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Flashcards in Soft TIssue Tumors Deck (45):

What is the embryological basis for sort-tissue tumors?

tey're mesenchymal proliferations


what general term do we use for malignant soft tissue tumors?



What are some envrionmental factors that have been associated with the development of soft tissue tumors?

1. trauma (from scar tissue)

2. enviornment agents like diosin and vinyl chloride

2. radiation therapy

4. oncogenic viruses

5. chronic lymphedema


What are common chromosomal mutations found in soft tissue tumors?

translocation abnormalities creation fusion genes that cause upregulation of transcription


What are some of the factors influencing the prognosis of soft tissue tumors?

1. histological classification

2. histologic grade

3. tumor stage (TNM staging)

4. location - superficial tumors have bette rprogrnosis

5. size - bigger is worse



What three factors go into the histological grade of a soft tissue tumor?

1. differentiation score (more differentiated is better)

2. mitotic count

3. amount of tumor necrosis


How do most soft tissue tumor metastases spread?



Which imaging technique is best for looking at soft tissue tumors?

MRI for everywhere but the abdomen - use CT for those


WHat are fatty soft tissue tumors called?



What are the 7 types of lipomas?

1. conventional

2. angiolipoma

2. pleomorphic/spindle cell lipoma

4. myolipoma

5. chondroid lipoma

6. angiomyolipoma

7. myelolipoma


WHere do liposarcomas usually occur?

deep soft tissues of proximal extremities and retroperitoneum

(one of the most common sarcomas of adulthood)


What are the 3 types of liposarcomas?

well-differentiated (least aggressive, but can still kill you if located in retroperitoneum)

myxoid/round cell liposarcoma (likely to recurr, somewhat aggressive)

pleomorphic liposarcoma (most aggressive)


Why are reactive pseudosarcomatous proliferations concerning clinically? Are they actually an issue?

THey are concerning clinically because they grow rapidly and can have an infiltrative appearance.

However, they are not concerning at all because they're not even neoplastic


What are the two forms of reactive pseudosarcomatous proliferation?

1. nodular fasciitis(proliferation of immature fibroblasts and myofibroblasts in myxoid stroma)

2. myositis ossificans (plump fibroblasts and myofibroblasts like in nocular fasciitis, except the outer margind evelopes osteoblasts which cause ossification of outer layer)


In general, what is a superficial fibromatosis?

a benign fibroblastic proliferation involving the fascia or aponeuroses

characterized by nodular or poorly defined broad fasicles of fibroblasts and myofibroblasts surrounded by dense collagen


What are the three most common superficial fibromatoses?

palmar fibromatosis (dupuytren's contracture)

plantar fibromatosis

penile fibromatosis (peyronie disease)


What do deep-seated fibromatosis (desmoid tumors) consist of?

infiltrative, poorly marginated proliferation of bland fibroblasts with collagen

don't metastasize, but will recur



what are the 3 general categories of the deep=seated fibromatosis (desmoid) tumors?

abdominal (in musculoaponeurotic structures of anterior abdominal wall - especially during pregnancy)

extra-abdominal fibromatosis (shoulders, chest, back and thigh)

intra-abdominal fibromatosis (mesentary or pelvic walls)


What syndrome is often associated with intra-abdominal fibromatoses?

familial adenomatosis polyposis = Gardner Syndrome


What do we call a malignant fibrous soft tissue tumor?

 a fibrosarcoma


Where are fibrosarcomas most often found?

In the deep soft tissues of the extremities - very aggressive


A fibrosarcoma will display what sort of pattern under staining?

a herringbone pattern


What is a fibrohistiocytic tumor?

WHat are the malignant types? the benign type?

it's a tumor that contains cellualr elements resembling both fibroblasts and histiocytes

benign: fibrous histiocytoma (dermatofibroma)

malignant: dermatofibrosarcoma protuberans and malignant fibrous histiocytoma


What is the benign version of a tumor with skeletal muscle differentiation?


actually very rare - occurs in the heart or in other sites

most occur in the heart of children with tuberous slcerosis


What is the most common soft tissue sarcoma of childhood?



What are the three types of rhabdomyosarcoma?

1. embryonal rhabdomyosarcoma (most common - usually in nasal, orbit, ear, and GU tract - subtype: botryoid)

2. alveolar rhabdomyosarcoma (deep tissue of extremities - you're better off with the PAX7 fusion gene than with the PAX3 fusion gene)

3. Plemorphic (rareest one...worst progrnosis)


What is a benign tumor of smooth muscle differentiation called? Where do the vast majority occur?

A leiomyoma

vast majoriy are in the uterus - called uterine fibroids

can also be in the arrector pili muscles, and some in the GI tract


WHat is a malignant tumor of smooth muscle differentiation called? Where do they occur most often?


most often in uteris or GI tract, retroperitoneum, and skin


What feature will both leiomyomas and leiomyosarcomas have on histology?

spindle cells arranged in fascicles intersecting at right angles

the leiomyosarcoma will have more nuclear pleomorphism and icnreased mitottic activity


What are the 6 main types of benign tumors with blood vessel/lymphatics differentiation?

1. hemangioma

2. pyogenic granuloma

3. lymphangioma (look like hemangiomas without the RBCs)

4. glomangioma

5. vascular ectasias (nevus flameus, spider angiomas, hereditary hermorrhagic telengiectasia)

6. Bacillary angiomatosis


A cavernous lymphngioma (cystic hygroma) in the neck of children is often associated with what syndrome?

Turner Syndrome


What blood vessel tumor-like lesion is associated with Osler-Weber-Rendu Disease?

Hereditary hemorrhagic telengiestasia


What causes a bacillary angiomatosis?

opportunistic infection from bartonella bacteria in immunoscompromise individuals


What are the three types of malignant tumors with blood vessel/lymphatic differentiation?

1. angiosarcomas (with endothelial differentiation - scalp, neck, breast, liver)

2. Hemangioendotheliomas (less aggressive than angiosarcomas)

3. Kaposi's sarcoma


Who often develops angiosarcomas in the axillary area?

women who have long-standing lymphedema following axillary lymph node resection for breast cancer

also those who have radiation therapy


What virus results in kaposi's sarcoma?

human herpesvirus 8


What are the four types of kaposi's sarcoma?

1. chronic KS (classic or European) - in ashkenazi jews or those of mediterranean descent

2. Lymphadenopathic KS (African or endemic) - affects young males and kids with involvement o flymphs nodes

3. Transplant-associated KS

4. AIDs associated KS


What are the two non-neoplastic peripheral nerve tumor-like lesions?

morton neuroma (repeated trauma of the interdigital plantar nerve between 3rd and 4th toes

traumatic neuroma (response to peripheral nerve tyring to regenerate and not reconnecting)


What are the two benign peripherla nerve sheath tumors we talked about?

What were the 2 malignant ones?

benign: Schwannoma, Solitary neurofibroma

malignant: plexiform neurofibroma, malignant peripheral nerve sheath tumor


What will you see in type one neurofroma (the more common)?

it's a mutation on chromosome 17 coding for neurofibromin

1. cafe au latte macules

2. optic gliomas

3. lisch nodules

4. Osseous lytic lesions

5. plexiform neurofibromas (this will never occur in NF2)

6. many cutaneous/subcutaneous neurofibromas

7. neural developmental problems


What is neurofibroma type 2 associated with?

bilateral acoustic schwannomas


spinal schwannomas

juvenile cataracts.


What is the benign tumor of sunovial original called? In the hands? In the kneed?

A tenosynovial giant cell tumor

In hands = giant cell tumor of tendon sheath

in kneed = villonodular synovitis


What is a malignant tumor of "synovial origina"


A synovial sarcoma

actually doesn't show any synovial cell differentiation, and desn't even occur in joint cavities - primarily located in paraarticular regions of extrmities


What characterizes a myxomatous lesion?

a soft tissue lesion with myxoid change - extracellular mucin accumultation with associated cellular proliferation

a ganglion cyst is an example

also digital mucous cysts