Open AccessCase Report
Diagnosis and Treatment of Langerhans Cell Sarcoma: A Case Report and Review of the Literature
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Giulia Pileggi, Sabrina Mariani, Valentina De Santis, Gianluca Maiorana, Federica Lubrano Lobianco, Chiara Togni, Monica Piedimonte, Caterina Tatarelli, Esmeralda Conte, Arianna di Napoli, Emanuela Pilozzi, Evelina Rogges, Agostino Tafuri and Giovanna Palumbo
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Abstract
Langerhans cell sarcoma (LCS) is a rare and aggressive neoplasm characterized by a clonal proliferation of Langerhans cells (LCs), with multi-organ involvement and poor prognosis. Diagnostic challenges arise from its rarity and overlapping features with Langerhans cell histiocytosis (LCH), requiring immunophenotypic and histological
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Langerhans cell sarcoma (LCS) is a rare and aggressive neoplasm characterized by a clonal proliferation of Langerhans cells (LCs), with multi-organ involvement and poor prognosis. Diagnostic challenges arise from its rarity and overlapping features with Langerhans cell histiocytosis (LCH), requiring immunophenotypic and histological analysis for differentiation. This case report discusses a 67-year-old male with multi-organ LCS involvement. Diagnosis was confirmed via liver biopsy and genetic analysis, revealing a MAP2K1 mutation. Treatment with subcutaneous cladribine and dexamethasone resulted in significant clinical and radiological improvement, despite hematological toxicity due to an underlying myelodysplastic neoplasm (MDS). This case proves the potential efficacy of cladribine for disseminated LCS and highlights the necessity for further research into optimal therapeutic approaches for this rare malignancy.
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