Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 2/2016

01-02-2016 | Endocrine Tumors

Limited Parathyroidectomy in Multiple Endocrine Neoplasia Type 1-Associated Primary Hyperparathyroidism: A Setup for Failure

Authors: Naris Nilubol, MD, Lee S. Weinstein, MD, William F. Simonds, MD, Robert T. Jensen, MD, Stephen J. Marx, MD, Electron Kebebew, MD

Published in: Annals of Surgical Oncology | Issue 2/2016

Login to get access

Abstract

Background

Recently, some surgeons have suggested that minimally invasive parathyroidectomy guided by preoperative localizing studies of patients with multiple endocrine neoplasia type 1 (MEN1)-associated primary hyperparathyroidism (pHPT) provides an acceptable outcome while minimizing the risk of hypoparathyroidism. This study aimed to evaluate the outcome for MEN1 patients who underwent limited parathyroidectomy compared with subtotal parathyroidectomy.

Methods

The authors performed a retrospective analysis of 99 patients with MEN1-associated pHPT who underwent at least one parathyroid operation at their institution. Preoperative imaging studies, intraoperative findings, and clinical outcomes for patients were compared.

Results

A total of 99 patients underwent 146 operations. Persistent pHPT was significantly higher in patients whose initial operations involved removal of 1 or 2 glands (69 %) or 2.5 to 3 glands (20 %) compared with those who had 3.5 or more glands removed (6 %) (P < 0.01). Persistent pHPT occurred in 5 % of all operations that cumulatively removed 3.5 or more parathyroid glands compared with 40 % of operations that removed 3 or fewer glands (P < 0.01). The single largest parathyroid gland was correctly identified preoperatively in 69 % (22/32) of the patients. However, preoperative localizing studies missed enlarged contralateral parathyroid glands in 86 % (19/22) of these patients. Preoperative localizing studies missed the largest contralateral parathyroid gland in 16 % (5/32) of the patients.

Conclusions

Limited parathyroidectomy in MEN1 is associated with a high failure rate and should not be performed. Preoperative identification of a single enlarged parathyroid gland in MEN1 is not reliable enough to justify unilateral neck exploration because additional enlarged contralateral parathyroid glands are frequently missed.
Literature
1.
Brandi ML, Marx SJ, Aurbach GD, Fitzpatrick LA. Familial multiple endocrine neoplasia type I: a new look at pathophysiology. Endocr Rev. 1987;8:391–405.PubMedCrossRef
2.
3.
Uchino S, Noguchi S, Sato M, et al. Screening of the MEN1 gene and discovery of germ-line and somatic mutations in apparently sporadic parathyroid tumors. Cancer Res. 2000;60:5553–7.PubMed
4.
Vasen HF, Griffioen G, Lips CJ, Struyvenberg A, van Slooten EA. Screening of families predisposed to cancer development in The Netherlands. Anticancer Res. 1990;10:555–63.PubMed
5.
Pieterman CR, Schreinemakers JM, Koppeschaar HP, et al. Multiple endocrine neoplasia type 1 (MEN1): its manifestations and effect of genetic screening on clinical outcome. Clin Endocr. 2009;70:575–81.PubMedCrossRef
6.
Carty SE, Helm AK, Amico JA, et al. The variable penetrance and spectrum of manifestations of multiple endocrine neoplasia type 1. Surgery. 1998;124:1106–13; discussion 1113–1104.PubMedCrossRef
7.
Marx SJ. Multiple endocrine neoplasia type 1. In: Scriver CB, Beaudet AL; Sly WS, et al (ed) The metabolic and molecular bases of inherited disease. 8th ed. New York: McGraw-Hill; 2001. P. 943–66.
8.
Lee CH, Tseng LM, Chen JY, Hsiao HY, Yang AH. Primary hyperparathyroidism in multiple endocrine neoplasia type 1: individualized management with low recurrence rates. Ann Surg Oncol. 2006;13:103–9.PubMedCrossRef
9.
Pieterman CR, van Hulsteijn LT, den Heijer M, et al. Primary hyperparathyroidism in MEN1 patients: a cohort study with longterm follow-up on preferred surgical procedure and the relation with genotype. Ann Surg. 2012;255:1171–8.PubMedCrossRef
10.
Elaraj DM, Skarulis MC, Libutti SK, et al. Results of initial operation for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Surgery. 2003;134:858–64; discussion 864–855 PubMedCrossRef
11.
Schreinemakers JM, Pieterman CR, Scholten A, Vriens MR, Valk GD, Rinkes IH. The optimal surgical treatment for primary hyperparathyroidism in MEN1 patients: a systematic review. World J Surg. 2011;35:1993–2005.PubMedCrossRef
12.
Katai M, Sakurai A, Ikeo Y, Hashizume K. Primary hyperparathyroidism in patients with multiple endocrine neoplasia type 1: comparison with sporadic parathyroid adenomas. Horm Metab Res. 2001;33:499–503.PubMedCrossRef
13.
Kraimps JL, Duh QY, Demeure M, Clark OH. Hyperparathyroidism in multiple endocrine neoplasia syndrome. Surgery. 1992;112:1080–6; discussion 1086–8.PubMed
14.
Norton JA, Cornelius MJ, Doppman JL, Maton PN, Gardner JD, Jensen RT. Effect of parathyroidectomy in patients with hyperparathyroidism, Zollinger-Ellison syndrome, and multiple endocrine neoplasia type I: a prospective study. Surgery. 1987;102:958–66.PubMed
15.
Prinz RA, Gamvros OI, Sellu D, Lynn JA. Subtotal parathyroidectomy for primary chief cell hyperplasia of the multiple endocrine neoplasia type I syndrome. Ann Surg. 1981;193:26–9.PubMedPubMedCentralCrossRef
16.
Proye C, Carnaille B, Quievreux JL, Combemale F, Oudar C, Lecomte-Houcke M. Late outcome of 304 consecutive patients with multiple gland enlargement in primary hyperparathyroidism treated by conservative surgery. World J Surg. 1998;22:526–9; discussion 529–30.PubMedCrossRef
17.
Slepavicius A, Beisa V, Janusonis V, Strupas K. Focused versus conventional parathyroidectomy for primary hyperparathyroidism: a prospective, randomized, blinded trial. Langenbecks Arch Surg. 2008;393:659–66.PubMedCrossRef
18.
Hellman P, Skogseid B, Juhlin C, Akerstrom G, Rastad J. Findings and long-term results of parathyroid surgery in multiple endocrine neoplasia type 1. World J Surg. 1992;16:718–22; discussion 722–713.PubMedCrossRef
19.
Versnick M, Popadich A, Sidhu S, Sywak M, Robinson B, Delbridge L. Minimally invasive parathyroidectomy provides a conservative surgical option for multiple endocrine neoplasia type 1 primary hyperparathyroidism. Surgery. 2013;154:101–5.PubMedCrossRef
20.
Prichard RS, O’Neill CJ, Oucharek JJ, et al. Is focused minimally invasive parathyroidectomy appropriate for patients with familial primary hyperparathyroidism? Ann Surg Oncol. 2011;19:1264–8.PubMedCrossRef
21.
Marx SJ, Agarwal SK, Kester MB, et al. Multiple endocrine neoplasia type 1: clinical and genetic features of the hereditary endocrine neoplasias. Recent Prog Horm Res. 1999;54:397–438; discussion 438–99.PubMed
22.
Brandi ML, Gagel RF, Angeli A, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86:5658–71.PubMedCrossRef
23.
Nilubol N, Weinstein L, Simonds WF, et al. Preoperative localizing studies for initial parathyroidectomy in MEN1 syndrome: is there any benefit? World J Surg. 2012;36:1368–74.PubMedCrossRef
24.
Chen CC, Skarulis MC, Fraker DL, Alexander R, Marx SJ, Spiegel AM. Technetium-99 m-sestamibi imaging before reoperation for primary hyperparathyroidism. J Nucl Med. 1995;36:2186–91.PubMed
25.
Feingold DL, Alexander HR, Chen CC, et al. Ultrasound and sestamibi scan as the only preoperative imaging tests in reoperation for parathyroid adenomas. Surgery. 2000;128:1103–9; discussion 1109–10.PubMedCrossRef
26.
O’Riordain DS, O’Brien T, Grant CS, Weaver A, Gharib H, van Heerden JA. Surgical management of primary hyperparathyroidism in multiple endocrine neoplasia types 1 and 2. Surgery. 1993;114:1031–7; discussion 1037–9.PubMed
27.
Burgess JR, David R, Greenaway TM, Parameswaran V, Shepherd JJ. Osteoporosis in multiple endocrine neoplasia type 1: severity, clinical significance, relationship to primary hyperparathyroidism, and response to parathyroidectomy. Arch Surg. 1999;134:1119–23.PubMedCrossRef
28.
Dotzenrath C, Cupisti K, Goretzki PE, et al. Long-term biochemical results after operative treatment of primary hyperparathyroidism associated with multiple endocrine neoplasia types I and IIa: is a more or less extended operation essential? Eur J Surg. 2001;167:173–8.PubMedCrossRef
29.
Cooper DS, Doherty GM, Haugen BR, et al. Revised American Thyroid Association management guidelines for patients with thyroid nodules and differentiated thyroid cancer. Thyroid. 2009;19:1167–214.PubMedCrossRef
30.
Goudet P, Cougard P, Verges B, et al. Hyperparathyroidism in multiple endocrine neoplasia type I: surgical trends and results of a 256-patient series from Groupe D’etude des Neoplasies Endocriniennes Multiples Study Group. World J Surg. 2001;25:886–90.PubMedCrossRef
31.
Nilubol N, Weisbrod AB, Weinstein LS, et al. Utility of intraoperative parathyroid hormone monitoring in patients with multiple endocrine neoplasia type 1-associated primary hyperparathyroidism undergoing initial parathyroidectomy. World J Surg. 2013;37:1966–72.PubMedCrossRef
Metadata
Title
Limited Parathyroidectomy in Multiple Endocrine Neoplasia Type 1-Associated Primary Hyperparathyroidism: A Setup for Failure
Authors
Naris Nilubol, MD
Lee S. Weinstein, MD
William F. Simonds, MD
Robert T. Jensen, MD
Stephen J. Marx, MD
Electron Kebebew, MD
Publication date
01-02-2016
Publisher
Springer US
Published in
Annals of Surgical Oncology / Issue 2/2016
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-015-4865-9

Other articles of this Issue 2/2016

Annals of Surgical Oncology 2/2016 Go to the issue

Translational Research and Biomarkers

Relationship Between Increased Expression of the Axl/Gas6 Signal Cascade and Prognosis of Patients with Upper Tract Urothelial Carcinoma

Gastrointestinal Oncology

Conditional Survival After Cytoreductive Surgery with Heated Intraperitoneal Chemotherapy for Low- and High-Grade Appendiceal Primaries

Pancreatic Tumors

Systematic Review and Meta-Analysis of Enucleation Versus Standardized Resection for Small Pancreatic Lesions

Pancreatic Tumors

Outcomes of Distal Pancreatectomy for Pancreatic Ductal Adenocarcinoma in the Netherlands: A Nationwide Retrospective Analysis

Endocrine Tumors

Patients Treated at Low-Volume Centers have Higher Rates of Incomplete Resection and Compromised Outcomes: Analysis of 31,129 Patients with Papillary Thyroid Cancer

Breast Oncology

Microcalcifications in 1657 Patients with Pure Ductal Carcinoma in Situ of the Breast: Correlation with Clinical, Histopathologic, Biologic Features, and Local Recurrence