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Endolymphatic Non-Langerhans Cell Histiocytosis of the Larynx: Report of an Uncommon Disease Manifestation

Classen, Carl Friedrich MD, PhD*; Haffner, Dieter MD, PhD*; Nizze, Horst MD, PhD; Möller, Peter MD, PhD; Prall, Friedrich MD, PhD

Journal of Pediatric Hematology/Oncology: July 2010 - Volume 32 - Issue 5 - p e173-e176
doi: 10.1097/MPH.0b013e3181d746eb
Online Articles: Original Articles

We report on an uncommon laryngeal non-Langerhans cell histiocytosis. An 11-year-old boy presented with a 6 months history of progressive breath inhibition. Magnetic resonance imaging showed diffuse laryngeal and local lymph node swelling. Histology first resembled sarcoidosis, however, corticosteroids were ineffective. Lymphoma, infection, immunodeficiency, and autoimmune disease were excluded. Six months later, biopsies were repeated, now showing numerous ectatic lymph vessels with clusters of histiocytes bearing stellate extensions and emperipolesis. S100 protein and CD1a were negative. Indomethacin treatment led to a gradual improvement. In conclusion, we observed a nonmalignant non-Langerhans cell endolymphatic reticulohistiocytosis, not fitting into any of the described categories.

*Department of General Pediatrics, University Children's Hospital Rostock

Institute for Pathology, University of Rostock, Rostock

Institute for Pathology, University Ulm, Ulm, Germany

Reprints: Carl Friedrich Classen, MD, PhD, University Children's Hospital Rostock, Ernst-Heydemann Straße 8, D-18057 Rostock, Germany (e-mail:

Received for publication August 29, 2009; accepted January 30, 2010

© 2010 Lippincott Williams & Wilkins, Inc.