Korean J Dermatol.  2019 Nov;57(9):538-543.

Langerhans Cell Sarcoma of the Scalp with Concomitant Multiple Cervical Lymph Node and Adjacent Soft Tissue Involvement: A Case Report

• Han MH
• Kim JY
• Lee WJ
• Lee SJ

Abstract

Currently, the World Health Organization classifies Langerhans cell tumors into Langerhans cell histiocytosis and Langerhans cell sarcoma (LCS). LCS is a neoplastic proliferation of Langerhans cells showing malignant cytological features and aggressive clinical behavior with grave prognosis. Only a few cases have been reported in the available literature; therefore, to date, no definitive treatment has been established. A 64-year-old woman presented with a 1-year history of an asymptomatic, slow-growing erythematous nodule measuring 0.7 cm on her scalp. The patient also reported a 3-month history of a painful swelling on the right side of her neck. Histopathological examination of a scalp biopsy specimen revealed sheets of atypical cells with hyperchromatic nucleoli and clear cytoplasm. Immunohistochemical studies revealed malignant cells positive for CD1a, CD31, CD68, and S-100 expression. Additionally, positron emission tomography-computed tomography and fine-needle aspiration revealed LCS of the cervical lymph nodes and surrounding soft tissue. We recommended surgical excision and adjunctive chemotherapy; however, the patient refused treatment and died of the disease 28 months later.

Keyword

Langerhans cell sarcoma; Lymph node; Parotid gland; Scalp; Soft tissue

MeSH Terms

Biopsy
Biopsy, Fine-Needle
Cytoplasm
Drug Therapy
Electrons
Female
Histiocytosis, Langerhans-Cell
Humans
Langerhans Cell Sarcoma*
Langerhans Cells
Lymph Nodes*
Middle Aged
Neck
Parotid Gland
Prognosis
Scalp*
World Health Organization