Langerhans Cell Sarcoma Arising from Langerhans Cell Histiocytosis: A Case Report

Abstract

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomographic (CT) scan revealed multiple tiny nodules in both lungs. The thoracoscopic lung biopsy revealed LCH. The patient quit smoking, but he received no other specific treatment. One year later, the follow up chest CT scan showed a 4 cm-sized mass in the left lower lobe of the lung. A lobectomy was then performed. Microscopic examination of the mass revealed an infiltrative proliferation of large cells that had malignant cytologic features. Immunohistochemical stains showed a strong reactivity for S-100 and CD68, and a focal reactivity for CD1a. We think this is the first case of LCS arising from LCH.

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

“…Langerhans cell sarcoma may develop de novo or by progression from previous Langerhans cell histiocytosis (LCH) [1,9]. Furthermore, a case of LCS occurring in a patient who underwent liver transplantation was also reported [14].…”

“…Inflammatory cell infiltration, consisting of lymphocytes and eosinophil gran- Table I. Summary of the reported cases of Langerhans cell sarcoma [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35] ulocytes, was also found, and isolated mitotic figures were identified ( Fig. 3A and 3B).…”

Cutaneous histiocytic sarcoma with E-cadherin expression in a Pembroke Welsh Corgi dog

“…2,7,15,32 In the present case, the high mitotic rate may be associated with the repeated recurrence after lomustine treatment.…”

Langerhans Cell Sarcoma in the Cervical Lymph Node: A Case Report and Literature Review

“…In 1984, Wood et al [2] first reported a 71-year-old patient with primary LCS exhibiting a diffusing lung infiltration and multiple skin nodules. In 2006, Jong-Sil Lee et al [4] reported the first case of a 34-year-old patient with pulmonary LCH that progressed into LCS 1 year later. The etiology and pathogenesis of LCS, although not fully understood, is reported to be related to immune abnormalities [1].…”