Cushing syndrome as presenting symptom of calcifying nested stromal–epithelial tumor of the liver in an adolescent boy: a case reportReportar como inadecuado




Cushing syndrome as presenting symptom of calcifying nested stromal–epithelial tumor of the liver in an adolescent boy: a case report - Descarga este documento en PDF. Documentación en PDF para descargar gratis. Disponible también para leer online.

Journal of Medical Case Reports

, 10:160

First Online: 16 June 2016Received: 26 January 2016Accepted: 12 May 2016DOI: 10.1186-s13256-016-0951-2

Cite this article as: Weeda, V.B., de Reuver, P.R., Bras, H. et al. J Med Case Reports 2016 10: 160. doi:10.1186-s13256-016-0951-2

Abstract

BackgroundEctopic adrenocorticotropic hormone-producing primary liver tumors are rare, especially in children. We report the case of an adolescent boy of mixed Dutch and Moroccan descent with an adrenocorticotropic hormone-producing calcifying nested stromal–epithelial tumor with long-term follow-up. Thus far, only two such cases have been reported.

Case presentationA 16-year-old boy of mixed Dutch and Moroccan descent presented with Cushing syndrome and a palpable abdominal mass. A calcifying nested stromal–epithelial tumor was diagnosed. Postoperatively, his plasma adrenocorticotropic hormone concentration normalized. He remains in complete remission 13 years after tumor resection.

ConclusionsCalcifying nested stromal–epithelial tumor should be in the differential diagnosis of liver tumors, especially if associated with Cushing syndrome as significant morbidity and mortality may be associated. Literature on the topics involved is comprehensively reviewed.

KeywordsEctopic ACTH production Calcifying nested stromal–epithelial tumor of the liver Desmoplastic nested spindle cell tumor of liver Liver tumor Pediatric  Download fulltext PDF



Autor: V. B. Weeda - Ph. R. de Reuver - H. Bras - J. Zsíros - W. H. Lamers - D. C. Aronson

Fuente: https://link.springer.com/







Documentos relacionados