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Open Access Case Report

Multifocal Langerhans cell sarcoma involving epidermis: a case report and review

Changsong Wang1*, Yanping Chen1, Chunfang Gao1, Jian Yin1 and Hong Li2

Author Affiliations

1 Department of Pathology, 150th Hospital of PLA, Luoyang, China

2 Department of Otorhinolaryngology and Head-Neck Surgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China

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Diagnostic Pathology 2012, 7:99  doi:10.1186/1746-1596-7-99

Published: 14 August 2012

Abstract

Objective

To study the clinico-pathological characteristics of Langerhans cell sarcoma (LCS) which involving epidermis.

Methods

A case of primary multifocal LCS was analyzed in histopathology and immunophenotype.

Results

A 41-year-old man with multifocal cutaneous LCS involving the inguina and waist was reported. Clinical and pathology data were available. Neoplastic cells with markedly malignant cytological features were observed. Tumor cells exhibited irregular shape with abundant and eosinophilic red staining cytoplasm; large, irregular-shaped, showing lobulated or dented nucleus and some cells with a longitudinal nuclear groove and prominent nucleoli. The tumor cells expressed CD1a, Langerin (CD207), S-100 protein, CD68 and vimentin, and did not express pan-T or B cell markers and epithelial markers. The patient died less than 1 year after diagnosis due to local recurrence and metastasis to the lung, despite the administration of local radiation and chemotherapy.

Conclusions

LCS is a tumor with markedly malignant cytological features that originates from Langerhans cells. Primary multifocal neoplasms involving epidermis is even rare. Accurate diagnosis is based on the histopathological and immunohistochemical of the tumor cells.

Virtual slide

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1182345104754765.

Keywords:
Langerhans cell sarcoma; Multiple focus; Langerin; CD1a