Journal Article

CD56/NCAM-Positive Langerhans Cell Sarcoma : A Clinicopathologic Study of 4 Cases

OGURA Michinori, NAOE Tomoki, KAGAMI Yoshitoyo, MURAYAMA Toshihiko, TATENO Masatoshi, TAKAHASHI Toshitada, HAMAZAKI Minoru, KAWASE Yoshiaki, NAGAI Hirokazu, MORISHIMA Yasuo, MORI Yoshio, TAJI Hirofumi, KAMIYA Yoshikazu, KAWASE Takakazu, NAKAMURA Shigeo, OYAMA Takashi

2005

Overview

This report concerns the clinicopathologic features of 4 patients with CD56/neural cell adhesion molecule (NCAM)-positive Langerhans cell sarcoma (LCS). Three of the patients were elderly, between 59 and 62 years of age at presentation, and the other was 35 years old. The presenting symptoms included fever, bone pain, and weakness. The patients shared some clinical findings, such as multiorgan involvement of lymph nodes, skin, lung, bone marrow, and spleen. LCS carries a poor prognosis, and 3 of the patients died of the disease within several years of presentation despite multiagent chemotherapy and radiotherapy. Of special interest is that all of the cases showed CD56 expression on the tumor cells in addition to expression of CD1a, S100beta, and langerin, the presence of which suggests derivation from Langerhans cells. For control, CD56 was also examined in 8 cases of Langerhans cell histiocytosis (LCH), a single-system unifocal or multifocal disease, and the results of staining of the tumor cells were negative. Our findings indicated that CD56 may be a clinically relevant biologic marker for predicting an intractable course of Langerhans cell neoplasms, although it is often difficult to draw a definite morphologically-based distinction between LCS and LCH.

• Publisher: Springer Nature B.V
• Subject: Adult / CD56 / CD56 Antigen - analysis / Female / Fever - etiology / Histiocytosis, Langerhans-Cell - immunology / Histiocytosis, Langerhans-Cell - pathology / Histiocytosis, Langerhans-Cell - therapy / Humans / Langerhans cell histiocytosis / Langerhans cell neoplasm / Langerhans cell sarcoma / Male / Middle Aged / Muscle Weakness - etiology / Pain - etiology / Sarcoma - immunology / Sarcoma - pathology / Sarcoma - therapy