Cushing syndrome as presenting symptom of calcifying nested stromal-epithelial tumor of the liver in an adolescent boy: a case report.
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.


METHODS
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.
Tytgat Institute at the Academic Medical Center, Meibergdreef 69-71, S-building, 1105 BK, Amsterdam, The Netherlands. v.b.weeda@amc.uva.nl.|Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands. v.b.weeda@amc.uva.nl.
V B
VB
Weeda
Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands.
Ph R
PR
de Reuver
Department of Pathology, Academic Medical Center, Meibergdreef 9, 1105, AZ, Amsterdam, The Netherlands.
H
H
Bras
Division of Pediatric Oncology, Emma Children's Hospital at the Academic Medical Center, Meibergdreef 9, 1105, AZ, Amsterdam, The Netherlands.
J
J
Zsíros
Tytgat Institute at the Academic Medical Center, Meibergdreef 69-71, S-building, 1105 BK, Amsterdam, The Netherlands.
W H
WH
Lamers
Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands.|Department of Paediatric Surgery, Leeds Children's Hospital at the Leeds Teaching Hospitals NHS Trust, Leeds, West Yorkshire, LS1 3EX, UK.
D C
DC
Aronson
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000097:blood; D002114:Calcinosis / Q000150:complications / Q000175:diagnosis; D003480:Cushing Syndrome / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D003937:Diagnosis, Differential; D004848:Epithelium / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008113:Liver Neoplasms / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D008297:Male; D009018:Morocco; D009426:Netherlands; D017154:Stromal Cells / Q000473:pathology
D000324:Adrenocorticotropic Hormone
Calcifying nested stromal–epithelial tumor of the liver; Desmoplastic nested spindle cell tumor of liver; Ectopic ACTH production; Liver tumor; Pediatric