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

Abstract

We present the case of a 62-year-old male patient with a three-month history of pain in the left shoulder. Magnetic resonance imaging of the left scapula showed an osteo-destructive lesion. H&E stained sections revealed a Langerhans cell sarcoma, and immunohistochemistry was performed additionally; CD68, CD163, CD14, fascin, HLA-DR, lysozyme, S100 CD1a and langerin showed a positive reaction, while CD20, CD30, CD34, CD31, pan-cytokeratin, AE/1AE3, SMA, desmin, EMA, ERG, INI-1, CD21, CD4, PLAP, MPO and CD117c were negative. We suggested palliative treatment with chemotherapy and radiation. The patient refused any treatment and died 2 weeks later.

Successful treatment of an elderly Langerhans cell sarcoma patient by EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) chemotherapy

“…Patients with progressive, systemic LCS often receive chemotherapy regimens, e.g. CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) according to malignant lymphomas, as previously reported 4 , 5 , 10 , 12 - 15 11 .…”

“…Here, we report an elderly LCS patient treated by etoposide-containing chemotherapy. LCS lesions mostly arise in the lymph nodes, skin, lung, bone and spleen 3 , 4 . In a relatively large study of histiocytic or dendritic cell neoplasms, the median age of patients with LCS (41 years) was older than that of those with LCH (33 years) 5 .…”

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.…”