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msk-derm > Neoplasia > Flashcards

Neoplasia Flashcards

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Dermatology Board Prep flashcards
1
Q

What is an osteochondroma?

A

A benign cartilage-capped bony projection that contains a marrow space contiguous with the underlying medulla of the bone

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2
Q

What genetic abberations are linked to osteochondromas?

A

Abberations of chromosome 8q22-24 (site of suppressor gene EXT)

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3
Q

Where is the tumor located in osteochondromas?

A

It pokes out of the bone, away from the joint space

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4
Q

What is the typical age of onset of osteochondromas?

A

Within the first 20 years of life

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5
Q

What is the sex ratio of osteochondromas?

A

M:F –> 2:1

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6
Q

Where are osteochondromas usually located?

A

Distal femur, proximal tibia, proximal humerus

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7
Q

What is the typical x-ray finding of osteochondromas?

A

Cartilage cap on a bony stalk (little mushroom) that is contiguous with the bone and grows away from growth plate

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8
Q

What is the treatment and prognosis for osteochondromas?

A

Treatment: none unless symptomatic or secondary to another problem
Prognosis: benign, low recurrence rate when removed

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9
Q

What is an osteoid osteoma?

A

A benign bone-producing tumor, characteristically < 2 cm

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10
Q

What is the classic history for osteoid osteomas?

A

Increasing duration and severity of pain, worse at night, relieved by aspirin or NSAIDs

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11
Q

What is the typical age of onset of osteoid osteomas?

A

2nd-3rd decades, rarely after 30 years old

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12
Q

What is the sex ratio for osteoid osteomas?

A

M:F –> 2:1

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13
Q

What is the typical location of osteoid osteomas?

A

Any bone except skull or sternum, often in femur/tibia, mostly in the cortex of the metadiaphysis

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14
Q

What are the x-ray findings of osteoid osteomas?

A

Zone of sclerotic bone surrounding well-defined lucent foci

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15
Q

What is a Nidus?

A

Woven bone lined by osteoblasts

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16
Q

What are the microscopic findings of osteoid osteomas?

A

Zone 1: Nidus lined by osteoblasts
Zone 2: rich fibrovascular stroma
Zone 3: surrounding dense cortical bone

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17
Q

What is the treatment and prognosis of osteoid osteomas?

A

Treatment: Nidus removal, often via thermal ablation
Prognosis: Excellent when removed completely

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18
Q

What is a chondroma/enchondroma?

A

Benign hyaline cartilaginous lesion that is subtyped by location

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19
Q

What is the age range for enchondroma formation?

A

Wide age range, mostly in 2nd-4th decades

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20
Q

What is the sex ratio for enchondromas?

A

M:F –> 1:1

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21
Q

What is the typical location for enchondromas?

A

Mostly in small bones of hands/feet, also in humerus, proximal and distal femurs

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22
Q

What are the x-ray findings of enchondromas?

A

well-circumscribed, lytic, lobulated lesions with arc and ring calcification

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23
Q

What are the microscopic findings of enchondromas?

A

Lobules of normal hyaline cartilage separated by bone marrow

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24
Q

What is the treatment and prognosis of enchondromas?

A

Treatment: Curettage

Prognosis: Excellent when fully removed

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25
Q

What are multiple enchondromatas?

A

Nonhereditary disorder characterized by multiple cartilaginous tumors with unilateral distribution in the appendicular skeleton

also known as Ollier’s disease or Maffuci’s syndrome

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26
Q

What are giant cell tumors of bone?

A

Locally aggressive neoplasm composed of mononuclear cells and osteoclast-like giant cells

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27
Q

What is the age range of giant cell tumors?

A

Young adults, peak in 3rd decade

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28
Q

What is the sex ratio of giant cell tumors?

A

M:F –> 2:3 (F>M)

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29
Q

What is the common location of giant cell tumors?

A

Distal femur, proximal tibia, distal radius, and proximal humerus

Can also include flat bones

30
Q

What are the x-ray findings of giant cell tumors?

A

Eccentric, lytic with ill-defined margins

Tumor growing from metaphysis into epiphysis

31
Q

What are the microscopic findings of giant cell tumors?

A

Spindled cells with ill-defined cellular margins and osteoclast-like giant cells evenly distributed

32
Q

What is the treatment and prognosis of giant cell tumors?

A

High recurrence rate, better outcomes with bloc excision, cryosurgery, and supervoltage radiation

33
Q

What is fibrous dysplasia?

A

Developmental fibro-ossueos lesions composed of fibroblast-like cells and woven bone without osteoblastic rimming

34
Q

What genes lead to fibrous dysplasia?

A

Activating mutations in GNAS1, a GTPase the leads to elevated cAMP

35
Q

What is the common age group for fibrous dysplasia?

A

Children and young adults

36
Q

What is the common sex ratio for fibrous dysplasia?

A

M=F

37
Q

What is the common location for fibrous dysplasia?

A

Femor, craniofacial bones, tibia, ribs

38
Q

What are the x-ray findings of fibrous dysplasia?

A

Metadiaphyseal/diaphyseal, expansile lesion with variable density

39
Q

What are the microscopic findings of fibrous dysplasia?

A

Irregularly shaped C and S spicules of woven bone within a low/moderately cellular stroma

40
Q

What is the treatment/prognosis for fibrous dysplasia?

A

May recur if not completely excised

41
Q

What is McCune-Albright syndrome?

A

Polyostotic fibrous dyplasia with cafe-au-lait lesions and endocrinopathies

42
Q

What are osteosarcomas?

A

Primary malignant tumors of bone where malignant neoplastic cells produce osteoid and/or woven bone

43
Q

What age groups are most affected by osteosarcomas?

A

Peak in 2nd decade, usually < 25 years of age

44
Q

What is the sex ratio of osteosarcomas?

A

M:F –> 2:1 or 3:1

45
Q

What is the common location of osteosarcomas?

A

Metaphysis of distal femur followed by proximal tibia and proximal humerus

46
Q

What are the x-ray findings of osteosarcomas?

A

Mixed blastic and lytic with cortical destruction and soft tissue mass

47
Q

What are the microscopic findings of osteosarcomas?

A

Malignant cells with osteoid or woven bone

48
Q

What is the treatment/prognosis of osteosarcomas?

A

Surgery and chemotherapy with relatively good prognosis

49
Q

What is the age range for chondrosarcoma?

A

Peak in 5th-7th decades

50
Q

What is the sex ratio of chondrosarcomas?

A

M:F –> 2:1

51
Q

What is the typical location of chondrosarcomas?

A

Pelvis, proximal femur, proximal humerus, distal femur, and ribs

52
Q

What are the x-ray findings of chondrosarcomas?

A

Lobulated, lytic lesion with arc and ring calcification and areas of cortical thickening and/or destruction

53
Q

What are microscopic findings of chondrosarcomas?

A

Lobules of variably hypercellular hyaline cartilage with tumor lobules that encase, infiltrate and destroy native bone

54
Q

What is the treatment and prognosis of chondrosarcomas?

A

Rare metastases, surgical treatment with fairly good prognosis

55
Q

What is ewing sarcoma?

A

Primitive tumors of bone and/or soft tissue with neuroectodermal differentiation

56
Q

What is the typical age range for ewing sarcoma?

A

Mostly children under 20

57
Q

What is the sex ratio of ewing sarcoma?

A

M:F –> 2:1

58
Q

What are the most common locations for ewing sarcoma?

A

Appendicular skeleton, femur, tibia, fibula, and humerus followed by pelvis and ribs

59
Q

What are the x-ray findings of ewing sarcoma?

A

Ill-defined lytic lesions in diaphysis or meta-diaphysis

60
Q

What are the microscopic findings of ewing sarcoma?

A

Sheets/nests of small polygonal cells with rounded nuclei and cytoplasmic glycogen

61
Q

What is the treatment and prognosis for ewing sarcoma?

A

Treatment: neoadjuvant multiagent chemotherapy with excision or radiation

Prognosis: 40% survival

62
Q

What is a plasma cell myeloma?

A

Neoplasm composed of clonal proliferation of plasma cells, called multiple myeloma if there are multiple findings

63
Q

What is the common age range of plasma cell myelomas?

A

> 40 years old with a peak in 6th/7th decades

64
Q

What is the sex ratio of plasma cell myelomas?

A

M>F

65
Q

What are the common locations of plasma cell myelomas?

A

Vertebra, ribs, skull, pelvic bones, femur, clavicle, and scapula

66
Q

What are the x-ray findings of plasma cell myelomas?

A

Multiple, sharply circumscribed “punched out” lytic lesions

67
Q

What is the prognosis of plasma cell myeloma?

A

Incurable disease with a 10% 10 year survival rate

68
Q

All statements are true of osteosarcomas except:

a) in the vast majority of cases, the radiologic appearance of conventional osteosarcoma mimics other benign osseous tumors
b) a relatively higher percentage of pelvic and craniofacial osteosarcomas occur in patients over the age of 30 years
c) by definition, an osteosarcoma is a tumor in which bone matrix is produced by malignant mesenchymal cells
d) patients exhibiting at least a 90% histological response to chemo have a much improved survival rate
e) in children, osteosarcoma is a more commonly encountered primary bone tumor than chondrosarcoma

A

a) in the vast majority of cases, the radiologic appearance of conventional osteosarcoma mimics other benign osseous tumors

69
Q

All statements are true of osteochondroma except:

a) recurrences may occur if the cartilaginous cap isnot completely excised
b) radiologically and grossly, the tumor projects away fromthe joint space
c) the cartilaginous cap exhibit microscopic features similar to the cartilage composing the growth plate
d) presence of a cartilaginous cap > 2cm, especially in an adult, is worrisome for sarcomatous transformation
e) in multiple hereditary exostoses (osteochondromatosis), the incidence of malignant transformation of the cartilaginous cap is > 50%

A

e) in multiple hereditary exostoses (osteochondromatosis), the incidence of malignant transformation of the cartilaginous cap is > 50%

70
Q

A 10 yo male has a destructive lytic lesion of the proximal femur. Needle core biopsy of the lesion shows small cells with high nucleocytoplasmic ratio and clear cytoplasm. Which is the true statement regarding this lesion?

a) large cell lymphoma is a common primary bone tumor in this age group
b) this tumor sample should be sent for analysis of a specific translocation
c) cytoplasm of these cells do not have glycogen
d) tumor is adequately treated with surgery alone
e) cartilage is commonly formed by the tumor cells

A

b) this tumor sample should be sent for analysis of a specific translocation

msk-derm (41 decks)

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