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?
2. pleomorphic/spindle cell lipoma
5. chondroid lipoma
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)
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?
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?
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?
2. pyogenic granuloma
3. lymphangioma (look like hemangiomas without the RBCs)
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?
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
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