Soft Tissue and Joint Tumors - Gupta Flashcards by Jacob McMillan

List the (3) joint soft tissue swelling/tumors

Ganglion cysts

Giant cell tumor of tendon sheath

Tenosynovial giant cell tumor

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

Tumors of Adipose

Fibrous Tumors

Tumors of Muscle

Tumors of Smooth Muscle

Tumors of Uncertain origin

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List the adipose soft tissue tumors

Lipoma

Liposarcoma

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List the fibrous soft tissue tumors

Nodular Fasciitis

Myositis Ossificans

Fibromatosis (Superficial and Deep)

Fibrosarcoma

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List the muscle tumors

Rhabdomyoma

Rhabdomyosarcoma

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List the smooth muscle tumors

Leiomyoma

Leiomyosarcoma

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List the tumors of uncertain origin

Synovial sarcoma

Undifferentiated pleomorphic sarcoma

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What pathology is being shown here?

Describe it

Is this malignant?

Ganglion cysts, not malignant

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

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What is this called?

Usually located where in body?

What cells are commonly present in these tumors?

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

Multinucleated giant cells in a background of histiocytes

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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?

Hemosiderin pigment deposition, Villi formation

Commonly involves knee

Causes destruction (and loss of function) of joint

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Most common benign soft tissue tumor in adults?

Lipoma

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Most common malignant soft tissue tumor in adults

Liposarcoma

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Most common malignant soft tissue tumor in children?

Rhabdomyosarcoma

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

mature adipose

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Common location of lipoma?

superficial extremity or trunk

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Are lipomas associated with pain? Name an exception

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

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

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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Treatment for lipoma?

cut that bitch out. they be good…usually

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

Lipoblast, hallmark cell for liposarcoma

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Key feature regarding location of liposarcomas within the body?

Found in deep soft tissue of the proximal extremities.

Also can be found in the retroperitoneum

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

Molecular testing for liposarcoma?

MDM2 and CDK4

12q13-15 amplification

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3 histologic subtypes of liposarcoma? Which is worst?

Well differentiated liposarcoma (bad)

Myxoid liposarcoma (worse)

Pleomorphic liposarcoma (you’re fucked)

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Are fibrous tumors common or rare?

Which one is the exception?

Relatively rare, except nodular fasciits (more common)

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What is the key to identifying nodular fasciitis?

Clinical history

Rate of growth in nodular fasciitis? Benign or malignant?

Rapid growth Benign

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

Trauma

What cells are commonly found in nodular fasciitis

Since its rapid growth, Rapid proliferation of immature fibroblasts, tons of mitotic figures, highly cellular

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

Subdermal fat

What condition is this associated with

Eggshell calficiation seen in Myositis ossificans

Fibromatosis can be divided into what 2 types?

Superficial, deep

Superficial fibromatosis definition?

infiltratitive fibroblastic proliferation that causes local deformity

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)

Palmar (Dupuytren contracture), part of Superficial fibromatosis

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

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

Deep Fibromatosis definition?

Large infiltrative masses that **recur** but **do not metastasize**

Epidemiology of Deep Fibromatosis?

Teens-30's, female

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

Gardner's Syndrome Osteomas also associated with Gardner's

What genes are associated with deep fibromatosis?

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

Severity of deep fibromatosis?

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)

Immunostain + for what in deep fibromatosis?

Beta catenin + on immunostain

Microscopy of deep fibromatosis?

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

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

malignant neoplasm of fibroblasts in a herringbone pattern Seen in Fibrosarcoma

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

hematogenous spread to lungs

Most frequent primary tumor of pediatric heart?

Rhabdomyoma

Tubular sclerosis is associated with what tumor?

Rhabdomyomas, seen in 50%

Mutations associated with Rhabdomyoma?

TSC1 (hamartin) TSC2 (tuberin)

What is this cell? What is it associated with?

Spider cell, Rhabdomyoma

Prognosis of rhabdomyoma?

Most spontaneously regress

Sarcoma means what?

Malignant mesenchymal tumor

Rhabdomyo means what?

Muscle

Rhabdomyosarcoma means what?

Malignant mesenychmal tumor with skeletal muscle differentiation

3 types of rhabdomyosarcoma? Which 2 are more common in people \< 20 years old? Which is more common in adults?

\<20 years old: alveolar, embryonal Adults: pleomorphic

Pediatric forms of rhabdomyosarcoma commonly arise where in body?

sinuses, head and neck, GU tract

What are these cells? What tumor are they associated with?

Rhabdomyoblasts (eosinophilic inclusions) associated with Rhabdomyosarcoma

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

Contain sarcomes (thick and thin filaments) and Z-bands Can use actin and myosins stains

Besides rhabdomyoblasts, what other cells are associated with rhabdomyosarcoma?

Elongated "strap" cells

Dx of this gross picture? Variant of what? (be specific) What is another differential dx?

Sarcoma botyoides Variant of embryonal rhabdomyosarcoma Teratoma looks kinda similar, but isnt coming out of vagina

Translocations associated with alveolar rhabdomyosarcoma? Which has a worse prognosis?

t(1:13) t(2:13) PAX3-FKHR = worse prognosis

Benign tumor of smooth muscle?

Leiomyoma

Most common neoplasm in women?

Leiomyoma

Key feature of leiomyomas on histology?

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

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

Hereditary leiomyomatosis, renal cell cancer syndrome

Most common site of leiomyoma?

Uterus most common (wall of uterus is made up of smooth muscle) However, they can be found all over body

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

Leiomyoma

Describe gross pathology of leiomyoma

Well-circumscribed, cut surface shows homogeneity, "white and whorled" appearance, degeneration in middle If you see hemorrhage or necrosis, start thinking leiomyosarcoma

Leiomyosarcomas Account for what % of soft tissue sarcomas? More common in men or women? Painful or painless? Deadly form can arise if what involved?

10-20% of soft tissue sarcomas Women\>men Typicall painless (can be large and bulky) Deadly form can arise from the great vessels

What gross feature of leiomyosarcomas can distinguish them from leiomyomas?

will have hemorrhage, necrosis

In general, sarcomas tend to spread via what route? Commonly spread to what organ?

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

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

Synovial Sarcoma

Are synovial sarcomas common?

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

Why is synovial sarcoma a misnomer?

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

Synovial sarcoma subtypes?

Monophasic, Biphasic

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

Monophasic Biphasic has spindle cells **AND** gland like structures

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

Undifferentiated pleomorphic sarcoma - cannot be identified based on histology, immunohistochemical profile, ultrastructure, or molecular genetics