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Neuroendocrine Tumor in Liver With Positive ACTH Receptor: A Case Report

Zein, Mohamad El MD; Vali, Reza MD; Charron, Martin MD; Manson, David MD; Perlman, Kusiel MD; Shammas, Amer MD

Journal of Pediatric Hematology/Oncology: January 2014 - Volume 36 - Issue 1 - p e1–e4
doi: 10.1097/MPH.0b013e318286d2ba
Online Articles: Clinical and Laboratory Observations

A rare case of possible primary ectopic adrenocorticotropic hormone (ACTH)-producing tumor in the liver mimicking a liver hemangioma is reported. A 9-year-old boy, with Cushing syndrome, was referred for the assessment of ectopic ACTH-producing tumor. Ultrasound, CT scan, and MRI of the abdomen revealed a liver lesion suggestive of a hemangioma. 111In-octreotide scintigraphy revealed focal activity in the liver, indicative of a somatostatin-positive lesion. 99mTc-labeled RBC scintigraphy was negative for hemangioma. After surgical resection of the tumor, the cortisol level converted to a normal range indicative of a rare possible primary ACTH-producing tumor in the liver, which was confirmed by histopathology.

Department of Diagnostic Imaging, Division of Nuclear Medicine, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

The authors declare no conflict of interest.

Reprints: Amer Shammas, MD, Department of Diagnostic Imaging, Division of Nuclear Medicine, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, Canada M5G 1X8 (e-mail: amer.shammas@sickkids.ca).

Received February 6, 2012

Accepted January 8, 2013

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