Abstract
Paragangliomas are tumors arising from the extra-adrenal paragangliar neural crest cells. The sympathoadrenal neuroendocrine system consists of extra-adrenal paragangliar cellular layer along the paravertebral and para-aortic axis, and the adrenal medullae. Paraganglioma should be included in the differential diagnosis of secondary erythrocytosis due to its possible ectopic erythropoietin (EPO) secretion. Thus, in this report we present a 24-year-old female patient with onset of unregulated ectopic EPO secretion, and consecutive erythrocytosis followed by hypertension, secondary to paraganglioma of multifocal retroperitoneal localization. Clinical, laboratory, and radiological investigations confirmed both an elevated EPO level and the presence of multiple paraganglioma. This paraneoplastic-mediated medical condition with high risk of cellular hyperviscosity syndrome (CHVS) requires prompt diagnosis and rapid therapeutic interventions. Initially, simple phlebotomy procedures were used; following that, tumors were surgically removed. In the course of the disease, this tumor relapsed, and urgent apheresis, as a treatment of life-threatening state, was used. The therapy performed resulted in a rapid blood viscosity depletion and a significant (P < 0.01) serum EPO reduction, as well as the general clinical benefit. Therefore, we conclude that the use of our own “multi-manner” apheresis (erythrocythapheresis plus plasma exchange), for long-time interval (until further causative therapy), effectively cross-bridged the possible hazards of EPO-dependent CHVS.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Tischler A. The adrenal medulla and extra-adrenal paraganglia. In: Kovacs K, Asa S, editors. Functional endocrine pathology. Oxford, UK: Blackwell Scientific; 1998. p. 550–95.
Ali-El-Dein B, El-Sobky A, El-Baz M, ShaaBan AA. Abdominal and pelvic extra-adrenal paraganglioma: a review of literature and a report of 7 cases. In Vivo 2002;16:249–54.
Kuaka N et al. Prediction of malignant behavior of pheochromocytomas and paragangliomas using immunohistochemical techniques. Endocr Pathol 2002;13(2):149–56.
Drenou B et al. Pheochromocytoma and secondary erythrocytosis: role of tumour erythropoietin secretion. Nouv Rev Fr Hematol 1995;37(3):197–9.
Jalil ND. Effectiveness and limits of preoperative imaging studies for the localization of pheochromocytomas and paragangliomas: a review of 282 cases. Eur J Surg 1998;164:23–8.
Terada N et al. Erythrocytosis complicated by multiple paraganglioma. Acta Haematol 1994;91(2):95–8.
Balint B et al. Cytapheresis in the treatment of cell-affected blood disorders and abnormalities. Transf Apher Sci 2006;35(1):25–31.
Balint B, and the National Apheresis Group. Apheresis in donor and therapeutic settings: recruitments vs. possibilities – a multicenter study. Transf Apher Sci 2005;33(2):229–37.
Buonuomo V et al. Malignant paraganglioma: a case report and review of the literature. Pediatr Hematol Oncol 2004;21:731–8.
Dionne JM et al. Malignant hypertension, polycythemia, and paragangliomas. J Pediatr 2006;148(4):540–5.
Balint B et al. Simple “closed-circuit” group-specific immunoadsorption system for ABO-incompatible kidney transplants. Transf Apher Sci 2007;36:225–33.
Edström Elder E et al. KI-67 and hTERT expression can aid in the distinction between malignant and benign pheochromocytoma and paraganglioma. Mod Pathol 2003;16(3):246–55.
Rezkalla MA, Rizk SN, Ryan JJ. Pheochromocytoma associated with polycythemia: case report. S D J Med 1995;48(10):349–51.
Chandra V, Thompson GB, Bower TC, Taler SJ. Renal artery stenosis and a functioning hilar paraganglioma: a rare cause of renovascular hypertension—a case report. Vasc Endovascular Surg 2004;38(4):385–90.
Donnelly S. Why is erythropoietin made in the kidney? The kidney functions as a “critmeter” to regulate the hematocrit. Adv Exp Med Biol 2003;543:73–87.
Bosio M, Barosi G. Postpheochromocytoma erythropoietin-dependent erythrocytosis. A postischemic mechanism? Am J Pediatr Hematol Oncol 1994;16(4):365–7.
Imai T et al. A case of retroperitoneal multiple functioning paraganglioma. Clin Endocrinol 1986;11:175–7.
Imai T et al. Multiple functioning paraganglioma associated with polycythemia. J Surg Oncol 1988;39(4):279–82.
Tekautz TM, Pratt CB, Jenkin JJ, Spunt SL. Pediatric extrarenal paraganglioma. J Pediatr Surg 2003;38:1317–21.
Messinezy M, Pearson TC. Apparent polycythaemia: diagnosis, pathogenesis and management. Eur J Haematol 1993;51:125–31.
Shih LY, Huang JY, Lee CT. Insulin-like growth factor I plays a role in regulating erythropoiesis in patients with end-stage renal disease and erythrocytosis. J Am Soc Nephrol 1999;10:315–22.
Myers B, Donohue SM. A case of sickle-cell erythrocytosis occurring following renal transplantation. Clin Lab Haematol 2002;24(3):175–7.
Author information
Authors and Affiliations
Corresponding author
Additional information
M. Todorovic and B. Balint contributed equally to this article.
Rights and permissions
About this article
Cite this article
Todorovic, M., Balint, B., Suvajdzic, N. et al. Triple-way therapeutic approach for paraganglioma-dependent erythrocytosis: drugs and surgery plus “multi-manner” apheresis. Med Oncol 25, 148–153 (2008). https://doi.org/10.1007/s12032-007-9008-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12032-007-9008-0