillary (30-50), follicular (40-60)

cowden syndrome

75%

surgery (even in M1 dz) -> observation vs adj treatment

all anaplastic thryoid cancer is considered stage IV - A-resectable, B- unresectable and C- metastatic

1. pT4 papillary and > 45
2. gross residual disease in neck after I-131
3. bulky mets after I-131
4. lesions with inadequate iodide uptake

thryoid, salivary glands and the GI tract

maximal safe resection --> adjuvant chemoRT

cisplatin/doxorubicin

familial polyposis
gardner syndrome
turcot syndrome
familial papillary carcinoma

unfavorable

II (If the patient were 65 yo, would be stage Ivc - separate staging for follicular/papillary < 45 - only I and II (M1))

800-1000 mCi

IVB (if <45 then stage I)

yes - females > males (3:1)

tall, columnar, insular, oxyphilic, poorly diff

80%

primary: central compartment (level VI), TE groove, delphian nodes

secondary: cervical/supraclavicular

tertiary: sup mediastinal/retropharyngeal nodes

papillary thyroid cancer (30%) > follicular (10%)

6 mos and 20%;

medullary much better prognosis: 10 yr OS 90%; +N 70%

5%

follicular epithelial derived (papillary>follicular> hurthle) (94%) > medullary > anaplastic

100-200 mCi (30-100 mCi for normal thyroid tissue)

gi irritation, sialadenitis, and cystitis

pulmonary fibrosis, oligospermia, and leukemia

follow-up iodide scans

I-131 txt of low-risk patients

less aggressive around the recurrent laryngeal nerve

gross: 70; mico: 60; nodal: 50

parafollicular C cells

TSH stimulation

> 1 cm tumor
N+ or DM
aggressive histologic subtypes
pT4+ papillary and age > 45

easier to follow up (whole body iodide scans and serum thyroglobulin)

80%

< 5%

epithelioid (40%) > mixed or biphasic (35%) > sarcomatous or mesenchymal

10%

dyspnea & nonpleuritic. Recurrent pleural effusion and/or pleural thickening

Superior: T1
Inferior: L2
Lateral: skin
medial: ipsi edge of vertebral body
need to block critical structures and supplement with electrons

8 mos

p16, p14, and (NF-2)

yes (based on retrospective)

positive: calretinin, vimentin, WT1, cytokeratin

negative: acid-schiff, CEA, Leu-M1

negative margin: 50 Gy
close/+ margin: 54-60 Gy
gross disease: > 60 Gy

NO PEAK, increases with increasing age

asbestos - ambiboles (rodlike) > chrysotile (serpentine)

pleurodesis with talc

local progression -> resp failure or infection

not a candidate (more adv disease, mixed histology, or medically highrisk)stripping of the pleura from the apex of the lung to the diaphragm, removing pericardium and parietal pleura

parietal pleura, lung, mediastinal nodes, pericardium, and ipsilateral diaphragm; with a graft to prevent herniation of abd contents through the defect; mediastinal nodal dissection should also be done

sarcomatous type

WBC > 8.3, Performance status, sarcomatous histology, male gender

peritoneum, pericardium, and tunica vaginalis testis

cisplatin and premetrexed (also gemcitabine)

20-40 years

historically RT 7Gy x 3 given to areas of invasive procedure to prevent needle tract seeding but proven to not reduce rate

yes. Men more affected

N3: contralat MN/IM/Hilar or SCLV
N2: ipsi MN, subcarinal, IM
N1: ipsi hilar

NO; smoking alone does not but exacerbates asbestos related mesothelioma

T1: ipsi parietal pleura and no or focal visceral pleura
T2: visceral or diaphragm mm or lung parenchyma
T3: mediastinal fat or soft tissue of CW or pericardium
T4: rib or diffuse CW or mediastinal organs or contral pleura or spinne or pericardial effu w/ + cytology or brachial plexus

high grade
size > 5 cm
deep location
recurrent dz
leiomyosarcoma

extremity (60%) > trunk (30%) > H&N (10%)

angiosarcoma that arises from chronic lymphedmea (most often as complication of mastecomy +/- radiotherapy)

MRI +/- CT of area
Chest imaging (CT)

50 Gy/2 Gy fx

75%

incisional

75%

IA: T1 G1
IB: T2 G1
IIA: T1, G2-3
IIB: T2, G2
III: T2, G3 or N1
IV: M1

high-grade extremity soft tissue sarcoma

> 70-80 Gy

20%

high grade undiff pleomorphic sarcoma (malignat fibrous histiocytoma) 20-30%
liposarcoma (10-20%)
leiomyosarcoma (5-10%)
synovial sarcoma (5-10%)
malignant peripheral nerve sheath tumors (5-10%)

T1 <= 5 cm (a - superficial ; b - deep)
T2 > 5 cm (a - superficial ; b - deep)

N1 regional mets

preop RT (35) > PORT (17)
No diff in LC (92), RFS (59), or OS (73 v 67)
In long term, PORT was associated with worse fibrosis and joint stiffness

Gardner - desmoid
RB - bone and STS
NF1 - benign neurofibromas and malignant periopheral nerve sheath tumors
Li Fraumeni - bone and STS

50 Gy + 10-16 Gy for negative marging
16-20 Gy for positive margin
20-26 Gy for grossly positive

50

Age > 50
recurrent disease
positive surgical margins
fibrosarcoma (including desmoid)
malignant peripheral nerve sheath tumor

primitive mesenchyme of the mesoderm

synovial t(X, 18)
clear cell sarcoma t(12,22)
ewing sarcoma/PNET t(11, 22)
alveolar rhabdomyosarcoma t(2,13), t(1,13)

T1, low grade s/p surgical resection w/ > 1 cm margins

SCARE: synovial, clear cell, angiosarcoma, rhabdomyosarcoma, epitheliodi
all around 15-25%

5%

RT
thorotrast
chlorophenols
vinyl chloride
arsenic
herbicides

lung; myxoid liposarcoma spreads to nonpulm sites

N-myc amplification, LOH 1p +11q, diploid DNA, telomerase

bones of the skull and orbit

< 1 yo of age best

no, in high risk only positive disease covered

urine catechol, U/A;
imaging - bone scan, abd u/s, CT c/a/p, MRI, I-131 (MIBG) scan
bone marrow biopsy
pathology - DNA content, n-myc amplification, and cytogenetics

wilms! (neuroblastoma rarely metastatsizes to the lungs)

75%

neuroblastoma

constitutional symptoms
periorbital ecchymosis ("raccoon eyes")
"blueberry muffin" sign (nontender blue skin mets)
scalp nodules
bone pain
diarrhea (increased VIP)
Horner syndrome
opsomyoclonus truncal ataxia
Kerner-Morrison syndrome (diarrhea, low K)

can be used for refractory NB

40%

neuroblastoma

NF 1, hirschsprung disease, fetal hydantoin syndrome

SANDS
Stage
Age
N-myc
DNA ploidy
Shimada classification

high risk (5%) then low risk

cytoxan
doxorubicin
etoposide
carboplatin
ifosfamide

adrenal medulla > paraspinal > post mediastinum

neuroblastoma
ganglioneuroblastoma
ganglioneuroma

2B; IV (not S because older than 1 year)
I: GTR =/- microscopic dz, ipsi LN- but removed LNs can be positive
IIA: STR
IIB: GTR/STR with involved nonadherent ipsi LNs
III: unresectable
IV: distant dz
IVS: (stage 2B or less and distant dz only at liver, skin, and/or <10% of BM) in infants < 1 year

infants

age and stage and dx

neural crest cells of the sympathetic ganglion

neuroblastoma

bone > LNs> bm, liver, skin, orbits (lung mets rare)

urinary catecholamines (VMA/HVA)

post chemo, preop tumor bed to a dose of 21.6 Gy if GTR and 36 Gy if gross disease

17 mos

90%

homer-wright pseudorosettes, hemorrhage and calcification

anterior: terible T's - thyrmoma, thymoma, terrible lymphoma; germ ell tumors
middle: cysts > lymphoma, teratomas > sarcomas (osteosarcoma, fibrosarcoma, angiosarcoma, rhabdomyosarcoma, granuloma)
posterior: neurogenic tumors (PNET, schwannoma, neurofibroma, neuroblastoma, ganglioneuroma), pheochromocytoma

chemo (cisplatin, doxorubicin, cyclophosphamide) --> RT vs surgery (--> PORT)

R0: 45-54 Gy
R1: 55-60 Gy
R2:: 60-70 Gy

completeness of resection
presence of LN mets

control signs and symptoms medically prior

less prevalent, more aggressive, and worse prognosis. 1 vs 30% LNs

pathogenesis: autoantibody to Ach receptor at the postsynaptic endplate
presentation: easy fatigability, ptosis, and diplopia (worse w/ movement, opposite of Lambert-Eaton)
diagnosis: tensilon test (edrophonium)
treatment: anticholinesterase or thymectomy

1/3rd of mediastinal tumors

50 years; no gender predilection

myasthenia gravis
red cell aplasia
hypogammaglobulinemia

I/II - 90%
III: 60%
IV: 30%

invasive: 50% noninvasive: 70%

40% (conversely 10% of patients with MG have thymoma)

stage III-IVA, +/close margins, or thymic carcinoma

stage I: encapsulated, no microscopic capsular invasion
II: macroscopic invasion into surrounding fat or mediastinal pleura or microscopic capsule
III: ext to surounding organs or great vessels
IVA: pleural or pericardial dissemination
IVB: +LNs or DM

median sternotomy

thymoma with gross residual dz
or thymic carcinoma with R1-R2

it is in the anterior mediastinum and it is involved in the processing and maturation of T lymphocytes to recognize foreign antigens from "self" antigens

3rd pharyngeal pouch

lung; (carcinoma to lung > bone, liver)

masaoka stage and completeness of resection

50% of anterior mediastinum and 20% of all mediastinal tumors

H&N region - upper aerodigestive tract (80%)

beta 2 microglobulin & albumin
stage I: beta 2 micro < 3.5 and albumin >3.5

stage III: beta 2 microglobulin > 5.5

I: 62 mos (5 years)
II: 44 mos (3-4 years)
III: 29 mos (2 years)

mgus - monoclonal gammapathy of unspefied significance; prolif of Ig in absecne of clinical, radiographic and lab evidence of MM; rate of trasnformation is 1% a year

yes (primary drainage)

SP is localized neoplasm and a curative paradigm is employed

1st line antimyeloma therapy b/c they decrease skeltal events and decrease bone pain

serum Cr: A - Cr < 2 mg/dl; B - Cr > 2 mg/dl

Blacks > white; no gender prelidection

1. clonal plasma cells of >= 10% (on either BM bx or bx from other tissue)
2. monoclonal protein in serum or urine
3. evidence of end organ damage

SBP - secretory
SEP - nonsecretory

CBC
LDH
Calcium
albumin, beta
SPEP, UPEP
BM biopsy (unilateral)
skeletal survey

high dose melphalan (proven vs TBI in randomized trial)

polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes -- variant of MM

25%

MM constitutes 90%; Solitary plasmacytoma 10%

SEP - 35% LN positivity
SBP - rare

lytic (negative on bone scan)

70%

it is a major histocompatibility complex class 1 molecules

T3/stage III

liver parenchymal mets - M1/IV

diarrhea (g3-4)

all decrease risk

superior: top of diaphragm
inferior: obturator foramen
lateral: peritoneal reflection

I: 80
II: 60
III: 30
IV: 10

yes; shown to reduce ovarian and fallopian tube malignancies

risk of primary peritoneal cancer persists

70%

nulliparity, advanced age at 1st birth, hormone replacement therapy, high-fat/lactose diet, family hx, BRCA 1/2 and HNPCC, older age

IA: limited to 1 ovary with capsule intact
IB: both ovaries with capsule intact
IC: rupture capsule, tumor on surface, malignant cells in ascites, or peritoneal washings

IIA: extension and/or implants on uterus and/or tubes
IIB: extension to and/or implants on other pelvic tissues
IIC: pelvic extension and or implants with malignant ascites or washings

IIIB (IIIA - is microscopic)
IIIC: peritoneal mets > 2 cm in size or regional LNs

3-6 mos

6% OS benefit and 11% RFS (ICON1 and ACTION trials)

diagnostic and therapeutic laparotomy for surgical staging and cytoreduction; goal to cytoreduce to < 1 cm of gross residual disease

80% are LOCAL

lymphocyte-rich

lymphocyte-depleted

patients with nodular lymhocyte predominant HL

classical more common

nodular sclerosing > mixed cellularity > lymphocyte depleted > lymphocyte rich

nodular sclerosing

bimodal peak - age 25 and 65

lymphocyte depleted

CD 15 and 30 : NEGATIVE
CD 45 and 20: POSITIVE

cervical chain (70%) and mediastinum (50%)

CD 15 and 30 : POSITIVE
CD 45 and 20: NEGATIVE

TLI - mantle field, inverted Y field and spleen field

STLI excludes iliac and inguinal regions

MOPE-ABV
Mechlorethamine
Oncovin (vincristine)
Prednisone
Etoposide
Adriamycin
Bleomycin
Vinblastine

Adriamycin
Bleomycin
Vinblastine
Dacarbazine

IFRT: involved lymphoid region
RFRT: involved regions + immediately adjacent LN regions
INRT: covers prechemo tumor volume

1. Chemo + planned IFRT
2. ABVD x 6 cycles +/- to initial bulky disease and/or residual PET+ sites at restaging

Bleomycin
Etoposide
Adriamycin
Cyclophosphagmide
Oncovin (vincristine)
Procarbazine
Prednisone

used in Europe for advanced-stage HD

leukemias < 5 yrs after txt, solid malignancies occur > 7 yrs after txt

1. chemo + planned IFRT
2. ABVD x 4 cycles +/- IFRT to residual PET+ sites at restaging

LN 10%; OS 85%

Asymmetry, Borders, Color, Diameter (> 6 mm), Enlargement

15%

increasing thickness
# of nodes involved
ulceration
Clark level (if < 1 mm)
satellitosis

female
young age
extremity location

15%

H&N highest; proximal lowest

lentigo maligna

T3b;
T1 <= a mm; T2 1-2 mm
T3 2-4 mm; T4 > 4 mm
a - no ulceration; b - ulceration

acral lentiginous; commonly affects palms/soles and subungual areas

uv
fair complexion, light hair/eyes
numerous benign nevi or large atypical
prior melanoma
family hx
polyvinyl chloride exposure

sentinel LN status

desmoplastic melanoma

superficial spreading (70%); nodular (25%)

full thickness or excisional biopsy with 1-3 mm margin is preferred for tissue diagnosis

avoid to permit accurate subsequent lymphatic mapping

superficial spreading

trunk: males
extremities: females

5%

hutchinson freckle

M1a: skin, SQ, distant LNs
M1b: lung only
M1c: viscera or other sites with increased LDH

S100
HNB-45
Melan-A

< 10%

intermittent intense exposure to UVA and UVB, such as hx of blistering burns in childhood

N1: 1+
N2: 2-3
N3: >= 4, or matted

30-40%

standard risk: > 3 yo, GTR/NTR < 1.5, and M0
high risk: < 3 yo or STR > 1.5 residual , or M+

MRI spine and CSF cytology (delay to day 10 to avoid a false + result from surgical debris)

bimodal age distribution - 7 and 25

small round blue cells and homer-wright rosettes; + stain for neuron specific enolase, synaptophysin, and nestin

large cell/anaplastic

downward deviation of gaze from increased cranial pressure (CN III, IV, and VI)

neuroectodermal cells from the sup medullary velum (germinal matrix of cerbellum) or cerebellar vermis

ha, n/v, altered mentation due to hydrocephalus, truncal ataxia, head bob, and diplopia (CN VI)

NO

deletion of 17p; delition of 16 q

Lympoma
Ewing
ALL
Rhabdomyosarcoma
Neuroblastoma
Neuroepithelioma
Medulloblastoma
Retinoblastoma

LEARN NMR

low grade glioma; 2nd is most common pediatric CNS tumor

younger patients

baseline audiometry, IQ testing, TSH and growth measures

midline cerbellar vermis (75)

medulloblastoma, ependymoma, astrocytoma, brainstem glioma, JPA, hemangioblastoma, mets

T2;
T1: <= 3 cm, confines
T2: > 3 cm, partial fill of 4th ventricle, invades 1 adj structure
T3a: invades 2 structures, complete fill of 4th ventricle
T3b: extends into brain stem, arises from floor of 4th ventricle, complete fill of 4th ventricle
T4: extends beyond aqueduct of slyvius or foramen magnum to involve 3rd ventricle/midbrain/upper cervical cord

classic, nodular/desmoplastic, and large cell/anaplastic

M2;
M0: no mets
M1: + CSF
M2: nodular seeding intracranially
M3: nodule in subarachnoid space in cord
M4: extraneural spread

desmoplastic

5%; gorlin (PTCH mutation) and turcot (APC mutation)

yes, males

rarely , can happen to bone

embryonal tumors (along with PNET and atypical teratoid rhabdoid tumor)

ventricular shunt/drain, steroids, acetazolamide (diamox)

early: pain, bleeding, diplopia, infection, edema
late: retinopathy (42% at 5 yrs, increasing to 80-90%), cataracts, keratitis, optic neuropathy

resection: lesions of the iris or ciliary body
not used for uveal lesions

apical height, and basal diameter
medium: AH 2.6-10 mm, BD 6-16 mm

macula is important for color vision

pt choice
salvage therapy
tumor involving > 40% of intraocular volume
tumor in a nonfunctional eye
symptomatic pt (pain)
eye with marked neovascularization
extrascleral extension

1-2%; liver is the most common site

outer fibrous layer (sclera), middle vascular layer (choroid), and inner nerve layer (retina)

uveal melanocytes of the uveal stroma (neural crest origin)

observation (33% of patients progress based on COMS)

98% are white

plaque brachytherapy

2 mm medial to the macula

ocular mets; ocular melanoma

ocular melanoma

50% at 15 years

intraocularly (vitreous, aqueous, or along ciliary vessels)
extraocularly (optic nerve, transsclerally, vascular tracking)
extrascleral extension

80+%

to the tumor apex (generally 85Gy); with a 2 mm margin around the tumor

no (risk of tumor seeding); dx made by exam and imaging

choroid, cilary body, and iris

ciliary body involvement/ extraocular extension

a: none
b: +cb
c: +exo
d: +cb +exo
e: ++exo

uvea, (mostly choroidal) > adnexa > conjunctiva

I-125;

enucleation (alternatively charged particles if available)

ora serrata

best - spindle cell; worse - epithelioid

NO differnce in OS or CSS; COMS study

30% present

13%; treatment failure or ocular pain from brachytherapy complications

peripapillary tumors

No

retinoblastoma

with RT: 50% at 50 yrs
w/o 25% at 50 years

enucleation with removal of 1.5 cm of optic nerve

1st hit is a germline deletion; 2nd hit is mitotic recombination error

leukocoria (abnl white reflection from the retina) > strabismus > painful glaucoma
developing countries more advanced - proptosis, orbital mass, and mets

sporadic (2 yo); familial (1 yo); majority (95%) < 5%

25% (bilateral) versus 75% (unilateral)

retinoblastoma

metastatic leukemia; retinoblastoma is the #1 primary tumor

bone, liver spleen; 15% present with DMs

RB1 tumor suppressor gene on chromosome 13

G1/S

90%

entire globe + optic nerve (8mm)

yes - 5% at 50 years

better orbital bone sparing; better lens sparing

bilateral retinoblastoma and midline PNET (pineal or suprasellar); represents 5% of hereditary RB

generally not (can lead to seeding)

individualize the treatment of each eye (preservation if possible)

reese-ellsworth grouping system; predicts visual preservation after EBRT (not survival)

neuroepithelial cells (from nucleated photoreceptor layer of the inner retina)

germline (60%) > heritable (40%)

preserve eye with chemotherapy x 6 cycles (VCE - vincristine/carboplatin/etoposide) -> focal therapy

osteosarcoma

YES; PFS 24 -> 69% (45%); OS 46 -> 76 (30%) with the addition of chemo to RT for NPX cancer; (criticized for poor RT alone results)

negative (w/ regards to LC and OS)

fossa of rossenmuller (fossa post to torus tubarius)

N3b (N3a is > 6 cm; in contrast to other H&N sites that just have N3 - > 6 cm)

bone > lungs, liver, and brain

T2 and above, N+ (al-sarraf and Lee)

T1: confined to NPX or extension to OPX, nasal cavity w/o paraphayngeal extension
T2: parapharyngeal extension
T3: bony structures/paranasal sinuses
T4: intracranial extension/CNs/infratemporal fossa, HPX, orbit, masticator

2b (undiff or lymphoepithelial)

50%

N2

petrosphenoidal syndrome: CN III-IV and VI involvement (oculomotor signs/Sx)
retroparotidian syndrome: CN IX-XII involvement

75%

hell no.

NO; multiple RCTs in asia showed no benefit

cisplatin IV 100 mg/m2 q3 weeks concurrently with RT then 3 additional cycles of cisplatin + 5FU

III (T1N2)
N2 - bilateral nodal dz < 6 cm
T1 - w/o parapharyngeal involvement

N3: IVB

NO; multiple RCTs in asia showed no benefit and 1 italian study

N1 - any # of nodes confined to the ipsi neck < 6 cm
N2 - bilateral

mean dose < 26 Gy

lower neck nodes involv, retropharyngeal LN involv, WHO type IIB

2.12 x 33 = 69.96 Gy to GTV
1.8 Gy x 33 = 59.4 Gy to intermediate risk ares
1.64 Gy x 33 = 54 Gy to low-risk areas

T1: designates lesions without parapharyngeal extension (can inv Opx/nasalcavity

T4; and other intracranial structures, infratemporal fossa, HPX, masticator space

50

nasopharyngeal cancer

neck mass > epistaxis, otalgia, nasal congestion, trismus

superiorly: invades cavernous sinus w/ predom CN VI involv
inferiorly/posteriorly: oropharyngeal structures
laterally: parapharyngeal space
anteriorly: nasal cavity

superior: sphenoid bone
inferior: soft palate
posterior: clivus/C1-2
anterior: post edge of choanae

WHO type I (keratinizing SCC)

WHO IIb (undiff or lymphoepithelial) (better LC but more distant spread)

N stage

CNs IX-XI

CNs III-IV, V1-2, VI

dermatomyositis

WHO I: keratizing squamous cell carcinoma (SCC) - 20%
WHO IIA: nonkeratizing SCC (nondifferentiated) (35%)
WHO IIb: undiffentiated or lymphoepithelial (45%)

consumption of salted fish and preserved meats, EBV infection, and smoking for keratinzing squamous cell type (no alcohol association)

retropharyngeal nodal involvement (node of Rouviere)

T3: denotes bony structures and/or paranasal sinus

can track through the foramen lacerum

deep-seated painless mass with hx of slow growth

> 50 Gy; rec 50-56 Gy

22% (7% if negative margin; 26% if positive)

well-diff (grade 1) fibrosarcoma

2% associated with APC --> familial adenomatous polyposis (FAP)

3rd or 4th

gardner syndrome

NO

surgical resection

women; no race or ethnic predilection

trauma, or high estrogen

retroperitoneal/intrabdominal

aggressvie fibromatosis

SOD - sebaceous cysts, osteomas, desmoid tumors

> 20 Gy

trunk/extremity
abdominal wall
intraabdominal compartment

bowel ischemia or obstruction

1. hormone ablation (tamoxifen)
2. NSAIDs (sulindac)
3. low-dose cytotoxic chemo (methotrexate or doxorubicin based)
4. targeted therapy (imatinib)

yes, up to 10+ years later

poor outcome

myxopapillary ependymoma

when resection is incomplete or anaplastic histology

ependymomas

completeness of surgical resection

ependymal cells lining the ventricles

like medulloblastoma/PNET: CSI 36Gy + vincristine; boost cavity/gross dz 45-50.4 (spine) and 54-59.4Gy if cranial --> adjuvant RT

NO (not until resection of primary)