Phaeochromocytoma in Nepal – A Single Centre Experience

Abstract

Background Phaeochromocytomas are rare tumors of chromaffin cells of neural crest that classically present with symptoms of catecholamine excess such as palpitations, headache and sweating. They are diagnosed by measuring plasma or urinary levels of catecholamines or their metabolites. Anatomic localization is done by computed tomographic scan or magnetic resonance imaging, or meta-iodobenzyl guanidine scan in certain cases. Adequate preoperative catecholamine blockade prevents perioperative hemodynamic instability. Objectives To study the clinical spectrum and management of phaeochromocytomas in a tertiary care centre, Tribhuvan University Teaching Hospital, in Nepal. Methods Retrospective review of case records of histologically proven cases of phaeochromocytomas from 2008 -2011 was done, and data collected on clinical spectrum, diagnostic modalities, perioperative management and follow-up. Results Tweleve cases of phaeochromocytomas were operated. The mean age was 36.41±14.07 years. There were 2 bilateral phaeochromocytomas and 1 extra-adrenal paraganglioma. Apart from the common symptoms of catecholamine excess, patients had atypical presentations like psychiatric manifestations and blurred vision. A combination of urinary Vanillyl Mandelic Acid and computed tomographic scan was used for diagnosis, and open surgery was done in all cases. Pre-operative blood pressure control was achieved by prazocin or calcium channel blockers. Ten patients had intraoperative surge in blood pressure. There were no major morbidity or mortality. Three patients had high blood pressure post-operatively, but were effectively managed with antihypertensives. Conclusion Phaeochromocytomas can have variable presentation. Good preoperative preparation and perioperative management can result in an excellent outcome. Kathmandu University Medical Journal | VOL.10 | NO. 3 | ISSUE 39 | JUL- SEP 2012 | Page 52-55 DOI: http://dx.doi.org/10.3126/kumj.v10i3.8020

Is there an optimal preoperative management strategy for phaeochromocytoma/paraganglioma?

“…recently reviewed studies comparing pretreatment with PBZ vs DX, and concluded that there was no evidence to suggest superiority of one agent over the other, and that alpha‐adrenoceptor blockade per se was efficacious. In another retrospective series, preoperative treatment with prazosin was associated with no deaths, although significant intraoperative hypertensive surges occurred in 83% of treated patients . Successful surgical outcomes following preoperative urapidil administration have also been reported.…”

“…Evidence for efficacy of selective α 1 ‐adrenoceptor antagonists in the preoperative management of PPGLs exists mainly for doxazosin (DX) . In one study DX performed as well as PBZ with respect to intraoperative haemodynamic stability, with fewer reported side‐effects, episodes of intraoperative tachycardia and postoperative fluid requirements, and no difference in mortality . In contrast, other groups observed that pretreatment with DX resulted in higher systolic blood pressures before and after anaesthetic induction compared with PBZ .…”

“…In another retrospective series, preoperative treatment with prazosin was associated with no deaths, although significant intraoperative hypertensive surges occurred in 83% of treated patients. 21 Successful surgical outcomes following preoperative urapidil administration have also been reported. However, hypertensive surges occurred at induction and/or tumour manipulation in all patients.…”

“…20,21 In one study DX performed as well as PBZ with respect to intraoperative haemodynamic stability, with fewer reported side-effects, episodes of intraoperative tachycardia and postoperative fluid requirements, and no difference in mortality. 21 In contrast, other groups observed that pretreatment with DX resulted in higher systolic blood pressures before and after anaesthetic induction compared with PBZ. 23 Van der Zee et al 22 recently reviewed studies comparing pretreatment with PBZ vs DX, and concluded that there was no evidence to suggest superiority of one agent over the other, and that alpha-adrenoceptor blockade per se was efficacious.…”

Does this patient have pheochromocytoma? A systematic review of clinical signs and symptoms

“…western region of Sweden. 1950–1997 121 47 (7–71) 53,68 [ 44 ] N. Sharma et al, 2001 Tertiary care hospital, Chandigarh, India, 1989–1996 30 24 (17–31) 17, 13 [ 45 ] Masky P et al 2012 Tribhuvan University Teaching Hospital, Maharajgunj, Kathmandu, Nepal. 2008–2011 12 36 (12–65) 5,7 [ 46 ] …”