Abstract
BackgroundSarcomas 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 presentationWe 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.ConclusionsThis 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.
Highlights
Sarcomas are a rare type of breast malignancies and malignant peripheral nerve sheath tumors of the breast are even rarer
We believe that malignant peripheral nerve sheath tumor of the breast can be managed by total mastectomy followed by adjuvant chemotherapy and radiotherapy
Its diagnosis can only be made in the presence of one of the following features: (1) the tumor arises from a peripheral nerve; (2) the tumor arises from a preexisting benign or other Malignant peripheral nerve sheath tumor (MPNST); (3) the tumor shows histological features of Schwann or perineurial cell differentiation [23, 24]
Summary
The unusual primary MPNST of the breast is often unsuspected and the diagnosis may be missed unless clinicians are aware of its signs and symptoms and confounding histopathology. Suspicion of MPNST should be raised in patients with stigmata of NF1 with a breast lump. Special attention should be paid when a patient without NF1 presents with a rapidly growing painless tumor in and around nerve tissue. A definitive therapeutic protocol needs to be charted but we believe that it can be managed by total mastectomy followed by adjuvant chemotherapy and radiotherapy. Long-term careful follow-up is required in order to generate an optimum treatment strategy
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