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MSS > Soft Tissue Tumors > Flashcards

Soft Tissue Tumors Flashcards

1
Q

How are mesenchymal tumors classified?

A

according to the tissue they recapitulate.

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2
Q

How common are sarcomas? (percentage of all cancers?)

What is the ratio of benign vs malignant soft tissue tumors?

A

Only 1% of all cancers

100:1

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3
Q

What are two of the most common soft tissue tumors?

A

lipoma; hemangioma

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4
Q

-What is the cause of most soft tissue tumors? What are some things that are associated with soft tissue tumors

A

mostly are unknown and sporadic. Associations are made with irradiation, trauma, chemical burns, and thermal burns on rare occasions

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5
Q

What are some genetic associations with soft tissue tumors?

A
  • neurofibromatosis type 1 (neurofibroma, malignant schwannoma)
  • Li-Fraumeni syndrome (soft tissue sarcomas)
  • Gardner syndrome (fibromatosis)
  • Osler-Weber-Rendu syndrome (telangiectasia)
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6
Q

most proportion of sarcomas occur where? (what percent)

  • How many percent of sarcomas are within children?
  • What’s the correlation of sarcomas with increase in age?
A

most sarcomas are in the thigh (40%)
15% of sarcomas are in children
and sarcomas increase in incidence with age.

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7
Q

What soft tissue sarcomas are in childhood?
What soft tissue sarcomas are within young adulthood?
What soft tissue sarcomas are within late adulthood?

A

rhabdomyosarcoma in children
synovial sarcomas within young adults
pleomorphic sarcomas and liposarcomas within elderly adults

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8
Q

What are important histological things for diagnosis of sarcomas?
What else can be helpful if these cannot determine the sarcoma subtype?

A

tissue architecture and cell morphology

  • ancillary tests can help:
    • immunohisto, electron microscopy, cytogenetics, molecular genetics…etc
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9
Q

What is “grade” of a tumor?

  • How is it classified?
  • what are 3 characteristic observations based on grade?
  • what does it predict in soft tissue sarcomas?
A

grade is the degree of differentiation, number of mitoses, and amount of necrosis upon microscopic examination.

  • it’s classified as grade 1-3
  • it predicts soft tissue sarcoma’s behavior
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10
Q

What are factors contributing to prognosis?

A

size, depth, and stage of the tumor

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11
Q

generally are prognoses better for superficial or deep tumors?

A

superficial

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12
Q

How are soft tissue sarcomas treated?

A

usually limb sparing wide excision (surgical).

systemic therapy/irradiation are usually only reserved for high grade or large tumors

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13
Q

what are some tissue sources for soft tissue tumors? (6)

A

adipose, nerves, muscles, vessels, fibrous tissue, other (myositis ossificans, angiofibroma, synovial, alveolar soft part sarcoma, epithelioid sarcoma…Etc)

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14
Q

what is the most common soft tissue tumor of adulthood?

A

Lipoma (benign)

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15
Q

Lipoma

  • how many are there usually? what if otherwise?
  • clinical presentation? (3)
  • treatment?
  • prognosis?
A

usually only 1. If more than 1–likely associated with rare genetic inheritance factors.

  • slow growing mass, painless, mobile
  • surgical excision.
  • surgical excision are usually curative.
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16
Q

Lipomas–what subtype is often associated with local pain?

A

angiolipoma–>local pain

17
Q 
How are lipoma subtypes classified?
What is the most common type? 
-what do they look like grossly? (3)
-what do they look like histologically?
-genetic associations?
A

subtypes are according to morphologic findings (spindle cell lipoma or angiolipoma…etc)

  • conventional lipoma
  • grossly: soft, yellow, well encapsulated masses
  • well differentiated adipose cells (mature white fat cells with no pleomorphism)
  • genetic association with 12q14 and 12q15. However not reliable for testing
18
Q

Liposarcoma

  • age range
  • are they common?
  • where do these occur on the body? (3)
  • histologic features of indolent (2), intermediate (5), and agressive liposarcomas (2)
  • What is a histological indication of liposarcoma vs. lipoma?
  • what is a concern with surgical treatment?
A
  • 40-60 year olds
  • yes they are one of the most common soft tissue sarcomas.
  • they occur in the proximal extremities and retroperitoneally.
  • Indolent: well differentiated liposarcoma (WD-LPS) with supernumerary ring nuclei.
  • Intermediate: myxoid/round cells with foamy edges. with t(12;16)(q13;p11). you can see pink stroma and fat cells and vessels
  • aggressive liposarcoma: pleomorphic
  • presence of lipoblast is an indication of liposarcoma!!! (smaller, bubbled cells with a central nucleus next to legit fat cells)
  • all stages of liposarcoma have high occurence rate unless well excised.
19
Q

Pseudosarcomatous proliferations

  • name 2
  • How do they develop oftenly? (2)
  • what makes them similar looking to sarcomas?
  • what is different about these?
A
  • Nodular fasciitis and myositis ossificans
  • they develop to local trauma or idiologically
  • hypercellularity, increased mitoses, primitive appearance (not well differentiated)
  • they appear suddenly and grow rapidly
20
Q

nodular fasciitis

  • How large is it?
  • where is it on the body?
  • what does it feel like?
  • what can it be mistaken for?
  • How is it different?
A
  • usually 2 cm ish
  • deep dermis, subQ, or in muscle
  • it feels very hard with ill defined margins
  • It can be mistaken for sarcoma… but it arises quickly and is rapid growing
21
Q

Myositis Ossificans

  • what is this?
  • how does this occur?
  • where on the body is it usually?
  • what age group?
  • becareful to distinguish this from what?
A
  • metastatic bone in soft tissue and is eventually filled with marrow
  • mostly post trauma
  • common in proximal extremities
  • young people
  • make sure to distinguish from extraskeletal osteosarcoma
22
Q

Fibromatoses

-locations (general)

A

superficial vs deep (desmoid tumors)

23
Q

Superficial Fibromatoses

  • Locations (3) (2 with special disease names)
  • prognosis/treatment?
A
  • palms (Dupuytren contractures), soles, and penis (Peyronie disease)
  • May resolve spontaenously, may recur
24
Q

Deep Fibromatosis (desmoid tumors))

  • behavior?
  • age group?
  • disease association? genetic association?
  • treatment/prognosis?
A
  • inbetween benign and lowgrade fibrosarcoma
  • teenagers to 30 year olds
  • Gardner’s disease; mutations in APC and beta catenin
  • frequent recurrence
  • surgical removal
25
Q

Fibrosarcoma

  • cell origin?
  • age group
  • locations (3)
  • prognosis in percentage of recurrence and metastasis?
  • molecular markers
A
  • fibroblast cells
  • mostly adults
  • retroperitoneal, thigh, knee,
  • aggressive tumors recur 50% of the time and metastisize 25% of the time
  • all markers are negative except for Vimentin
26
Q

Leiomyomas

  • what are they
  • how common?
  • locations
  • size?
  • treatment?
A
  • smooth muscle tumors (beign)
  • most common neoplasms in women
  • uterine, skin, deep soft tissue
  • LESS than 1-2 cm
  • easily cured if solitary
  • can be difficult to remove if there are many
27
Q

Leiomyosarcoma

  • what proportion of soft tissue sarcomas?
  • age/sex?
  • location
  • prognosis for different locations?
  • staining
A
  • 15-20% of soft tissue sarcomas
  • adults; females more than males
  • skin and deep tissue of extremities and retroperitoneum
  • if superficial good prognosis… deep lesions are hard to excise
  • SMA, desmin
28
Q

Rhabdomyosarcoma

  • age group
  • location
  • subtypes (percentages?)
A
  • most common soft tissue sarcoma in children. usually occurs in children under 20
  • Head, neck GU tract (usually where there’s little skeletal tissues)
  • Embryonic (49%) , alveolar (31%), pleomorphic
29
Q

Embryonal rhabdomyosarcoma

  • what percentage of rhabdomyosarcoma?
  • what age group
  • location (6)
  • variants (3)
  • histological finding (2)
  • staining? (2)
A
  • 49%
  • less than 10 years old
  • head/neck: periorbital and parameningeal
  • GU tract
  • deep soft tissue of extremities, pelvis, and retroperitoneum
  • sarcoma botryoides, spindle cell, anaplastic
  • small blue round cells, with occasional rhabdomyoblasts (spindle like)
  • stain with desmin (blue) and myogenin (brown)
30
Q

Alveolar Rhabdomyosarcoma

  • percentage of rhabdomyosarcoma?
  • age group?
  • location
  • histological findings
  • how to confirm diagnosis?
A
  • 39%
  • 10-25 y/o
  • mostly in soft tissue of extremities; but sometimes in head/neck, GU, retroperitoneum, perineum
  • also small round blue cells, but over all tissue architecture has “alveolar appearance”
  • translocation: t (2/13)/PAX3-FKHR or t(1/13)/PAX7/FKHR are present 8–85% of the time.
31
Q

name 3 soft tissue sarcomas of unknown histogenesis

A
  • synovial sarcoma
  • epithelioid sarcoma
  • alveolar soft part sarcoma
32
Q

Synovial Sarcoma

  • cells of origin?
  • what percent of soft tissue sarcomas?
  • what percent of soft tissue of extremities?
  • Age group/sex?
  • Location
  • genetic characteristic
  • histological characteristic (2)
  • radiographic appearance
  • treatment
  • sites of mets
  • 5 year survival rate; 10 year survival rate
A
  • Cells do NOT arise from synovium
  • 5-10% of soft tissue sarcomas
  • 80% of soft tissue sarcomas of the extremities
  • young adults; male
  • almost always around the knee… therefore “synovium” although not actual synovial
  • translocation of x and 18(p11; q11)
  • biphasic (glandular and spindle cells); monophasic (only spindle cells
  • scattered poorly definedcalcification in the soft tissue of the knee region
  • lungs, bones, and regional lymph nodes
  • treatment via limb sparing surgery and aggressive chemotherapy
  • 5 year survival rate 25%-65%; 10 year survival rate 10-30%

MSS (7 decks)

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