Laparoscopic adrenalectomy as an effective approach to massive bilateral pheochromocytomas

BMJ Case Rep. 2017 Sep 7:2017:bcr2017221009. doi: 10.1136/bcr-2017-221009.

Abstract

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.

Keywords: adrenal disorders; thyroid disease.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Gland Neoplasms / diagnostic imaging
  • Adrenal Gland Neoplasms / pathology
  • Adrenal Gland Neoplasms / surgery
  • Adrenalectomy / methods*
  • Carcinoma, Medullary / pathology
  • Carcinoma, Medullary / surgery
  • Carcinoma, Neuroendocrine / diagnosis
  • Carcinoma, Neuroendocrine / pathology
  • Carcinoma, Neuroendocrine / surgery
  • Female
  • Humans
  • Laparoscopy / methods*
  • Magnetic Resonance Imaging
  • Multiple Endocrine Neoplasia / genetics
  • Multiple Endocrine Neoplasia / pathology
  • Multiple Endocrine Neoplasia / surgery
  • Multiple Endocrine Neoplasia Type 2a / genetics
  • Multiple Endocrine Neoplasia Type 2a / pathology
  • Multiple Endocrine Neoplasia Type 2a / surgery
  • Mutation
  • Pheochromocytoma / diagnosis*
  • Pheochromocytoma / drug therapy
  • Pheochromocytoma / pathology
  • Pheochromocytoma / surgery*
  • Proto-Oncogene Proteins c-ret / genetics
  • Thyroid Neoplasms / diagnosis
  • Thyroid Neoplasms / pathology
  • Thyroid Neoplasms / surgery
  • Thyroidectomy
  • Treatment Outcome
  • Young Adult

Substances

  • Proto-Oncogene Proteins c-ret

Supplementary concepts

  • Thyroid cancer, medullary