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
- Regional Lymph nodes mets common
- can systematic met to lungs
- extremely poor
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
What is seen on imaging epitheloid sarcoma?
- Xray
- calcification within lesions 10-20%
- MRI
- indeterminate in appearance
- T1 dark, T2 bright
- calcification within lesions 10-20%
- indeterminate in appearance
- T1 dark, T2 bright
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
What tumours have the propensity to spread to local lymph nodes?
- epitheloid sarcoma
- Rhabdomyosarcoma
- Clear cell sarcoma
- synovial sarcoma
- Vascular sarcoma
What is the histology of epitheloid sarcoma?
- epitheial appearance, nodular pattern w central necrosis
-
immuomarkers for Keratin, vimetin , CD34
immuomarkers for Keratin, vimetin , CD34
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
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
Name any associated conditions with intramuscular myxomas?
-
Mazabraud's syndrome- multiple intramuscular myxomas assoc with monostotic/polyostotic fibrous dysplasia
- Myxoid liposarcomas
what is the presentation of intramuscular myxomas?
- Slow growing mass
- may/maynot be painful
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
intramuscular location of myxomas is important to differentiate from myxoid liposarcoma, which occurs in an intermuscular location
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