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Derm Rheum > Soft Tissue and Joint Tumors - Gupta > Flashcards

Soft Tissue and Joint Tumors - Gupta Flashcards

1
Q

List the (3) joint soft tissue swelling/tumors

A

Ganglion cysts

Giant cell tumor of tendon sheath

Tenosynovial giant cell tumor

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2
Q

Soft tissue tumors can be divided into 5 categories. List these categories

A

Tumors of Adipose

Fibrous Tumors

Tumors of Muscle

Tumors of Smooth Muscle

Tumors of Uncertain origin

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3
Q

List the adipose soft tissue tumors

A

Lipoma

Liposarcoma

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4
Q

List the fibrous soft tissue tumors

A

Nodular Fasciitis

Myositis Ossificans

Fibromatosis (Superficial and Deep)

Fibrosarcoma

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5
Q

List the muscle tumors

A

Rhabdomyoma

Rhabdomyosarcoma

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6
Q

List the smooth muscle tumors

A

Leiomyoma

Leiomyosarcoma

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7
Q

List the tumors of uncertain origin

A

Synovial sarcoma

Undifferentiated pleomorphic sarcoma

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8
Q

What pathology is being shown here?

Describe it

Is this malignant?

A

Ganglion cysts, not malignant

Small cyst-like spaces with no epithelial lining, containing myxoid mucinous material

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9
Q

What is this called?

Usually located where in body?

What cells are commonly present in these tumors?

A

Giant Cell Tumor of Tendon Sheath

Usually near joints, can interfere with function

Many lipid laden macrophages in tumor, gives it the yellow color

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10
Q

What do Giant Cell Tumors of Tendon Sheaths look like microscopically? Youve got this

A

Multinucleated giant cells in a background of histiocytes

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11
Q

List 2 gross pathologic features seen in Diffuse type tenosynovial giant cell tumors?

Joint most commonly involved?

Does this tumor inhibit function of the joint?

A

Hemosiderin pigment deposition, Villi formation

Commonly involves knee

Causes destruction (and loss of function) of joint

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12
Q

Most common benign soft tissue tumor in adults?

A

Lipoma

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13
Q

Most common malignant soft tissue tumor in adults

A

Liposarcoma

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14
Q

Most common malignant soft tissue tumor in children?

A

Rhabdomyosarcoma

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15
Q

By definition, lipomas are a tumor of ____ ____ tissue?

A

mature adipose

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16
Q

Common location of lipoma?

A

superficial extremity or trunk

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17
Q

Are lipomas associated with pain? Name an exception

A

No, most are painless, except angiolipomas will manifest with local pain

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18
Q

55-75% of lipomas are associated with what feature? (vague question)

A

molecular rearrangements of HMGA2/HMGIC at 12q13-15

  • she said not to memorize the specific rearrangment, just know they are associated with them
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19
Q

Treatment for lipoma?

A

cut that bitch out. they be good…usually

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20
Q

What is this cell? What is it a hallmark cell for?

A

Lipoblast, hallmark cell for liposarcoma

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21
Q

Key feature regarding location of liposarcomas within the body?

A

Found in deep soft tissue of the proximal extremities.

Also can be found in the retroperitoneum

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22
Q

What immunostain(s) can be used to identify liposarcoma?

Molecular testing for liposarcoma?

A

MDM2 and CDK4

12q13-15 amplification

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23
Q

3 histologic subtypes of liposarcoma? Which is worst?

A

Well differentiated liposarcoma (bad)

Myxoid liposarcoma (worse)

Pleomorphic liposarcoma (you’re fucked)

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24
Q

Are fibrous tumors common or rare?

Which one is the exception?

A

Relatively rare, except nodular fasciits (more common)

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25
Q

What is the key to identifying nodular fasciitis?

A

Clinical history

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26
Q

Rate of growth in nodular fasciitis?

Benign or malignant?

A

Rapid growth

Benign

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27
Q

Nodular fasciitis commonly is associated with a hx of what in patients?

A

Trauma

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28
Q

What cells are commonly found in nodular fasciitis

A

Since its rapid growth,

Rapid proliferation of immature fibroblasts, tons of mitotic figures, highly cellular

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29
Q

Within skin/subcutaneous layers, where is nodular fasciitis commonly found?

A

Subdermal fat

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30
Q

What condition is this associated with

A

Eggshell calficiation seen in Myositis ossificans

31
Q

Fibromatosis can be divided into what 2 types?

A

Superficial, deep

32
Q

Superficial fibromatosis definition?

A

infiltratitive fibroblastic proliferation that causes local deformity

33
Q

Irregular nodular thickening of palmar fascia, 50% bilateral, progressive contracture of 4th and 5th fingers. What is this condition called? What tumor is it associated with (be specific)

A

Palmar (Dupuytren contracture), part of Superficial fibromatosis

34
Q

What organ is involved in Peyronie Disease? What occurs? What tumor is it associated with (be specific)?

A

Palpable induration or mass on the dorsal penis. Eventual curvature of the shaft and/or constriction of the urethra.

Associated with Superficial Fibromatosis

35
Q

Deep Fibromatosis definition?

A

Large infiltrative masses that recur but do not metastasize

36
Q

Epidemiology of Deep Fibromatosis?

A

Teens-30’s, female

37
Q

What disorder is Deep Fibromatosis associated with?

Bonus question: What other tumor is also associated with this disorder?

A

Gardner’s Syndrome

Osteomas also associated with Gardner’s

38
Q

What genes are associated with deep fibromatosis?

A

APC gene or Beta-Catenin, both lead to increased WNT signaling

39
Q

Severity of deep fibromatosis?

A

The fibromatosis itself is not severe (does not metastize) but its the damage they can cause on other organ function that is severe. (i.e. can cause bowel obstructions etc)

40
Q

Immunostain + for what in deep fibromatosis?

A

Beta catenin + on immunostain

41
Q

Microscopy of deep fibromatosis?

A

DENSE collagen, spindle cell proliferation, no mitosis, no necrosis

42
Q

What is being shown here? What tumor is it associated with?

A

malignant neoplasm of fibroblasts in a herringbone pattern

Seen in Fibrosarcoma

43
Q

Over 25% of fibrosarcomas spread where? via what route?

A

hematogenous spread to lungs

44
Q

Most frequent primary tumor of pediatric heart?

A

Rhabdomyoma

45
Q

Tubular sclerosis is associated with what tumor?

A

Rhabdomyomas, seen in 50%

46
Q

Mutations associated with Rhabdomyoma?

A

TSC1 (hamartin)

TSC2 (tuberin)

47
Q

What is this cell? What is it associated with?

A

Spider cell, Rhabdomyoma

48
Q

Prognosis of rhabdomyoma?

A

Most spontaneously regress

49
Q

Sarcoma means what?

A

Malignant mesenchymal tumor

50
Q

Rhabdomyo means what?

51
Q

Rhabdomyosarcoma means what?

A

Malignant mesenychmal tumor with skeletal muscle differentiation

52
Q

3 types of rhabdomyosarcoma?

Which 2 are more common in people < 20 years old?

Which is more common in adults?

A

<20 years old: alveolar, embryonal

Adults: pleomorphic

53
Q

Pediatric forms of rhabdomyosarcoma commonly arise where in body?

A

sinuses, head and neck, GU tract

54
Q

What are these cells?

What tumor are they associated with?

A

Rhabdomyoblasts (eosinophilic inclusions)

associated with Rhabdomyosarcoma

55
Q

Rhabdomyoblasts contain what muscle components? As a result, what stains can you use to identify them?

A

Contain sarcomes (thick and thin filaments) and Z-bands

Can use actin and myosins stains

56
Q

Besides rhabdomyoblasts, what other cells are associated with rhabdomyosarcoma?

A

Elongated “strap” cells

57
Q

Dx of this gross picture?

Variant of what? (be specific)

What is another differential dx?

A

Sarcoma botyoides

Variant of embryonal rhabdomyosarcoma

Teratoma looks kinda similar, but isnt coming out of vagina

58
Q

Translocations associated with alveolar rhabdomyosarcoma?

Which has a worse prognosis?

A

t(1:13)

t(2:13) PAX3-FKHR = worse prognosis

59
Q

Benign tumor of smooth muscle?

60
Q

Most common neoplasm in women?

61
Q

Key feature of leiomyomas on histology?

A

Fascicles of densely eosinophilc spindle cells that often intersect at right angles

62
Q

Loss of function mutation in fumerate hydratase is associated with what 2 disorders?

A

Hereditary leiomyomatosis, renal cell cancer syndrome

63
Q

Most common site of leiomyoma?

A

Uterus most common (wall of uterus is made up of smooth muscle)

However, they can be found all over body

64
Q

What type of tumor is known to wrap around the esophagus?

65
Q

Describe gross pathology of leiomyoma

A

Well-circumscribed, cut surface shows homogeneity, “white and whorled” appearance, degeneration in middle

If you see hemorrhage or necrosis, start thinking leiomyosarcoma

66
Q

Leiomyosarcomas

Account for what % of soft tissue sarcomas?

More common in men or women?

Painful or painless?

Deadly form can arise if what involved?

A

10-20% of soft tissue sarcomas

Women>men

Typicall painless (can be large and bulky)

Deadly form can arise from the great vessels

67
Q

What gross feature of leiomyosarcomas can distinguish them from leiomyomas?

A

will have hemorrhage, necrosis

68
Q

In general, sarcomas tend to spread via what route?

Commonly spread to what organ?

A

hematogenous spread, as opposed to carcinomas that generally spread through lymphatics

Commonly spread to lungs

69
Q

Chromosomal translocation t(X,18) asocciated with what tumor?

A

Synovial Sarcoma

70
Q

Are synovial sarcomas common?

A

Yes, 10% of all soft tissue sarcomes, ranked #4

71
Q

Why is synovial sarcoma a misnomer?

A

B/c it was thought to start in synovium (first recognized in knee synovium), but now its known that they can present in areas without synovium

72
Q

Synovial sarcoma subtypes?

A

Monophasic, Biphasic

73
Q

Monophasic or biphasic described as uniform spindle cells with scant cytoplasm?

A

Monophasic

Biphasic has spindle cells AND gland like structures

74
Q

What is considered the malignant high grade waste basket of sarcomas?

A

Undifferentiated pleomorphic sarcoma

  • cannot be identified based on histology, immunohistochemical profile, ultrastructure, or molecular genetics

Derm Rheum (39 decks)

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