A rare case of extra adrenal retroperitoneal paraganglioma: case report
DOI:
https://doi.org/10.18203/2349-2902.isj20240754Keywords:
Extra adrenal retroperitoneal paraganglioma, Catecholamine excess, Retroperitoneal paraganglioma, Endocrine neoplasmAbstract
Extra adrenal retroperitoneal paragangliomas are neuro endocrine neoplasms with extremely rare incidence and a wide plethora of clinical presentations. They originate from the neural crest cells interspersed throughout the body. They can present with vague symptoms of pain abdomen, hypertension, palpitations and in severe cases with renal failure, and shock owing to catecholamine excess. On the other end of the spectrum they can be totally asymptomatic and detected incidentally. The multitude of clinical presentations and lack of specific diagnostic tests hence pose a great difficulty in the pre-operative diagnosis of the disease. We are presenting a case of a 20-year-old female with vague symptoms to highlight the management and clinical diagnosis of extra adrenal retroperitoneal paraganglioma.
References
Andersen KF, Altaf R, Krarup-Hansen A. Malignant pheochromocytomas and paragangliomas the importance of a multidisciplinary approach. Cancer Treat Rev. 2011;37(2):111-9.
Hemalatha AL, Geeta KA, Anoosha K, Ashok KP, Deepa MR. Extra- adrenal silent retroperitoneal paraganglioma: report of a rare case. J Clin Diagn Res. 2014;8(11):FD06-7.
Hayes WS, Davidson AJ, Grimley PM, Hartman DS. Extraadrenal retroperitoneal paraganglioma: clinical, pathologic, and CT findings. AJR Am J Roentgenol. 1990;155(6):1247-50.
Wen J, Li HZ, Ji ZG, Mao QZ, Shi BB, Yan WG. A decade of clinical experience with extra-adrenal paragangliomas of retroperitoneum: Report of 67 cases and a literature review. Urol Ann. 2010;2(1):12-6.
Date A, Khadav B, Nongmaithem M. Paraganglioma: A case report. J Evid Based Med Healthc. 2016;3(5):173-5.
Limaiem F, Bouraoui S, Bouslama S, Haddad I, Lahmar A, Regaya SM. Retroperitoneal non-functioning paraganglioma: A case report. J Interdiscipl Histopathol. 2013;1(3):168-71.
Elder EE, Elder G, Larsson C. Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. J Surg Oncol. 2005;89(3):193-201.
Unger N, Pitt C, Schmidt IL, Walz MK, Schmid KW, Philipp T, et al. Diagnostic value of various biochemical parameters for the diagnosis of pheochromocytoma in patients with adrenal mass. Eur J Endocrinol. 2006;154(3):409-17.
Timmers HJ, Pacak K, Huynh TT, Abu-Asab M, Tsokos M, Merino MJ, et al. Biochemically silent abdominal paragangliomas in patients with mutations in the succinate dehydrogenase subunit B gene. J Clin Endocrinol Metab. 2008;93(12):4826-32.
Sclafani LM, Woodruff JM, Brennan MF. Extraadrenal retroperitoneal paragangliomas: natural history and response to treatment. Surgery. 1990;108(6):1124-9.
Parmar K, Chandna A, Kumar S. Retroperitoneal paraganglioma: a chameleon masquerading as an adrenal pheochromocytoma. Ann R Coll Surg Engl. 2019;101(2):e62-5.
Ji XK, Zheng XW, Wu XL, Yu ZP, Shan YF, Zhang QY, et al. Diagnosis and surgical treatment of retroperitoneal paraganglioma: A single-institution experience of 34 cases. Oncol Lett. 2017;14(2):2268-80.
Lack EE, Cubilla AL, Woodruff JM, Lieberman PH. Extra-adrenal paragangliomas of the retroperitoneum: A clinicopathologic study of 12 tumors. Am J Surg Pathol. 1980;4(2):109-20.
