Published in:
Open Access 01-12-2016 | Case report
Cushing syndrome as presenting symptom of calcifying nested stromal–epithelial tumor of the liver in an adolescent boy: a case report
Published in: Journal of Medical Case Reports | Issue 1/2016
Login to get accessAbstract
Background
Ectopic 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 presentation
A 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.
Conclusions
Calcifying 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.