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Orthopaedic Surgery > Ortho Boards - Oncology > Flashcards

Ortho Boards - Oncology Flashcards

1
Q

Work up order for bone tumors?

A

H&P
Local Staging
Systemic Staging
Biopsy (Last!)

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2
Q

Enneking’s 4 questions

A

1) Location
2) Tumor bone interaction
3) Bone-tumor interaction
4) Matrix

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3
Q

How to better evaluate tumor-bone and bone-tumor interactions from Enneking’s 4 questions?

A

Lodwick Classification

1) Geographic - sclerotic, distinct, indistinct (NOF, UBC, GCT)
2) Moth eaten - OM, mets
3) Destructive - Ewings

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4
Q

Actual location of bone tumors?

A 
Epiphysis
Metaphysis
Diaphysis
Flat bones
Spine
Sacrum
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5
Q

What needs a bone marrow biopsy as part of the work up?

A

Ewings sarcoma

Myeloma

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6
Q

Systemic staging work up?

A

Bone scan - cold in myeloma, renal cell carcinoma, thyroid carcinoma (high flow lesions)
Chest CT scan (85% found in tumors of unknown origin) - if metastatic suspicion get CT C/A/P
Labs - CBC, CMP, ESR, CRP, SPEP, UPEP

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7
Q

Myeloma dx vs. staging?

A

Diagnosis - SPEP, UPEP, bone marrow

Staging - Skeletal survey

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8
Q

When to use a PET scan?

A

18F-FDG

Used for melanoma, lymphoma, carcinoma

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9
Q

Benign bone tumors with metastasis?

A

Chondroblastoma

Giant cell tumor

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10
Q

Most common tumor stage?

A

IIB - MSTS

High grade (II), extracompartmental (B), no mets (M0)

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11
Q

Malignant bone lesions <5yo, <30, >30

A

<5years - LCH (Letter-Siwe), LCH (Hand-Schuller-Christian), metastatic rhabdomyosarcoma, metastatic neuroblastoma

< 30 years - Ewings Sarcoma, osteosarcoma

> 30 years - Chondsarcoma, mets, lymphoma, myeloma, chordoma, adamantinoma

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12
Q

Benign bone lesions: < 5 yo, < 30, > 30

A

< 5 years: osteomyelitis, osteofibrous dysplasia

< 30 years: Osteoid osteoma, osteoblastoma, chondroblastoma, ABC, LCH, osteofibrous dysplasia, non-ossifying fibroma

> 30 years: giant cell tumor, pagets disease

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13
Q

Bone lesions found at any age?

A

Infection, fibrous dysplasia, simple bone cyst

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14
Q

Lesions treated with chemo + surgery?

A

Ewings and osteosarcoma

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15
Q

Lesions treated with radiation + surgery?

A

soft tissue sarcoma

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16
Q

Lesions treated with limb salvage/wide excision?

A

Chondrosarcoma, adamantinoma, chordoma, parosteal osteosarcoma, soft tissue sarcoma

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17
Q

Lesions treated with intralesional resection?

A

giant cell tumor, ABC, NOF, LCH, osteoblastoma, chondroblastoma

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18
Q

Lesions treated with radiofrequency ablation?

A

osteoid osteoma

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19
Q

Chromosomal Translocations?

t(11;22)
t(12;16)
t(2;13)
t(12;22)
t(X;18)
t(9;22)
A

t(11;22) - Ewings sarcoma/PNET - EWS-FL1
t(12;16) - Myxoid liposarcoma - TLS-CHOP
t(2;13) - alveolar rhabdomyosarcoma - PAX3-FKHR
t(12;22) - clear cell carcinoma - EWS-ATF1
t(X;18) - synovial sarcoma - SSX1-SYT
t(9;22) - myxoid chondrosarcoma - EWS-CHN

None for osteosarcoma (Rb gene)

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20
Q

Syndrome: McCune Albright
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: McCune Albright
Disease: polyostotic fibrous dysplasia, cafe au lait spots, endocrinopathy (precocious puberty)
MSK Neoplasm: None
Genetic Association: GNAS1, Chr 20, AR

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21
Q

Syndrome: Mazabraud Syndrome
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Mazabraud Syndrome
Disease: Fibrous dysplasia and soft tissue myxomas
MSK Neoplasm: None
Genetic Association: GNAS1

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22
Q

Syndrome: Jaffe-Campanacci
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Jaffe-Campanacci
Disease: multiple non-ossifying fibromas with cafe au lait skin lesions
MSK Neoplasm: None
Genetic Association: None

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23
Q

Syndrome: POEMS Syndrome
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: POEMS
Disease: Polyneuropathy, organomegaly, endocrinopathy, M-protein, Skin abnormalities (hyperpigmentation)
MSK Neoplasm: Myeloma
Genetic Association: None

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24
Q

Syndrome: Li-Fraumeni Syndrome
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Li-Fraumeni Syndrome
Disease: Sarcoma, breast, leukemia, adrenal glands
MSK Neoplasm: Osteosarcoma
Genetic Association: p53

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25
Q

Syndrome: Retinoblastoma
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Retinoblastoma
Disease: bilateral eye tumors in children
MSK Neoplasm: Osteosarcoma
Genetic Association: Rb1

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26
Q

Syndrome: Rothmund-Thompson
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Rothmund-Thompson
Disease: Sun-sensitive rash with poikiloderma and telangiectasias
MSK Neoplasm: Osteosarcoma
Genetic Association: RECQL4

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27
Q

Syndrome: Multiple hereditary exostosis
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Multiple hereditary exostosis
Disease: multiple osteochondromas
MSK Neoplasm: Chondrosarcoma
Genetic Association: ETX1, EXT2, EXT3

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28
Q

Syndrome: Ollier’s Disease
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Ollier’s Disease
Disease: Enchondromas
MSK Neoplasm: Chondrosarcoma
Genetic Association: PTHR1

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29
Q

Syndrome: Maffucci’s syndrome
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Maffucci’s syndrome
Disease: Enchondromas, angiomas and CNS, pancreatic, ovarian malignancies
MSK Neoplasm: Chondrosarcoma and angiosarcomas
Genetic Association: PTHR1

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30
Q

Syndrome: Hand-Schuller-Christian disease
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Hand-Schuller-Christian disease
Disease: Multifocal langerhans cell histiocytosis (LCH), exopthalmos, diabetes insipidus, lytic skull lesions
MSK Neoplasm: LCH
Genetic Association: None

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31
Q

Syndrome: Letterer-Siwe disease
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Letterer-Siwe disease
Disease: Multifocal langerhans cell histipcytosis (LCH), visceral and bone disease. FATAL
MSK Neoplasm: LCH
Genetic Association: ?

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32
Q

Syndrome: Stuart-Treves
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Stuart-Treves
Disease: chronic lymphedema
MSK Neoplasm: angiosarcoma
Genetic Association: None

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33
Q

Syndrome: Neurofibromatosis type 1
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: Neurofibromatosis type 1
Disease: multiple neurofibromas
MSK Neoplasm: malignant peripheral nerve sheath tumor (MPNST)
Genetic Association: NF1

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34
Q

Immunohistochemistry

Stain: SMA
Disease:

A

Smooth muscle actin - benign/malignant muscle

Leiomyosarcoma

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35
Q

Immunohistochemistry

Stain: Desmin
Disease:

A

Skeletal muscle

Rhabdomyosarcoma

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36
Q

Immunohistochemistry

Stain: S-100
Disease:

A

Neural tumors

Schwanoma
MPNST

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37
Q

Immunohistochemistry

Stain: CD34/31
Disease:

A

Endothelial cells/vascular tumors

Hemangioma
hemangioendothelioam
Angiosarcoma

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38
Q

Immunohistochemistry

Stain: B-catenin
Disease:

A

membrane marker, wnt pathway

Fibromatosis or extraskeletal desmoid

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39
Q

Immunohistochemistry

Stain: CD99
Disease:

A

Ewings sarcoma

Peripheral neuroectodermal tumor

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40
Q

Immunohistochemistry

Stain: Keratin
Disease:

A

Epithelpid sarcoma, synovial sarcoma, carcinoma, adamantinoma

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41
Q

Immunohistochemistry

Stain: EMA
Disease:

A

Epitheloid membrane antigen

Epitheloid or synovial sarcoma

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42
Q

Immunohistochemistry

Stain: MyoD1
Disease:

A

Myogenin

Rhabdomyosarcoma

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43
Q

Immunohistochemistry

Stain: Vimentin
Disease:

A

Soft tissue sarcoma

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44
Q

Immunohistochemistry

Stain: CD 20, CD 45
Disease:

A

Lymphoma

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45
Q

Immunohistochemistry

Stain: CD138
Disease:

A

Myeloma

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46
Q

Benign and malignant tumors?

Histologic type: Hematopoietic

A

Benign: None

Malignant: myeloma, lymphoma

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47
Q

Benign and malignant tumors?

Histologic type: Chondrogenic

A

Benign: Osteochondroma, chondroma, chondroblastoma, chondromyxoid fibroma

Malignant: Primary/secondary chondrosarcoma, defifferentiated chondrosarcoma, mesenchymal chondrosarcoma, clear cell chondrosarcoma

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48
Q

Benign and malignant tumors?

Histologic type: Osteogenic

A

Benign: Osteoid osteoma, osteoblastoma

Malignant: Osteosarcoma, Parosteal osteosarcoma, periosteal osteosarcoma, telangectatic osteosarcoma

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49
Q

Benign and malignant tumors?

Histologic type: Unknown origin

A

Benign: Giant cell tumor

Malignant: Ewings, adamantinoma

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50
Q

Benign and malignant tumors?

Histologic type: Fibrogenic

A

Benign: Fibroma, desmoplastic fibroma

Malignant: Fibrosarcoma, malignant fibrous histiocytoma/

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51
Q

Benign and malignant tumors?

Histologic type: Notocordal

A

Benign: None

Malignant: Chordoma

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52
Q

Benign and malignant tumors?

Histologic type: Vascular

A

Benign: hemangioma

Malignant: Hemangioendothelioma, hemangiopericytoma

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53
Q

Benign and malignant tumors?

Histologic type: Lipogenic

A

Benign: Lipoma

Malignant: Liposarcoma

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54
Q

Benign and malignant tumors?

Histologic type: Neurogenic

A

Benign: Neurilemoma

Malignant: Malignant peripheral nerve sheath tumor

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55
Q

Epiphyseal Tumors?

A

Chondroblastoma

Giant cell tumor

Clear cell chondrosarcoma (femoral head)

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56
Q

Metaphyseal tumors?

A

Osteosarcoma

Chondrosarcoma

Mets

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57
Q

Diaphyseal lesions?

A

AEIOUY + Mets

Adamantinoma
Eosinophilic granuloma
Infection
osteoid osteoma/osteoblastoma
Ewings sarcoma
mYleoma, lYmphona, fibrous dYsplasia
Mets
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58
Q

Flat bone lesions?

A

Chondrosarcoma

Fibrous dysplasia

Hemangioma

Pagets disease

Ewings sarcoma

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59
Q

Spine lesions?

A

Anterior column: Giant cell tumor metastatic disease

Posterior column: osteoid osteoma, Osteoblastoma, aneurysmal bone cyst

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60
Q

Sacrum lesions

A

Midline - Chordoma

Eccentric - ANC, GCT, mets

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61
Q

Syndromes associated with osteosarcoma

A

Li Fraumeni

Retinoblastoma

Rothmund-Thompson

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62
Q

Syndromes associated with chondrosarcoma?

A

MHE

Olliers

Maffucci’s

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63
Q

Syndrome: familial adenomatous polyposis
Disease: ?
MSK Neoplasm: ?
Genetic Association: ?

A

Syndrome: familial adenomatous polyposis
Disease: multiple intestinal polyps, colon cancer, hepatoblastomas
MSK Neoplasm: desmoid tumors
Genetic Association: APC

64
Q

Chemotherapy used for what soft tissue sarcoma?

A

Rhabdomyosarcoma

Controversial for STS

65
Q

When is radiation used?

A

Local control of Ewings sarcoma, lymphoma, myeloma, metastatic bone disease

66
Q

Chemotherapy agents for osteogenic sarcoma and Ewings sarcoma with toxicities?

A

Osteogenic Sarcoma - (DCM)

  • Doxorubicin (Adriamycin) - cardiotox
  • Cisplatin - audiotoxic, nephrotoxic, peripheral nerves
  • Methotrexate - oral cavity

Ewings Sarcoma - (DIVCE)

  • Doxorubicin (Adriamycin) - cardiotox
  • Ifosfamide - nephrotox, central neurotox
  • Vincristine - peripheral neurotox
  • Cyclophosphamide (Cytoxan) - leukemia/lymphoma, cystitis, bladder cancer
  • Etoposide - secondary leukemia
67
Q

Pre-operative (neoadjuvant) vs. post-operative radiation (adjuvant)

A

Pre-op - 50Gy (5000 cGy) - smaller field, wound complications

Post-op - 66 Gy (6600 cGy) - long term fibrosis, bone necrosis and fracture (esp subtroch)

68
Q

Most common soft tissue tumor in children?

A

Hemangioma

69
Q

Most common soft tissue tumor in adults?

A

Lipoma

70
Q

Most common malignant soft tissue tumor children

A

Rhabdomyosarcoma

71
Q

Most common malignant soft tissue tumor in adults?

A

Undifferentiated pleomorphic sarcoma (UPS)

72
Q

Most common primary benign bone tumor?

A

Osteochondroma

73
Q

Most common primary malignant bone tumor?

A

Osteosarcoma

74
Q

Most common secondary benign bone lesion?

A

ABC

75
Q

Most common secondary malignancies?

A

Malignant fibrous histiocytoma, osteosarcoma, fibrosarcoma

76
Q

Most common phalangeal tumor?

A

Enchondroma

77
Q

Most common soft tissue sarcoma of wrist/hand?

A

Epitheloid sarcoma

78
Q

Most common soft tissue sarcoma of the foot and ankle?

A

Synovial sarcoma

79
Q

Soft tissue sarcomas with lymph node mets?

A 
ESARC
Epithelioid sarcoma
Synovial Sarcoma
Angiosarcoma
Rhabdomysarcoma
Clear Cell Sarcoma
80
Q

Tumors of fibrous tissue?

A

Benign

  • Calcifying aponeurotic fibroma
  • Fibromatosis - Dupuytrens
  • Extraabdominal desmoid tumor - most locally invasive of all soft tissue masses, assoc with APC
  • Nodular fasciitis - painful rapidly enlarging mass

Malignant

  • UNdifferentiated pleomorphic sarcoma (previously MFH)
  • Fibrosarcoma

Dermatofibrosarcoma protuberans - nodular cutaneous tumor

81
Q

Tumors of fatty tissue?

A

Benign
- Lipoma - subtypes (spindle cell lipoma, pleomorphic lipoma, angiolipoma)

Malignant
- Liposarcoma - subtypes (well differentiated, myxoid, dedifferentiated, round-cell, pleomorphic)

82
Q

Tumors of neural tissue?

A

Neurilemoma (benign schwanoma)
Neurofibroma
Neurofibromatosis
Neurofibrosarcoma (MPNST)

83
Q

Tumors of muscle?

A

Leiomyosarcoma

Rhabdomyosarcoma

84
Q

Vascular tumors?

A

Hemangioma

Angiosarcoma

85
Q

Synovial timors?

A

Gangila
pigmented villonodular synovitis (PVNS) - nodular and diffuse - recurrent hemarthrosis
Giant cell tumor of tendon sheath
Synovial chondromatosis - multiple rice bodies with metaplastic change.

86
Q

Other random rare sarcomas?

A

Synovial sarcoma
Epitheloid sarcoma
Clear cell sarcoma - slow growing mass in association with tendon or aponeuroses
Alveolar clear cell sarcoma - anterior thigh

87
Q

Post traumatic masses?

A

Hematoma - sarcomas can spontaneously hemorrhage and necrosis may mimic hematoma

Myositis ossificans (HO) - as progression occurs look for peripheral mineralization with central clear area.

88
Q

Tumors with small round blue cells?

A 
Ewings sarcoma/PNET
Neuroblastoma
Rhabdomyosarcoma
Wilms tumor (nephroblastoma)
Synovial sarcoma
Myeloma
Lymphoma
89
Q

What is important in soft tissue sarcoma prognosis?

A

Size (>5cm or > 1.5cm in hand/wrist
Depth (deep to fascia)
Growth
Presentation

90
Q

Most common tested soft tissue sarcomas?

A

undifferentiated pleomorphic sarcoma (previously known as malignant fibrous histiocytoma), liposarcoma, synovial sarcoma, leiomyosarcoma

91
Q

Imaging of soft tissue sarcoma?

A

Dark on T1
Bright on T2

Except lipoma - always look exactly like fat

92
Q

Lipoma vs. liposarcoma?

A

MDM2+ or ring chromosomes

93
Q

Fibrosarcoma pathology?

A

Herringbone pattern

94
Q

Malignant peripheral nerve sheath tumor pathology?

A

Spindle cells in whirling patterns

S-100 positive

95
Q

Rhabdomyosarcoma pathology

A

Small round blue cells
Desmin positive
Chr t(2;13) - PAK-FKHR gene

96
Q

Benign and malignant lesions that mineralize in soft tissues?

A

Benign - Lipoma, hemangioma, myositis ossificans, CPPD, calcific tendonitis

Malignant - synovial sarcoma, liposarcoma, angiosarcoma

97
Q

Lesions that can occur on both sides of a joint?

A

HIP(P) CATS

Hemophilia (blood)
Infection (PUS)
Pannus (inflammatory arthropathy)
PVNS
CPPD
Arthritis
TB
Synovial chondromatosis
98
Q

Telangiectatic osteosarcoma vs ABC?

A

ABC is wider than the physis

TO - expands on cortex more than ABC. Path has malignant osteoid, vascular lakes/channels (similar to ABC), and giant cells.

99
Q

Types of osteosarcoma

A

Central - conventional, telangiectatic, low grade, small cell, giant cell rich, secondary, non-osteogenic

Surface - parosteal (stuck on) and periosteal

Extraosseus

100
Q

Pathology of osteosarcoma?

A

Parosteal - more bland, less aggressive

Periosteal - more aggressive, can have areas of cartilage

Conventional - immature looking blue cells, mitotic figures, immature woven bone

101
Q

Histology of enchondroma?

A

Bland looking cartilage - acellular

On hands and feet can be much more cellular

102
Q

Tx of benign cartilage lesions

A

Osteochondroma/Enchondroma - latent in adults - observation

Chondroblastoma and chondromyxoid fibroma - rare aggressive lesions, require treatment, curettage.

103
Q

Imaging of chondrosarcoma vs. benign lesions?

A

Will see endosteal scalloping, cortical expansion, cortical disruption.

If truly benign should be able to cover up lesion and rest of bone looks normal.

104
Q

NOF vs. chondromyxoid fibroma?

A

NOF usually has a definable rim of bone, CMF does not.

105
Q

NOF pathology

A

Spindle shaped fibroblasts, whorled pattern, giant cells and hemosiderin.

106
Q

Osteofibrous dysplasia vs. fibrous dysplasia on pathology?

A

OFD - loose stroma of fibroblasts/spindle cells with scattered bone trabecular WITH rimming osteoblasts

Fibrous dysplasia - similar but no rimming osteoblasts on histo - looks completely different on XR. “Chinese characters”

107
Q

Fibrous dysplasia vs OFD on XR?

A

FD - ground glass (looks like frosted glass) like someone smudged the bone on XR. More smooth, cortical expansion, no normal fat, it has been replaced with fibrous tissue.

OFD - Anterior cortical lesion usually on the tibia, multiloculated, soap bubble appearance with bowing of the tibia. DDx = adamantinoma.

108
Q

Chinese characters on histology?

A

Fibrous dysplasia - no rimming osteoblasts.

109
Q

Types of cafe au lait spots?

A

Maine - irreg - McCune Albright

California - smooth - neurofibroma

110
Q

Small round blue cell tumors?

A

LERN-M

Lymphoma
Ewings - look for CD99, t(9;22), and pseudorosettes (tumor trying to make blood vessels)
Rhabdomyosarcoma 
Neuroblastoma
Myeloma
111
Q

Markers for Myeloma?

A

CD56+

CD45 neg (Lymphoma)

112
Q

Histology of Chordoma?

A

Sheets/cords of cells in myxoid stroma, vacuolated cells, Physaliferous cells (vacuolated, signet ring looking cells)

113
Q

Other names for langerhans cell histiocytosis?

A

Eosinophilic granuloma (EG)

Histiocytosis X

114
Q

Punched out lesions in a skeletally immature patient?

A

Langerhans cell histiocytosis (LCH) - neoplastic proliferation of langerhans cells (look like a bi-lobed histiocyte - babys butt imprint…)

115
Q

Causes of vertebra plana?

A

MELT

Myeloma
Ewings
Lymphoma/LCH (EG)
Tuberculosis

116
Q

Histology of langerhans cell histiocytosis?

A

Eosinophils, plasma cells, langerhans cells (histiocyte with grooved nucleus - babys butt imprint)

EM - bierbeck granules (tennis racket)

117
Q

Giant cells in GCT?

A

Cells in the stroma look like the same as those in the giant cells. Think of chocolate chip cookies with chocolate chips spilled everywhere.

Stromal cells responsible for lysis

118
Q

Tumors with giant cells?

A 
ABC
Chondroblastoma
Fracture
GCT
Giant cell rich osteosarcoma
119
Q

Medical treatment for GCT?

A

Denosumab (Prolia) - human monoclonal antibody that binds RANKL

  • Stromal cells produce RANKL and giant cells express RANK to activate osteoclasts
120
Q

Adamantinoma vs. OFD on histo and XR?

A

Adamantinoma is larger along tibia, less bowing because it grows faster and the bone has not yet had time to deform.

Histology - OFD chinese characters with rimming osteoblasts and fibrous stroma, keratin negative.

Adamantinoma epithelial nests/glands and keratin positive.

121
Q

Stain to differentiate adamantinoma vs. OFD?

A

Keratin - positive in adamantinoma and negative in OFD.

122
Q

Systems to predict pathological fractures?

A

Harrington - 50% destruction, lytic lesion in proximal femur > 2.5cm, lesser troch avulsion, pain after radiation

Mirels - score of 4-12, site, pain, lesion, size (each with score of 1-3)
Site - upper, lower, peritroch
Pain - mild, moderate, functional
Lesion - blastic, mixed, lytic
Size - <1/3, 1/3-2/3, >2/3

> /= 9 - operate
8 = borderline
= 7 - non-op

123
Q

ABCs can be seen with what other lesion?

A

GCT, CB, CMF, FD, OB, Telangtatic OS

124
Q

ABC vs. UBC?

A

ABC - can be associated with other lesions, no fallen leaf sign, eccentric, width > physis, multiple fluid-filled lesions, open curettage

UBC - no other lesions, fallen leaf sign, central, width < physis, aspiration/injection. Simple bone cyst.

125
Q

5 lesions that can look like anything?

A 
FD - the great immitator
Infection
Mets
Cartilage
Langerhans cell histiocytosis (EG or HX)
126
Q

CMF vs chondroblastoma

A

CMF - rarely crosses the physis, chondroid with myxoid background

CB - crosses physis, chicken wire pathology

127
Q

Fibrous lesions of bone?

A

NOF
desmoplastic fibroma
Fibrosarcoma
Malignant fibrous histiocytoma

128
Q

Another name for malignant fibrous histiocytoma?

A

Non-osteogenic osteosarcoma

129
Q

Small round blue cell tumors with molecular markers?

A

LERNM
Lymphoma - CD45+, CD20+, leucocyte common antigen +, look for large soft tissue mass on MRI

Ewings Sarcoma - CD99+, t(11;22), FLI-1+

Rhabdomyosarcoma - Desmin+, XR normal, mass on MRI

Neuroblastoma - Neuron-specific enolase (NSE+), XR normal, mass on MRI

Myeloma - Kappa/lambda light chain, monoclonal spike on SPEP/UPEP, CD138+

130
Q

Genetic abnormality in fibrous dysplasia?

A

Activating mutation of the GS-alpha surface protein - GNAS1

- Increased production of cyclic adenosine monophosphate.

131
Q

Treatment of fibrous dysplasia?

A

Internal fixation - usually plate

Do not use autogenous bone graft - will quickly be transformed into woven bone of fibrous dysplasia

132
Q

Pagets disease is the result of what?

A

Abnormal bone remodeling

133
Q

Vicious cycle of metastatic bone disease

A

Bone destruction by activated osteoclasts

parathyroid hormone-related peptide (PTHrP) - stimulates release of RANKL from osteoblasts and marrow stromal cells and binds to RANK on osteoclast precursor cells

Granulocyte colony stimulating factor (G-CSF) from cancer cells causes further osteoclast differentiation

With bone resorption - transforming growth factor beta (TGF-B), insulin like growth factor (IGF-1), and calcium are released and cause more PTHrP to be released from cancer cells.

134
Q

Wide excision for what tumors?

A

Chondrosarcoma, adamantinoma, parosteal osteosarcoma, chordoma

135
Q

Most common malignant sarcoma of soft tissue in adults?

A

Undifferentiated pleomorphic sarcoma (previously known as malignant fibrous histiocytoma - not to be confused with MFH of bone).

136
Q

MRI imaging for PVNS?

A

Low on T1 and T2

137
Q

Histology of synovial sarcoma?

A

Biphasic - epithelial cells (carcinoma) and spindle cells (fibrosarcoma)

138
Q

When is chemotherapy used in chondrosarcoma?

A

Only used as an adjunct with dedifferentiated and mesenchymal types.

139
Q

Dedifferentiated chondrosarcoma on histology?

A

Look for high grade spindle cell sarcoma associated with low-grade cartilage component

140
Q

Most common primary malignant lesions of the spine

A

1) Chondrosarcoma
2) Chordoma
3) Ewings Sarcoma
4) Multiple Myeloma
5) Lymphoma

141
Q

Most common primary spine tumors in the posterior elements

A

ABC
OO
OB

142
Q

Primary Benign Spine Tumors

A

8 Total

1) Aneurysmal Bone Cyst
2) Eosinophilic granuloma
3) Giant cell tumor
4) Osteoid osteoma
5) Hemangioma
6) Neurofibroma
7) Osteoblastoma
8) Osteochondroma

143
Q

Radiosensitivity of common spine mets

A

Radiosensitive: Myeloma, Lymphoma, neuroblastoma, seminoma

Moderate RS: Breast, Prostate

Resistant: Lung, GI, renal cell, sarcoma, melanoma

144
Q

Bone cancer staging system??

A

Enneking System or MSTS
Grade I - Low Grade
Grade II - High Grade
Grade III - Any grade with distant mets

A: Intracompartmental
B: Extracompartmental

Most common = IIB

145
Q

IHC for leiomyosarcoma

A

Smooth muscle actin

146
Q

Markers for rhabdomyosarcoma

A

Desmin and MyoD1/myogenin

147
Q

Markers for schwanoma or malignant peripheral nerve sheath tumor

A

S-100

148
Q

Markers for vascular tumors?

A

CD34 and CD31

149
Q

Markers for Ewing’s sarcoma or Peripheral neuroectodermal tumor (PNET)

A

CD99

150
Q

Markers for soft tissue sarcoma

A

Vimentin

151
Q

Keratin and epithelial membrane antigen (ema) staining

A

Epitheloid sarcoma, synovial sarcoma, or adamantinoma (keratin only)

152
Q

Markers for lymphoma

A

CD20 and CD45

153
Q

Markers for myeloma?

A

CD138

154
Q

Bone malignancies with a high grade spindle component?

A

De-differentiated chondrosarcoma

Osteosarcoma

Fibrosarcoma

MFH

155
Q

Rare subtype of heterotopic ossification and genetics?

A

fibrodysplasia ossificans progressiva (FOP) - ACVR1 gene (Activin A type I receptor gene a BMP-1 receptor)

156
Q

In AJCC grading for bone and sarcomas what is the difference between Stage III and Stage IV?

A

Stage III - High grade with regional lymph note mets

Stage IV - any mets

Orthopaedic Surgery (2 decks)

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