Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 16, 2015; 3(5): 474-478
Published online May 16, 2015. doi: 10.12998/wjcc.v3.i5.474
Composite pheochromocytoma masquerading as solid-pseudopapillary neoplasm of pancreas
Geetanjali Gupta, Ravindra Kumar Saran, Satyajit Godhi, Siddharth Srivastava, Sundeep Singh Saluja, Pramod Kumar Mishra
Geetanjali Gupta, Ravindra Kumar Saran, Department of Pathology, Academic Block, GB Pant Hospital, New Delhi 110002, India
Satyajit Godhi, Sundeep Singh Saluja, Pramod Kumar Mishra, Department of Gastrointestinal Surgery, Academic Block, GB Pant Hospital, New Delhi 110002, India
Siddharth Srivastava, Department of Gastroenterology, Academic Block, GB Pant Hospital, New Delhi 110002, India
Author contributions: Gupta G, Godhi S, Saran RK and Saluja SS equally contributed; Gupta G and Godhi S wrote paper; Srivastava S and Saluja SS contributed in management of the patient; Saluja SS and Mishra PK revised the paper along with managing the case.
Ethics approval: We did not take any ethics committee approval as we are describing a histological surprise and not conducting an experimental study.
Informed consent: A written informed consent was taken from the patient and his relatives.
Conflict-of-interest: All the authors declare that they do not have any conflict of interest in the subject of the case report.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Sundeep Singh Saluja, Associate Professor, Department of Gastrointestinal Surgery, Academic Block, GB Pant Hospital, 2, Jawaharlal Nehru Marg, New Delhi 110002, India. sundeepsaluja@yahoo.co.in
Telephone: +91-97-18599259 Fax: +91-23-239442
Received: August 8, 2014
Peer-review started: August 9, 2014
First decision: October 14, 2014
Revised: November 10, 2014
Accepted: February 10, 2015
Article in press: February 12, 2015
Published online: May 16, 2015
Processing time: 272 Days and 23 Hours
Abstract

Pheochromocytoma and ganglioneuroma form rare composite tumours of the adrenal medulla comprising less than 3% of all sympathoadrenal tumours. We present a case of intraoperatively detected adrenal medullary tumour of composite pheochromocytoma and ganglioneuroma diagnosed on histopathology, in a normotensive patient. A 50-year-old male with a past history of chronic obstructive pulmonary disease presented with abdominal pain and significant weight loss since one month. Ultrasound and contrast-enhanced computed tomography abdomen revealed a large lobulated lesion in the distal body and tail of pancreas suggestive of solid and papillary neoplasm of body and tail of pancreas. Intra-operatively, a 15 cm × 10 cm solid lesion with cystic areas was seen arising from the left lower pole of the adrenal gland pushing the pancreas which appeared unremarkable. In our case, exploratory laparotomy with tumour excision was done. Extensive sectioning and microscopic examination of this adrenal tumour confirmed a diagnosis of composite Pheochromocytoma with Ganglioneuroma on histopathology. Immunophenotyping with S-100 further supported the diagnosis. The goal of this report is to increase the awareness of this rare disease and to further identify its variable presentation.

Keywords: Ganglioneuroma; Pheochromocytoma; Adrenal

Core tip: Adrenal composite pheochromocytomas may present in various ways without hypertension and conclusive symptoms. We present a case of intraoperatively detected adrenal medullary tumour of composite pheochromocytoma and ganglioneuroneuroma diagnosed on histopathology, in a normotensive patient. The goal of this report is to increase the awareness of this rare disease and to further identify its variable presentation.