Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature

Abstract

In the following, we describe the very rare case of Langerhans cell sarcoma (LCS) in the lung. Throughout the medical literature, only a few cases have been published, and, to the best of our knowledge, this is the first case to be reported in Germany. The patient was an 81-year-old man who showed symptoms such as chronic cough and weight loss. Clinical examination including needle biopsy indicated a high possibility of carcinoma in the right lung and in the mediastinum; however, the final histopathological diagnosis after immunohistochemistry gave evidence of LCS. LCS is a neoplastic proliferation of Langerhans cells with malignant cytological features exhibiting a very aggressive behaviour. LCS can be distinguished from other carcinomas, lymphomas and sarcomas by the typical morphological features of Langerhans cells and the immunophenotype with a consistent expression of S-100 protein and CD1a. In contrast to Langerhans cell histiocytosis, the LCS consists of Langerhans cells with high atypia and a very high mitotic rate.

ESHAP therapy effective in a patient with Langerhans cell sarcoma

“…CHOP or CHOP-like regimens were administered in four patients, and CR or PR was achieved in three of them [2,6,7]. Because LCS in the present case became refractory to the CHOP regimen, we selected the ESHAP regimen, which is one of the salvage regimens for relapsing or refractory lymphoma [14], and a marked improvement in LCS lesions was observed.…”

“…Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with overtly malignant cytologic features, and is considered a higher-grade variant of LCH [1]. Neoplasms of the Langerhans cell phenotype occur rarely, and, to the best of our knowledge, only 21 cases of LCS have been reported in the English literature [1][2][3][4][5][6][7][8][9][10][11][12]. We thought that the present LCS case was unusual in that she was diagnosed after living-related liver transplantation (LRLT), and so it was thought to have arisen from LCH.…”

Unusual cutaneous Langerhans cell sarcoma without extracutaneous involvement

“…Verdijk et al found a lack of Birbeck granules in Langerhans cells to be associated with a mutation in the langerin gene [16]. The lack of Birbeck granules under the electron microscopy might be due to the damage of Birbeck granules during histological process [3] or poor differentiation of tumor cells [10]. Thus, langerin is a useful marker for distinguishing Langerhans cell tumors from indeterminate cell tumors even in Birbeck granules-negative cases [12,17].…”

“…It can occur at any age and may involve multiple systems or tissues. Skin and underlying soft tissue are the most common involvement, but other unusual locations, such as bone [2], lung [3], gallbladder and peritoneal lymph nodes [4] have also been described in the literature. LCS may be limited to cutaneous involvement or may progress to affect other organs.…”

Cutaneous Langerhans Cell Sarcoma: A Case Report

“…They can occur at any age, although incidence peaks around the fourth decade of life. Skin and the underlying soft tissues are the most common sites of tumor origin, but the involvement of bone, lung, gallbladder, and peritoneal lymph nodes has also been described [5][6][7]1].…”