Non-syndromic pheochromocytoma: From post-operatory scores to lifetime follow-up

Mihai Cristian Dumitrascu,Diana Elena Becheru,Adina Ghemigian,Mara Cârșote ,Florica Șandru ,Е С Петрова ,M Popescu ,Anda Dumitrașcu

doi:10.37897/rjmp.2021.3.14

Abstract

We introduce a case report of an adult male diagnosed with non-syndromic pheochromocytoma with a first pathological post-operatory report of malignant type with further re-considerations during follow-up for a 6-year period of time. This is 46 - year old male admitted for: post-adrenalectomy status reassessment. In 2013 he was diagnosed with high blood pressure requiring a complex regime of anti-hypertensive drugs to control it. In 2015 he was referred for an endocrine check-up which revealed a pheochromocytoma (noradrenaline type). Computed tomography imaging of the abdomen showed a right adrenal tumor of 28/38 mm, a mass that was clearly contoured, with heterogeneous pattern, and with moderate caption of intravenous contrast. Laparoscopic right adrenalectomy is performed with normalization of high pre-operatory normetanephrines and a dose reduction of anti-hypertensive medication which was still necessary. Pathological report suggested a malignant pheochromocytoma; the initial PASS score of 8 was later re-calculated, and a GAPP score of 5 was achieved showing a moderately differentiated tumor. No genetic backup was identified. Within the first year after tumor removal, the patient suffered a stroke, proving the higher cardiovascular risk than general population even after hormonal imbalance is restored. Lifelong surveillance is the rule.

Highlights

There are multiple endocrine causes of high blood pressure like pituitary related causes, adrenal-related causes, some thyroid conditions, especially those with hyper-function or overtreatment with levothyroxine, and, particular types of neuroendocrine neoplasia [1,2,3,4,5]
Article History: Received: 16 September 2021 Accepted: 25 September 2021 approach and advanced imaging like 68Ga-DOTATATE and 64Cu-DOTATATE, pheochromocytoma has usually a strong genetic background, and the potential of aggressive profile remains an open issue, lifelong surveillance is indicated in majority of cases [10,11]
We introduce a case report of an adult male diagnosed with non-syndromic pheochromocytoma with a first pathological report of malignant type with further considerations during follow-up

Summary

INTRODUCTION

There are multiple endocrine causes of high blood pressure like pituitary related causes (acromegaly, Cushing disease), adrenal-related causes (like pheochromocytoma, Conn’s syndrome, adrenal Cushing syndrome, certain types of bilateral macronodular hyperplasia, adrenocortical carcinoma, etc.), some thyroid conditions, especially those with hyper-function or overtreatment with levothyroxine, and, particular types of neuroendocrine neoplasia [1,2,3,4,5]. Pheochromocytoma is a rare, yet potentially deadly condition, due to increased cardiovascular risk, as well as potential malignant status in some cases as shown by high GAPP/ PASS scores [6,7]. Malignant pheochromocytoma is traditionally considered if the positive metastatic status is found [8,9]. Article History: Received: 16 September 2021 Accepted: 25 September 2021 approach and advanced imaging like 68Ga-DOTATATE and 64Cu-DOTATATE, pheochromocytoma has usually a strong genetic background, and the potential of aggressive profile remains an open issue, lifelong surveillance is indicated in majority of cases [10,11]

AIM

Findings

DISCUSSION

CONCLUSIONS