Abstract
Introduction: Alveolar soft part sarcoma (ASPS) is a soft tissue tumour found primarily in adolescents and young adults. It has an incidence of 0.5 – 1.0 % of all soft tissue sarcomas and a predilection for females. The lesion is inherently malignant and may occur throughout the body however, reports suggest they are predominantly detected in the anterior thigh. Symptoms include a painless slow-growing mass but ASPS can commonly present with brain or lung metastases due to the high vascularity of the lesion. Microscopically, cellular groups exist with centralised areas of necrosis, thus resulting in a pseudoalveolar appearance.
Methods: We identified six cases of histologically proven ASPS. The mean age was 28.5 years (21–36). Four patients were male. All patients had a primary presentation of a mass, two of which were painful. All patients, except one, presented within 8 months of the onset of symptoms (mean 3.8 months) with the other seeking medical advice only after 72 months. The location of the mass included the thigh in three patients and the triceps, soleus and retroperitoneal space in the others. Four patients underwent pre-operative diagnostic biopsies. Surgical excision was performed in all patients.
Results: The mean size of tumour was 9.4 x 9.4 x 6.3cm but there was no correlation between lesion size and duration of symptoms. The patient who presented late was found to have multiple lung metastases prior to surgery. Despite appropriate adjuvant therapy, three patients developed post-operative metastases at a mean of 5.2 months (1–24), two of which died along with the patient who presented with metastases at a mean of 2.6 years.
Discussion: This series demonstrates that this tumour may present as a painful mass. Despite early detection and appropriate treatment, ASPS remains a highly malignant neoplasm with a high associated mortality rate.
Correspondence should be addressed to BOOS at the Royal College of Surgeons, 35–43 Lincoln’s Inn Fields, London WC2A 3PE, England.
