Open AccessVaginal delivery in a patient with pheochromocytoma, medullary thyroid cancer, and primary hyperparathyroidism (multiple endocrine neoplasia type 2A, Sipple′s syndrome)
Author(s) -
Muhammad Anas Muzannara,
Nasser Tawfeeq,
Nasir Mahmood,
Mohammed K Al Harbi,
Georges Geldhof,
V. Dimitriou
Publication year - 2014
Publication title -
saudi journal of anaesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.416
H-Index - 24
eISSN - 1658-354X
pISSN - 0975-3125
DOI - 10.4103/1658-354x.136652
Subject(s) - medicine , pheochromocytoma , multiple endocrine neoplasia , medullary thyroid cancer , primary hyperparathyroidism , pregnancy , thyroid carcinoma , hyperparathyroidism , thyroidectomy , parathyroidectomy , thyroid cancer , thyroid , gynecology , parathyroid hormone , biochemistry , chemistry , genetics , biology , gene , calcium
Multiple endocrine neoplasia 2A (MEN 2A), or Sipple's syndrome is a rare inherited dominant syndrome, characterised by medullary thyroid carcinoma, adrenal pheochromocytoma and hyperparathyroidism, due to specific RET proto-oncogene mutations. The women with MEN 2A syndrome are at risk of complicated pregnancy because of unrecognised pheochromocytoma and transmission of RET mutation to the progeny. We report a case of a woman with MEN 2A diagnosed in early pregnancy. Alpha-blockade medical therapy was used effectively and time was given for fetal maturation. Uncomplicated vaginal delivery performed under epidural analgesia. Six weeks postpartum adrenalectomy, thyroidectomy and parathyroidectomy were performed uneventfully.