Bolognia Vascular Tumors

Front 1

Benign Vascular Neoplasms

Back 1

1. infantile hemangioma
2. congenital hemangioma
3. cherry angioma
4. sinusoidal hemangioma
5. hobnail hemangioma
6. tufted hemangioma
7. multifocal lymphangioendotheliomatosis with thrombocytopenia

Front 2

borderline and low-grade malignant vascular neoplasms

Back 2

1. kaposiform hemangioendothelioma
2. dabska-type hemangioendothelioma
3. retiform hemangioendothelioma
4. epithelioid hemangioendothelioma
5. kaposi's sarcoma

Front 3

malignant vascular neoplasms

Back 3

angiosarcoma

Front 4

perivascular neoplasms and neoplastic-like conditions

Back 4

1. glomous tumor (proper)
2. glomuvenous malformation (glomangioma)
3. infantile-type hemangiopericytoma/myofibromatosis
4. adult-type hemangiopericytoma
5. glomangiopericytoma
6. myopericytoma

Front 5

?

Back 5

reactive angioendotheliomatosis

Front 6

?

Back 6

reactive angioendotheliomatosis

Front 7

glomeruloid hemangioma

Back 7

POEMS

mult small firm dome-shaped red-purple papules
TRUNK, PROXIMAL EXTREMITIES (only few glomeruloid hemangiomas, most cherry angiomas)

Front 8

major criteria POEMS

Back 8

1. polyneuropathy
2. monoclonal plasma cell proliferative disorders (almost always lambda)
3. sclerotic bone lesions
4. castleman disease
5. vascular endothelial growth factor elevation

Front 9

minor criteria POEMS

Back 9

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

Front 10

other sx of POEMS

Back 10

clubbing, wt loss, hyperhyidrosis, pulmonary hypertenstion/restrictive lung disease, throbotic diatheses, diarrhea, low vitamin B12

Front 11

possible POEMS associations

Back 11

arthralgias
cardiomyopathy (systolic dysfunctio)
fever

Front 12

POEMS

Back 12

polyneuropathy and plasma cell disorder
and one other criteria

Front 13

POEMS

Back 13

Polyneuropathy
organomegaly
endocrinopathy
M-protein
skin lesions

Front 14

glomeruloid hemangioma

Back 14

reactive, not neoplastic
increased VEGF
85% POEMS in assoc with multicentric Castleman's HHV8
IL6 produced by HHV8 = angiogenesis induces VEGF expression

Front 15

glomeruloid hemangioma assoc with virus?

Back 15

HHV8
Castleman's
increase VEGF = angiogenesis

Front 16

?

Back 16

glomeruloid hemangioma

Front 17

?

Back 17

glomeruloid hemangioma

Front 18

glomeruloid hemangioma histo

Back 18

dilated vessels w grapelike capillaries looks like glomeruli
stroma w large cells and PAS + eosinophilic globules

Front 19

microvenular hemangioma

Back 19

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

Front 20

?

Back 20

microvenular hemangioma

Front 21

microvenular hemangioma histo

Back 21

prolif of capillaries and venules w collapsed lumina
slit like spaces
full thickness reticular dermis

Front 22

angiolymphoid hyperplasia w eosinophilia (ALHE)

Back 22

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

Front 23

?

Back 23

angiolymphoid hyperplasia w eosinophilia (ALHE)

Front 24

?

Back 24

angiolymphoid hyperplasia w eosinophilia (ALHE)

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?

Back 25

angiolymphoid hyperplasia w eosinophilia (ALHE)

Front 26

?

Back 26

angiolymphoid hyperplasia w eosinophilia

Front 27

angiolymphoid hyperplasia with eosinophilia (ALHE) histo

Back 27

lobular prolif of vessels
lined with HOBNAIL CELLS
dense perivascular infiltrate w eos and lymphoid aggregates
fibrous stroma, myxoid degeneration larger vessels

Front 28

Kimura's Disease

Back 28

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

Front 29

Kimura's Disease clinical

Back 29

subQ nodules head/neck
painless cervical LAD

Front 30

spindle cell hemangio(endothelio)ma

Back 30

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

Front 31

?

Back 31

spindle cell hemangio(endotheloma

Front 32

spindle cell hemangio(endothe)oma histo?

Back 32

hemorrhagic nodules w 2 elements
1. thin walled cavernous spaces w organizing thrombi +/- focal masson's lesions
2. cellular areas containing spindle cells

Front 33

lobular capillary hemangioma

Back 33

pyogenic granuloma

Front 34

pyogenic granuloma

Back 34

lobular capillary hemangioma
assoc w trauma, PWS,preg, drugs
rapid growth wks mths then stable
HANDS, FACE, sites of trauma

Front 35

epulis

Back 35

pyogenic granuloma on gingival lesion

Front 36

pyogenic granuloma
assoc w drugs?

Back 36

retinoids
indinavir
EGFR inhibitors

Front 37

Campbell-DeMorgan spot

Back 37

cherry angioma

Front 38

cherry angioma

Back 38

campbell-demorgan spot
MC acquired cutaneous vascular lesion
increase 20s or preg
POEMs

Front 39

sinusoidal hemangioma

Back 39

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

Front 40

targetoid hemosiderotic hemangioma

Back 40

hobnail hemangioma

Front 41

hobnail hemangioma

Back 41

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

Front 42

?

Back 42

targeroid hemosideric hemangioma
hobnail hemangioma

Front 43

?

Back 43

targetoid hemosideric hemangioma
hobnail hemangioma

Front 44

?

Back 44

tuffed hemangioma

Front 45

?

Back 45

tuffed hemangioma

Front 46

tuffed hemangioma GLUT1?

Back 46

GLUT1 negative

Front 47

?

Back 47

tuffed hemangioma
cannonballs

Front 48

congenital cutaneovisceral angiomatosis w thrombocytopenia

Back 48

multifocal lymphangioendotheliomatosis w thrombocytopenia

Front 49

multifocal lymphangioendotheliomatosis w thrombocytopenia

Back 49

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

Front 50

?

Back 50

multifocal lymphangioendothelimatosis hemangiomas

Front 51

multifocal lymphangioendotheliomatosis histo

Back 51

CD31, CD34, LYVE 1+
Light-absent D2-40, GLUT-1 neg

Front 52

multifocal lymphangioendotheliomatosis hemangiomas

Back 52

diffuse neonatal hemangiomatosis
blue rubber bleb nevus syndrome
glomuvenous malformations
maffucci's
extramedullary hematopoiesis

Front 53

Kaposiform Hemangioendothelioma

Back 53

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

Front 54

Kaposiform Hemangioendothelioma + factor?

Back 54

VEGFR3+ lymphatic endothelial phenotype

Front 55

Kaposiform Hemangioendothelioma treatment

Back 55

WLE
If large, IFN alpha-2a, vincristine, radiation, chemo
CS not effective

Front 56

?

Back 56

Kapasiform Hemangioendotelioma

Front 57

?

Back 57

Kapasiform Hemangioendothelioma

Front 58

Kapsiform histo

Back 58

spindle cells CD31+
podoplanin +

Front 59

Kapasiform Hemangioendothelioma vs KS histo

Back 59

KS no lobules

Front 60

Dabska's Tumor

Back 60

papillary intralymphatic angioendothelioma (PILA) or hobnail hemangioendothelioma

Front 61

hobnail hemantioendothelioma

Back 61

Dabska's Tumor

Front 62

Papillary intralymphatic angioendoendothelioma

Back 62

Dabska's Tumor

Front 63

Dabska's Tumor

Back 63

VEGFR3+

Front 64

Dabska's Tumor
clinical

Back 64

enlarging dermal or SQ mass or plaque
somtimes arising in preexisting lymangioma
low metastatic

Front 65

Dabska's Tumor location?

Back 65

MC head/neck or distal extremities of kids

Front 66

Dabska's Tumor histo

Back 66

rosette pattern

Front 67

?

Back 67

Dabsko's Tumor

Front 68

?

Back 68

Dabsko's Tumor
rosette pattern

Front 69

Retiform Hemangioendothelioma

Back 69

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+

Front 70

Retiform Hemangioendothelioma histo

Back 70

rete testis-like pattern