Asymptomatic Presacral Paraganglioma: Management of an Unpredictable Intraoperative Finding

Abstract

Paragangliomas are rare neuroendocrine tumors originating from the embryological neural crest. We report a rare case of a patient with an asymptomatic presacral mass (incidentaloma) who experienced an unpredictable intraoperative hypertensive crisis after manipulation of the tumor. Presacral neoplasms pose a diagnostic and therapeutic challenge due to their obscure anatomical location and the difficulty in performing an R0 excision. Furthermore, the management of asymptomatic paragangliomas requires a high level of clinical suspicion and expertise due to potential life-threatening intraoperative complications.

Keywords

paraganglioma - secreting tumor - presacral paragangliomas - 131-I MIBG - neoplasm

• References

• 1 Delellis RA, Lloyd RV, Heitz PU. , et al. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Endocrine Organs. Lyon: IARC Press; 2004
• 2 Mantas D, Kandilis A, Charalampoudis P. Nonfunctioning symptomatic paraganglioma: is there an optimal follow-up for patients with extra-adrenal benign paragangliomas. J Surg Case Rep 2014; 2014 (09) rju092
• 3 Pedroso C, Robalo R, Sereno P, Barros C, Marques C. A rare abdomino-pelvic tumor: paraganglioma. Acta Med Port 2015; 28 (01) 114-116
• 4 Guo Q, Li B, Guan J, Yang H, Wu Y. Intraoperative diagnosis of functional retroperitoneal multiple paraganglioma: a case report. Oncol Lett 2012; 4 (04) 829-831
• 5 Saxena D, Pandey A, Bugalia RP. , et al. Management of presacral tumors: our experience with posterior approach. Int J Surg Case Rep 2015; 12: 37-40
• 6 Lenders JW, Duh QY, Eisenhofer G. , et al; Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014; 99 (06) 1915-1942
• 7 Fishbein L. Pheochromocytoma and paraganglioma: genetics, diagnosis, and treatment. Hematol Oncol Clin North Am 2016; 30 (01) 135-150
• 8 Lee JA, Duh QY. Sporadic paraganglioma. World J Surg 2008; 32 (05) 683-687
• 9 Plouin PF, Amar L, Dekkers OM. , et al; Guideline Working Group. European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. Eur J Endocrinol 2016; 174 (05) G1-G10
• 10 NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines). J Natl Compr Canc Netw 2018;16(06):693–702
• 11 Carrasquillo JA, Pandit-Taskar N, Chen CC. I-131 metaiodobenzyl guanidine therapy of pheochromocytoma and paraganglioma. Semin Nucl Med 2016; 46 (03) 203-214
• 12 van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP. (131)I-MIBG therapy for malignant paraganglioma and phaeochromocytoma: systematic review and meta-analysis. Clin Endocrinol (Oxf) 2014; 80 (04) 487-501
• 13 Jimenez C, Erwin W, Chasen B. Targeted radionuclide therapy for patients with metastatic pheochromocytoma and paraganglioma: from low-specific-activity to high-specific-activity Iodine-131 metaiodobenzylguanidine. Cancers (Basel) 2019; 11 (07) E1018
• 14 Ramachandran R, Rewari V. Factors affecting the haemodynamic behaviour of patients undergoing pheochromocytoma and paraganglioma removal: a review. Cardiovasc Endocrinol 2017; 6 (02) 73-80
• 15 Dziki Ł, Włodarczyk M, Sobolewska-Włodarczyk A. , et al. Presacral tumors: diagnosis and treatment—a challenge for a surgeon. Arch Med Sci 2019; 15 (03) 722-729
• 16 Sasikumar A, Bhan C, Jenkins JT, Antoniou A, Murphy J. Systematic review of pelvic exenteration with en bloc sacrectomy for recurrent rectal adenocarcinoma: R0 resection predicts disease-free survival. Dis Colon Rectum 2017; 60 (03) 346-352