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

Abstract

Langerhans cell sarcoma (LCS) is extremely rare, with only 36 cases reported in English literature. In this report we represent the case of a 77-year-old woman with a 1-month history of left neck swelling and pain. A diagnosis of LCS was rendered from pathological findings of the cervical lymph node biopsy. The patient’s condition deteriorated rapidly and she died 2 days after diagnosis. A literature review in the context of the present case was performed to better enhance understanding of the early diagnosis and treatment of this unusual lesion.

Case study interpretation-Fort Lauderdale: Case 1

“…Several recent reviews of Langerhans cell sarcoma have been published in the context of case reports and a small case series (8)(9)(10)(11). The cells have characteristic oval, "coffee bean" grooved nuclei, lack nuclear atypia, and express Langerhans cell associated markers CD1a, S100, and Langerin.…”

Langerhans cell sarcoma originating from left knee subcutaneous tissue: A case report and literature review

“…Sparse reports of LCS are available in English literature; to the best of our knowledge, only 55 cases have been reported to date. The available reports are summarized, together with the present case, in Table I [(2) and references therein; (3–39)]. A search of Pubmed was performed to find these reports using the keyword ‘Langerhans cell sarcoma’ and manual screening was applied.…”

“…LCS is a neoplastic proliferation of Langerhans cells with markedly malignant cytological features, whereas LCH is proliferative disorder of Langerhans cells (1). LCS may occur in individuals of a broad age range, and is highly metastatic (2). The disease may involve multiple organs or tissues (3), including bone, lung, skin, lymph nodes, gallbladder, tonsil and other soft tissues.…”

“…The disease may involve multiple organs or tissues (3), including bone, lung, skin, lymph nodes, gallbladder, tonsil and other soft tissues. In the clinic, LCS is an extremely rare disease that is unfamiliar to dermatologists (2). The clinical behavior of the disease is aggressive and the overall survival rate for affected patient's is <50% (3).…”

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

“…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).…”