Objective: Elucidation of the natural history and optimal management of neurogenic sarcomas is not straightforward, because of diagnostic difficulties and the low incidence of this disease. The majority of neurogenic sarcomas are categorized and treated as soft tissue sarcomas, and only a few centers have sufficient experience to add to our understanding of these malignant tumors. This article proposes an algorithm for the management of these tumors.
Methods: A cohort of 18 cases of neurogenic sarcoma (overall mean follow-up period after oncological diagnosis, 33.7 mo), representing approximately 3% of all soft tissue sarcomas treated at the University of Toronto Musculoskeletal Oncology Unit (Toronto, Canada) between 1989 and 1995, were reviewed. The clinical and pathological criteria used to establish the diagnosis of neurogenic sarcoma, the presenting symptoms, the tumor size and grade, the ability to obtain tumor-free margins during en bloc resection, the time to recurrence, and the overall survival rates were noted. A centralized pathological review of the biopsy was undertaken in all cases.
Results: A metastatic survey demonstrated localized disease for 16 of the 18 patients. The overall management strategy for these 16 neurogenic sarcomas with localized disease was to obtain local control by en bloc resection. Tumor size, tumor grade, and, most importantly, the ability to obtain tumor-free margins were all relevant prognostic factors for survival. Tumor-free surgical margins were obtained for 11 of 16 patients, with 9 of these 11 patients remaining disease-free. In contrast, all patients for whom tumor-free margins could not ultimately be achieved died as a result of their disease.
Conclusion: Based on our experience, we propose an algorithm for the management of these rare tumors, for both optimal patient care and oncological research.