Ectopic Pheochromocytoma: Does the Rule of Tens Apply?

ABSTRACT. We report here the course and treatment of a patient with ectopic pheochromocytoma. The patient was cured after treatment with respiratory and circulatory support, multiple-organ protection, and continuous renal replacement therapy (CRRT) for approximately 2 weeks. After misdiagnosis, a patient with ectopic pheochromocytoma who is being treated should undergo aggressive fluid supplementation and CRRT instead of central venous pressure measurement, which has limited value in guiding fluid supplementation. The main priority is maintaining hypotension. Hypertension may be controlled with rapidacting agents. A good outcome can be anticipated for patients who undergo comprehensive intensive care unit therapy.

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“…The patient's BP in this case met the criteria for a diagnosis of critical hypertension (Madani et al, 2007).…”

Section: Discussionmentioning

confidence: 68%

“…The best approach to diagnose or exclude pheochromocytoma is determination of catecholamine and vanillylmandelic acid levels in 24-h urine samples (Madani et al, 2007). Obviously, it was the insufficient diagnosis and differential diagnosis in this case that led to inadequate preoperative preparation and intraoperative anesthesia.…”

Section: Discussionmentioning

confidence: 93%

Case Report Post-surgical treatment of a patient with ectopic pheochromocytoma

et al. 2015

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“…Pheochromocytoma originates in chromaffin cells derived from primitive neural crest cells, which may react and stain with chromic salts (8). Furthermore, pheochromocytoma or paraganglioma in the pancreas, to a certain extent, is a type of pancreatic neuroendocrine tumor (pNET), which means that the tumor cells of resected tumors may demonstrate positive reactions for CgA and Syn following immunohistochemical staining (12).…”

Section: Discussionmentioning

confidence: 99%

“…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)(7)(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).…”

Section: Introductionmentioning

confidence: 99%

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

Ding¹,

Min²,

He³

et al. 2016

Oncology Letters

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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...

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“…The 10% rule is no longer applied. The ectopic pheochromocytoma is more prevalent than 10% (Madani et al, 2007). Extra-adrenal tumors are extra-adrenal paragangliomas and the majority is located in the head and neck area (69%), followed by intra-abdominal (22%) and intrathoracic locations (10%) (Van Der Horst-Schrivers et al, 2010).…”

Section: Introductionmentioning

confidence: 99%