Primary Cutaneous Langerhans Cell Sarcoma

Abstract

Langerhans cell sarcoma (LCS) is a rare but potentially life-threatening neoplastic condition. The diagnosis of LCS requires morphological and immunophenotypic characterization to distinguish it from other epithelioid-appearing malignancies. Four cases of LCS were encountered in the consultative practices of 2 of the authors. The patients ranged in age from 54 to 88 years of age. In 2 of the cases the patients had a history of acute myelogenous leukemia with eruptions occurring after initiation of decitabine. One patient died within 3 months of presenting with the skin eruption, whereas the other patient is in remission. In the other 2 patients, there was no antecedent history; the presentation was in the context of a solitary nodule. One patient declined treatment and died of disseminated metastatic disease. The other patient had complete excision with no evidence of recurrent or metastatic disease. In all cases, the biopsies showed a sheet-like growth of large atypical epithelioid cells. Phenotypic studies revealed positivity for CD4, CD1a, and S100 in all and variable staining for langerin, lysozyme, CD83, CD31, and CD14. Cutaneous LCS represents a terminally differentiated myeloid tumor with a variable but potentially aggressive clinical course. It may be related to a common stem cell defect given the association with acute leukemia. The morphology ranges from atypical appearing Langerhans cell to a high-grade large cell epithelioid malignancy mimicking amelanotic nodular melanoma.

Langerhans Cell Sarcoma: A Case Report Demonstrating Morphological and Immunophenotypical Variability within a Single Lesion

“…These cells are normally found in the epidermis and also along with the mucosal lining of the oral cavity, pharynx, oesophagus, upper airways, urethra, and female reproductive tract [6]. LCS has a high degree of cytological atypia and usually a clinically aggressive course [2]; most cutaneous Langerhans cell sarcomas are described as having large atypical cells, some of which have an epithelioid appearance with pleomorphic nuclei; these are usually found in the dermis and the subcutaneous tissue [4]. In this case, morphologically the lesion contained pleomorphic bizarre cells with epidermotropism which has so far not been described in cutaneous Langerhans cell sarcoma.…”

“…The tumour cells in LCS are reported to express CD1a, S100, and Langerin [7]. These tumours may arise de novo or may complicate myeloproliferative disorders and also rarely Langerhans cell histiocytosis [2]. Bohn et al reviewed a total of 20 cases and reported that purely cutaneous presentation was seen in only three cases whilst the remaining cases showed involvement of both the skin and other organ systems [4].…”

“…Typically, involvement of multiple sites is seen [2]. The tumour cells have an overtly malignant cytomorphology and it is only the immunophenotype and/or the ultrastructure which may suggest and confirm Langerhans cell lineage [3].…”

Langerhan's Cell Sarcoma: Two Case Reports

“…They function as antigen presenting cells and are most commonly located in the epidermis, mucous membranes, and lymphatics. 1 , 2 Langerhan’s Cell Sarcoma (LCS) is a rare, but aggressive tumor with a poor prognosis. It was first reported by Wood et al .…”

“…Langerhan’s cells sarcoma is often difficult to diagnose by morphology alone and immunohistochemical markers play an important role in its diagnosis. 2 , 4 LCS stains positive for CD1a, CD4, CD207 (Langerin), lysozyme, and S-100 protein. 1 , 2 , 5 , 6 A high mitotic rate, cellular atypia, and the presence of Birbeck granules on electron microscopy are also present.…”