Adult Langerhans cell histiocytosis presenting with multisystem involvement and sarcomatoid features: A case report

Luis E. Aguirre, Ingrid Schwartz, Jennifer Chapman, Marcelo F. Larsen, Alvaro Alencar

Research output: Contribution to journalArticlepeer-review


Background: Langerhans cell tumors are rare clonal disorders characterized by neoplastic proliferation of dendritic cells that can be further classified into the subtypes Langerhans cell histiocytosis and Langerhans cell sarcoma, which are rare neoplasms exhibiting aggressive features and a poor prognosis. In addition to illustrating the refractoriness and poor outcomes of multisystem Langerhans cell histiocytosis in adults, specific events in this case highlight important characteristics of disease biology that warrant detailed discussion and exposition to a wider audience. Case presentation: We describe the case of a 42-year-old Caucasian man with Langerhans cell histiocytosis diagnosed from a lesion on the left arm that presented with constitutional symptoms, early satiety, and weight loss. Esophagogastroduodenoscopy showed extensive esophageal and duodenal involvement by Langerhans cell histiocytosis with features of Langerhans cell sarcoma. He was initially treated for Langerhans cell histiocytosis with low doses of cytarabine until he eventually presented clear transformation to acute monoblastic leukemia with complex karyotype that could not be properly controlled, leading eventually to death. Conclusions: Langerhans cell histiocytosis remains an exceedingly rare entity in adults, frequently presenting as multisystem disease with risk organ involvement. Langerhans cell sarcoma represents an aggressive subtype with extremely poor prognosis for which intensive acute myeloid leukemia induction should be strongly considered.

Original languageEnglish (US)
Article number169
JournalJournal of medical case reports
Issue number1
StatePublished - Sep 27 2020


  • Acute myeloid leukemia
  • Central nervous system
  • Chemotherapy
  • Colony-stimulating factor
  • Cytogenetics
  • Langerhans cell histiocytosis
  • Langerhans cell sarcoma
  • Leukemic transformation

ASJC Scopus subject areas

  • Medicine(all)


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