Background: Non secretory adrenal adenomas constitute majority of adrenal incidentalomas. 5% of these are pheochromocytomas (PCC) with the classical triad of headache, palpitations and diaphoresis. Atypical presentations are seen in 9-10% of these patients.
Aim: To highlight unusual presentations with the difficulties associated with diagnosis and management.
Materials and methods: Out of a total of 18 cases of PCC/ paragangliomas (PGL), over a period of 2 years from June 2017 to 2019, we report a series of 6 unusual cases. The diagnostic pitfalls and difficult management strategies have been described.
Results: Our series had 2 males and 4 females. The varied presentations in our cases were: acute intestinal pseudoobstruction, right sided neck mass, colossal adrenal incidentaloma, right hemiparesis with acute coronary events, congestive cardiac failure and a rare abdominal infrarenal tumour. Amongst the 6 cases, 3 of them had normal blood pressure. Normotensive PCC are rare entities, described in a few studies. All of our cases had a biochemical and histopathological confirmation of PCC/PGL with surgical excision and cure. PCC presenting with intestinal pseudoobstruction and a tumour of 20x16 cm were the rarest of them all, which have been only reported anecdotally.
Conclusion: This series reconfirms the masquerading nature of pheochromocytomas. Two extremely rare presentations were the highlights of our study. A keen suspicion and adequate preoperative evaluation dictates management in such cases. Diagnostic and management protocols may vary based on the presentation contributing for a learning experience.