Soft Tissue Tumors Flashcards
How are mesenchymal tumors classified?
according to the tissue they recapitulate.
How common are sarcomas? (percentage of all cancers?)
What is the ratio of benign vs malignant soft tissue tumors?
Only 1% of all cancers
100:1
What are two of the most common soft tissue tumors?
lipoma; hemangioma
-What is the cause of most soft tissue tumors? What are some things that are associated with soft tissue tumors
mostly are unknown and sporadic. Associations are made with irradiation, trauma, chemical burns, and thermal burns on rare occasions
What are some genetic associations with soft tissue tumors?
- neurofibromatosis type 1 (neurofibroma, malignant schwannoma)
- Li-Fraumeni syndrome (soft tissue sarcomas)
- Gardner syndrome (fibromatosis)
- Osler-Weber-Rendu syndrome (telangiectasia)
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?
most sarcomas are in the thigh (40%)
15% of sarcomas are in children
and sarcomas increase in incidence with age.
What soft tissue sarcomas are in childhood?
What soft tissue sarcomas are within young adulthood?
What soft tissue sarcomas are within late adulthood?
rhabdomyosarcoma in children
synovial sarcomas within young adults
pleomorphic sarcomas and liposarcomas within elderly adults
What are important histological things for diagnosis of sarcomas?
What else can be helpful if these cannot determine the sarcoma subtype?
tissue architecture and cell morphology
- ancillary tests can help:
- immunohisto, electron microscopy, cytogenetics, molecular genetics…etc
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?
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
What are factors contributing to prognosis?
size, depth, and stage of the tumor
generally are prognoses better for superficial or deep tumors?
superficial
How are soft tissue sarcomas treated?
usually limb sparing wide excision (surgical).
systemic therapy/irradiation are usually only reserved for high grade or large tumors
what are some tissue sources for soft tissue tumors? (6)
adipose, nerves, muscles, vessels, fibrous tissue, other (myositis ossificans, angiofibroma, synovial, alveolar soft part sarcoma, epithelioid sarcoma…Etc)
what is the most common soft tissue tumor of adulthood?
Lipoma (benign)
Lipoma
- how many are there usually? what if otherwise?
- clinical presentation? (3)
- treatment?
- prognosis?
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.
Lipomas–what subtype is often associated with local pain?
angiolipoma–>local pain
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?
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
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?
- 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.
Pseudosarcomatous proliferations
- name 2
- How do they develop oftenly? (2)
- what makes them similar looking to sarcomas?
- what is different about these?
- 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
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?
- 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
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?
- 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
Fibromatoses
-locations (general)
superficial vs deep (desmoid tumors)
Superficial Fibromatoses
- Locations (3) (2 with special disease names)
- prognosis/treatment?
- palms (Dupuytren contractures), soles, and penis (Peyronie disease)
- May resolve spontaenously, may recur
Deep Fibromatosis (desmoid tumors))
- behavior?
- age group?
- disease association? genetic association?
- treatment/prognosis?
- inbetween benign and lowgrade fibrosarcoma
- teenagers to 30 year olds
- Gardner’s disease; mutations in APC and beta catenin
- frequent recurrence
- surgical removal
Fibrosarcoma
- cell origin?
- age group
- locations (3)
- prognosis in percentage of recurrence and metastasis?
- molecular markers
- 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
Leiomyomas
- what are they
- how common?
- locations
- size?
- treatment?
- 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
Leiomyosarcoma
- what proportion of soft tissue sarcomas?
- age/sex?
- location
- prognosis for different locations?
- staining
- 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
Rhabdomyosarcoma
- age group
- location
- subtypes (percentages?)
- 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
Embryonal rhabdomyosarcoma
- what percentage of rhabdomyosarcoma?
- what age group
- location (6)
- variants (3)
- histological finding (2)
- staining? (2)
- 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)
Alveolar Rhabdomyosarcoma
- percentage of rhabdomyosarcoma?
- age group?
- location
- histological findings
- how to confirm diagnosis?
- 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.
name 3 soft tissue sarcomas of unknown histogenesis
- synovial sarcoma
- epithelioid sarcoma
- alveolar soft part sarcoma
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
- 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%
