Abstract
Familial pheochromocytomas are commonly associated with multiple endocrine neoplasia type 2 (MEN 2) syndrome. Majority of the patients present with normal clinical and biochemical parameters in the preoperative period, the incidence of hypertension being only 50 %. Even though patients may be clinically asymptomatic, surveillance and proper preoperative evaluation is important, as surgery for associated tumors may precipitate a hypertensive crisis and result in severe complications. A family of 19 members, of which 12 were positive for MEN 2A syndrome, presented to our hospital. Seven of the 12 patients had pheochromocytoma and medullary thyroid carcinoma (MTC), while the other 5 had only raised plasma calcitonin levels. Two of the 7 patients presented with bilateral pheochromocytoma and underwent an open adrenalectomy. The other 5 patients had a left-sided adrenal tumor and underwent left laparoscopic adrenalectomy under combined general and epidural anesthesia. We present our experience with four of these five cases. We here state that how paucity of literature on perioperative preparation of clinically and biochemically silent pheochromocytomas led to serious intraoperative complications in one of four cases.
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Nguyen L, Niccoli P, Caron P, Bastie D, Maes B, Chabrier G (2001) Pheochromocytoma in multiple endocrine neoplasia type 2: a prospective study. Eur J Endocrin 144:37–44
Pacak K, Ilias I, Adams KT, Eisenhofer G (2005) Biochemical diagnosis, localization and management of pheochromocytoma: focus on multiple endocrine neoplasia type 2 in relation to other hereditary syndromes and sporadic forms of the tumour. J Intern Med 257:60–68
Eisenhofer G, Lenders JWM, Linchan WM, Walther MM, Goldstein DS, Keisir HR (1999) Plasma normetanephrine and metanephrine for detecting pheochromocytoma in von Hippel-Lindau disease and multiple endocrine neoplasia type 2. N Engl J Med 340:1872–1879
Bajwa SJS, Bajwa SK (2011) Implications and considerations during pheochromocytoma resection: a challenge to the anesthesiologist. Indian J Endocrinol Metab 15:S337–S344
Ramakrishna H (2015) Pheochromocytoma resection: current concepts in anesthetic management. J Anaesthesiol Clin Pharmacol 31(3):317–323
Dortzbach K, Gainsburg D, Frost E (2010) Variants of pheochromocytoma and their anesthetic implications. MEJ Anesth 20(6):897–905
Kumar SK, Krishna KS, Sandip P, Kritikumar M (2012) Pheochromocytoma: an uncommon presentation of an asymptomatic and biochemically silent adrenal incidentaloma. Malays J Med Sci 19(2):86–91
Trivedi P, Roy PS, Choudhary SR, Narayan S (2013) Anesthetic management of previously non-diagnosed pheochromocytoma: clinical vigilance, the ultimate saviour of anesthesiologist. IJA 57(3):295–297
Shen SJ, Cheng HM, Chiu AW, Chou C, Chen JY (2005) Perioperative hypertensive crisis in clinically silent pheochromocytomas: report of four cases. Chang Gung Med J 28:44–50
Dubey RK, Verma N, Pandey CK (2014) Anesthetic management of a dopamine-secreting pheochromocytoma in multiple endocrine neoplasia 2B syndrome. IJA 58(2):217–219
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The authors declare that they have no competing interests.
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This work is credited to the Department of Anesthesia and Intensive Care, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
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Aggarwal, S., Talwar, V., Virmani, P. et al. Anesthetic Management of Clinically Silent Familial Pheochromocytoma with MEN 2A: A Report of Four Cases. Indian J Surg 78, 414–417 (2016). https://doi.org/10.1007/s12262-016-1539-1
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DOI: https://doi.org/10.1007/s12262-016-1539-1