Case report - multimodality treatment of the metastatic ASPS

[Olga]

Alveolar soft – part sarcoma of the extremity: a case report
http://www.pubmedcentral.nih.gov/articl ... id=2580048

An 11– year–old boy with ASPS which presented with a markedly vascular tumor in the left thigh, and multiple bilateral pulmonary metastases 8 months after diagnosis is described. The patient has remained disease–free for over 5 years since the initial diagnosis after he had the following treatment:
1. Initial resection of the primary tumor, no metastases found.
1. The soft tissue sarcoma chemotherapy protocol of MMT–89 (SIOP).
2. 6 bilateral lung metastases found 8 month after the Dx.
3. Complete resection of all visible lung metastases (viable tumor tissue, chemoresistant).
4. Post-surgery 15Gy of bilateral pulmonary irradiation.
5. Maintenance chemotherapy regimen of the German Soft Tissue Tumour Trial 2002 with: daily oral cyclophosphamide (2 x 25 mg/ m2) in combination with weekly vinblastine (3 mg/m2) iv on days 1, 8 and 15 to be repeated starting on day 28 for a year.

The patient remains disease–free and clinically well with full activity over 5 years since the initial diagnosis. Now he is in a follow up every 6 months. The movement of the left arm, the heart and renal function are normal and our patient has an excellent quality of life.

My comment:
there are known cases of the long term disease-free survival with the surgery only when the metastatic disease is limited so it is hard to draw any conclusions from this case, although regimen with the weekly vinca alkaloid component has already noted to have an activity in our ASPS patient Amanda Minderlein (Celebrex+vincristine).