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Flashcards in Other soft tissue tumours Deck (11):
1

What is epitheloid sarcoma?

  • A rare Malignant slow growing nodular soft tissue tumour
  • age 10-35 yrs
  • M>F 2:1
  • **Most common soft tissue sarcoma of Hand/wrist**
  • also in forearm, buttock/thigh, knee , foot
  • mass is deep or superificial
  • Genetics- unknown
  • Mets
    • Regional Lymph nodes mets common
    • can systematic met to lungs
  • Prognosis
    • extremely poor

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2

What are the symptoms of epitheloid sarcoma?

  • Small, firm, painless, slow growing mass
  • often in upper extremity

0/E

  • 3-6cm firm , painless mass
  • amy have skin ulceration

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3

What is seen on imaging epitheloid sarcoma?

  • Xray
    • calcification within lesions 10-20%
  • MRI
    • indeterminate in appearance
    • T1 dark, T2 bright

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4

What is the tx of epitheloid sarcoma?

OPerative

  • Wide excision with adjuvant Radiotherapy
    • all operative tumours
    • prefrom sentinel node biopy to evaluate for lymph note mets
    • high rate of multiple recurrences if mistaken for a benign lesion and inadequate excision
  • Amputation
    • maybe necessary to prevent dpread of disease in cases of multiple recurrences

5

What tumours have the propensity to spread to local lymph nodes?

  • epitheloid sarcoma
  • Rhabdomyosarcoma
  • Clear cell sarcoma
  • synovial sarcoma
  • Vascular sarcoma

6

What is the histology of epitheloid sarcoma?

  • epitheial appearance, nodular pattern w central necrosis



  • immuomarkers for Keratin, vimetin , CD34
     

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7

What is intramuscular myxoma?

  • A benign soft tissue tumour that presents as a slow growing deeply seated mass confined within skeletal tissue
  • likely develop from premature mesenchymal stem cells which differentiate into benign fibroblasts which loose their capacity to secrete collagen

  • age 40-60yrs

  • >F

  • location

    • thigh, shoulder, buttocks, upper arms

  • Prognosis

    • local recurrence and mets uncommon

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8

Name any associated conditions with intramuscular myxomas?

  • Mazabraud's syndrome- multiple intramuscular myxomas assoc with monostotic/polyostotic fibrous dysplasia
  • Myxoid liposarcomas

9

what is the presentation of intramuscular myxomas?

  • Slow growing mass
  • may/maynot be painful

10

What is seen on imaging intramuscular myxomas?

  • Homogenous appearance
  • Bright T2, dark T1

  • intramuscular location of myxomas is important to differentiate from myxoid liposarcoma, which occurs in an intermuscular location
     

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11

what is the tx of intramuscular myxomas?

Non operative

  • Observation
    • asymptomatic lesions

Surgery

  • marginal surgical resection
    • symptomatic low grade
    • neoadjuvant not required as low risk of recurrence/mets