B6.016 - Soft Tissue Tumors Prework Part 2 Flashcards
vascular tumors
hemangioma
kaposi sarcoma
angiosarcoma
what are hemangiomas
majority are superficial lesions, often head and neck, may occur internally especially the liver
types of hemangiomas
capillary hemangioma
pyogenic granuloma (lobular capillary hemangioma)
cavernouse hemangioma

capillary hemangioma
closely packed aggregates of thin walled capillaries
juvenile hemangiomas of the skin of newborns is extremely common

cavernous hemangioma
larger, more frequently involve deep structures like liver
histo: large cavernous vasvcular spaces filled with blood

capillary hemangioma

capillary hemangioma

cavernous hemangioma

pyogenic granuloma (lobular capillary hemangioma)
kaposi sarcoma feautures
HHV8 associated
low to intermediate grade sarcoma, locally aggressive
what are the 4 forms of KS
classic or european
african/endemic
transplant associated
AIDS associated
describe classic/european KS
chronic, indolent coures of older men, skin lesions, not associated with HIV
describe african/endemic KS
protracted, progressive coures, lymphadenopathy, not associated with HIV
describe transplant associated KS
aggressive coures, involves LN, mucosa and internal organs, may resolve after withdrawal of immunosuppression
describe AIDS associated KS
HIV-1 most aggressive form, most common AIDs associated cancer of US
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
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

KS

KS

KS
feaures of angiosarcoma
more often in older adults
most commonly involves skin, soft tissue, breast and liver
what is hepatic angiosarcoma associated with
hepatic angiosarcoma: associated with chemical carcinogens (arsenic, thorotrast, polyvinyl chlloride PVC)
what is upper extremity angiosarcoma associated with
lymphedema following axillary lymph node dissection in radical mastectomy for breast cancer
what can angiosarcoma be associated with
radiation (post radiation angiosarcoma)

angiosarcoma

angiosarcoma
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

angiosarcoma

angiosarcoma

angiosarcoma

angiosarcoma

angiosarcoma

angiosarcoma
benign nerve sheath tumors
schwannoma
neurofribroma
malingnant nerve sheath tumors
malignant peripheral nerve sheath tumor
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
histo and IHC of shwannoma
histo: spindle cells show nuclear palisading, Antoni A and Antoni B areas
IHC: S100 positive in all cells

Schwannoma

schannoma

schannoma
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
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

neurofibroma

neurofibroma

plexiform neurofibroma NF1

plexiform neurofibroma NF1

plexiform neurofibroma NF1

plexiform neurofibroma

plexiform neurofibroma
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%

MPNST
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

MPNST

MPNST

MPNST with heterologous elements (chondro)

MPNST with heterologous elements (rhabdo/triton)
tumors of uncertain histogenesis
synovial sarcoma
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
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

synovial sarcoma

synovial sarcoma

synovial sarcoma

synovial sarcoma
epithelial marker

synovial sarcoma
mesenchymal marker
describe UPS
undifferentiated pleomorphic sarcoma
previously called MFH
odler adults
proximal extremities and retroperitoneum
usually large grey-white unencapsulated mass in muscle
UPS histo
no differentiation toward specific tissue type
malignant spindle cells in storiform pattern, large bizarre mulitnucleated pleomorphic cells
aggressive, may recur metastasize

UPS

UPS

UPS