Abstract
Purpose: Many cases of Alveolar Soft Part Sarcoma have been reported on the trunk or head and neck region; we present two cases of the tumour in the soft tissues of the extremities.
Case Series: Alveolar Soft Part Sarcoma is a very rare tumour representing only 0.2–1 percent of soft tissue sarcomas. Patients commonly present with distant metastases both at the time of diagnosis and late in the course of disease. Many of the reported cases involve the trunk or head and neck region, especially around the orbit.
We present cases of Alveolar Soft Part Sarcoma, one in the upper limb the other in the lower limb, in two young females. Both were treated in the same centre by the same consultant.
The first is the case of a 33 year old female with a swelling in the right triceps present for 7 months before presentation to our centre. The time between diagnosis of possible tumour and biopsy was less than a month. Biopsy revealed a trojani grade 2 tumour and there were no metastases prior to wide excision. She received radiotherapy and has no metastases on follow up. Post therapy she is left with neurological pain and a rash on her face after radiotherapy.
The second case is that of a 25 year old female presenting, with a swelling in the right thigh, after 12 months including a 6 month history of shortness of breath. She had a family history of pancreatic cancer. Biopsy revealed a trojani grade 3 tumour and imaging revealed lung metastases. She went on to have chemotherapy. She later developed bone metastases.
Conclusion: Alveolar Soft Part Sarcoma must be suspected in all tumours and there aggressive nature must be realised.
Correspondence should be addressed to BOOS at the Royal College of Surgeons, 35 - 43 Lincoln’s Inn Fields, London WC2A 3PN
