Surgical Therapy for Familial Hyperparathyroidism

2009 ◽  
Vol 75 (7) ◽  
pp. 579-583 ◽  
Author(s):  
Jyotirmay Sharma ◽  
Collin J. Weber

Isolated familial hyperparathyroidism (FHPT) not associated with multiple endocrine neoplasia is a rare and aggressive form of primary hyperparathyroidism. The traditional management of FHPT is a bilateral neck exploration with an increased rate of multigland hyperplasia, supernumerary glands, and recurrence. A prospective database was queried, which included 1383 consecutive parathyroidectomies between 1992 and 2008, and 28 patients with FHPT were identified. Patient demographics, pathology, intraoperative parathyroid hormone (IOPTH) kinetics, recurrence patterns, and accuracy of localization studies were analyzed. Twenty-one patients underwent bilateral neck explorations as an initial surgery, and seven patients had nine unilateral neck explorations for recurrent hyperparathyroidism. Overall cure rate was 89.2 per cent with a mean follow-up of 2.9 years (range: 6 months to 9.2 years); 64.3 per cent of patients had multigland disease. IOPTH helped identify supernumerary glands in three (12.5%) patients and accurately lateralized recurrent disease in eight of nine surgeries (88.8%). Tc-99m-Sestamibi failed to identify multigland disease in 11 patients (52.3%). FHPT has a greater prevalence of multigland disease, decreased utility of sestamibi scanning, and a higher recurrence rate than sporadic primary hyperparathyroidism. In FHPT, IOPTH is a useful adjunct in identifying additional tumors and in select cases may play a role in tumor localization.

2010 ◽  
Vol 162 (2) ◽  
pp. 399-406 ◽  
Author(s):  
Janneke E Witteveen ◽  
Job Kievit ◽  
Hans Morreau ◽  
Johannes A Romijn ◽  
Neveen A T Hamdy

ObjectiveCure rate for primary hyperparathyroidism (PHPT) is reported to be 94–100% 1 year after surgery, but recent data suggest recurrence in 4% of the patients 1–5 years post-operatively. The aim of our study was to establish the cure rate and its maintenance in the long-term after parathyroidectomy (PTx) in patients with sporadic PHPT.DesignEvaluation of recurrence in patients with sporadic hyperparathyroidism who underwent PTx 1–24 years prior to the study.Patients and methodsWe identified 111 patients who underwent initial PTx between 1984 and 2008, and had no MEN-1, MEN-2, or CaR mutation; parathyroid carcinoma; a history of lithium use; or renal failure. Thirty-eight patients were lost to follow-up or were unwilling or unable to participate in the study. Cure was defined as maintenance of normal serum calcium and parathyroid hormone concentrations 6 months after PTx.ResultsCure was achieved in 68 of 73 patients studied (93%) and was sustained in all for 6±5 years.ConclusionThe cure rate of sporadic PHPT after initial surgery is 93%. When cure is achieved, this is sustained in 100% of the patients for up to 24 years post-operatively. Our data suggest that closer early follow-up is advocated in all patients undergoing PTx to definitively establish cure and to provide a safety net for those with residual gland pathology. The data do not support the need for long-term follow-up when cure is established 6 months after PTx.


2019 ◽  
Vol 85 (2) ◽  
pp. 214-218 ◽  
Author(s):  
Anatoliy V. Rudin ◽  
Travis J. McKenzie ◽  
Roberta Wermer ◽  
Geoffrey B. Thompson ◽  
Melanie L. Lyden

Primary hyperparathyroidism is the most common cause of hypercalcemia. Follow-up can be resource-intensive and costly. The aim of this study was to determine if there is a subset of patients who can be defined cured earlier than six months. This was a retrospective study of patients who underwent parathyroidectomy between January 2012 and March 2014. Patients with a history of multiple endocrine neoplasia syndrome, and secondary or tertiary hyperparathyroidism were excluded. Patients with normal preoperative calcium and parathyroid hormone (PTH) and those without six months follow-up were excluded. Patients were divided into two groups: cured and not cured. Data analysis was performed between the two groups. A total of 509 patients were screened, and 214 met our inclusion criteria: 202 in the cured category and 12 in the not cured category (94% cure rate). There was no significant difference between age, gland weight, or pre-operative PTH. There was a statistically significant difference between final intraoperative PTH (IOPTH) (37 vs 55, P = 0.008) and per cent PTH decrease (69 vs 43%, P < 0.0001). There was a significant difference between intraoperative cure rate (P < 0.0006), imaging concordance (P = 0.0115), and solitary versus multiglandular disease (P = 0.0151). Subgroup analysis in patients with concordant imaging, solitary parathyroid adenoma, and IOPTH decrease by 50 per cent to normal or near-normal correlated with a six-month cure rate of 97 per cent. Patients with primary hyperparathyroidism with concordant imaging, single-adenoma pathology, and IOPTH decrease by 50 per cent to normal or near-normal levels (15–65 pg/mL) can be considered cured and may need less frequent follow-up.


2019 ◽  
Vol 404 (4) ◽  
pp. 431-438 ◽  
Author(s):  
Emmanuelle Trébouet ◽  
Sahar Bannani ◽  
Matthieu Wargny ◽  
Christophe Leux ◽  
Cécile Caillard ◽  
...  

2021 ◽  
pp. 000313482110488
Author(s):  
Ehab Alameer ◽  
Mahmoud Omar ◽  
Marcus Hoof ◽  
Hosam Shalaby ◽  
Mohamed Abdelgawad ◽  
...  

Background Normocalcemic primary hyperparathyroidism (NCpHPT) and normohormonal primary hyperparathyroidism (NHpHPT) are recently recognized variants of primary hyperparathyroidism. Current guidelines for the management hyperparathyroidism recognize NCpHPT as one of the areas that are recommended for more research due to limited available data. Methods A retrospective review of patients who had parathyroidectomy between 2014 and 2019. We excluded patients with multiple endocrine neoplasia syndromes and secondary and tertiary hyperparathyroidism. Included patients were classified based on the biochemical profile into classic or normocalcemic hyperparathyroidism group. Collected data included demographics, preoperative localizing imaging, intraoperative parathyroid hormone levels, and postoperative cure rates. Results 261 patients were included: 160 patients in the classic and 101 patients in the normocalcemic group. Patients in the normocalcemic group had significantly more negative sestamibi scans (n = 58 [8.2%] vs 78 [51.3%], P = <.01), smaller parathyroid glands (mean weight 436.0 ± 593.0 vs 742.4 ± 1109.0 mg, P = .02), higher parathyroid hyperplasia rates (n = 51 [50.5%] vs 69 [43.1%]), and significantly higher intraoperative parathyroid hormone at 10 minutes (78.1 ± 194.6 vs 43.9 ± 62.4 1, P = .04). Positive predictive value of both intraoperative parathyroid hormone and cure rate was lower in the normocalcemic group (84.2% vs 95.7%) and (80.5% vs 95%), respectively. Conclusion Normocalcemic hyperparathyroidism is a challenging disease. Surgeons should be aware of the lower cure rate in this group, interpret intraoperative parathyroid hormone with caution, and have a lower threshold for bilateral neck exploration and 4 glands visualization.


1998 ◽  
Vol 77 (5) ◽  
pp. 404-407 ◽  
Author(s):  
Justin A. Bergman ◽  
Ronald Pallant

Over a ten-year period 26 patients were evaluated by the authors for elevated serum calcium and parathormone levels. All patients were clinically asymptomatic and were referred for parathyroid evaluation following a diagnosis of hypercalcemia made on routine SMA-12 studies. Twenty-four patients had positive thallium/technetium subtraction studies; two were suggestive of bilateral adenomata, one of which was confirmed at surgery. In two patients both thallium/technetium and technetium sestamibi scanning studies were negative. These patients were managed medically without surgical exploration. In all operative cases the patients remained normocalcemic during a six-month follow-up period. Operative times in all cases were less than one hour.


2018 ◽  
Vol 84 (8) ◽  
pp. 325-327
Author(s):  
Andrew A. Rosenthal ◽  
Rachele J. Solomon ◽  
Thomas Capasso ◽  
Stephanie A. Eyerly-Webb

1970 ◽  
Vol 117 (541) ◽  
pp. 693-698 ◽  
Author(s):  
Thomas A. Flanagan ◽  
Donald W. Goodwin ◽  
Philip Alderson

In 1964 Cutler et al. (1) reported on a family in which 11 members apparently had primary hyperparathyroidism. Seven cases were diagnosed at surgery; four other members had elevated serum calcium and other evidence of the disease. It was noted as an incidental finding that two of the affected members also suffered from severe psychiatric illness. The present study is a six-year follow-up investigation of the family from a psychiatric viewpoint, using non-hyperparathyroidism family members as controls to explore the possible relation of hyperparathyroidism to psychiatric illness.


1978 ◽  
Vol 48 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Donald O. Quest ◽  
Ronald Brisman ◽  
Joao L. Antunes ◽  
Edgar M. Housepian

✓ Ninety-nine patients with medulloblastoma who received surgery and radiotherapy, and had a statistically sufficient follow-up period were analyzed for factors influencing survival and the relevance of the “period of risk for recurrence” hypothesis. This postulate states that the period of risk for recurrence of a congenital tumor is equal to the age at presentation of illness plus 9 months' gestational time. The assumption is made that a tumor of embryonic origin will become manifest after a period of time determined by its inherent rate of growth and that tumor cells surviving treatment will multiply and present with recurrence in an equal period of time. Ten of 43 patients survived the period of risk, a presumed cure rate of 23%. None of these patients has subsequently developed evidence of tumor recurrence. Older patients at initial surgery had a somewhat greater survival rate for the first 5 years after treatment (10 of 26 older patients (38%) versus 15 of 54 younger patients (28%)), but by 10 years there was no appreciable difference in survival rates between those over 16 years of age and those younger. The beneficial effect of total neuraxis megavoltage radiotherapy is indicated by the improved 5-year survival rate from 9 of 41 patients (22%) to 16 of 39 patients (41%) with the newer techniques.


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