Soft Tissue Tumors (6) Flashcards
What do neoplasms arise from in soft tissues
Extraskeletal mesodermal tissues
What is the cellular origin of STT
multipotent mesenchymal stem cells
where do malignant soft tissue tumors tend to metastize to
sarcomas tend to metastasize via the bloodstream usually to the lungs
Benign vs malignant tumor vascularity
benign- relatively avascular
malignant- hypervascular
What are desmoid tumors
-locally agressive proliferation of fibroblasts that do not metasize
What is desmoid tumors due to
Inactivating mutations in the APC gene
mostly in familial adenomatous polyposis
tx of desmoid tumors
complete surgical excisions is usually curative
Nodular fasciitis- type, cause
- benign but rapidly growing reactive lesion
- probably results from trauma and commonly affects the superficial tissues of the forearm, trunk, back
What is dupuytrens contracture and what does it cause
- progressive fibrosis of palmar fascia
- benign
-jt stiffness and loss of full ext develop insidiously over decades
end stage dupuytrens contracture
nodules progeress to form longitudinal bands (cords on palmar fascia)
-fingers gradually lose ext and draw fingies into flex
Dupuytrens contracture- age, gnder
most pts over 50
-80% men
Pathology of dupytrens contracture
fibroblastic proliferation and disorderly collagen deposition with palmar fascial thickening
-increased conc of prostaglandins lead to pain
What is plantar fibromatosis
plantar equivalent of dupuytrens (generally less agressive and recurrent tendency)
What are examples of plantar fibromatosis (3)
- Ledderhose disease
- Proteus syndrome
- Jevenile aponeurotic fibroma
What is peyronie disease
Penile fibromatosis
-Aquired, localized fibrotic disorder of the tunica albuginea resulting in penile deformity
What is the mc Soft tissue sarcoma
Malignant fibrous histiocytoma
mc sarcomas in >40
What is a angiolipoma
- histologically indistinguishable from normal tissues
- small, well circumscribed lipoma, often multiple and painful
What is the second most common sarcoma in adults
Liposarcomas- comprise 20% of all malignant soft tissue tumors
Age of liposarcomas and where are the mc found
> 50
Deep thigh/retroperitoneum, may become large
what is a lipoblast
a malignant appearing cell with uniacuolated or multivacuolated cytoplamic fat cells– defines a liposarcoma
local recurrence of liposarcomas?
local recurrence and metastases after surgery are common from round cell and pleomorphic liposarcomas
what is a rhabdomyosarcoma and who is it mc in
Malignant tumor that displays featires of striated mm differentiation
mc soft tissue sarcoma of children + young adults
What are the 4 subtypes of rhabdomyosarcoma
- Embryonal
- Botryoid embryonal
- Alveolar
- Pleomorphic
Embryonal rhabdomyosarcoma- age, where is it located
mc in children between 3-12
loc- head/neck, genitourinary tract, retroperitoneum
what do botryoid embryonal rhabdomyosrcomas look like and where are they located
Polypoid, grape like tumor masses
loc- hollow visceral organs (bladder, vag)
Alveolar rhabdomyosarcoma- ages, loc
10-25 y old
mc in upper and lower extremities (similar to embryonal type)
What is the least common rhabadomyosarcoma
Pleomorphic
tx of rhabadomyosarcomas
80% cured with combined therapeutif modalities, surgery, radiation therapy, chemotherapy
What is a leiomyoma and where does it arise
benign smooth muscle tumor
-arises in subcutaneous tissues or from blood vessel walls
What is a leiomyosarcoma and where is it found
agressive smooth muscle sarcoma
typically of uterine, gastrointestinal or soft tissue origin
leiomyosarcome gender and why
2:1 W:M
gender distribution may reflect the proliferation of smooth mm that can occur in response to estrogen
What is the S/S of leiomyosarcoma and where are most generally found
s/s: abdominal mass, pain, swelling, weight loss etc
mc site- retroperitonium
Synovial sarcoma age + mc site
adolescents, young adults
mc site- around the knee jt (but can be anywhere around axial skeleton)
What is the tripple sign and what does it represent
Synovial sarcoma
-areas of high, intermediate and low signal intensity on T2W images
What predicts a poor outcome in synovial sarcomas
poorly differentiated histologically predicts poor outcomes
Where are lipomas usually found
trunk, neck, prox extremities
