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NMSP Exam 2 > Tumors > Flashcards

Tumors Flashcards

1
Q

what are the benign bone tumors?

A 
osteochondroma
chondroma
osteoid osteoma
osteoblastoma
giant cell tumors
aneurysmal bone cyst
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2
Q

what is the most common benign bone tumor?

A

osteochondroma

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3
Q

describe osteochondroma

A

benign tumors that arise in early adulthood

men > women

location: metaphysis near growth plate of tubular bone

hereditary tumors are AD and may progress to osteosarcoma

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4
Q

what gene is associated with osteochondroma?

A

EXT1 or EXT2

encode heparin sulfate glycosaminoglycans

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5
Q

describe chondromas

A

benign hyaline cartilage, solitary metaphyseal lesion

20-50 y/o

may be within medullary cavity (enchondroma)

blue/grey and translucent

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6
Q

what syndromes are associated with chondromas?

A

Ollier syndrome

Maffucci syndrome: chondromas + angiomas

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7
Q

what gene is associated with chondromas?

A

IDH1 and IDH2

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8
Q

describe chondrosarcomas

A

malignant tumor that may arise de novo or from preexisting osteochondroma or enchondroma

M > F

40 y/o +

axial skeleton (pelvis, shoulders, ribs)

painful and may metastasize

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9
Q

what genes are associated with chondrosarcomas?

A

EXT gene

IDH gene

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10
Q

what causes osteoid osteoma?

A

excess prostaglandin E2 production by osteoblasts

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11
Q

describe osteoid osteomas

A

less than 2 cm painful mass

appendicular skeleton

young men, teens and 20s

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12
Q

what will you see on XR with osteoid osteoma?

A

thick rind of reactive cortical bone

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13
Q

what will be seen on histo with osteoid osteoma?

A

haphazardly interconnecting trabeculae of woven bone that are rimmed by prominent osteoblasts

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14
Q

what is the treatment for osteoid osteoma?

A

radiofrequency ablation

responsive to ASA and NSAIDs

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15
Q

you see a XR of a lesion within the spine that is >2cm without bony reaction. the patient says he has an achy pain that doesn’t respond to ASA. what do you suspect?

A

osteoblastoma

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16
Q

what is the most common malignant tumor of bone?

A

osteosarcoma

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17
Q

what is the classic presentation of osteosarcomas?

A

young male with painful, enlarging mass around the knee

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18
Q

who is at risk for developing osteosarcoma?

A

older males with Pagets

people who have previously had radiation

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19
Q

what genes are associated with osteosarcoma?

A

Rb - sporadic osteo
TP53 - Le Fraumani syndrome
INK4a
MDM2 and CDK4

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20
Q

what will be seen on XR with osteosarcoma?

A

mixed lytic and blastic mass

*Codman triangle - elevation of the periosteum

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21
Q

where is osteosarcoma most likely to metastasize?

A

lungs
other bones
brain

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22
Q

what is the classic presentation for Ewing sarcoma?

A

white person <20 y/o with painful, tender, warm, swollen, enlarging mass with fever and increased ESR

*mimics infection!!

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23
Q

what is seen on histo with Ewing sarcoma?

A

undifferentiated small blue round cell tumor

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24
Q

what is seen on XR with Ewing sarcoma?

A

periosteal rxn - reactive bone with “onion skin”

seen in diaphysis of long bones and flat bones

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25
Q

what is the gene and mutation associated with Ewing sarcoma?

A

EWS-FL11 fusion gene

t(11;22)(q24:q12)

26
Q

what is fibrous dysplasia?

A

benign proliferation of fibrous tissue and bone that do not mature

monostotic is asymptomatic (70%)
polyostotic has crippling deformities

27
Q

what is the typical presentation of fibrous dysplasia?

A

early adolescent child with incidental pain, fracture or discrepancy in limb length

found in cranio facial bones, long bones, or ribs

28
Q

what will be seen on XR with fibrous dysplasia?

A

ground glass appearance with well defined margins

29
Q

what syndromes are associated with fibrous dysplasia?

A

McCune-Albright

Mazabraud syndrome

30
Q

what will be seen on histo with fibrous dysplasia?

A

curvilinear trabeculae

described as “Chinese characters”

31
Q

describe giant cell tumor of bone

A

benign osteoclastoma multinucleated osteoclast-type giant cells

arise in epiphyses in the knee

tends to recur after removal

32
Q

what is the typical presentation of giant cell tumor?

A

20-40 y/o with arthritic knee symptoms

33
Q

what should also be on the differential when considering giant cell tumor?

A

brown tumor of hyperparathyroidism

34
Q

what is the most common form of skeletal malignancy?

A

metastatic tumors

35
Q

what is a lipoma?

A

benign tumor of adipose tissue

most common soft tissue tumor of adults

36
Q

what is seen on histo in lipoma?

A

encapsulated mass of normal-appearing adipose tissue

37
Q

what is liposarcoma?

A

common malignant soft tissue tumor in adults

occurs in deep soft tissue of proximal extremities and retroperitoneum

recur unless completely excised

38
Q

what mutation is associated with liposarcoma?

A

12q13-q15 and t(12;16)

MDM2 encoding p53

39
Q

what is seen on histo in liposarcoma?

A

well-differentiated
myxoid
round cell
pleomorphic

40
Q

what is nodular fasciitis?

A

self-limited fibroblastic and myofibroblastic proliferation

UE of young adults

t(17;22) encoding MYH9-USP6 fusion gene

41
Q

what are the fibromatoses?

A

palmar - Dupuytren’s contracture
plantar
penile - Peyronie disease

42
Q

what is deep fibromatosis?

A

desmoid tumor

large, infiltrative painful tumor that frequently recurs

occurs in women ages teens-30s

APC or B-catenin genes

43
Q

who is predisposed to deep fibromatosis?

A

familial adenomatous polyposis (Gardner syndrome) with germline APC mutations

44
Q

what is seen on histo with nodular fasciitis?

A

stellate and spindle cells separated by collagen bundles

45
Q

what is fibrosarcoma

A

malignant, deep-seated, slow-growing tumor in thigh or trunk

in children: arises in extremities

46
Q

what is seen on histo in fibrosarcoma?

A

uniform, spindled cells arranged in herringbone fascicles

47
Q

what is rhabdomyosarcoma?

A

malignant skeletal muscle tumor

3 types:
alveolar
embryonal (60%)
pleomorphic

48
Q

what gene is associated with alveolar rhabdomyosarcoma?

A

fusion of FOX01 to either PAX3 or PAX7

49
Q

what forms of rhabdomyosarcoma arise in kids?

A

alveolar

embryonal

50
Q

what is seen on histo in embryonal rhabdomyosarcoma?

A

rhabdomyoblasts causing cross striations

sarcoma botryoides causing cambium layer

51
Q

where is rhabdomyosarcoma likely to localize in kids?

A

sinuses
head
neck
GU

52
Q

what is leiomyoma?

A

benign smooth muscle tumor

most commonly found in the uterus

53
Q

what syndromes are associated with leiomyomas?

A

hereditary leiomyomatosis

renal cell cancer syndrome

54
Q

what is leiomyosarcoma?

A

malignant smooth muscle tumor found in adult woman

can metastasize to the lungs

55
Q

what is synovial sarcoma?

A

malignant tumor adjacent to joint

presents in 20s-40s

can metastasize to lungs

56
Q

what is seen on histo in synovial sarcoma?

A

keratins
epithelial cells
spindle cells

57
Q

what is tuberous sclerosis complex?

A

AD mutation in TSC1 or TSC2

hamartomas
renal angiomyolipomas
pulmonary lymphangioleiomyomatosis
cardiac rhabdomyomas
angiofibromas
subungal fibromas
58
Q

what is von hippel-lindau disease?

A

AD mutation in VHL tumor suppressor gene

hemangioblastomas of cerebellum and retina
cysts of viscera
RCC
pheochromocytoma

59
Q

what is NF1?

A 
AD mutation
neurofibromas
optic nerve gliomas
lisch nodules
cafe au lait spots
60
Q

what is NF2?

A

AD mutation
bilateral schwannomas
meningiomas
ependymomas

61
Q

what are the peripheral nerve sheath tumors?

A

schwannoma
neurofibroma
malignant PNST

NMSP Exam 2 (14 decks)

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