Evolution of surgical treatment of primary hyperparathyroidism in patients with multiple endocrine neoplasia type 2A.
OBJECTIVE
To determine the best surgical strategy for patients with multiple endocrine neoplasia type 2A (MEN 2A) who have primary hyperparathyroidism (PHPT).


METHODS
We performed a systematic literature review and conducted a retrospective cohort study that included patients with PHPT identified from the MEN 2A database at the University Medical Center of Utrecht, Utrecht, the Netherlands, between 1979 and 2009.


RESULTS
The review describes the course of worldwide parathyroid surgical management in MEN 2A PHPT over the past 75 years, which has evolved from aggressive parathyroid resections to minimally invasive parathyroidectomy (MIP). The study cohort included 20 patients. Primary surgery for parathyroid disease in patients with MEN 2A (n = 16) included MIP (n = 6), conventional neck exploration with resection of enlarged parathyroid gland(s) (n = 4), and resection of 1 or more enlarged gland(s) during total thyroidectomy (n = 6). Thirteen patients were initially cured after the primary operation. Five patients experienced persistent or recurrent PHPT. After MIP, 1 patient had persistent PHPT, but no patient developed recurrent PHPT during 5 years of follow-up. Five patients had hypoparathyroidism after subtotal or total parathyroidectomy with autotransplantation, but only 1 patient had transient hypoparathyroidism after MIP. One patient had transient recurrent laryngeal nerve injury after MIP.


CONCLUSIONS
Surgery for PHPT in patients with MEN 2A has evolved from aggressive conventional exploration of all 4 glands to focused MIP, which appears to be a feasible approach. MIP has low rates of persistent and recurrent PHPT, and the complications are minimal.
Scholten
Anouk
A
Department of Surgical Oncology and Endocrine Surgery, University Medical Center Utrecht, the Netherlands.
Schreinemakers
Jennifer M J
JM
Pieterman
Carolina R C
CR
Valk
Gerlof D
GD
Vriens
Menno R
MR
Borel Rinkes
Inne H M
IH
D000328:Adult; D015331:Cohort Studies; D005260:Female; D006801:Humans; D049950:Hyperparathyroidism, Primary / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D019060:Minimally Invasive Surgical Procedures / Q000379:methods*; D018813:Multiple Endocrine Neoplasia Type 2a / Q000601:surgery*; D016105:Parathyroidectomy / Q000379:methods*; D016896:Treatment Outcome; D055815:Young Adult