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Connective Tissue Oncology Society |
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| 2001 CTOS Annual Meeting Posters— Medical Oncology ALVEOLAR SOFT PART
SARCOMA IN ADULTS: ANALYSIS OF CLINICAL FEATURES AND TREATMENT OUTCOME
IN 19 CASES. OBJECTIVE: Alveolar soft part sarcoma (ASPS) is a rare tumour, accounting for approximately 1% of malignant soft tissue tumours. Most centres see very few cases and the literature pertaining to adults is sparse. The purpose of this study was to review the experience of our institution in treating adults presenting with ASPS and to assess strategies for management of metastatic disease, including systemic therapy and surgery. METHODS: The case records of 19 consecutive patients presenting with alveolar soft part sarcoma aged 15 years and over between 1984 and 2000 were reviewed retrospectively. Data were evaluated with respect to patient characteristics, location of primary tumour, frequency and site of metastases, recurrence, treatment outcome and death. Survival was defined as the interval between diagnosis and death or last follow-up visit. PFS was defined as the time from diagnosis to relapse, death, disease progression, or most recent follow up visit. The last follow up visit in the group was November 16, 2000 and the median follow up duration of surviving patients is 68.1 months (range, 21.8 – 222.6). RESULTS: The 12 male and 7 female patients had a median age of 28 years (range 15 -51). 68% (13/19) of patients presented with extremity tumours. Local recurrence was rare (1 patient) but 74% of the patients (14/19) had developed metastatic disease. 47% (9/19) had pulmonary metastatic disease at presentation with 26% (5/19) developing metastatic disease more than 3 years after diagnosis. At last follow up, 69% (13/19) were alive, 32% (6/19) were disease free and 37% (7/19) were alive with metastatic disease. No objective responses to chemotherapy were observed in the 47% (9/19) of patients who received chemotherapy for metastatic disease but of the 9 who underwent pulmonary metastasectomy, 7 achieved complete clearance, with only 2 of these recurring in the thorax after surgery. 4 patients of the 9 (44%) remain disease free at last follow up. CONCLUSION: ASPS is a rare disease entity in adults, characterised by a high metastatic rate but indolent disease course. There is currently no effective systemic treatment but resection of pulmonary metastases may be curative and should be pursued aggressively even if repeated metastasectomies are required.
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