Ahmad Younes, Souad Ghattas, Samar Semaan, Hani Maalouf, Adham Al Kadirri, Hind Rahban, Ziad El Rassi
Keywords :
Case report, Giant, Malignant, Pheochromocytoma
Citation Information :
Younes A, Ghattas S, Semaan S, Maalouf H, Al Kadirri A, Rahban H, El Rassi Z. A Rare Case of Giant Malignant Pheochromocytoma. World J Endoc Surg 2023; 15 (2):46-49.
A pheochromocytoma (PCC) is a tumor that arises from an embryonic neural crest and produces catecholamines at high levels. The mean size of these tumors is approximated to be around 7.2 cm, with an average weight of 222 g. In this case, a 61-year-old female patient was referred to our medical center to investigate multiple episodes of hypertensive crisis associated with a huge 18 cm right adrenal mass identified on a computed tomography (CT) scan and magnetic resonance imaging (MRI). Twenty-four-hour urine analysis and blood level exhibited elevated total metanephrine and normetanephrine levels. Mass resection was performed via laparotomy en bloc. Histopathological analysis of the resected specimen confirmed PCC of the right adrenal gland weighing 1900 g. The Adrenal gland Scaled Score (PASS) was 13. PCC may reach sizes much larger than the average observed in the literature and size tends to be positively correlated to malignancy.
Karagiannis A, Mikhailidis DP, Athyros VG, et al. Pheochromocytoma: an update on genetics and management. Endocr Relat Cancer 2007;14(4):935–956. DOI: 10.1677/ERC-07-0142
Tsuboi I, Ogawa K, Kadota K, et al. Right adrenal giant cystic pheochromocytoma: a case report. Urol Case Rep 2023;48:102398. DOI: 10.1016/j.eucr.2023.102398
Wang HL, Sun BZ, Xu ZJ, et al. Undiagnosed giant cystic pheochromocytoma: a case report. Oncol Lett 2015;10(3):1444–1446. DOI: 10.3892/ol.2015.3484
Uysal E, Kırdak T, Gürer AO, et al. Giant multicystic malignant pheochromocytoma. Turk J Surg 2017;33(4):296–298. DOI: 10.5152/UCD.2015.3011
Kantorovich V, Eisenhofer G, Pacak K. Pheochromocytoma: an endocrine stress mimicking disorder. Ann N Y Acad Sci 2008;1148:462–468. DOI: 10.1196/annals.1410.081
Thompson LD. Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases. Am J Surg Pathol 2002;26(5):551–566. DOI: 10.1097/00000478-200205000-00002
Andersen KF, Altaf R, Krarup-Hansen A, et al. Malignant pheochromocytomas and paragangliomas - the importance of a multidisciplinary approach. Cancer Treat Rev 2011;37(2):111–119. DOI: 10.1016/j.ctrv.2010.07.002
Dahia PL. Evolving concepts in pheochromocytoma and paraganglioma. Curr Opin Oncol 2006;18(1):1–8. DOI: 10.1097/01.cco.0000198017.45982.06
Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014;99(6):1915–1942. DOI: 10.1210/jc.2014-1498
Pan Z, Repertinger S, Deng C, et al. A giant cystic pheochromocytoma of the adrenal gland. Endocr Pathol 2008;19(2):133–138. DOI: 10.1007/s12022-008-9016-4
Elder EE, Elder G, Larsson C. Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. J Surg Oncol 2005;89(3):193–201. DOI: 10.1002/jso.20177
Gimm O, Koch CA, Januszewicz A, et al. The genetic basis of pheochromocytoma. Front Horm Res 2004;31:45–60. DOI: 10.1159/000074657
Alexandraki KI, Grossman AB. Adrenal incidentalomas: ‘the rule of four’. Clin Med (Lond) 2008;8(2):201–204. DOI: 10.7861/clinmedicine.8-2-201
Kota SK, Kota SK, Panda S, et al. Pheochromocytoma: an uncommon presentation of an asymptomatic and biochemically silent adrenal incidentaloma. Malays J Med Sci 2012;19(2):86–91.
Kudva YC, Sawka AM, Young WF. Clinical review 164: the laboratory diagnosis of adrenal pheochromocytoma: the Mayo Clinic experience. J Clin Endocrinol Metab 2003;88(10):4533–4539. DOI: 10.1210/jc.2003-030720
Roizen MF, Horrigan RW, Koike M, et al. A prospective randomized trial of four anesthetic techniques for resection of pheochromocytoma. Anesthesiol. 1982;57(3):A43. DOI: 10.1097/00000542-198209001-00043