Other soft tissue tumours

Question 1

What is epitheloid sarcoma?

Answer 1

• 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

Question 2

What are the symptoms of epitheloid sarcoma?

Answer 2

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

0/E

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

Question 3

What is seen on imaging epitheloid sarcoma?

Answer 3

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

Question 4

What is the tx of epitheloid sarcoma?

Answer 4

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

Question 5

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

Answer 5

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

Question 6

What is the histology of epitheloid sarcoma?

Answer 6

• epitheial appearance, nodular pattern w central necrosis
• immuomarkers for Keratin, vimetin , CD34

Question 7

What is intramuscular myxoma?

Answer 7

• 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

Question 8

Name any associated conditions with intramuscular myxomas?

Answer 8

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

Question 9

what is the presentation of intramuscular myxomas?

Answer 9

• Slow growing mass
• may/maynot be painful

Question 10

What is seen on imaging intramuscular myxomas?

Answer 10

• Homogenous appearance
• Bright T2, dark T1
• intramuscular location of myxomas is important to differentiate from myxoid liposarcoma, which occurs in an intermuscular location

Question 11

what is the tx of intramuscular myxomas?

Answer 11

Non operative

• Observation
  
  • asymptomatic lesions

Surgery

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