Leiomyosarcoma
Neurosarcoma / Malignant peripheral nerve sheath tumour
Clear cell sarcoma
Fibrosarcoma
Epitheliod sarcoma
Leiomyosarcoma
Definition
Tumour of smooth muscle
5 - 10% of sarcomas
Location
Most common retroperitoneal / intra-abdominal / uterus (65%)
- extremities 19%
- trunk 16%
Clinical
Most common in elderly
Most common peripherally in groin and popliteal fossa
Management
Wide resection + radiotherapy
Prognosis
Kannan et al World J Surg Oncol 2022
- meta-analysis
- metastatic rate 40%
Neurofibrosarcoma / Malignant peripheral nerve sheath tumour
Epidemiology
10% of sarcomas
- 50% seen in neurofibromatosis
- radiation induced
- sporadic
Neurofibromatosis
NF-1
- 10% life risk
- increases with age
Clinical
Enlarging painful mass
Neurological symptoms
- pain / paraesthesia / weakness
Site
- brachial plexus
- lumbosacral plexus
- major nerve trunks - especially the sciatic nerve
MRI
Management
Wide resection + radiotherapy
Prognosis
Aggressive, malignant tumours
Kolberg et al Neuro Oncol 2013
- meta-analysis of 1,800 patients
- overall survival 44%
Clear Cell Sarcoma
Definition
Exceedingly rare
Arise from neural crest cells
- clear cells contain accumulated glycogen
- share characteristics with malignant melanoma
Characteristic translocation - t(12:22)
Characteristics
Arise from tendon sheaths and aponeurosis
Common around foot & ankle (50%)
Young patients 20 - 40 years
Management
Wide resection + radiotherapy
Prognosis
- 5 year survival 47%
- 10 year survival 36%
Fibrosarcoma
Definition
Malignant neoplasm composed of fibroblasts
Produces variable amount of collagen
Epidemiology
Rare - < 1% of sarcomas
MRI
Epithelioid Sarcoma
Characteristics
50% arise forearm / wrist
- most common sarcoma of the hand
Young adult
Odd small sarcoma
- can arise in the dermis
- nodule / ulceration
- often misdiagnosed as benign ganglion