deck_5375697 Flashcards by Shane Hagen

What do soft tissue tumors arise from?

not mature cells, but pluripotent stem cells (soft tissue tumors are not that common)

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Where are soft tissue tumors typically found?

up to 40% can be found on the thigh

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What are the joint tumors?

ØGanglion cystØGiant cell tumor of tendon sheathØTenosynovial Giant Cell Tumor

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What is a ganglion cyst?

psuedocyte (because theres no true epithelial lining)arising from the joint capsule or tenden sheath and is considered a myxoid degeneration

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What is this?

Ganglion cyst with the myxoid degeneration (light-blue)treated by excision

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What is a tenosynovial giant cell tumor?

collective name for a family of benign related tumors including localized neoplasms (aka giant cell tumor of tendon sheath) or diffuse (pigmented villa-nodular synovitis)

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What is this?

a giant cell tumor of tendon sheath - multinucleated giant cells in a background of histiocytes and cells that resemble synoviocytesthis is the localized type of tenosynovial giant cell tumors

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What dis?

This is the diffuse type of tenosynovial giant cell tumor previously called pigmented villanodular synovitis

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Where doespigmented villanodular synovitis show up most commonly?

the knee and causes synovium proliferation

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What are the tumors of adipose?

-lipomas-liposarcomas

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What dat?

lipoma- a benign tumor of fat (most common soft tissue tumor in adults) commonly found on the trunk in older men

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How can you differentiate lipomas from angiolipomas?

most lipomas are mobile, slow growing, and PAINLESS (angiolipomas can have pain)

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What this?

Lipoma- complete excision usually curativeremember: mobile, slow growing and PAINLESS

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What is a common mutation assoicated with lipomas?

55-75% of solitary lipomas have rearrangements of HMGA2/HMGIC at 12q13-15

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What is this? How to get rid of?

liposarcoma with the hallmark ‘lipoblast’ cell (lipid vacuoles that intent the central nucleus)surgery is the mainstay

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What is a liposarcoma?

common soft tissue tumor in late adulthood (50-60s) that RECUR LOCALLY AND REPEATEDLY UNLESS ADEQUATELY EXCISEDmost common soft tissue sarcoma of adults

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Where are liposarcomas commonly found?

DEEP soft tissue of the proximal extremities and in the retroperitoneum (bad!)

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Immunostains for liposarcomas?

MDM2 and CDK4 for molecular testing for 12q13-15 amplification

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What are the fibrous tumors?

-Nodular fasciitis-Myositis Ossificans-Fibromatosis (Superficial and Deep)-Fibrosarcoma

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What is nodular fasciitis?

a reactive pseudosarcoma consisting of plump fibroblasts that are solitary, RAPIDLY GROWING AND THERE IS A HX OF TRAUMA

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What is the classic spot for nodular fasciitis?

the subdermal fat, commonly in the arms and antibrazos

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What is Myositis ossificans?

a neoplasm distinguished by the presence of metaplastic bone that arises in the proximal extremities after TRAUMA, typically in YOUNG ATHLETESlooks like an osteosarcoma

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What is superficial fibromatosis?

A benign,infiltrative fibroblastic proliferation that causes local deformity common in males

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What dis?

Deep fibromatosis

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What are deep fibromatoses?

large infiltrative masses that occur but do not metastasize in females from teens-30s

Deep fibromatoses have an association with what?

APC gene or Beta-Catenin, both leading to increased WNT signaling-Gardner's Syndrome

What are fibrosacromas?

malignant neoplasm of fibroblasts in adults

Where are fibrosarcomas commonly found?

deep in the thigh or retroperitoneum

Where do fibrosarcomas go to?

heme spread to the lungs most commonly (25%)local recurrence is common

What dis?

Fibrosarcomas- patterns vary from resemblin fibromatosis to herringbone patterns to architectural disarray

How do fibrosarcomas appear grossly?

unencapsulated with necrosis and hemorrhage

What are the tumors of muscle?

-rhabdomyoma-rhabdomyosarcoma

rhabdomyoma with 'spider' cells

What is the most frequent primary tumor of the pediatric heart?

rhabdomyomamay cause valvular or cardiac chamber obstruction

What is the progression of rhabdomyomas?

most spontaneously regress (more of a hamartoma than a true neoplasm)

50% of rhabdomyomas are associated with what?

tuberous sclerosis -mutations of TSC1 (hamartin) and TSC2 (tuberin)

Rhabdomyosarcomathese eosinophilic inclusions are the hallmark to histo diagnosis= rhabdomyoblasts! (these have sarcomeres and Z bands)

What is a rhabdomyosarcoma?

a MALIGNANT mesenchymal tumor with skeletal muscle differentiations

What are the subtypes of rhabdomyosarcomas?

-alveolar-embryonal-pleomorphic

What are the most common soft tissue sarcomas of childreen (under 20 yo)?

alveolar and embryonal rhabdomyosarcomas the pediatric forms typically arise in the sinuses, head, neck, and GU tract

Pleomorphic rhabdomyosarcomas are in what patient pop?

adults

What is this?

pleomorphic rhabdomyosarcoma- more common in ADULTS and there are numerous, multinucleated bizarre eosinophilic tumor cells

What stains can comfirm a pleomorphic rhabdomyosarcoma?

Myogenin (IHC stain)

What is this?

alveolar rhabdomyosarcoma- seen in children and kind of looks like lung

What is this?

Embryonal rhabdomyosarcoma- seen in children and characterized by soft gray infiltrative mass compound of spindled and round cells in myxoid stroma

What is sarcoma botyoides?

A variant of embryonal rhabdomyosarcoma that develops in the wall of hallow mucosa lined area (nasopharynx and vagina) and form a CAMBLUM layer

What mutations are common in alveolar rhabdomyosarcoma? Which one has the worst prognosis?

t(1,13) and t(2,13) with t(2,13)= worse prognosis

What is this?

Sarcoma botyoides (variant of embryonal rhabdomyosarcoma)

What is this showing?

In sarcoma botyoides, you seen a polypoid mass covered by mucosa, with an underlying hypercellular zone of pporly differentiated cells (cambium layer)

What are the tumors of smooth muscle?

-leiomyoma-leiomyosarcoma

What is this?

Leiomyoma- a benign tumor of smooth muscle (the most common neoplasm in women)- tend to be positive for estrogen receptor Image: Fascicles of densely eosinophilic spindle cells that often intersect at right angles

What causes Hereditary leiomyomatosis and renal cell cancer syndrome?

– germ line loss-of-function mutation in the fumerate hydratase (enzyme in Krebs cycle) gene

What is this?

Leiomyomas arise almost anywhere in the body, since smooth muscle is ubiquitous and rarely transform to leiomyosarcoma.

What is the most common location for a leiomyomas?

Uterus is the most common site; the wall of the uterus is made up of smooth muscle.

What is this?

Leiomyosarcoma - look at how non-uniform this looks

Where are leiomyosarcomas common?

Deep soft tissue of the extremities and retroperitoneum (more in women than men)10-20% of soft tisse sarcomas they are typically painless and can be large and bulky

Whats going on here?

Leiomyosarcoma metastatic to lung

T or F. Sarcomas tend to spread through the bloodstream (hematogenous spread) rather than through the lymphatic system

T, so one very characteristic place for them to metastasize to is the lungs, where the capillary bed acts as a filter for circulating tumor cells. Once filtered, some tumor cells are able to grow.

Pulmonary metastases are generally multiple, frequently numerous and tend to be larger and more numerous in the basal lower lobes because of the gradient in pulmonary arterial blood flow, which goes from highest in the bases to lowest in the apices.

Pulmonary metastases are a major cause of death for patients with sarcomas of all kinds.

What is a synovial sarcoma?

A misnomer, this tumor is of uncertain origin and can present in locations that lack synovium, often as painless deep seated masses for several years these represent 10% of all soft tissue sarcomas

What is the most common mutation in synovial sarcomas?

t(X,18)

Facts about synovial sarcomas

most common in 20s-40sMETs common to lung and lymph nodesTreatment: aggressive with limb-sparing surgery and often chemo

What is this?

Undifferentiated pleomorphic sarcoma- Malignant high grade waste basket – these tumors don’t fit anywhere and can not be identified based on histology, immunohistochemical profile, ultrastructure or molecular genetics.

Dx?

Giant cell tumor of tendon sheath

Dx?

Squamous cell carcinoma

What is this?

McCune Albright

Dx?

radiolucent nidus of osteoid osteoma

deck_5375697 Flashcards

(67 cards)