Alveolar soft part sarcoma: clinicopathological findings in a series of 11 cases

Adrien Daigeler¹ , Cornelius Kuhnen² , Joerg Hauser¹ , Ole Goertz¹ , Daniel Tilkorn¹ , Lars Steinstraesser¹ , Hans-Ulrich Steinau¹ and Marcus Lehnhardt¹

¹Department of Plastic Surgery, Burn Center, Hand surgery, Sarcoma Reference Center, BG-University Hospital Bergmannsheil, Ruhr University Bochum, Buerkle-de-la-Camp-Platz 1, 44789 Bochum, Germany

²Institute for Pathology, BG-University Hospital Bergmannsheil, Ruhr-University Bochum, Bürkle-de-la-Camp-Platz 1, 44789 Bochum, Germany

author email corresponding author email

World Journal of Surgical Oncology 2008, 6:71doi:10.1186/1477-7819-6-71


Published:	1 July 2008

Abstract

Background

Alveolar sarcoma of the soft parts (ASPS) represents a very rare entity of soft tissue sarcoma with special features such as young peak age incidence and frequent metastasis to the brain. The aim of this study was a clinicopathological analysis with special reference to treatment and outcome.

Methods

From the database of the BG-University Hospital Bergmannsheil, 1597 soft tissue sarcoma (STS) cases were reviewed and 11 consecutive patients with ASPS were isolated. Data was acquired from patients' charts and contact to patients, their relatives or general practitioners, with special reference to treatment and clinical course. The average follow up time from the time of the definite operation for the primary tumor was 6.5 years. Kaplan-Meier method was used to calculate survival.

Results

Patients with localized disease who received complete resection and adjuvant radiation and who did not develop recurrence or metastatic disease within 2 years after surgery had a positive outcome. The size of the tumor, its localization, and the time of untreated growth before treatment did not influence the long-term results. All patients who developed recurrent disease also suffered from distant metastasis, reflecting the aggressive biology of the tumor. All patients with distant metastasis had the lungs and the brain affected.

Conclusion

Due to the limited number of patients with ASPS, prospective studies would have to span decades to gather a significant collective of patients; therefore, it is not possible to comment meaningfully on a possible benefit of neoadjuvant or adjuvant therapy.

We recommend wide surgical excision and, in the absence of data telling otherwise, adjuvant radiation. In cases with recurrent disease or metastasis, the prognosis is bad and further treatment will be restricted to palliation in most cases.