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Derm Rheum > Tumors of Bone and Cartilage - Gupta > Flashcards

Tumors of Bone and Cartilage - Gupta Flashcards

1
Q

What are the tumors of bone?

A

1) Osteoma
2) Osteoid osteoma/osteoblastoma
3) Osteosarcoma

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2
Q

What is an osteoma?

A

Benign bone forming tumor composed of compact or mature trabecular bone –> usually involves facial bones

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3
Q

What is the clinical presentation of a pt with osteoma?

A

1) Pain
2) Headache
3) Vision changes

Associated with Gardner syndrome (familial colorectal polyposis)

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4
Q

What is the microscopic appearance of an osteoma?

A

Dense compact bone with paucicellular stroma (see very few cells)

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5
Q

What is Gardner syndrome?

A

Autosomal dominant (chromosome 5q21 - APC gene) disease leading to multiple colon polyps and tumors in thyroid, bone, epidermoid cysts, fibromas, and desmoid tumors

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6
Q

What are desmoid tumors?

A

Tumors that grow in b/t muscle fibers and can cause obstruction –> surgical resection causes more tumors to form

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7
Q

What is osteoid osteoma?

A

Benign tumor of osteoblasts

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8
Q

What population are osteoid osteomas seen in?

A

Males < 25 years old

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9
Q

What is the clinical presentation of osteoid osteoma?

A

Bone pain that resolves with aspirin

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10
Q

What is the microscopic appearance of osteoid osteoma?

A

Randomly interconnected trabeculae of woven bone, prominently rimmed by a single cell layer of osteoblasts

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11
Q

Where do osteoid osteomas occur and what do they look like on imaging?

A

Cortex of long bones

Bony mass (<2cm) with radiolucent center (osteoid, black arrow) with surrounding sclerosis (white arrow)

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12
Q

How does osteoblastoma differ from osteoid osteoma?

A

Osteoblastoma usually larger (>2cm)

Bone pain doesn’t respond to aspirin

Arise in the vertebrae

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13
Q

What does osteoblastoma look like microscopically?

A

Comprised of anastomosing trabeculae of osteoid and woven bone rimmed by osteoblasts

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14
Q

How is osteoblastoma treated?

A

Curettage (scooping) or excision en bloc

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15
Q

What is osteosarcoma?

A

Malignant prolferation of osteoblasts

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16
Q

What is the epidemiology of osteosarcoma?

A

Mostly in teenage males

Associated with Pagets disease and post-radiation in elderly

Rb gene = increased risk and poor prognosis

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17
Q

What characteristic feature does osteosarcoma show on imaging?

A

Codman’s triangle –> periosteol reaction to tumor destroying new bone before it ossifies

Indicates aggresive tumor

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18
Q

What does osteosarcoma look like microscopically?

A

Invades normal bone producing poorly formed bony spicules in a hypercellular matrix of osteoid and numerous pleomorphic malignant cells

Described as “lace-like” –> dainty little tumors

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19
Q

What is seen in chondroblastic osteosarcoma?

A

Malignant cartilage formation

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20
Q

What is the pathogenesis of osteosarcoma?

A

70% have acquired genetic mutation

Rb –> germline Rb 1000x increased risk

TP53 –> DNA repair and apoptosis

INK4a –> encodes tumor suppressor

MDM2 and CDK4 –> cell cycle regulators that inhibit p53 and Rb

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21
Q

What are the cartilage-forming tumors?

A

1) Chondroma
2) Osteochondroma
3) Chondrosarcoma

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22
Q

What is a chondroma?

What are the types of chondroma?

A

Benign cartilaginous tumor

1) Enchondroma –> arises from diaphyseal medullary cavity
2) Subperiosteal/juxtacortical
3) Soft tissue chondroma

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23
Q

What genetic mutations are associated with enchondromas?

A

IDH1 and IDH2 (isocitrate dehydrogenase)

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24
Q

What does a chondroma look like grossly?

A

Grey-white mass with color consistent with cartilage –> usually well circumscribed

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25
Q

What does chondroma look like microscopically?

A

Numerous chondrocytes closely packed near periphery in a pink ground substance that resembles normal cartilage

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26
Q

What is an osteochondroma?

A

Tumor of bone with an overlying cartilage cap –> mushroom cap appearance on X-ray is pathognomonic

27
Q

What is the epidemiology of osteochondroma?

A

Mostly in males 10-20 years old

28
Q

What is the epidemiology and location of enchondroma?

A

Age 20-49, no gender preference

Usually found in small bones of hands and feet

29
Q

What is Maffuci syndrome?

A

Multiple enchondromas and soft tissue hemangiomas; also ovarian carcinoma and brain glioma

30
Q

What is chondrosarcoma and where is it typically found?

A

Malignant cartilage forming tumor (no osteoid)

Arises in medulla of pelvis or central/axial skeleton

31
Q

What is the epidemiology of chondrosarcoma?

A

Men in their 40s

32
Q

What is the gross appearance of chondrosarcoma?

A

Invasive cartilage growth in bone

This example shows cartilage growing across joint space

33
Q

What is the microscopic appearance of chondrosarcoma?

A

Cells mimic normal chondrocytes –> appear to float in chondroid-like matrix

34
Q

What are the fibrous and fibroosseous tumors?

A

1) Fibrous cortical defect
2) Fibrous dysplasia

35
Q

What is a fibrous cortical defect (nonossifying fibroma)?

A

Benign fibrous lesion of bone

36
Q

What is the epidemiology of fibrous cortical defect?

A

Children and adolescents

37
Q

What is the microscopic appearance of fibrous cortical defect?

A

Fibrous spindle cells with pinwheel appearance

Scattered giant cells, histiocytes, cholesterol clefts, and hemosiderin

38
Q

What does fibrous cortical defect look like grossly?

A

Lesion >5cm with intermedullary component

39
Q

What is fibrous dysplasia?

A

A problem with osteoblastic maturation that causes fibrous tissue to form instead of medullary bone

40
Q

How does fibrous dysplasia present and what are the most common sites?

A

Presents with painful swollen bones prone to fracture

Ribs and femur are most commonly affected

41
Q

What is the gross appearance of fibrous dysplasia?

A

Variable size, well circumscribed, medullary, tan-white, gritty mass lesions

42
Q

What is the microscopic appearance of fibrous dysplasia?

A

Poorly formed islets of trabeculae of woven bone without a rim of osteoblasts –> resemble chinese characters

Fibroblastic activity surrounding trabeculae with lots of collagen

43
Q

What is McCune-Albright syndrome?

A

Fibrous dysplasia plus cafe-au-laite spots and endocrine dysfuntion (especially early onset puberty)

44
Q

What is a giant cell tumor of bone?

A

Tumor composed of multinucleated giant cells and stromal cells

45
Q

What is the epidemiology of giant cell tumor of bone and where does it typically arise?

A

Young adults (20-40)

Epiphysis of long bones, typically in the knee

46
Q

What is the characterstic appearance of giant cell tumor on X-ray?

A

“Soap bubble” appearance –> dude is fucked

47
Q

What feature of giant cell tumor cells makes them a good candidate for targeted therapy?

A

High level of RANK-L expression

48
Q

What is the microscopic appearance of giant cell tumor?

A

Regular and uniform distribution of stromal and giant cells –> nuclei of mononuclear cells and osteoclasts are ovoid with prominent nucleoli (top pic)

Fibroblastic activity and formation of reactive bone (bottom pic, pink shit)

49
Q

What is Ewing sarcoma (primitive neuroectodermal tumor)?

A

Malignant proliferation of poorly-differentiated cells derived from neuroectoderm

50
Q

What is the epidemiology and typical location of Ewing sarcoma?

A

Usually in males <15 years old

Typically found in diaphysis of long bones

51
Q

What is the gross appearance of Ewing sarcoma?

A

White, fleshy, ill-defined tumor with involvement of medulla and cortex with periosteal elevation

52
Q

What is the microscopic appearance of Ewing sarcoma?

A

Sheets of small, round, uniform cells (resemble lymphocytes)

Indistinct cell membranes

53
Q

What is the characteristic appearance of Ewing sarcoma on X-ray?

A

Onion skin appearance of periosteum - layered periosteum

54
Q

What translocation is seen in Ewing sarcoma?

A

t(11,22) EWS-FLI1 fusion

55
Q

What cellular organization pattern is sometimes seen in Ewing sarcoma?

A

Homer Wright rosettes –> dark blue tumor cells surrounding pink fibrillar material resembling neuropil

56
Q

What is a solitary bone cyst?

A

Benign lytic lesion, fluid-filled cyst with a thin wall

57
Q

What is the epidemiology and typical location of solitary bone cysts?

A

Males <20 years old

Medullary in metaphysis of proximal humerus or femur –> typicaly presents as a fracture

58
Q

What is an anuerysmal bone cyst?

A

Exactly what is sounds like asshole

Benign, but grows rapidly

Looks like a sponge filled with blood

59
Q

What is the epidemiology of anuerysmal bone cysts?

A

No gender preference; ages 1-20

Removed by curettage

60
Q

What is the microscopic appearance of anuerysmal bone cysts?

A

Richly vascularized bone tissue –> differentiating feature is rich capillary bed

61
Q

What do metastatic bone tumors do to bone? What is the exception?

A

Produce punched out lesions radiographically

Prostatic carcinoma produces osteoblastic (sclerotic) lesions

62
Q

What is the mnemonic to remember sites of metastasis for bone shit

A

BLT with a Kosher Pickle

Breast

Lung

Thyroid

Kidney

Prostate

63
Q

Whats does metastatic bone tumor from prostatic carcinoma look like radiographically?

A

Osteoblastic lesions –> highly sclerotic bone

Derm Rheum (39 decks)

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