Pheochromocytoma of the pancreas: A report of three cases and a literature review

Abstract

Abstract. Pheochromocytoma is primarily derived from the adrenal medulla. The majority of extra-adrenal pheochromocytoma cases occur in the superior para-aortic region and para-adrenal area. However, pheochromocytoma originating from the pancreas is rare. The present study reports the cases of three patients who had no history of hypertension but were post-operatively diagnosed with pheochromocytoma located in the pancreas. Of the three patients, two were admitted to hospital due to abdominal pain, and imaging examinations revealed a soft-tissue lesion in the head of pancreas. Local resection of the pancreatic tumor was successfully performed and a diagnosis of pheochromocytoma derived from the pancreas was subsequently made by pathologists. The third patient was admitted to hospital for surgical treatment due to the identification of a continuously growing lesion in the tail of pancreas during physical examinations. Distal resection of the pancreas was stopped during surgery when the patient's blood pressure and heart rate suddenly increased to 180/110 mmHg and 140 beats/min, respectively. Due to a marked rise in noradrenaline and adrenaline levels in the blood subsequent to surgery, the patient was diagnosed with pancreatic pheochromocytoma. The present study additionally reviewed the associated literature concerning pheochromocytoma in order to improve the understanding of this rare clinical phenomenon. The aim of the present study is to highlight to surgeons that although patients may not present with typical clinical manifestations due to the non-functional status of the tumor, undiagnosed pheochromocytoma of the pancreas should be considered when surgeons observe an unexpected hypertensive crisis during pancreatic tumor surgery. IntroductionPheochromocytoma is a catecholamine-secreting tumor that originates from chromaffin cells. The majority of these tumors arise in the adrenal medulla (1), and when they occur outside the adrenal gland they are known as an extra-adrenal pheochromocytoma or a paraganglioma (2). Pheochromocytoma is primarily sporadic, but may additionally be associated with multiple endocrine neoplasia syndrome (3) and mutations of mitochondrial complex 2 succinate dehydrogenase enzymes (4).Extra-adrenal pheochromocytoma has been previously reported to account for ~10% of all pheochromocytoma cases during adulthood and for 30-40% of cases in children (5). These tumors may occur in the superior para-aortic region, the para-adrenal area and the inferior para-aortic region, as well as the organ of Zuckerkandl and the urinary bladder (6-8). Pancreatic localization of extra-adrenal pheochromocytoma is rare, and to the best of our knowledge, only one case has been previously reported in the Chinese literature (9). In the present study, the cases of three patients (Table I) with pheochromocytoma involving the pancreas are presented. To the best of our knowledge, the present report is the first to present three definite and comprehensive cases of this rare clinical phenomenon. In addition, the rele...

Metastatic pheochromocytoma to the pancreas diagnosed by endoscopic ultrasound‐guided fine needle aspiration: A case report and review of literature

“…17,18 Despite the potential risk of causing hemorrhage within the lesion and precipitating a hypertensive crisis, the use of FNA in diagnosing chromaffin cell tumors can be useful particularly in atypical presentations, for example, in diagnosing a retroperitoneal extra-adrenal pheochromocytoma located in the organ of Zuckerkandl. 19 Most patients in these reports did not present with hypertension, diagnosis was made after resection of the pancreas, and an adrenal mass was not mentioned in these cases. 19 Most patients in these reports did not present with hypertension, diagnosis was made after resection of the pancreas, and an adrenal mass was not mentioned in these cases.…”

“…2 Primary pancreatic pheochromocytoma is another possibility in this case but is a rare entity described in few case reports, mainly from China. 19 Most patients in these reports did not present with hypertension, diagnosis was made after resection of the pancreas, and an adrenal mass was not mentioned in these cases. We describe a case of metastatic pheochromocytoma to the pancreas diagnosed by EUS-FNA, the first reported case as per the literature review.…”