Biochemically silent normotensive colossal pheochromocytoma
DOI:
https://doi.org/10.18203/2349-2902.isj20243462Keywords:
Giant pheochromocytoma, Paraganglinoma, NormotensiveAbstract
A case presentation about rare giant pheochromocytoma of about a 50 year old female patient came to our out-patient department of general surgery with epigastric and right flank discomfort for past 1 day, abdominal distension and constipation for past 6 months. Patient’s blood pressure was normal without any postural variations and with no features of androgenic excess. All routine investigations were normal. Biochemical profile of urinary [VMA, metanephrine and normetanephrine], serum [cortisol, metanephrine, normetanephrine], serum aldosterone and plasma renin activity are measured normal. Contrast enhanced CT abdomen done suggestive of large well defined heterogeneously enhancing soft tissue lesion of size 21×22×28 cm noted involving abdominal cavity more on right side arising from right lumbar region was identified. Patient underwent exploratory laparotomy and identified that mass was arising from right adrenal gland and right kidney which was difficult to be dissected from the mass, for which right nephrectomy was also done along with mass removal of size 28×28×20 cm and weight of 4200 gm. Postoperative period was uneventful. Histopathological examination showed gross feature of single large encapsulated globular structure with right kidney attached to it. Mass was encapsulated with total size of 24×25×14 cm, greyish in colour and soft to firm in consistency. On cut section focal areas of greyish yellow friable tissue with few small cystic areas, large hemorrhagic area and focal greyish white areas was seen. Microscopic feature of these tumor cells was arranged in well defined nests (zellballen) bound by highly vascularized stroma. Cells were polygonal shaped with finely granular amphophilic cytoplasam, round to oval nuclei with salt and pepper chromatin and at places prominent nucleoli were seen. Special staining with PAS done and it was negative.
References
Wheeler MH, Sadler GP. Disorders of the adrenal glands. In: Cuschieri SA, Steele RJ, Moossa AR, editors. Essential surgical practice. (4th edn) UK: Arnold Ltd. 2002;131-36.
Mansmann G, Lau J, Balk E, Rothberg M, Miyachi Y, Bornstein SR. The clinically inapparent adrenal mass: updatein diagnosis and management. Endocr Rev. 2004;25(2):309-40.
Rothmund M (2008) Adrenal glands and other endocrine disorders. In:Williams NS, Bulstrode CJK, O’Connell PR, editors. Bailey and Love’s short practice of surgery. (25th edn) UK: Edward Arnold Ltd, 813-14.
Zuber SM, Kantorovich V, Pacak K. Hypertension in pheochromocytoma: characteristics and treatment. Endocrinol Metab Clin North Am. 2011;40:295-311.
Guerrero MA, Schreinemakers JM, Vriens MR, Insoo S, Jimmy H, Wen TS, et al. Clinical spectrum of pheochromocytoma. J Am College Surgeons. 2009;209:727-32.
Lu Y, Li P, Gan W, Zhao X, Shen S, Feng W, et al. Clinical and pathological characteristics of hypertensive and normotensive adrenal pheochromocytomas. Exp Clin Endocrinol Diabetes. 2016;124(6):372-9.
Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet. 2005;366:665-75.
Bravo EL, Tagle R. Pheochromocytoma: state-of-the-art and future prospects. Endocr Rev. 2003;24:539-53.
Mannelli M, Lenders JW, Pacak K, Parenti G, Eisenhofer G. Subclinical phaeochromocytoma. Best Pract Res Clin Endocrinol Metab. 2012;26:507-15.
Kopetschke R, Slisko M, Kilisli A. Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma. Eur J Endocrinol. 2009;161:355-61.
Duh QY, Liu C, Tyrrell JB, Adrenals. In: Doherty GM, Current Diagnosis and treatment: surgery. (13th edn) USA: McGraw Hill companies. 2011;737-50.
Därr R, Lenders JW, Hofbauer LC, Naumann B, Bornstein SR. Pheochromocytoma-update on disease management. Ther Adv Endocrinol Metab. 2012;3:11-26.
Agarwal A, Gupta S, Mishra AK, Singh N, Mishra SK. Normotensive pheochromocytoma: institutional experience. World J Surg. 2005;29(9):1185-8.
Shao Y, Chen R, Shen ZJ, Teng Y, Huang P, Rui WB, et al. Preoperative alpha blockade for normotensive pheochromocytoma: is it necessary? J Hypertens. 2011;29(12):2429-32.
Kantorovich V, Pacak K. Pheochromocytoma and paraganglioma. Progress Brain Res. 2010;182:343-73.
Noshiro T, Shimizu K, Watanabe T, Akama H, Shibukawa S, Miura W, et al. Changes in clinical features and long-term prognosis in patients with pheochromocytoma. Am J Hypertension. 2000;13:35-43.
Bravo EL, Tagle R. Pheochromocytoma: state-of-the-art and future prospects. Endocrine Rev. 2003;24:539-53.
Zhu Y, He HC, Su TW. Selective a1-adrenoceptor antagonist (controlled release tablets) in preoperative management of pheochromocytoma. Endocrine Connections. 2010;38:254-9.
Fung MM, Viveros OH, O’Connor DT. Diseases of the adrenal medulla. Acta Physiol (Oxf). 2008;192:325-35.
Manger WM. The protean manifestations of pheochromocytoma. Hormone Metabol Res. 2009;41:658-63.
Ito Y, Fujimoto Y, Obara T. The role of epinephrine, norepinephrine, and dopamine in blood pressure disturbances in patients with pheochromocytoma. World J Surg. 1992;16:759-63.
Ariton M, Juan CS, AvRuskin TW. Pheochromocytoma: clinical observations from a Brooklyn tertiary hospital. Endocrine Practice. 2000;6:249-52.
Eisenhofer G, Huynh TT, Elkahloun A, John CM, Gennady B, Linehan WM, et al. Differential expression of the regulated catecholamine secretory pathway in different hereditary forms of pheochromocytoma. Am J Physiol Endocrinol Metab. 2008;295(5):E1223.
Eisenhofer G, Pacak K, Huynh TT, Thanh-Truc H, Nan Q, Gennady By, et al. Catecholamine metabolomic and secretory phenotypes in phaeochromocytoma. Endocrine-Related Cancer. 2011;18(1):97-111.
Eisenhofer G, Walther MM, Huynh TT, Li ST, Bornstein SR, Vortmeyer A, et al Pheochromocytomas in von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2 display distinct biochemical and clinical phenotypes. J Clin Endocrinol Metabol. 2001;86(5):1999-2008.
Lenders JW, Duh QY, Eisenhofer G, Anne-Paule GR, Stefan KGG, Mohammad HM, et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metabol. 2014;99(6):1915-42.
Kimura N, Watanabe T, Noshiro T, Soichiro S, Yukio M. Histological grading of adrenal and extra-adrenal pheochromocytomas and relationship to prognosis: a clinicopathological analysis of 116 adrenal pheochromocytomas and 30 extra-adrenal sympathetic paragangliomas including 38 malignant tumors. Endocrine Pathol. 2005;16(1):23-32.
Haissaguerre M, Courel M, Caron P, Sophie D, Christophe D, Philippe G, et al. Normotensive incidentally discovered pheochromocytomas display specific biochemical, cellular, and molecular characteristics. J Clin Endocrinol Metabol. 2013;98(11):4346-54.
Eiden LE, Schafer MK, Weihe E. The vesicular amine transporter family (SLC18): amine/proton antiporters required for vesicular accumulation and regulated exocytotic secretion of monoamines and acetylcholine. Pflugers Archiv: Eur J Physiol. 2004;447(5)636-40.
Sane CS-Jn T, Raade M. Is biochemical screening for pheochromocytoma in adrenal incidentalomas expressing low unenhanced attenuation on computed tomography necessary? J Clin Endocrinol Metab. 2012;97(6):2077-83.
Leung K, Stamm M, Raja A. Pheochromocytoma: the range of appearances on ultrasound, CT, MRI, and functional imaging. Am J Roentgenol. 2013;200:370-8.
Neumann HP, Berger DP, Sigmund G, Blum U, Schmidt D, Parmer RJ, Volk B, Kirste G. Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. N Engl J Med. 1993;329(21):1531-8.
Basso L, Lepre L, Melillo M, Fora F, Mingazzini PL, Tocchi A. Giant pheochromocytoma: case report. Ir J Med Sci. 1996;165(1):57-9.
Arcos CT, Luque VR, Luque JA, Gracia PM, Jimenez AB, Munoz MM. Malignant giant pheochromocytoma: a case report and review of the literature. Can Urol Assoc J. 2009;3(6):E89-91.
Maharaj R, Parbhu S, Ramcharan W, Baijoo S, Greaves W, Harnanan D, et al. Giant cystic pheochromocytoma with low risk of malignancy: a case report and literature review. Case Rep Oncol Med. 2017;2017:4638608.
Grissom JR, Yamase HT, Prosser PR. Giant pheo-chromocytoma with sarcoidosis. SouthMed J. 1979;72(12):1605-7.
Zelinka T, Musil Z, Duskova J, Burton D, Merino MJ. Metastatic pheochromocytoma: does the size and age matter? Eur J Clin Invest. 2011;41(10):1121-8.
Garg MK, Kharb S, Brar KS, Gundgurthi A, Mittal R. Medical management of pheochromocytoma: Role of the endocrinologist. Indian J Endocrinol Metab. 2011;15(4):S329-36.
Bellezza G, Giansanti M, Cavaliere A. Pigmented ‘‘Black’’ pheochromocytoma of the adrenal gland-a case report and review of the literature. Arch Pathol Lab Med. 2004;128:125-8.
Steinhoff M, Wells S Jr, DeSchryver-Kecskemeti K. Stromal amyloid in pheochromocytomas. Hum Pathol 1992;23:33-6.
Harach H, Laidler P. Combined spindle cell sarcoma/phaeochromocytoma of the adrenal. Histopathology. 1993;23(6):567-9.
Juarez D, Brown R, Ostrowski M, Reardon MJ, Lechago J, Truong LD. Pheochromocytoma associated with neuroendocrine carcinoma. A new type of composite pheochromocytoma. Arch Pathol Lab Med. 1999;123(12):1274-9.
Erlic Z, Neumann H. Clinical question: When should genetic testing be obtained in a patient with pheochromocytoma or paraganglioma? Clin Endocrinol (Oxf). 2009;70(3):354-7.
