Page 1 - Long-term surveillance following resection of pheochromocytoma

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CUA BEST PRACTICE REPORT
ORIGINAL RESEARCH

Canadian Urological Association Best Practice Report: Long-term

surveillance following resection of pheochromocytoma

Ravi M. Kumar, MD ; Philippe D. Violette, MD ; Christopher Tran, MD ; Eva Tomiak, MD ; Jason Izard, MD ;
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Varun Bathini, MD ; Neal E. Rowe, MD 1
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1 Division of Urology, Department of Surgery, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada; Departments of Health Research Methods Evidence and Impact, and Surgery, McMaster,
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Hamilton, ON, Canada; Division of Endocrinology and Metabolism, Department of Medicine, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada; Department of Genetics, Children’s Hospital
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of Eastern Ontario, Ottawa, ON, Canada; Department of Urology, Kingston Health Sciences Centre, Queen’s University, Kingston, ON Canada; Division of Urology, Department of Surgery, University of
Saskatchewan, Saskatoon, SK, Canada
Cite as: Can Urol Assoc J 2019;13(12):372-6. http://dx.doi.org/10.5489/cuaj.6254 the scope of this review, but there are recent published
guidelines on this topic. 7
Following surgery, patients are at risk for tumor persis-
Published online September 9, 2019 tence and recurrence. Despite an overall good prognosis, the
disease can recur in up to 16% of patients within 10 years
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following surgery. Recurrences may be local or metastatic
Introduction and have been reported up to 53 years post-initial resection,
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making long-term followup essential. Extra-adrenal disease,
hereditary pheochromocytomas, right-sided tumors, bilateral
Background tumors, and larger tumors are thought to be risk factors for
recurrence. Currently, there is no consensus on the proper
Pheochromocytoma is a tumor of the catecholamine-produ- methodology for followup. There have been no randomized
cing cells of the adrenal medulla. The incidence is estimated studies addressing optimal followup nor prospective registries
to be 1–2 cases per 100 000 individuals and they make up to provide higher-quality evidence for this issue. Important
approximately 5% of incidental adrenal masses. 1 clinical questions remain regarding duration of followup and
Classical symptoms of pheochromocytomas include which tests should be used to detect and monitor recurrences.
headache, episodic perspiration, tachycardia, flushing,
nausea, and hypertension, although many tumors can be Objective
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asymptomatic. These tumors can be sporadic or heredi-These tumors can be sporadic or heredi-
tary. Familial cases account for up to 30% of tumors. 3 This Best Practice Report (BPR) aims to standardize clinical
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Pheochromocytomas can also occur outside the adrenal care regarding the long-term surveillance following surgery
gland, known as paragangliomas, in up to 25% of cases. 4 for pheochromocytoma, specifically with respect to the dur-
About 10% of pheochromocytomas are malignant. 5 ation and monitoring methods (Fig. 1).
Although there are several clinical and genetic factors asso-
ciated with an increased risk of malignancy, at this time, Methodology
there are no molecular, cellular, or histological criteria that
can reliably differentiate benign from malignant disease. 5 This BPR was developed using methodology consistent with
Therefore, malignancy is defined by the presence of clini- GRADE. We limited our evidence synthesis to previously pub-
cal metastases. The most common sites for metastases are lished studies examining the long-term followup of surgical
lymph nodes, bones, liver, and lungs. 6 patients with pheochromocytoma using PubMed, Medline,
Evaluation of suspected pheochromocytoma begins with and the Cochrane Library database. The bibliographies of rel-
confirming a biochemical disturbance by measuring plasma- evant articles were searched to avoid exclusion of meaningful
free metanephrines or urinary fractionated metanephrines, articles. In this narrative review, focus was given to system-
followed by computed tomography (CT) imaging. Once a atic reviews, related guidelines, and comparative studies. In
pheochromocytoma is confirmed, complete surgical resec- particular, a freely accessible clinical practice guideline on
tion of the tumor is advised, preferably via laparoscopic this topic was published in 2016 by the European Society
or robot-assisted adrenalectomy. The full details regarding of Endocrinology. This guideline addressed similar subject
workup and treatment of pheochromocytomas is beyond matter and scope that is of interest to the Canadian urology

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