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BMJ Case Reports 2017; doi:10.1136/bcr-2017-221009
  • Rare disease
  • CASE REPORT

Laparoscopic adrenalectomy as an effective approach to massive bilateral pheochromocytomas

  1. Francisco Carrilho1
  1. 1Department of Endocrinology, Diabetes and Metabolism, Coimbra Hospital and University Centre, Coimbra, Portugal
  2. 2Faculty of Medicine, University of Coimbra, Coimbra, Portugal
  1. Correspondence to MD Diana Martins, diana.cruzm{at}gmail.com
  • Accepted 23 August 2017
  • Published 7 September 2017

Summary

Pheochromocytomas are catecholamine-secreting neoplasms, arising from adrenomedullary chromaffin cells. In type 2 multiple endocrine neoplasia (MEN2) syndrome, pheochromocytomas are usually benign but with predisposition to be bilateral (50%–80% of cases).

The authors present the case of a young patient diagnosed with uncommonly large bilateral cystic pheochromocytomas and simultaneous detection of medullary thyroid carcinoma. Molecular testing confirmed germline RET codon C634 mutation, consistent with MEN2A syndrome. The patient underwent bilateral laparoscopic adrenalectomy plus total thyroidectomy with central lymph node dissection without associated complications. The histopathological study of the surgical specimens revealed bilateral benign pheochromocytomas (Ki67 of 2%) and a medullary carcinoma of the thyroid T1bN0M0; R0, respectively. One year after surgery, the patient was considered free of disease.

This case demonstrates that bilateral laparoscopic adrenalectomy can be a safe and feasible approach for phechromocytomas in MEN2 syndrome, even in lesions with large diameter. However, due to elevated possibility of recurrence, patients should maintain lifelong follow-up.

Footnotes

  • Contributors DM contributed to the diagnosis, management of the patient and manuscript writing; DR contributed significantly to managing the patient; MM contributed with the genetic evaluation of the patient and relatives and is responsible for the follow-up in the setting of medullary thyroid carcinoma; FC contributed to the manuscript editing.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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