Langerhan's Cell Sarcoma: Two Case Reports

Abstract

Langerhan’s cell sarcoma (LCS) is a rare neoplasm with a poor prognosis. To our knowledge, only sixty-six cases have been published. We discuss two patients who presented very differently with LCS, as well as a recently published review of all sixty-six cases. Our first case had a complicated history of metastatic, high-grade myxofibrosarcomas and presented with a single skin lesion of LCS which was treated with resection to a positive margin and adjuvant radiotherapy. The LCS recurred locoregionally and was again resected. The patient is alive two years after initial diagnosis. The second case presented with bone marrow and splenic involvement, leukocytosis, and thrombocytopenia. This patient had an excellent response to etoposide, prednisone, oncovorin, cyclophosphamide, and adriamycin, with normalization of the complete blood count, negative bone marrow biopsy at follow up, and splenectomy without viable neoplasm. This patient is alive without signs of disease at 16 months after initial diagnosis.

Successful treatment of an elderly Langerhans cell sarcoma patient by EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) chemotherapy

“…To our knowledge, characteristic chromosomal abnormalities or cytogenetic mutations are unknown in LCS. PET/CT is a good modality for staging and evaluating LCH, 7 and can be used to identify LCS lesions 8 . However, in this current case, the lesions were unevaluable by PET/CT.…”

“…EPOCH chemotherapy has been reported to be highly effective for refractory or relapsed non-Hodgkin’s lymphoma 17 . Kaleem et al demonstrated that EPOCH was effective for an LCS patient with multi-organ lesions 8 . We speculate that EPOCH chemotherapy was highly effective due to a pathogenic similarity with Merkel cell carcinoma (MCC).…”

Langerhans Cell Histiocytosis of the Temporal Bone

“…While CD1a and/or langerin are considered pathognomonic for LCH, they also stain positive in Langerhans cell sarcoma, an extremely rare disease with fewer than 100 cases reported. 19 Treatment of temporal bone LCH is dependent on the stage and severity of disease. 12 While no consensus treatment regimen is available, several treatment modalities have been proven effective.…”

“…While CD1a and/or langerin are considered pathognomonic for LCH, they also stain positive in Langerhans cell sarcoma, an extremely rare disease with fewer than 100 cases reported. 19 …”