Published online May 16, 2015. doi: 10.12998/wjcc.v3.i5.474
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
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.
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.