Langerhans cell sarcoma of the right calf: a case report.

Abstract

Background: Langerhans cell sarcoma (LCS) is an extremely rare malignant tumor originating from the neoplastic proliferation of Langerhans cells. To report a rare case of LCS with a prolonged clinical course following surgical excision and chemotherapy, we present this case report.

Case description: We report the case of a 46-year-old female Han Chinese patient who presented with persistent right calf pain. Surgical excision was performed, and the diagnosis of LCS was confirmed histopathologically and by immunohistochemical evaluation. Postoperative positron emission tomography/computed tomography (PET/CT) performed 24 days after surgery revealed bilateral femoral and tibial lesions, which are consistent with LCS in conjunction with pathological findings. The patient received six cycles of anthracycline chemotherapy. Subsequently, abdominal contrast-enhanced computed tomography (CT) and PET/CT indicated liver dissemination, prompting a switch to six cycles of ifosfamide chemotherapy. Follow-up evaluations demonstrated stable disease. After a treatment-free interval of 23 months, repeat abdominal contrast-enhanced CT showed complete resolution of the liver dissemination. The patient remains in stable condition during regular follow-up, with overall disease duration exceeding 9 years.

Conclusions: Given the rarity of LCS, there are no standardized recommendations for adjuvant therapy, including radiotherapy and chemotherapy regimens for both localized and systemic disease. This case suggests that surgical excision followed by chemotherapy may be effective in preventing and controlling recurrence and systemic progression. Further research is needed to establish evidence-based management guidelines for LCS.