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MM1 Leesy > B6.016 - Soft Tissue Tumors Prework Part 2 > Flashcards

B6.016 - Soft Tissue Tumors Prework Part 2 Flashcards

1
Q

vascular tumors

A

hemangioma

kaposi sarcoma

angiosarcoma

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2
Q

what are hemangiomas

A

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

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3
Q

types of hemangiomas

A

capillary hemangioma

pyogenic granuloma (lobular capillary hemangioma)

cavernouse hemangioma

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4
Q
A

capillary hemangioma

closely packed aggregates of thin walled capillaries

juvenile hemangiomas of the skin of newborns is extremely common

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5
Q
A

cavernous hemangioma

larger, more frequently involve deep structures like liver

histo: large cavernous vasvcular spaces filled with blood

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6
Q
A

capillary hemangioma

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7
Q
A

capillary hemangioma

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8
Q
A

cavernous hemangioma

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9
Q
A

pyogenic granuloma (lobular capillary hemangioma)

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10
Q

kaposi sarcoma feautures

A

HHV8 associated

low to intermediate grade sarcoma, locally aggressive

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11
Q

what are the 4 forms of KS

A

classic or european

african/endemic

transplant associated

AIDS associated

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12
Q

describe classic/european KS

A

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

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13
Q

describe african/endemic KS

A

protracted, progressive coures, lymphadenopathy, not associated with HIV

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14
Q

describe transplant associated KS

A

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

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15
Q

describe AIDS associated KS

A

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

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16
Q

pathogenesis of KS

A

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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17
Q

histology of KS and 3 stages/clinical coures

A

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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18
Q
A

KS

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19
Q
A

KS

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20
Q
A

KS

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21
Q

feaures of angiosarcoma

A

more often in older adults

most commonly involves skin, soft tissue, breast and liver

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22
Q

what is hepatic angiosarcoma associated with

A

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

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23
Q

what is upper extremity angiosarcoma associated with

A

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

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24
Q

what can angiosarcoma be associated with

A

radiation (post radiation angiosarcoma)

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25
Q
A

angiosarcoma

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26
Q
A

angiosarcoma

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27
Q

histo and prognosis of angiosarcoma

A

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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28
Q
A

angiosarcoma

29
Q
A

angiosarcoma

30
Q
A

angiosarcoma

31
Q
A

angiosarcoma

32
Q
A

angiosarcoma

33
Q
A

angiosarcoma

34
Q

benign nerve sheath tumors

A

schwannoma

neurofribroma

35
Q

malingnant nerve sheath tumors

A

malignant peripheral nerve sheath tumor

36
Q

shwannoma features

A

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

37
Q

histo and IHC of shwannoma

A

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

IHC: S100 positive in all cells

38
Q
A

Schwannoma

39
Q
A

schannoma

40
Q
A

schannoma

41
Q

neurofibroma features

A

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

42
Q

histo and IHC of neurofibroma

A

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

43
Q
A

neurofibroma

44
Q
A

neurofibroma

45
Q
A

plexiform neurofibroma NF1

46
Q
A

plexiform neurofibroma NF1

47
Q
A

plexiform neurofibroma NF1

48
Q
A

plexiform neurofibroma

49
Q
A

plexiform neurofibroma

50
Q

feautres of malignant peripheral nerve sheath tumor

A

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%

51
Q
A

MPNST

52
Q

histo, IHC of MPNST

A

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
53
Q
A

MPNST

54
Q
A

MPNST

55
Q
A

MPNST with heterologous elements (chondro)

56
Q
A

MPNST with heterologous elements (rhabdo/triton)

57
Q

tumors of uncertain histogenesis

A

synovial sarcoma

58
Q

features of synovial sarcoma

A

cell of origin unclear

most occur in pts 20-40 yrs

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

59
Q

histo/features of synovial sarcoma and tx

A

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

60
Q
A

synovial sarcoma

61
Q
A

synovial sarcoma

62
Q
A

synovial sarcoma

63
Q
A

synovial sarcoma

epithelial marker

64
Q
A

synovial sarcoma

mesenchymal marker

65
Q

describe UPS

A

undifferentiated pleomorphic sarcoma

previously called MFH

odler adults

proximal extremities and retroperitoneum

usually large grey-white unencapsulated mass in muscle

66
Q

UPS histo

A

no differentiation toward specific tissue type

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

aggressive, may recur metastasize

67
Q
A

UPS

68
Q
A

UPS

69
Q
A

UPS

MM1 Leesy (6 decks)

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