Soft Tissue Tumors (6) Flashcards

1
Q

What do neoplasms arise from in soft tissues

A

Extraskeletal mesodermal tissues

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2
Q

What is the cellular origin of STT

A

multipotent mesenchymal stem cells

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3
Q

where do malignant soft tissue tumors tend to metastize to

A

sarcomas tend to metastasize via the bloodstream usually to the lungs

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4
Q

Benign vs malignant tumor vascularity

A

benign- relatively avascular

malignant- hypervascular

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5
Q

What are desmoid tumors

A

-locally agressive proliferation of fibroblasts that do not metasize

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6
Q

What is desmoid tumors due to

A

Inactivating mutations in the APC gene

mostly in familial adenomatous polyposis

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7
Q

tx of desmoid tumors

A

complete surgical excisions is usually curative

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8
Q

Nodular fasciitis- type, cause

A
  • benign but rapidly growing reactive lesion

- probably results from trauma and commonly affects the superficial tissues of the forearm, trunk, back

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9
Q

What is dupuytrens contracture and what does it cause

A
  • progressive fibrosis of palmar fascia
  • benign

-jt stiffness and loss of full ext develop insidiously over decades

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10
Q

end stage dupuytrens contracture

A

nodules progeress to form longitudinal bands (cords on palmar fascia)

-fingers gradually lose ext and draw fingies into flex

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11
Q

Dupuytrens contracture- age, gnder

A

most pts over 50

-80% men

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12
Q

Pathology of dupytrens contracture

A

fibroblastic proliferation and disorderly collagen deposition with palmar fascial thickening

-increased conc of prostaglandins lead to pain

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13
Q

What is plantar fibromatosis

A

plantar equivalent of dupuytrens (generally less agressive and recurrent tendency)

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14
Q

What are examples of plantar fibromatosis (3)

A
  • Ledderhose disease
  • Proteus syndrome
  • Jevenile aponeurotic fibroma
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15
Q

What is peyronie disease

A

Penile fibromatosis

-Aquired, localized fibrotic disorder of the tunica albuginea resulting in penile deformity

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16
Q

What is the mc Soft tissue sarcoma

A

Malignant fibrous histiocytoma

mc sarcomas in >40

17
Q

What is a angiolipoma

A
  • histologically indistinguishable from normal tissues

- small, well circumscribed lipoma, often multiple and painful

18
Q

What is the second most common sarcoma in adults

A

Liposarcomas- comprise 20% of all malignant soft tissue tumors

19
Q

Age of liposarcomas and where are the mc found

A

> 50

Deep thigh/retroperitoneum, may become large

20
Q

what is a lipoblast

A

a malignant appearing cell with uniacuolated or multivacuolated cytoplamic fat cells– defines a liposarcoma

21
Q

local recurrence of liposarcomas?

A

local recurrence and metastases after surgery are common from round cell and pleomorphic liposarcomas

22
Q

what is a rhabdomyosarcoma and who is it mc in

A

Malignant tumor that displays featires of striated mm differentiation

mc soft tissue sarcoma of children + young adults

23
Q

What are the 4 subtypes of rhabdomyosarcoma

A
  1. Embryonal
  2. Botryoid embryonal
  3. Alveolar
  4. Pleomorphic
24
Q

Embryonal rhabdomyosarcoma- age, where is it located

A

mc in children between 3-12

loc- head/neck, genitourinary tract, retroperitoneum

25
what do botryoid embryonal rhabdomyosrcomas look like and where are they located
Polypoid, grape like tumor masses loc- hollow visceral organs (bladder, vag)
26
Alveolar rhabdomyosarcoma- ages, loc
10-25 y old | mc in upper and lower extremities (similar to embryonal type)
27
What is the least common rhabadomyosarcoma
Pleomorphic
28
tx of rhabadomyosarcomas
80% cured with combined therapeutif modalities, surgery, radiation therapy, chemotherapy
29
What is a leiomyoma and where does it arise
benign smooth muscle tumor -arises in subcutaneous tissues or from blood vessel walls
30
What is a leiomyosarcoma and where is it found
agressive smooth muscle sarcoma typically of uterine, gastrointestinal or soft tissue origin
31
leiomyosarcome gender and why
2:1 W:M gender distribution may reflect the proliferation of smooth mm that can occur in response to estrogen
32
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
33
Synovial sarcoma age + mc site
adolescents, young adults mc site- around the knee jt (but can be anywhere around axial skeleton)
34
What is the tripple sign and what does it represent
Synovial sarcoma -areas of high, intermediate and low signal intensity on T2W images
35
What predicts a poor outcome in synovial sarcomas
poorly differentiated histologically predicts poor outcomes
36
Where are lipomas usually found
trunk, neck, prox extremities