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general Flashcards

1
Q

5 epiphyseal lesions

A

chondroblastoma, giant cell tumor, clear cell chondrosarcoma, PVNS, infection

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2
Q

diaphyseal lesions

A

AEIOU +/- Y adamantinoma, EG, infection, osteoid osteoma, U-ing’s (Ewing’s), lYmphoma

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3
Q

“chicken wire calcification” and “cobblestone appearance”

A

chrondroblastoma

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4
Q

treatment for chondroblastoma

A

intra-lesional curretage and bone grafting

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5
Q

synovial cell sarcoma mutation and translocation

A

X;18 translocation defect, SYT-SSX1 or SYT-SSX2 fusion product

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6
Q

“herringbone” pattern of spindle cells

A

fibrosarcoma

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7
Q

chromosomal translocation in myxoid liposarcoma

A

t(12;16), genes CHOP/TLS

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8
Q

CD99 positive

A

ewing’s/PNET (11, 22… 99)

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9
Q

CD1a positive

A

eosinophilic granuloma

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10
Q

translocation and genes in ewing’s sarcoma

A

t(11;22), genes EWS FLI1

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11
Q

translocation in myxoid chondrosarcoma

A

t(9;22), EWS-CHN

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12
Q

translocation and genes in clear cell sarcoma

A

t(12;22)(q13;a12), genes EWS, AFT1

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13
Q

treatment sequence for high grade bone sarcoma

A

chemo, surgery, chemo

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14
Q

soft tissue sarcoma treatment sequence

A

radiation and surgery, +/- chemo

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15
Q

rhabdomyosarcoma translocation and gene

A

t(2;13), gene Pax3-FKHR

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16
Q

describe the MSTS classification system for benign lesions

A

Arabic numbers.
1 = latent
2 = active
3 = aggressive

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17
Q

describe the MSTS classification system for malignant lesions

A

roman numerals. I - low grade, II - high grade, III - metastatic. A - intracompartmental, B - extracompartmental

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18
Q

What are the Mirel’s Criteria?

A 
score > 8 --> prophylactic fixation
site (1 upper limb, 2 lower limb, 3 peritroch)
pain (1 mild, 2 moderate, 3 functional)
lesion (1 blastic, 2 mixed, 3 lytic)
size (1  <1/3, 1  1/3-2/3, 3  >2/3)
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19
Q

How does osteoid osteoma present?

A

small, discrete, painful, benign bone lesion. constant progressive pain worse at night, relieved by NSAIDs

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20
Q

what does osteoid osteoma look like on histo?

A

distinct demarcation between nidus and reactive bone

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21
Q

what lab may be abnormal in intramedullary osteosarcoma?

A

elevated alk phos

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22
Q

where does osteosarcoma usually metastasize to?

23
Q

what does intramedullary osteosarcoma look like on histo?

A

tumor cells produce “lacey” osteoid, stroma cells have malignant characteristics

24
Q

what is the treatment for intramedullary osteosarcoma?

A

high grade - chemo, limb salvage, chemo

low grade - wide surgical resection

25
what does MDR gene do in osteosarcomas?
lets cells pump chemo out
26
where are enchondromas most commonly found?
hands (60%) and feet
27
what are the differences between ollier's and maffucci's?
both are sporadic, not genetic, multiple enchondromas. maffucci's includes soft tissue angiomas. risk of malignant transformation <30% in ollier's, up to 100% in maffucci's
28
what are the genetics of osteochondroma/MHE?
autosomal dominant mutation in EXT tumor suppressor gene
29
what is the characteristic histo of chondroblastoma?
chondroblasts arranged in "cobblestone" or "chicken-wire" pattern, scattered multinucleated giant cells
30
treatment of chondromyxoid fibroma
intra-lesional curettage and bone grafting
31
treatment of chondrosarcoma
wide surgical excision, doesn't respond to chemo/radiation
32
what are the characteristic findings of multiple myeloma?
CRAB - hyperCalcemia, renal insufficiency, anemia, bone lesions (multiple, punched-out)
33
CD 38+
multiple myeloma
34
histo of multiple myeloma
round plasma cells with eccentric nuclei and clockface chromatin, clear area (Hoffa zone) next to nucleus due to prominent Golgi apparatus
35
what are the immunohistochemical markers for lymphoma?
CD20+, CD45+, lymphocyte common antigen+
36
labs in malignant fibrous histiocytoma
elevated WBC, eosinophilia, abnormal LFTs, hypoglycemia
37
fallen leaf sign
unicameral bone cyst, pathologic fracture with fallen cortical fragment in base of empty cyst
38
mutation in primary ABC
upregulation of USP6, translocation of t(16;17)(q22;p13)
39
genetics of fibrous dysplasia
GNAS mutation, GSalpha protein activating --> increased cAMP production
40
what is a verocay body and what is it pathognomonic for?
neurilemmoma/Schwannoma, 2 rows of aligned nuclei in palisading formation
41
what sarcomas spread to lymph nodes?
Rhabdomyosarcoma, angiosarcoma, clear cell sarcoma, epithelioid sarcoma, synovial sarcoma (RACES)
42
translocation in alveolar rhabdomyosarcoma
t(2;13) -> Pax3-fKHR fusion protein
43
what genetics are associated with dermatofibrosarcoma protruberans?
t(17;22)
44
what is a medical treatment for dermatofibrosarcoma protruberans?
imatinib - inhibits PGDF-receptor tyrosine kinase, no response if patient lacks t(17;22)
45
genetics of well-differentiated liposarcoma
amplification of MDM2
46
myxoid liposarcoma translocation and genes
t(12;16), CHOP, TLS
47
what cell type is pathognomonic for liposarcoma?
signet-ring cell
48
what stage does osteosarcoma usually present at?
stage IIB
49
Paget's treatment
bisphosphonates
50
top two most common bony lesions in kids
1. osteosarcoma, 2. ewing's
51
most common soft tissue sarcoma in kids < 15
rhabdomyosarcoma
52
rhabdomyosarcoma treatment
chemo and surgery
53

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