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70 Cards in this Set
- Front
- Back
Benign Vascular Neoplasms
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1. infantile hemangioma
2. congenital hemangioma 3. cherry angioma 4. sinusoidal hemangioma 5. hobnail hemangioma 6. tufted hemangioma 7. multifocal lymphangioendotheliomatosis with thrombocytopenia |
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borderline and low-grade malignant vascular neoplasms
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1. kaposiform hemangioendothelioma
2. dabska-type hemangioendothelioma 3. retiform hemangioendothelioma 4. epithelioid hemangioendothelioma 5. kaposi's sarcoma |
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malignant vascular neoplasms
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angiosarcoma
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perivascular neoplasms and neoplastic-like conditions
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1. glomous tumor (proper)
2. glomuvenous malformation (glomangioma) 3. infantile-type hemangiopericytoma/myofibromatosis 4. adult-type hemangiopericytoma 5. glomangiopericytoma 6. myopericytoma |
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?
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reactive angioendotheliomatosis
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?
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reactive angioendotheliomatosis
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glomeruloid hemangioma
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POEMS
mult small firm dome-shaped red-purple papules TRUNK, PROXIMAL EXTREMITIES (only few glomeruloid hemangiomas, most cherry angiomas) |
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major criteria POEMS
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1. polyneuropathy
2. monoclonal plasma cell proliferative disorders (almost always lambda) 3. sclerotic bone lesions 4. castleman disease 5. vascular endothelial growth factor elevation |
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minor criteria POEMS
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organomegaly (spleen, liver, LAD)
extravascular volume overload (edema, pleural effusion, ascites) endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic) skin changes (hyperpigmenation, hyertrichosis, glomeruloid hemangiomas, plethora, acrocyanosis, flushing, white nails) papilledema thrmobocytosis/polycythemia |
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other sx of POEMS
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clubbing, wt loss, hyperhyidrosis, pulmonary hypertenstion/restrictive lung disease, throbotic diatheses, diarrhea, low vitamin B12
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possible POEMS associations
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arthralgias
cardiomyopathy (systolic dysfunctio) fever |
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POEMS
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polyneuropathy and plasma cell disorder
and one other criteria |
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POEMS
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Polyneuropathy
organomegaly endocrinopathy M-protein skin lesions |
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glomeruloid hemangioma
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reactive, not neoplastic
increased VEGF 85% POEMS in assoc with multicentric Castleman's HHV8 IL6 produced by HHV8 = angiogenesis induces VEGF expression |
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glomeruloid hemangioma assoc with virus?
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HHV8
Castleman's increase VEGF = angiogenesis |
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?
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glomeruloid hemangioma
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?
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glomeruloid hemangioma
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glomeruloid hemangioma histo
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dilated vessels w grapelike capillaries looks like glomeruli
stroma w large cells and PAS + eosinophilic globules |
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microvenular hemangioma
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POEMS, but not classic
assoc w preg, OCPs small solitary slow evolving asymptomatic red papule FOREARMS also on trunk and legs, rare face ddx: patch KS |
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?
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microvenular hemangioma
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microvenular hemangioma histo
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prolif of capillaries and venules w collapsed lumina
slit like spaces full thickness reticular dermis |
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angiolymphoid hyperplasia w eosinophilia (ALHE)
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epitheloid hemangioma
young middle age females reactive, not neoplastic ssoc w trauma or AV shunting tan/brown/red papules or nodules grouped EARS or forehead/scalp asymptomatic, painful, pruritic, pulsatile some w regional LAD and eosinophilia 1/3 recurr |
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angiolymphoid hyperplasia w eosinophilia (ALHE)
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angiolymphoid hyperplasia w eosinophilia (ALHE)
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angiolymphoid hyperplasia w eosinophilia (ALHE)
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?
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angiolymphoid hyperplasia w eosinophilia
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angiolymphoid hyperplasia with eosinophilia (ALHE) histo
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lobular prolif of vessels
lined with HOBNAIL CELLS dense perivascular infiltrate w eos and lymphoid aggregates fibrous stroma, myxoid degeneration larger vessels |
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Kimura's Disease
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10 - 30 yr old asian males
subQ nodules head/neck (preauricular, parotid, or submandibular) PAINLESS cervical LAD assoc w atopic diathesis and elevated IgE marked tissue and blood eosinophilia histo w reactive lymphoid follicles w germinal centers and eos less prominent reactive endothelial changes |
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Kimura's Disease clinical
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subQ nodules head/neck
painless cervical LAD |
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spindle cell hemangio(endothelio)ma
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kids, young adults
M = F bule red firm ASYMPTOMATIC DISTAL EXTREMITIES solitary but then multiply vascular proliferations in traumatized vessel 60% recurr after excision assoc w Maffucci's, KTS, congenital lymphedema |
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?
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spindle cell hemangio(endotheloma
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spindle cell hemangio(endothe)oma histo?
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hemorrhagic nodules w 2 elements
1. thin walled cavernous spaces w organizing thrombi +/- focal masson's lesions 2. cellular areas containing spindle cells |
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lobular capillary hemangioma
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pyogenic granuloma
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pyogenic granuloma
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lobular capillary hemangioma
assoc w trauma, PWS,preg, drugs rapid growth wks mths then stable HANDS, FACE, sites of trauma |
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epulis
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pyogenic granuloma on gingival lesion
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pyogenic granuloma
assoc w drugs? |
retinoids
indinavir EGFR inhibitors |
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Campbell-DeMorgan spot
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cherry angioma
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cherry angioma
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campbell-demorgan spot
MC acquired cutaneous vascular lesion increase 20s or preg POEMs |
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sinusoidal hemangioma
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aquired, rare
middle-age females variant of cavernous hemangioma well-demardcated, solitary, mobile, deep dermal or subq papules/nodules arms and torso of women ddx angiosarcoma |
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targetoid hemosiderotic hemangioma
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hobnail hemangioma
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hobnail hemangioma
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targetoid hemosiderotic hemangioma
solitary lesion TRUNK or EXTREMITY central red-brown to blue papule w surrounding thin pale ring and ecchymotic halo surrounding halo fades |
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?
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targeroid hemosideric hemangioma
hobnail hemangioma |
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targetoid hemosideric hemangioma
hobnail hemangioma |
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tuffed hemangioma
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tuffed hemangioma
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tuffed hemangioma GLUT1?
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GLUT1 negative
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?
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tuffed hemangioma
cannonballs |
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congenital cutaneovisceral angiomatosis w thrombocytopenia
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multifocal lymphangioendotheliomatosis w thrombocytopenia
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multifocal lymphangioendotheliomatosis w thrombocytopenia
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sporadic
congenital cutaneous and visceral vascular lesions progeressive hundreds of flat or indurated red-brown to burgundy plaques/papules w central pallor and scar-like areas severe GI bleed mild-moderate thrombocytopenia |
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?
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multifocal lymphangioendothelimatosis hemangiomas
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multifocal lymphangioendotheliomatosis histo
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CD31, CD34, LYVE 1+
Light-absent D2-40, GLUT-1 neg |
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multifocal lymphangioendotheliomatosis hemangiomas
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diffuse neonatal hemangiomatosis
blue rubber bleb nevus syndrome glomuvenous malformations maffucci's extramedullary hematopoiesis |
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Kaposiform Hemangioendothelioma
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rare
< 2 yrs old Kasabach-Merritt esp retroperitoneal KHEs VEGFR3+ lymphatic endothelial phenotype vascular macules/plaques develop nodules deep start as bulging indurated masses no regress present later than infantile hemangiomas locally aggressive but not metastatic MCC death: thrombocytopenia and local tumor invasion |
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Kaposiform Hemangioendothelioma + factor?
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VEGFR3+ lymphatic endothelial phenotype
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Kaposiform Hemangioendothelioma treatment
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WLE
If large, IFN alpha-2a, vincristine, radiation, chemo CS not effective |
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?
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Kapasiform Hemangioendotelioma
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Kapasiform Hemangioendothelioma
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Kapsiform histo
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spindle cells CD31+
podoplanin + |
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Kapasiform Hemangioendothelioma vs KS histo
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KS no lobules
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Dabska's Tumor
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papillary intralymphatic angioendothelioma (PILA) or hobnail hemangioendothelioma
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hobnail hemantioendothelioma
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Dabska's Tumor
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Papillary intralymphatic angioendoendothelioma
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Dabska's Tumor
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Dabska's Tumor
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VEGFR3+
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Dabska's Tumor
clinical |
enlarging dermal or SQ mass or plaque
somtimes arising in preexisting lymangioma low metastatic |
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Dabska's Tumor location?
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MC head/neck or distal extremities of kids
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Dabska's Tumor histo
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rosette pattern
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?
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Dabsko's Tumor
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?
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Dabsko's Tumor
rosette pattern |
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Retiform Hemangioendothelioma
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Hobnail hemangioendothelioma (together wit Dabska's tumor)
rare LYMPHATIC differentiation low metastatic ADULTS solitary exophytic nodule or plaque on TRUNK or EXTREMITIES (distal LE) VEGFR3+ |
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Retiform Hemangioendothelioma histo
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rete testis-like pattern
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