Unusual primary breast cancer - malignant peripheral nerve sheath tumor: a case report and review of the literature.

Background: Sarcomas are a rare type of breast malignancies and malignant peripheral nerve sheath tumors of the breast are even rarer. There are no specific clinical and radiological features for the diagnosis of this tumor and histological features are also reported to be nonspecific. Therefore, immunohistochemistry is required for its diagnosis. A definitive treatment protocol is unavailable because of its rarity.

Case Presentation: We report a case of a sporadic form of breast malignant peripheral nerve sheath tumor found in a 16-year-old Asian Bangladeshi girl. She experienced local recurrence and she had multiple left breast lumps four times in a very short period after repeated surgeries. However, she was later managed successfully with chemotherapy and locoregional radiotherapy. A chemotherapy protocol with ifosfamide, vincristine, and actinomycin was used and radiotherapy was given with a total dose of 50 Gy given in 25 fractions of 2 Gy by a 6 MV photon linear accelerator followed by 10 Gy boost given in 5 fractions of 2 Gy by 9 MeV electron energy. With more than 3 years of periodic follow-up, she is still well without any locoregional and metastatic recurrence.

Conclusions: This report suggests proper immunohistochemical analysis whenever a breast sarcoma is found in order to find a rare histological variety. We believe that malignant peripheral nerve sheath tumor of the breast can be managed by total mastectomy followed by adjuvant chemotherapy and radiotherapy. Long-term meticulous follow-up is required to develop an optimum therapeutic strategy.