Primary hyperparathyroidism in multiple endocrine neoplasia type 1: individualized management with low recurrence rates.
BACKGROUND
To evaluate the outcomes in different surgical modalities for primary hyperparathyroidism in multiple endocrine neoplasia type 1 (MEN1) patients, intraoperative findings from a single surgeon were studied to investigate a potentially improved modality of parathyroidectomy (PTx).


METHODS
All 22 patients had PTx by a single surgeon in the past 21 years. Three modalities of PTx were used, depending on the operative findings, after all parathyroids and the thymus were identified. If fewer than three glands were enlarged, selective removal of the enlarged glands with or without biopsy of a normal-appearing gland was performed (selective PTx); if all glands were enlarged, either a subtotal PTx leaving a 50-mg remnant in situ or a total PTx with autotransplantation (TPTx + AT) was performed.


RESULTS
There were 7 men and 15 women, aged 22 to 67 years (average, 43 years). Sixteen had familial and six had sporadic MEN1. They underwent 23 operations, including 11 selective PTx, 6 subtotal PTx, and 6 TPTx + AT. On follow-up for 1 to 19 years, only one patient (4.6%) had recurrent hyperparathyroidism 5.5 years after subtotal PTx. Others had either normocalcemia (n = 14; 63.6%) or hypocalcemia (n = 7; 31.8%). Those who had either a subtotal PTx or TPTx + AT had a significantly higher rate of postoperative hypocalcemia than those who had a selective PTx (9.9% vs. 54.5%; P = .032; Fisher's exact test).


CONCLUSIONS
Primary hyperparathyroidism in our MEN1 patients was less aggressive than that reported in the literature. Selective PTx according to the intraoperative findings achieved optimal outcomes.
Lee
Chen-Hsen
CH
Department of Surgery, Taipei-Veterans General Hospital, 201, Sec. 2, Shih-Pai Rd, Taipei, Taiwan. chlee@vghtpe.gov.tw
Tseng
Ling-Ming
LM
Chen
Jui-Yu
JY
Hsiao
Hsin-Yun
HY
Yang
An-Hang
AH
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D006961:Hyperparathyroidism / Q000209:etiology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications*; D012008:Recurrence; D016896:Treatment Outcome