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MSK 2 - AH > Bones, Joints, ST - Dobson > Flashcards

Bones, Joints, ST - Dobson Flashcards

1
Q

What has morphology of monophonic to uniform spindle cells w/scant cytoplasm, is biphasic and has + gland like structures and immunochemistry is (+) for keratin?

A

Synovial sarcoma

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3
Q

What tumor occurs in the deep soft tissue of extremities most commonly in the thigh of middle aged/older adults and is aggressive w/poor prognosis?

A

Undifferentiated pleomorphic sarcoma (UPS)

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5
Q

What are the main causes of osteonecrosis (AVN)?

A 
Alcohol abuse
Steroids
Fracture
Pregnancy 
Radiation
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6
Q

What is the most common benign bone tumor?

Occurs where?

What age group, in whom?

A

Osteochondroma (exostosis), 85% solitary

Metaphysis of long bones

M > F, late adolescence

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8
Q

Monostotic fibrous dysplasia is what?

Causes what?

CFs?

A

Single bone due to GNAS1 GOF

B9 tumor, well-circumscribed lesion

Causes pain, fracture, discrepancies in limb length

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9
Q

What is a multiloculated blood-filled cystic space that occurs in 1st 2 decades of life mostly in females and presents w/pain and swelling most often in the metaphysis of long bones?

Mutation?

A

Aneurysmal bone cyst (ABC)

USP6 –> Increased NF-KB

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11
Q

What is the most common sarcoma in adults 50-60 and occurs in deep soft tissues, prox extremities and retroperitoneum?

Translocation?

Fusion inhibits what?

A

Liposarcoma

t(12;16) well-diff and myxoid

MDM2 inhibits p53

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12
Q

What tumor has morphology of sheets of primitive small round cells?

Mutation?

A

Ewing Sarcoma

T(11; 22)
FLI1 - EWS

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13
Q

Osteopetrosis mutation in what?

Inheritance?

A

CAII, CLCN7

AR

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15
Q

What tumor occurs in 20s-40s, present w/deep seated mass and has t(X;18)?

A

Synovial sarcoma

SS18-SSX1,2, 4 fusion genes

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16
Q

What has morphology of gray-white, firm, bland fibroblasts arranged in broad sweeping fascicles amid dense collagen?

Resembles what?

A

Fibromatosis

Scar

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17
Q

What tumor (b9) has a ground glass appearance?

Cured how?

A

Monostotic fibrous dysplasia

Curettage

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20
Q

Acute presentation of osteomyelitis?

Other presentation?

A

Localized pain, fever, chills

FUO or localized pain

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25
Q

What is a pseudocyst w/synovial fluid w/no lining and no connection?

A

Ganglion cyst

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28
Q

Leiomyosarcoma occurs where?

A

Deep soft tissue extremities
Retroperitoneum
IVC

W > M

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29
Q

Osteoblastoma size?

Occurs where?

Treatment?

A

> 2 cm

Posterior spine

Curette or excise

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30
Q

How to diagnose ABC?

1/3 have what?

A

CT or MRI

Blue bone

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31
Q

Osteoporosis causes what skeletal deformity?

This causes what?

A

Kyphoscoliosis

Restrictive lung disease

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32
Q

Which type of OI does death occur in utero or shortly after birth?

A

Type 2

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33
Q

What has the morphology of cement lines and mosiac pattern of lamellar bone?

A

Paget’s

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36
Q

Ewing sarcoma occurs in whom?

Presents how?

Clinical course?

A

< 20, boys > females

Painful enlarging mass that is tender, warm, swollen, often mimics infection

Aggressive

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37
Q

Multiple hereditary exostosis disease what inheritance?

Mutation?

Can progress to what?

A

AD

EXT1/EXT2

Chondrosarcoma

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38
Q

What is a polyostotic disease assoc w/cafe au lait spots, endocrine abnormalities like precocious puberty?

Due to what?

A

McCune-Albright syndrome

GNAS1 mutation in embryogenesis

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39
Q

What has morphology of lytic tumor that destroys overlying cortex producing bulging soft tissue?

A

Giant cell tumor

Osteoclastoma

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41
Q

Newly deposited bone can form a shell of living tissue known as what?

A

Involucrum

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42
Q

Cleidocranial dysplasia mutation?

Inheritance?

CF?

A

RUNX2

AD

Short height, no clavicle

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43
Q

What arises from arrector pili muscle and is painful and found in skin, nipples, scrotum, labia?

The hereditary and RCC have what mutation?

A

Leiomyoma

LOF ch. 1, fumarate hydratase

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45
Q

What type of fracture are the ends of the bone at the fracture site unaligned?

A

Displaced

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46
Q

What is the collagen defect in type 1 OI?

A

Decreased synthesis of pro-a1(1)

Abnromal pro-a1(1) or proa2(1)

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47
Q

What has morphology of hemosiderin or foamy lipid due to macrophages w/patchy fibrosis?

A

Tenosynovial giant cell tumor

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48
Q

What is Mazabraud syndrome?

A

Fibrous dysplasia (polyostotic) and soft tissue myxomas (IM)

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50
Q

What is the most common soft tissue tumor of adulthood?

Described how?

A

Lipoma

Soft, mobile, painless

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51
Q

Osteosarcoma occurs in what age group?

Occurs where?

What mutation?

A

< 20 and older adults w/Paget’s

Long bone metaphysis (femur/prox tibia)

RB

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52
Q

10% of joint replacements are for what?

What is the best test to see?

A

AVN

MRI

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53
Q

What tumor has morphology of soft gray, primitive round and spindle cells in a myxoid stroma w/visible cross-striations?

A

Embryonal rhabdomyosarcoma

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54
Q

What causes a more destructive osteomyelitis than pyogenic form?

Spreads how?

75% develop st infection where?

A

Mycobacterium

Hematogenous through ant. Vertebral bodies

Psoas

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56
Q

What has a t(x;18)?

A

Synovial sarcoma

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57
Q

What type of fracture is a slowly developing rapture that follows a period of increased physical activity in which bone is subjected to repetitive loads?

A

Stress

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60
Q

What has morphology of extending from medulla through cortex into soft tissue, chondrocytes w/increased cellularity and atypia?

A

Chondrosarcoma

61
Q

What has morphology of well encapsulated mass of mature adipocytes, subcutis of prox extremities and trunk?

A

Lipoma

62
Q

Long bones form how?

Flat bones?

A

Endochondral ossification

Intramembranous

63
Q

What tumor has morphology of mature adipocytes and scattered spindle cells w/hyperchromatic nuclei?

A

Well-differentiated liposarcoma

64
Q

Which karyotype makes up the majority?

A

Complex

70
Q

What are the most common sites of metastatic tumors that go to bones?

A

Prostate, breast, kidney, lung

Peanut Butter, K(L)S

74
Q

What tumor has morphology of numerous spaces lined by discohesive uniform round tumor cells w/little cytoplasm and a t(2; 13)?

A

Alveolar rhabdomyosarcoma

75
Q

Osteoid osteoma size?

Occurs where?

Mostly what?

Main symptom?

A

< 2 cm

Appendicular skeleton (50% femur/tibia)

Cortex w/reactive bone

Severe nocturnal pain

76
Q

What can cause Dupuytren contracture, plantar and penile (Peyronie disease)

Clinical course?

Demographic?

A

Superficial fibromatosis

Innocuous

M > F

77
Q

What has a t(2;13) or t(1;13)?

Gene fusion?

A

Rhabdomyosarcoma - alveolar type

PAX3-FOXO1
PAX7-FOXO1

78
Q

What large, infiltrative masses occurs in teens-30’s in women that frequently recur but rarely metastasize and may be occasionally painful?

Associations with what?

A

Deep fibromatosis (Desmoid tumor)

APC, B-catenin mutation
Gardner syndrome

80
Q

CF of osteopetrosis

A

Fracture
Anemia, leukopenia
CN 2, 7, 8 nerve compression

83
Q

Alveolar soft-part sarcoma has what t?

A

t(X;17)

TFE3-ASPL

84
Q

Diffuse tenosynovial giant cell tumor occurs where?

Localized?

A

Large joints (knee 80%)

Small joints (hand)

85
Q

What tumor has morphology of large multinucleated, bizarre eosinophilic tumor cells w/+ immunochemistry for myogenin?

Prognosis?

A

Pleomorphic rhabdomyosarcoma

Fatal - poor, occurs in adults

87
Q

Osteopetrosis AD gene mutation?

A

LRP5

89
Q

Sarcoma botryoides is a variant of what?

May develop where?

A

Embryonal rhabdomyosarcoma

Nasopharynx, bile duct, bladder, vagina

91
Q

What morphology is pathognomonic for osteosarcoma?

Presents how?

10-20% of pts have what at time of dx?

A

Malignant osteoid (formation of bone by tumor cells)

Painful progressively enlarging mass

Metastases to lungs (poor prognosis)

93
Q

IV drug users or gu tract infections w/osteomyelitis more likely to have what bacterial infection?

A

E coli
Pseudomonas
Klebsiella

94
Q

What has morphology of a painless, firm mass, eosinophilic spindle cells w/blunted ends and is (+) immunostain w/Abs to smooth m. Actin and desmin?

A

Leiomyosarcoma

95
Q

What type of fracture extends only partially through the bone, common in infants?

A

Green stick

96
Q

What tumor (b9) has morphology composed of curvilinear trabeculae of woven bone that lack conspicuous osteoblastic rimming and arise in a background of fibrous tissue?

A

Fibrous dysplasia

97
Q

Chondrosarcoma types w/aggressive behavior?

Most common type (90%)?

A

Dedifferentiated and mesenchymal

Conventional central

98
Q

What is the most common soft tissue sarcoma of childhood and teenagers, < 20 y/o?

Types?

A

Rhabdomyosarcoma

Alveolar (20%)
Embryonal (60%)
Pleomorphic (20%)

99
Q

What presents w/severe nocturnal pain and is relieved by ASA/NSAIDS?

A

Osteoid osteoma

100
Q

What has the morphology of no medullary canal, compressed neural foramen a, deposited bone is not remodeled and is woven?

What other key feature?

A

Osteopetrosis

Erlenmeyer flask deformity

102
Q

What has morphology of railroad tracks - dissecting osteitis w/osteoclasts boring into center of trabeculum?

A

Hyperparathyroidism

103
Q

Which type of osteoporosis has morphology of thinned cortex, widened Haversian?

A

Senile

105
Q

What is thanotophoric dwarfism?

Mutation?

A

Lethal version of achondroplasia

FGFR3

106
Q

What is the low-turnover variant of Osteoporosis?

Due to what?

A

Senile

low calcium intake

108
Q

Bone metastasis has what changes to the following?

Ca

PO4

ALP?

A

inc

Inc/normal

Inc

109
Q

What tumor has morphology of abundant ground substance and a rich capillary network in which are scattered immature adipocytes and more primitive round to stellate cells?

A

Myxoid liposarcoma

111
Q

What translocation in tenosynovial giant cell tumor?

Overexpress what?

A

T(1;2) fusion of type 6 collagen to a-3

M-CSF

112
Q

Chondroma occurs where?

Age group?

Risk of what?

A

Tubular bones hands and feet

30-50

Sarcoma

113
Q

What is a lytic tumor that is locally aggressive, b9, and occurs in 20-40s around the knee?

Most are what?

Tx?

A

Giant cell tumor (osteoclastoma)

Solitary

RANKL-I —> denosumab

114
Q

Infantile fibrosarcoma has what t?

A

t(12;15)

ETV6-NTRK3

116
Q

Presence of Homer-Wright Rosettes in Ewing sarcoma indicates what?

Known as what?

A

Increased degree of of neuroectodermal differentiation

Small blue cell tumor

117
Q

What has a t(12;16)?

Gene fusion?

A

Liposarcoma myxoid and round cell type

FUS-DDIT3

120
Q

What type of fracture is the bone fragmented?

A

Comminuted

125
Q

Chondrosarcoma age group and sex?

Presents how?

15% arise from what?

Mutation?

A

40-60

Painful enlarging mass (axial skeleton)

Preexisting b9 cartilaginous tumor

IDH1 and IDH2

126
Q

Osteoid osteoma and osteoblastoma occur in what age group?

A

10-20

128
Q

What has the morphology of circumscribed hyaline cartilage nodule in medulla?

A

Chondroma

130
Q

What is the high turnover variant of osteoporosis?

What hormones or inflammatory agents are increased/dec?

A

Postmenopausal

INC: IL-6, IL-1, TNF, RANK, RANKL, osteoclast

DEC: estrogen, OPG

131
Q

What is an important prognostic finding in Ewing sarcoma?

A

The amount of chemo-induced necrosis

134
Q

OI aka brittle bone disease mutation?

Inheritance?

A

COL1A1 or COL1A2

AD

135
Q

Brachydactyly types D and E mutation?

CF?

A

HOXD13

Short fingers and toes

138
Q

Achondroplasia mutation?

inheritance?

CF?

A

FGFR3

AD

Dwarfism

139
Q

How does osteomyelitis spread in children?

Localization of infection where?

A

Hematogenous

Metaphysis in long bone

142
Q

What is Ollier disease?

What morphology?

A

Multiple endochondromas

More cellular, have atypia

143
Q

What has the hallmark of histologically normal bone w/decrease in quantity, loss of horizontal trabeculae and thickened vertical trabeculae?

A

Osteoporosis

145
Q

What is a small intraosseous abscess that frequently involves the cortex and is walled off by reactive bone?

A

Brodie abscess

151
Q

What occurs mainly in axial skeleton and femur in pts > 70, most commonly in whites in U.S./Europe?

Mutation in familial form?
Juvenile form?

A

Paget’s (osteitis Deformans)

SQSTM1 –> Inc NF-KB
RANK or OPG

152
Q

How does osteomyelitis occur in adults?

Seed what regions?

Organism?

A

Open fractures, surgery, diabetic foot

Epiphysis and subchondral regions

S. Aureus

154
Q

Maffuci syndrome has what?

A

Enchondromatosis, spindle cell hemangiomas, ovarian carcinomas, and at risk for brain gliomas

156
Q

What confirms osteoporosis on xray?

A

30-40% loss

166
Q

What is the result of impaired formation/fx of osteoclasts?

A

Osteopetrosis

167
Q

Dead bone is known as what?

A

Sequestrum

176
Q

What presents as deep bone pain, deformity or nerve entrapment?

What complications?

A

Paget’s

sarcoma (osteosarcoma or fibrosarcoma)

184
Q

Postnatal fracture, skeletal fragility, hearing impairment, and joint laxity indicate what condition?

A

OI

Blue sclera gives it away

MSK 2 - AH (7 decks)

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