Case Report: Anlotinib combined with radiotherapy achieves sustained disease control in advanced malignant melanotic nerve sheath tumor

Background: Malignant melanotic nerve sheath tumor (MMNST) is a rare and aggressive subtype of malignant peripheral nerve sheath tumor (MPNST) characterized by melanin-producing cells. Due to its rarity, there is currently no standardized therapeutic strategy, posing significant challenges in selecting effective treatment modalities. Case summary: We report the first documented case of a 54-year-old female with recurrent MMNST and multiple pulmonary metastases. Following local radiotherapy combined with systemic anlotinib treatment, the patient achieved prolonged progression-free survival (PFS) exceeding 32 months. Conclusion: This case suggests that anlotinib combined with radiotherapy may be an effective therapeutic option for advanced MMNST, providing valuable clinical evidence for managing this malignancy.