B6.016 - Soft Tissue Tumors Prework Part 2 Flashcards Preview

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Flashcards in B6.016 - Soft Tissue Tumors Prework Part 2 Deck (69):
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vascular tumors

hemangioma

kaposi sarcoma

angiosarcoma

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what are hemangiomas

majority are superficial lesions, often head and neck, may occur internally especially the liver

 

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types of hemangiomas

capillary hemangioma

pyogenic granuloma (lobular capillary hemangioma)

cavernouse hemangioma

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capillary hemangioma 

closely packed aggregates of thin walled capillaries

juvenile hemangiomas of the skin of newborns is extremely common

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cavernous hemangioma

larger, more frequently involve deep structures like liver

histo: large cavernous vasvcular spaces filled with blood

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capillary hemangioma

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capillary hemangioma

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cavernous hemangioma

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pyogenic granuloma (lobular capillary hemangioma)

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kaposi sarcoma feautures

HHV8 associated

low to intermediate grade sarcoma, locally aggressive

 

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what are the 4 forms of KS

classic or european

african/endemic

transplant associated

AIDS associated

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describe classic/european KS

chronic, indolent coures of older men, skin lesions, not associated with HIV

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describe african/endemic KS

protracted, progressive coures, lymphadenopathy, not associated with HIV

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describe transplant associated KS

aggressive coures, involves LN, mucosa and internal organs, may resolve after withdrawal of immunosuppression

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describe AIDS associated KS

HIV-1 most aggressive form, most common AIDs associated cancer of US

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pathogenesis of KS

regardless of clinical subtype 95% of KS are infected iwth HHV8, aka KSHV

KSHV can be trnasmitted sexually as well as by poorly understood nonsexual routes

KSHV is a necessary requirement for KS development but tumor progression also requires a cofactor like HIV

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histology of KS and 3 stages/clinical coures

patches

plaques

nodules

proliferation of slit like vascular channels, lined by plump spindle cells with extravasated RBCs

frequent mitoses

clinically variable 

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KS

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KS

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KS

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feaures of angiosarcoma

more often in older adults

most commonly involves skin, soft tissue, breast and liver

 

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what is hepatic angiosarcoma associated with

hepatic angiosarcoma: associated with chemical carcinogens (arsenic, thorotrast, polyvinyl chlloride PVC)

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what is upper extremity angiosarcoma associated with

lymphedema following axillary lymph node dissection in radical mastectomy for breast cancer

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what can angiosarcoma be associated with

radiation (post radiation angiosarcoma)

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angiosarcoma

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angiosarcoma

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histo and prognosis of angiosarcoma

histo: vascular channels lined by atypical plump malignant endothelial cells

IHC: CD31+

Prognosos: poor outcome with frequent local recurrence and distant metastasis

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angiosarcoma

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angiosarcoma

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angiosarcoma

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angiosarcoma

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angiosarcoma

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angiosarcoma

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benign nerve sheath tumors

schwannoma

neurofribroma

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malingnant nerve sheath tumors

malignant peripheral nerve sheath tumor

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shwannoma features

encapsulated benign nerve sheath tumor composed of schwann cells; age 20-40

eccentric mass loosly attached to a nerve

usually 90% sporadic; 3-10% associated with NF2

excision curative

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histo and IHC of shwannoma

histo: spindle cells show nuclear palisading, Antoni A and Antoni B areas

IHC: S100 positive in all cells

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Schwannoma

 

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schannoma

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schannoma

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neurofibroma features

non encapsulated benign nerve sheath tumor composed of admixture of axons, schwann cells, perineural cells and fibroblasts

Most (90%) are sporadic, 10% occur in NF1 and may be solitary multiple large or plexiform tumors

 

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histo and IHC of neurofibroma

histo: bundles of spindle cells with angulated, elongated nuclei and wavy cell process in a loose myxoid and collagenous background

IHC: S100 positive in scattered cells

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neurofibroma

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neurofibroma

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plexiform neurofibroma NF1

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plexiform neurofibroma NF1

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plexiform neurofibroma NF1

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plexiform neurofibroma 

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plexiform neurofibroma

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feautres of malignant peripheral nerve sheath tumor

sarcoma arising from a nerve or from a benign nerve sheath tumor or showing nerve sheath differentiation

50% sporadic

50% associated with NF1

adults 20-50

70% arise in major nerve trunks

local recurrence >40%, metastasis 30-60%

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MPNST

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histo, IHC of MPNST

histo: spindle and epitheliod cell variants

IHC: foal S100 in 60%

15% may be mixed with other sarcoma types (heterologous elements)

- rhabdomyosarcoma (triton tumor)

-cartilage or bone

-angiosarcoma, glandular elements

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MPNST

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MPNST

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MPNST with heterologous elements (chondro)

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MPNST with heterologous elements (rhabdo/triton)

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tumors of uncertain histogenesis

synovial sarcoma

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features of synovial sarcoma

cell of origin unclear

most occur in pts 20-40 yrs

most occur in deep soft tissue around large joints of extremities, mainly knees

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histo/features of synovial sarcoma and tx

histo: biphasic tumor with both epithelial and mesenchymal (sarcomatous) components

mesenchymal component may predominate

characteristic chromosomal transloacation (t(x;18))

treated with wide excision and chemo

commonly metastasizes to lung, bone, regional LN

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synovial sarcoma

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synovial sarcoma

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synovial sarcoma

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synovial sarcoma

epithelial marker

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synovial sarcoma

mesenchymal marker

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describe UPS

undifferentiated pleomorphic sarcoma

previously called MFH

odler adults

proximal extremities and retroperitoneum

usually large grey-white unencapsulated mass in muscle

 

 

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UPS histo

no differentiation toward specific tissue type 

malignant spindle cells in storiform pattern, large bizarre mulitnucleated pleomorphic cells

aggressive, may recur metastasize

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UPS

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UPS

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UPS