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Video Assisted Thoracoscopic Thymectomy Langerhans Cell Histiocytosis

Contributors: Gary Nace, Juan Calisto and Marcus Malek

Langerhans Cell Histiocytosis (LCH) is an exceedingly rare proliferative disorder in which pathologic histiocytic cells accumulate in nearly every organ. Our patient, a five-month-old, six kilogram female with mild pulmonary valve stenosis, had both thymic and lung tissue involvement. To date there has never been a report of a thymic LCH with lung metastases in an infant. She underwent a video assisted thoracoscopic thymectomy.

DOI: http://dx.doi.org/10.17797/2qbbejhisy

Video Assisted Thoracoscopic Thymectomy
Metastatic Langerhans Cell Histiocytosis
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Preoperative echocardiogram shows a large thymic mass.
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Bax, N.M.A. Endoscopic Surgery in Infants and Children. Berlin:Springer, 2008.Print. �  Ladisch, Stephan (2011). "Histiocytosis Syndromes of Childhood". In Kliegman, Robert M.; Stanton, Bonita F.; St. Geme, Joseph; Schor Nina; Behrman, Richard E. Nelson Textbook of Pediatrics (19th ed.). Saunders. pp.� 1773�¢7. �  Sholl, LM; Hornick, JL; Pinkus, JL; Pinkus, GS; Padera, RF (June 2007) �¢Immunohistochemical analysis of langerin in langerhans cell histiocytosis and pulmonary inflammatory and infectious diseases.". The American journal of surgical pathology 31 (6): 947�¢52. �  Haupt, Riccardo; Minkov, Milen; Astigarraga, Itziar; Sch�¤fer, Eva; Nanduri Vasanta; Jubran, Rima; Egeler, R. Maarten; Janka, Gritta; Micic, Dragan; Rodriguez-Galindo, Carlos; Van Gool, Stefaan; Visser, Johannes; Weitzman, Sheila; Donadieu, Jean (February 2013). "Langerhans cell histiocytosis (LCH): Guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years". Pediatric Blood & Cancer 60 (2): 175�¢184

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