Primary perioperative haemodynamic effects of ß-receptor blockade in patients with catecholamine-secreting tumours.

Introduction: Guidelines for the treatment of catecholamine-producing tumours strictly recommend starting ß-receptor blocking medication only after α-receptor blockade has been established. This recommendation is supported only by non-surgical case reports. However, in clinical practice ß-receptor blockade is often started before the diagnosis of a phaeochromocytoma is made. As we routinely treat patients with catecholamine-producing tumours without α-receptor blockade, our aim was to evaluate haemodynamic changes in such patients with and without ß-receptor blockade. Methods: Perioperative blood pressure was assessed prospectively for all patients. The primary outcome was the highest pre-, intra-, and postoperative systolic blood pressure in patients with or without a ß-receptor blockade. Secondary outcomes were the incidence of intraoperative systolic blood pressure peaks >250 mm Hg and hypotensive episodes. Subsequently, a propensity score matching (PSM) analysis was performed. Results: Out of 584 phaeochromocytoma and paraganglioma resections, 383 operations were performed without α-receptor blockade (including 84 with ß-receptor blockade). Before operation and intraoperatively, patients with ß-receptor blockade presented with higher systolic blood pressure (155 [25] and 207 [62] mm Hg) than patients without ß-receptor blockade (147 [24] and 183 [52] mm Hg; P=0.006 and P=0.001, respectively). Intraoperatively, patients with ß-receptor blockade demonstrated a higher incidence of hypotensive episodes (25% without vs 41% with ß-blockade; P<0.001). After propensity score matching no difference between the groups could be confirmed. Conclusion: Overall, patients with isolated ß-receptor blockade developed higher blood pressure before operation and intraoperatively. After propensity score matching a difference could no longer be detected. Overall, ß-receptor blockade seems to be more a sign for severe disease than a risk factor for haemodynamic instability.

Use of fluorescence imaging and indocyanine green during thyroid and parathyroid surgery: Results of an intercontinental, multidisciplinary Delphi survey.

BACKGROUND: In recent years, fluorescence imaging-relying both on parathyroid gland autofluorescence under near-infrared light and angiography using the fluorescent dye indocyanine green-has been used to reduce risk of iatrogenic parathyroid injury during thyroid and parathyroid resections, but no published guidelines exist regarding its use. In this study, orchestrated by the International Society for Fluorescence Guided Surgery, areas of consensus and nonconsensus were examined among international experts to facilitate future drafting of such guidelines. METHODS: A 2-round, online Delphi survey was conducted of 10 international experts in fluorescence imaging use during endocrine surgery, asking them to vote on 75 statements divided into 5 modules: 1 = patient preparation and contraindications to fluorescence imaging (n = 11 statements); 2 = technical logistics (n = 16); 3 = indications (n = 21); 4 = potential advantages and disadvantages of fluorescence imaging (n = 20); and 5 = training and research (n = 7). Several methodological steps were taken to minimize voter bias. RESULTS: Overall, parathyroid autofluorescence was considered better than indocyanine green angiography for localizing parathyroid glands, whereas indocyanine green angiography was deemed superior assessing parathyroid perfusion. Additional surgical scenarios where indocyanine green angiography was thought to facilitate surgery are (1) when >1 parathyroid gland requires resection; (2) during redo surgeries, (3) facilitating parathyroid autoimplantation; and (4) for the predissection visualization of abnormal glands. Both parathyroid autofluorescence and indocyanine green angiography can be used during the same procedure and employing the same imaging equipment. However, further research is needed to optimize the dose and timing of indocyanine green administration. CONCLUSION: Though further research remains necessary, using fluorescence imaging appears to have uses during thyroid and parathyroid surgery.

Surgical Outcomes After Neoadjuvant Chemoradiation Followed by Curative Surgery in Patients With Esophageal Cancer: An Intergroup Phase III Trial of the Swiss Group for Clinical Cancer Research (SAKK 75/08).

OBJECTIVE: To assess the impact of surgical technique in regard to morbidity and mortality after neoadjuvant treatment for esophageal cancer. BACKGROUND: The SAKK trial 75/08 was a multicenter phase III trial (NCT01107639) comparing induction chemotherapy followed by chemoradiation and surgery in patients with locally advanced esophageal cancer. METHODS: Patients in the control arm received induction chemotherapy with cisplatin and docetaxel, followed by concomitant chemoradiation therapy with cisplatin, docetaxel, and 45Gy. In the experimental arm, the same regimen was used with addition of cetuximab. After completion of neoadjuvant treatment, patients underwent esophagectomy. The experimental arm received adjuvant cetuximab. Surgical outcomes and complications were prospectively recorded and analyzed. RESULTS: Total of 259 patients underwent esophagectomy. Overall complication rate was 56% and reoperation rate was 15% with no difference in complication rates for transthoracic versus transhiatal resections (56% vs 54%, P = 0.77), nor for video assisted thoracic surgeries (VATS) versus open transthoracic resections (67% vs 55%, P = 0.32). There was a trend to higher overall complication rates in squamous cell carcinoma versus adenocarcinoma (65% vs 51%, P = 0.035), and a significant difference in ARDS in squamous cell carcinoma with 14% versus 2% in adenocarcinoma (P = 0.0002). For patients with involved lymph nodes, a lymph node ratio of ≥0.1 was an independent predictor of PFS (HR 2.5, P = 0.01) and OS (HR 2.2, P = 0.03). CONCLUSIONS: This trial showed no difference in surgical complication rates between transthoracic and transhiatal resections. For patients with involved lymph nodes, lymph node ratio was an independent predictor of progression free survival and overall survival.

X-chromosome variants are associated with aldosterone producing adenomas.

Aldosterone-producing adenomas (APAs) are a major cause of primary aldosteronism (PA) and are characterized by constitutively producing aldosterone, which leads to hypertension. Several mutations have been identified in ion channels or ion channel-associated genes that result in APAs. To date, no studies have used a genome-wide association study (GWAS) approach to search for predisposing loci for APAs. Thus, we investigated Scandinavian APA cases (n = 35) and Swedish controls (n = 60) in a GWAS and discovered a susceptibility locus on chromosome Xq13.3 (rs2224095, OR = 7.9, 95% CI = 2.8-22.4, P = 1 × 10-7) in a 4-Mb region that was significantly associated with APA. Direct genotyping of sentinel SNP rs2224095 in a replication cohort of APAs (n = 83) and a control group (n = 740) revealed persistently strong significance (OR = 6.1, 95% CI = 3.5-10.6, p < 0.0005). We sequenced an adjacent gene, MAGEE1, of the sentinel SNP and identified a rare variant in one APA, p.Gly327Glu, which is complementary to other mutations in our primary cohort. Expression quantitative trait loci (eQTL) were investigated on the X-chromosome, and 24 trans-eQTL were identified. Some of the genes identified by trans-eQTL point towards a novel mechanistic explanation for the association of the SNPs with APAs. In conclusion, our study provides further insights into the genetic basis of APAs.

A somatic mutation in CLCN2 identified in a sporadic aldosterone-producing adenoma.

OBJECTIVE: To screen for CLCN2 mutations in apparently sporadic cases of aldosterone-producing adenomas (APAs). DESCRIPTION: Recently, CLCN2, encoding for the voltage-gated chloride channel protein 2 (ClC-2), was identified to be mutated in familial hyperaldosteronism II (FH II). So far, somatic mutations in CLCN2 have not been reported in sporadic cases of APAs. We screened 80 apparently sporadic APAs for mutations in CLCN2. One somatic mutation was identified at p.Gly24Asp in CLCN2. The male patient had a small adenoma in size but high aldosterone levels preoperatively. Postoperatively, the patient had normal aldosterone levels and was clinically cured. CONCLUSION: In this study, we identified a CLCN2 mutation in a sporadic APA comprising about 1% of all APAs investigated. This mutation was complementary to mutations in other susceptibility genes for sporadic APAs and may thus be a driving mutation in APA formation.

Pattern of and reason for postoperative residual disease in patients with advanced ovarian cancer following upfront radical debulking surgery.

OBJECTIVE: Describing the pattern of and reasons for post-operative tumor residuals in patients with advanced epithelial ovarian cancer (AOC) operated in a specialized gynecologic cancer center following a strategy of maximum upfront debulking followed by systemic chemotherapy. METHODS: All consecutive AOC-patients treated between 2005 and 2015 due to stages FIGO IIIB/IV were included in this single-center analysis. RESULTS: 739 patients were included in this analysis. In 81 (11.0%) patients, chemotherapy had already started before referral. Of the remaining 658 patients, upfront debulking was indicated in 578 patients (87.8%), while 80 patients (12.8%) were classified ineligible for upfront debulking; mostly due to comorbidities. A complete tumor resection was achieved in 66.1% of the 578 patients with upfront surgery, 25.4% had residuals 1-10mm and 8.5% had residuals exceeding 10mm, and 12.5% of patients had multifocal residual disease. Most common localization was small bowel mesentery and serosa (79.8%), porta hepatis/hepatoduodenal ligament (10.1%), liver parenchyma (4.3%), pancreas (8.0%), gastric serosa (3.2%), and tumor surrounding/infiltrating the truncus coeliacus (2.7%); 14.9% of the patients had non-resectable supra diaphragmatic lesions. Size of residual tumor was significantly associated with progression-free and overall survival. CONCLUSIONS: Upfront debulking for AOC followed by systemic chemotherapy was our main treatment strategy in almost 90% of all patients. The majority experienced a benefit by this approach; while 11.7% of patients probably did not. Understanding sites and reason for residual disease may help to develop adequate surgical training programs but also to identify patients that would better benefit from alternative treatment strategies.

Prognostic and predictive value of the Arbeitsgemeinschaft Gynaekologische Onkologie (AGO) score in surgery for recurrent ovarian cancer.

OBJECTIVES: The Arbeitsgemeinschaft Gynaekologische Onkologie (AGO) score was developed as selection criteria and validated as predictor of a high probability for complete resection in recurrent ovarian cancer. It is not defined whether the predictive value is independent from underlying tumor biology or is solely based on a selection of good prognostic risks. METHODS: We performed an exploratory analysis of all consecutive patients with cytoreductive surgery in recurrent ovarian cancer in a tertiary referral center 1999-2013, before and after introduction of the AGO score. RESULTS: 217 consecutive patients were included of whom 112 patients were AGO score positive and 105 patients were score negative. Corresponding complete resection rates were 89.3% and 66.7%, respectively, and confirm the score's predictive value. However, a positive AGO score was also associated with better outcome after adjustment for surgical outcome. Patients with complete resection and a positive AGO score showed a median overall survival of 63.9 months (95% CI 48.1-79.6) compared to 48.4 months (95% CI 30.3-66.5) after complete resection and negative score (log-rank p=0.10). However, in multivariate analysis the only independent prognostic factor was complete resection (HR 2.450; 95% CI: 1.542-3.891). CONCLUSIONS: The AGO score could identify suitable candidates for secondary cytoreductive surgery but failed to prove an independent prognostic value thus suggesting an effect of successful surgery on its own. However, the latter has to be proven prospectively. In addition, further studies should evaluate the predictive and prognostic impact of a negative score.

External branch of the superior laryngeal nerve monitoring during thyroid and parathyroid surgery: International Neural Monitoring Study Group standards guideline statement.

UNLABELLED: Intraoperative neural monitoring (IONM) during thyroid surgery has gained widespread acceptance as an adjunct to the gold standard of visual identification of the recurrent laryngeal nerve (RLN). Contrary to routine dissection of the RLN, most surgeons tend to avoid rather than routinely expose and identify the external branch of the superior laryngeal nerve (EBSLN) during thyroidectomy or parathyroidectomy. IONM has the potential to be utilized for identification of the EBSLN and functional assessment of its integrity; therefore, IONM might contribute to voice preservation following thyroidectomy or parathyroidectomy. We reviewed the literature and the cumulative experience of the multidisciplinary International Neural Monitoring Study Group (INMSG) with IONM of the EBSLN. A systematic search of the MEDLINE database (from 1950 to the present) with predefined search terms (EBSLN, superior laryngeal nerve, stimulation, neuromonitoring, identification) was undertaken and supplemented by personal communication between members of the INMSG to identify relevant publications in the field. The hypothesis explored in this review is that the use of a standardized approach to the functional preservation of the EBSLN can be facilitated by application of IONM resulting in improved preservation of voice following thyroidectomy or parathyroidectomy. These guidelines are intended to improve the practice of neural monitoring of the EBSLN during thyroidectomy or parathyroidectomy and to optimize clinical utility of this technique based on available evidence and consensus of experts. LEVEL OF EVIDENCE: 5

Continuous monitoring of the recurrent laryngeal nerve in thyroid surgery: a critical appraisal.

BACKGROUND AND PURPOSE: Intraoperative neuromonitoring (IONM) contributes in several ways to recurrent laryngeal nerve (RLN) protection. Notwithstanding these advantages, surgeons must be aware that the current, intermittent, mode of IONM (I-IONM) has relevant limitations. To overcome these I-IONM limitations, a continuous IONM (C-IONM) technology has been proposed. METHODS: A PubMed indexed literature review of the current limitations of I-IONM is presented and a commentary about C-IONM is provided presenting the preliminary results of research on this topic. MAIN FINDINGS: I-IONM, despite the advantages it produces, presents some important limitations; to overcome these drawbacks a C-IONM technology has been introduced. CONCLUSIONS: RLN traction injury is still the most common cause of RLN injury and is difficult to avoid with the application of I-IONM in thyroid surgery. C-IONM is useful to prevent the imminent traction injury by detecting progressive decreases in electromyographic amplitude combined with progressive latency increases. C-IONM seems to be a technological improvement. Likely, C-IONM by vagal nerve stimulation should enhance the standardization process, RLN intraoperative information, documentation, protection, training, and research in modern thyroid surgery. Although C-IONM is a promising technology at the cutting edge of research in thyroid surgery, we need more studies to assess in an evidence-based way all its advantages.

Intraoperative neuromonitoring does not reduce the incidence of recurrent laryngeal nerve palsy in thyroid reoperations: results of a retrospective comparative analysis.

PURPOSE: Recurrent laryngeal nerve (RLN) palsy is the major concern of reoperative thyroid surgery, and the introduction of neuromonitoring could reduce the rate of this complication. The present study is a retrospective analysis of the experience with completion thyroidectomy with and without neuromonitoring in a referral center. METHODS: Between October 1999 and April 2011, 246 patients [37 men, 209 women; mean age, 55 ± 12.5 (range, 25-80) years] underwent 250 reoperations for recurrent goiter (n = 203), hyperthyroidism (n = 26), or recurrent thyroid cancer (n = 17). The mean interval between the initial and the reoperative procedure was 17.5 years. According to the availability of the neuromonitoring system and to the surgeon preference, 91 operations were performed with neuromonitoring (NM-group), whereas 159 were performed with direct nerve visualization (NV-group) alone. Patients' characteristics, perioperative data, and postoperative complications were collected in a prospectively maintained database. RESULTS: In the NM-group, 51 unilateral and 40 bilateral resections were performed. The NV-group included 122 unilateral and 37 bilateral procedures. The number of nerves at risk after previous surgery was 128 (NM-group) and 161 (NV-group), respectively. We registered eight RLN palsy in the NM-group (6.2 %) and four in the NV-group (2.5 %; p = 0.1). CONCLUSIONS: The routine use of intraoperative neuromonitoring seems not to reduce the incidence of RLN during redo thyroid surgery, at least in the setting of a tertiary referral center.

Minimally invasive video-assisted parathyroidectomy (MIVAP) for secondary hyperparathyroidism: report of initial experience.

BACKGROUND: Minimally invasive video-assisted parathyroidectomy (MIVAP) has become a well-accepted procedure for patients with primary hyperparathyroidism. Because it allows bilateral neck exploration, the authors began using this technique for patients with secondary hyperparathyroidism. In this preliminary study, the authors report their initial experience. METHODS: From July 2006 to November 2008, 12 patients (6 women, 6 men; mean age, 45.5 +/- 16.9 years (range, 23-71 years) underwent MIVAP with bilateral exploration for secondary hyperparathyroidism. The operation was performed through a central 2-cm to 3-cm skin incision; a 30 degrees 5-mm endoscope was used for magnification. RESULTS: MIVAP was successfully completed in 11 patients (92%). The mean operative time was 81 +/- 37 minutes (range, 35-130 minutes). No major complications were registered. After a mean follow-up period of 11.4 months (range, 3-30 months), 1 patient showed recurrence. CONCLUSIONS: MIVAP appears to be a safe and feasible procedure in patients with secondary hyperparathyroidism.

Single-access retroperitoneoscopic adrenalectomy (SARA) versus conventional retroperitoneoscopic adrenalectomy (CORA): a case-control study.

BACKGROUND: Stimulated by the concept of Natural Orifice Transluminal Endoscopic Surgery (NOTES), minimizing the access even further has become a new trend in minimally invasive surgery. We compare our recently described new method of endoscopic single-access adrenalectomy with the conventional retroperitoneoscopic approach in a matched-pairs study. METHODS: Fifty single-access retroperitoneoscopic adrenalectomies (SARA) were performed in 47 selected patients suffering from Conn's adenomas (n = 20), pheochromocytomas (n = 15), Cushing's adenomas (n = 6), and other diseases (n = 6). For SARA, a single 2-cm skin incision beneath the 12th rib was used. Following creation of the retroperitoneal space with the rigid endoscope, dissection was carried out single-handed. Another 47 patients served as control group; they were treated by the traditional retroperitoneoscopic three-port approach (CORA). Patients were matched with respect to gender, body mass index, diagnoses, tumor size, and tumor site. RESULTS: Mortality was zero and no major complications occurred in both groups. SARA was completed in 41 cases (86%). The overall complication rate was 8.5% in SARA and 6.4% in CORA. Operative time was longer for SARA (56 +/- 28 min) than for CORA (40 +/- 12 min) (P < 0.05). Postoperatively, pain medication was administered in 47% of SARA patients and in 75% of CORA patients (P = 0.01). Mean hospital stay was 2.4 +/- 0.7 days (SARA) and 3.1 +/- 1.2 days (CORA) (P < 0.01). CONCLUSIONS: Because feasibility and safety of SARA could be demonstrated in a large group of selected patients, this surgical technique may represent a new milestone in minimally invasive endocrine surgery.

Single access retroperitoneoscopic adrenalectomy (SARA)--one step beyond in endocrine surgery.

INTRODUCTION: Today, endoscopic adrenalectomy has become a gold standard in endocrine surgery. METHOD AND RESULTS: To minimize the morbidity and improve cosmesis, a single access retroperitoneoscopic adrenalectomy (SARA) has been developed. Patients were placed in prone position. Through a single 1.5 cm skin incision at the tip of the 12th rib the creation of the retroperitoneal space and the dissection of the adrenal gland were performed. SARA was used in five patients with adrenal tumors (size: 1-4 cm; four right, one left). Operating time ranged from 35 to 70 min, mean blood loss was <10 ml. No perioperative or postoperative complications were noticed. Mean hospital stay was 2 days. CONCLUSIONS: SARA is feasible and represents a new horizon in endocrine surgery.

Retroperitoneoscopic adrenalectomy in Conn's syndrome caused by adrenal adenomas or nodular hyperplasia.

BACKGROUND: In patients with primary hyperaldosteronism, solitary adrenal adenomas are an indication for surgical intervention. In contrast, adrenal hyperplasia is almost exclusively treated by drugs. PATIENTS AND METHODS: In a prospective clinical study 183 patients (81 men, 102 women; age 49.6+/-12.8 years) with Conn's syndrome were operated on using the posterior retroperitoneoscopic approach. Tumor size ranged from 0.2 to 5.0 cm (mean 1.5+/-0.8 cm). Final histology described a solitary adenoma in 127 patients and adrenal hyperplasia in 56 patients. Partial adrenalectomies were performed in 47 operations. RESULTS: The perioperative complication rate was 4%, mortality zero. In none of the cases was conversion to open surgery necessary. The mean operating time was 58+/-32 minutes (range 20-230 minutes) and was associated with sex (p<0.001) but not with the extent of resection (partial vs. total, p=0.51) or with tumor size (1.5 cm; p=0.43) or tumor site (p=0.77). Median blood loss was 15 ml. Median duration of postoperative hospitalization was 4 days. After a mean follow-up of nearly 5 years, 96% of patients are normokalemic, 30% of patients are cured (normotensive without medication), and 87% showed an improvement of hypertension (normotensive without or with reduced medication). Cure of hypertension depended on the patient's age (p<0.001) and sex (p<0.001), duration of hypertension (p<0.05), and histomorphology (p<0.001). Improvement of hypertension was not associated with any of these factors. CONCLUSIONS: Retroperitoneoscopic removal of adrenal glands in patients with Conn's syndrome is a safe, rapidly performed surgical procedure and can thus be considered as first choice option for treatment of both solitary adrenal adenomas and hyperplasia presenting with a clinically predominating nodule.

Posterior retroperitoneoscopic adrenalectomy--results of 560 procedures in 520 patients.

BACKGROUND: The posterior retroperitoneoscopic adrenalectomy is less popular than the laparoscopic transabdominal method. Due to the direct approach to the adrenal glands, however, the posterior retroperitoneal access is easy to use and may offer advantages not available with other endoscopic procedures for adrenalectomy. METHODS: Between July 1994 and March 2006, we performed 560 adrenalectomies (right side: n = 258; left side: n = 302) by the posterior retroperitoneoscopic approach in 520 patients (200 male, 320 female; age, 10 to 83 years). Of the 520 patients, 21 suffered from Cushing's disease, 499 patients had adrenal tumors (157 Conn's adenomas, 120 pheochromocytomas [13 bilateral], 110 Cushing's adenomas [6 bilateral], and 112 other tumors). Tumor size ranged from 0.5 to 10 cm (mean, 2.9 +/- 1.7 cm). The procedures were performed with the patients in the prone position usually with 3 trocars. RESULTS: Mortality was zero. Conversions to open or laparoscopic lateral surgery were necessary in 9 patients (1.7%). Major complications occurred in 1.3% of patients, minor complications in 14.4%. Mean operating time was 67 +/- 40 min and declined significantly (P < .001) from the early procedures (106 +/- 46 min) to the later operations (40 +/- 15 min). CONCLUSIONS: The posterior retroperitoneoscopic adrenalectomy is a safe and fast procedure. In experienced hands, this method represents the ideal approach in adrenal surgery.

Laparoscopic and retroperitoneoscopic treatment of pheochromocytomas and retroperitoneal paragangliomas: results of 161 tumors in 126 patients.

BACKGROUND: Laparoscopic and retroperitoneoscopic excisions of pheochromocytomas and retroperitoneal paragangliomas are challenging surgical procedures because of extensive intraoperative catecholamine release, extreme vascularization, and demanding localization. MATERIALS: In a prospective clinical study 161 chromaffine neoplasias (134 pheochromocytomas, 27 paragangliomas) were removed endoscopically in 126 patients (67 males, 59 females, age 41.7 +/- 16.4 years; 130 operations). Six patients showed multiple (2-5) tumors. Tumor size ranged from 0.5 to 12 cm (mean 3.5 +/- 1.9 cm). Forty-two patients suffered from hereditary diseases. Twenty-four patients had bilateral adrenal diseases; in 14 patients pheochromocytomas were removed on both sides synchroneously. Ten neoplasias were local or loco-regional recurrences (7 pheochromocytomas, 3 paragangliomas). The laparoscopic route was chosen in 16 operations; the retroperitoneoscopic technique was performed in 128 others. Partial adrenalectomies were performed in 57 operations (in all but one of the patients with bilateral disease). High-dosage alpha-blockade with phenoxybenzamine was routinely used. RESULTS AND DISCUSSION: Conversion to open surgery occurred once. Perioperative complications were minor (17%); mortality was zero. Operating time for unilateral retroperitoneoscopically removed primary pheochromocytomas (n = 113) was 82 +/- 49 minutes (range: 20-300 minutes) and depended on tumor size (< 3 cm vs. > or = 3 cm; P < 0.05) and gender (P < 0.001), but not on extent of resection (partial vs. total, P = 0.266). Operating time for paragangliomas ranged from 55 to 600 minutes. Median blood loss was 20 ml. Median duration of postoperative hospitalization was 4 days. In 22 of 24 patients with bilateral disease, complete preservation of cortical function was achieved. Locoregional and/or distant metastatic recurrence were found in 5 patients. CONCLUSIONS: Endoscopic removal of solitary, bilateral, multiple, and recurrent pheochromocytomas and retroperitoneal paragangliomas is feasible and safe, but surgeons need extensive experience in minimally invasive techniques, as well as in endocrine surgery.

Long-term results of less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1.

BACKGROUND: Our aim was to assess long-term results after less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1. METHODS: Of 1888 patients undergoing operation at our institution for primary hyperparathyroidism between 1972 and 2001, 83 (4.4%) had multiple endocrine neoplasia type 1. Outcome data were available for 79; 66 underwent subtotal parathyroidectomy, 55 (83%) of these with bilateral thymectomy. In 13 patients, only grossly enlarged glands were resected (mean 1.1 per patient) as the syndrome of multiple endocrine neoplasia type 1 was not yet evident or the initial exploration was performed elsewhere. RESULTS: Follow-up has been 48 +/- 51 months (mean + SD). Intraoperative serum PTH assay decay in 20 patients was suggestive of cure in 18 patients, none of whom required reoperation. Nine patients (11%) required reoperation (3 required reoperation twice) after a mean interval of 77 +/- 53 months. Subtotal parathyroidectomy resulted in a lesser reoperation rate than resection of grossly enlarged glands (7% vs 30%, P =.02). At the time of review, 63 patients (80%) were normocalcemic, 10 (13%) hypocalcemic (2 after unsuccessful delayed autograft), and 7% hypercalcemic (none after reoperation). By Kaplan-Meier analysis, the rate of surgical cure (patients who are nonhypercalcemic) is 60% and 51% at 10 and 15 years, respectively. CONCLUSION: Subtotal parathyroidectomy reduces the need for reoperation. Selective reoperation leads to long-lasting biochemic cure.