Langerhans cell sarcoma originating from left knee subcutaneous tissue: A case report and literature review

Abstract

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with notably malignant cytological features. Reports of LCS are sparsely available in English literature; to the best of our knowledge, only 55 cases have been reported. The present study reports a case of LCS originating from subcutaneous tissue of the left knee in a 75-year-old man. The diagnosis of LCS was supported by the results of magnetic resonance imaging, histological and immunohistochemical studies. The tumor began to metastasize to inguinal lymph nodes and eventually involved multiple organs. Chemotherapy and radiotherapy were administered but were ineffective, and the patient died within 2 years of diagnosis. The present case should aid in expanding the currently available knowledge concerning LCS.

Nodal Langerhans cell neoplasm: detailing the diagnostic quandaries

“…The discriminating attributes as accounted for in the literature and accepted by the WHO are a markedly higher degree of cytological atypia, more frequent mitotic features and a higher rate of Ki67 proliferation index (usually more than 30%) in LCS compared with LCH. 2,4,6,8 The tumor in our study did not exhibit high cellularity, cellular crowding, marked pleomorphism and atypia neither in the primary nor in the recurrent pleural lesion, as is the wont of LCS. Thus, despite a relatively rapid recurrence, the neoplasm could not be categorized as unequivocal LCS.…”

“…There appears to be no clear difference in phenotypic profiles between LCH and LCS, expressing the typical immunophenotypic profile of Langerin (CD207), CD1a, S-100 protein, and similar ultra-structure characteristics of Birbeck granules within the cytoplasm. 2,4,6,7 Although not pathognomonic, the disease is found to harbor BRAF V600E mutation.…”

Histiocytic lesions of the orbit: A study of 9 cases

“…Our patient with LCS succumbed to death within 1 month after ocular diagnosis almost 1.5 years after initial diagnosis despite intensive systemic chemotherapy. Due to the rarity of LCS, no standard treatment with good efficacy has been suggested to date especially in cases with orbital involvement 20 . Complete or subtotal tumor resection should be done.…”

“…Complete or subtotal tumor resection should be done. Chemotherapeutic regimens, such as a modified ESHAP (etoposide, carboplatin, cytarabine, and methylprednisolone) and MAID (mesna, doxorubicin, ifosfamide, dacarbazine) have been demonstrated to be effective in a proportion of patients 20 . High-dose radiotherapy is also used alongside chemotherapy in the management of LCS 20 …”

Langerhans cell sarcoma presenting as a mediastinal mass in a young infant: a case report

“…It may occur de novo or may develop from an antecedent Langerhans cell histiocytosis (LCH) [1]. About 70 cases of LCS have been reported worldwide [2][3][4][5][6]. Very little data are available on its manifestations, therapy and prognosis in children.…”