Langerhans cell sarcoma involving skin and showing epidermotropism: A comprehensive review

Abstract

Langerhans cell sarcoma (LCS) is rare and aggressive; patients have an overall survival rate of less than 50%. We present a 62-year-old man with a history of superficial spreading melanoma of the upper back with sentinel lymph node metastasis, Langerhans cell histiocytosis, and LCS. The patient presented with erythematous papules and scaly areas on his face, neck, arms, chest, abdomen, and legs. A skin biopsy revealed a proliferation of large neoplastic cells involving the dermis and with epidermotropism. These cells had atypical bean-shaped nuclei, with ample cytoplasm and abundant mitotic figures including atypical forms. Immunohistochemical studies showed the tumor to be diffusely positive for CD1a, S100 protein, and langerin (CD207) and negative for melanocytic markers. Some tumor cells were positive for cyclin D1. A diagnosis of LCS involving the skin was established. The present study is a very unusual case of LCS showing epidermotropism. The patient's history of metastatic melanoma posed additional challenges for diagnosis, underlying the need of immunophenotyping in these cases. Consensus for optimal standard therapy has not been established in LCS, and thus, early recognition is important since these neoplasms tend to recur and metastasize. LCS in skin is discussed and published cases are comprehensively reviewed.

Left Forearm Langerhans Cell Sarcoma Demonstrated by 18F-FDG PET/CT

“…[1][2][3] Diagnosis of primary Langerhans cell sarcoma is difficult, and it is necessary to differentiate it from other malignancies of the skin, including anaplastic large cell lymphoma, 4 malignant melanoma, 5 metastatic cancer, myeloid sarcoma, and malignant fibrous histiocytoma. 6 Langerhans cell sarcoma occurs at any age and involves many organs or tissues, including bone marrow, 7 lung, 8 lymph nodes, 9 liver, 10 skin, [11][12][13] and subcutaneous tissue. 14 Our case indicated that the differential diagnosis of Langerhans cell sarcoma should be borne in mind for cases with high FDG uptake of skin mass.…”

Exploring the current status and trends of research on Langerhans Cell Sarcoma: A bibliometric analysis

“…Development usually occurs at 30-40 years of age; however, the disease may have a wider age of onset and may also present in children [4,5]. LCS affects multiple organ systems; however, previously published literature has identified the skin, lymph nodes, bone marrow, lungs, spleen, and liver as common sites of involvement [6][7][8]. Diagnosis of this disease has proved to be difficult since its marked cytologic atypia and pleomorphism is coherent with several other diseases such as malignant melanoma, lymphomas, and other dendritic cell tumors.…”