Preoperative calcitonin testing improves the diagnosis of medullary thyroid carcinoma in female and male patients.

AIM: Calcitonin (Ctn) measurement in patients with thyroid disease could potentially increase the detection rates of medullary thyroid carcinoma (MTC) but remains a controversial issue. The aim of this study was to evaluate routine preoperative Ctn measurements. METHODS: All patients with thyroid surgery documented in the prospective StuDoQ|Thyroid registry between March 2017 and September 2020 were included. Cutoff levels for Ctn were determined with receiver-operating characteristic analyses to assess the preoperative diagnosis of MTC in subgroups for females and males. FINDINGS: In 29 590 of 39 679 patients (75%) participating in the registry, routine preoperative Ctn testing was performed. In 357 patients (227 females and 130 males), histopathology confirmed MTC with a mean tumor size of 14.7 mm (±12.43). Biochemical cure was achieved in 71.4% of the patients. Ctn levels between 11 and 20 pg/mL were seen in 2.6% of the patients, and only 0.7% of the patients had Ctn levels above 21 pg/mL. Cutoff levels for the diagnosis of MTC were 7.9 pg/mL for females and 15 pg/mL for males (P < 0.001). The sensitivity and specificity for females were 95 and 98%, and 96 and 97% for males, respectively. CONCLUSION: Routine Ctn testing is a reliable predictor for MTC and provides the opportunity for earlier thyroidectomy before lymph node metastases occur, resulting in a better prognosis. Females with Ctn levels >7.9 pg/mL and males >15 pg/mL without any other extrathyroidal sources for an elevated Ctn should be monitored. Thyroid surgery should be considered if Ctn levels are increasing or ultrasound detects suspicious thyroid lesions.

Total Versus Near-total Thyroidectomy in Graves Disease: Results of the Randomized Controlled Multicenter TONIG-trial.

BACKGROUND: Previous data suggest that the incidence of hypoparathyroidism after surgery for Graves disease (GD) is lower after subtotal thyroidectomy compared to total thyroidectomy (TT). The present study evaluated the incidence of postoperative hypoparathyroidism after near-total (NTT) versus TT in GD. METHODS/DESIGN: In a multicenter prospective randomized controlled clinical trial, patients with GD were randomized intraoperatively to NTT or TT. Primary endpoint was the incidence of transient postoperative hypoparathyroidism. Secondary endpoints were permanent hypoparathyroidism, transient recurrent laryngeal nerve palsy (RLNP), reoperations for bleeding, inadvertently removed parathyroid glands, and recurrent hyperthyroidism after 12 months. RESULTS: Eighteen centers randomized 205 patients to either TT (n = 102) or NTT (n = 103) within 16 months. According to intention-to-treat postoperative transient hypoparathyroidism occurred in 19% (20/103) patients after NTT and in 21% (21 of 102) patients after TT (P = 0.84), which persisted >6 months in 2% and 5% of the NTT and TT groups (P = 0.34). The rates of parathyroid autotransplantation (NTT 24% vs TT 28%, P = 0.50) and transient RLNP (NTT 3% vs TT 4%, P = 0.35) was similar in both groups. The rate of reoperations for bleeding tended to be higher in the NTT group (3% vs 0%, P = 0.07) and the rate of inadvertently removed parathyroid glands was significantly higher after NTT (13% vs 3%, P = 0.01). An existing endocrine orbitopathy improved in 35% and 24% after NTT and TT (P = 0.61). Recurrent disease occurred in only 1 patient after TT (P = 0.34). CONCLUSION: NTT for GD is not superior to TT regarding transient postoperative hypoparathyroidism.

Total Parathyroidectomy With Routine Thymectomy and Autotransplantation Versus Total Parathyroidectomy Alone for Secondary Hyperparathyroidism: Results of a Nonconfirmatory Multicenter Prospective Randomized Controlled Pilot Trial.

OBJECTIVE: This randomized controlled multicenter pilot trial was conducted to find robust estimates for the rates of recurrence of 2 surgical strategies for secondary hyperparathyroidism (SHPT) within 36 months of follow-up. BACKGROUND: SHPT is a frequent consequence of chronic renal failure. Total parathyroidectomy with autotransplantation (TPTX+AT) and subtotal parathyroidectomy (SPTX) are the standard surgical procedures. Total parathyroidectomy alone (TPTX) might be a good alternative, as morbidity and recurrence rates are low according to small-scale retrospective studies. METHODS: The trial was performed as a nonconfirmatory randomized controlled pilot trial with 100 patients on long-term dialysis with otherwise uncontrollable SHPT to generate data on the rate of recurrent disease within a 3-year follow-up period after TPTX or TPTX+AT. Parathyroid hormone (PTH) and calcium levels, recurrent or persistent hyperparathyroidism, parathyroid reoperations, morbidity, and mortality were evaluated during a 3-year follow-up. RESULTS: A total of 52 patients underwent TPTX and 48 TPTX+AT. Patient characteristics, preoperative baseline data, duration of surgery (02:29 vs 02:47 hrs, P = 0.17) and mean hospital stay (10 ± 7.1 vs 8 ± 3.7 days, P = 0.11) did not differ significantly. Persistent SHPT developed in 1 TPTX and 2 TPTX+AT patients. None of the TPTX patients required delayed parathyroid AT to treat permanent hypoparathyroidism. Serum-calcium values were similar (2.1 ± 0.3 vs 2.1 ± 0.2, P = 0.95) whereas PTH rose by time in the TPTX+AT group and was significantly higher at the end of follow-up when compared with the TPTX group (31.7 ± 43.6 vs 98.2 ± 156.8, P = 0.02). Recurrent SHPT developed in 4 TPTX+AT and none of the TPTX patients. CONCLUSIONS: TPTX+AT and TPTX seem to be safe and equally effective for the treatment of otherwise uncontrollable SHPT. TPTX seems to suppress PTH more effectively and showed no recurrences after 3 years. The hypothesis that TPTX is superior to TPTX+AT referring to the rate of recurrent SHPT has to be tested in a large-scale confirmatory trial. Nevertheless, TPTX seems to be a feasible alternative therapeutic option for the surgical treatment of SHPT.

Endoscopic vacuum therapy for esophageal perforations and leakages.

BACKGROUND AND STUDY AIMS: Injuries to the esophageal wall, such as perforations and anastomotic leaks, are serious complications of surgical and endoscopic interventions. Since 2006, a new treatment has been introduced, in the form of endoscopically placed vacuum sponge therapy. PATIENTS AND METHODS: Between April 2012 and October 2014, 10 patients (5 men and 5 women) aged 57 to 94 years were treated at our institution using endoscopic vacuum therapy (EVT) in the upper gastrointestinal tract. RESULTS: The defect in the esophageal wall was successfully closed in seven of the 10 patients (70 %). No severe complications occurred. CONCLUSIONS: EVT is a valuable tool for management of defects in the esophageal wall and should be considered as a treatment option for patients with this condition.

Shortness: an unknown phenotype of multiple endocrine neoplasia type 1.

OBJECTIVE: An observation of shortness among the female participants of a regular screening program in multiple endocrine neoplasia type 1 (MEN1) patients has raised the question as to whether shortness represents a phenotype characteristic of the disease. METHODS: The body height (cm) of genetically confirmed MEN1 patients at the time of diagnosis was compared with the body height of their unaffected relatives (parents, siblings, and children), the midparental body height, and the body height of the age-matched German population. Univariate analysis of the clinical variables was performed using the t-test, Mann­Whitney U test, and ANOVA as appropriate, and multivariate analysis was performed as a logistic regression analysis. P values <0.05 were considered statistically significant. RESULTS: The mean body height of 22 female MEN1 patients (mean age 33.5 years) was 161 +/- 5 cm and thus significantly lesser than the body heights of their unaffected female relatives (mean 165.5 +/- 7.3 cm, P=0.027) and the age-matched German female population (mean 167 cm, P=0.0001) and mid-parental height (177.5 cm, P<0.0001). The mean body height of 24 male MEN1 patients (mean age 34.8 years) was also lesser (177 +/- 6.5 cm) than the average body height of German males in this age group (180 cm, P=0.031) and tended to be lesser than that of their unaffected male relatives (178.5 +/- 5.8 cm, P=0.0915) and the mid-parental body height (177.5 cm, P=0.124). CONCLUSIONS: Small body height is a yet unrecognized phenotype characteristic of MEN1 patients, especially in women. The mechanisms behind this phenotypical characteristic warrant further investigation.

Parathyroidectomy, elevated depression scores, and suicidal ideation in patients with primary hyperparathyroidism: results of a prospective multicenter study.

OBJECTIVE: To assess anxiety and depression symptoms, suicidal ideation, and health-related quality of life (HRQOL) in a large series of consecutive patients with primary hyperparathyroidism (pHPT) before and after parathyroidectomy. DESIGN: This prospective multicenter study investigated preoperative and postoperative depression, anxiety, suicidal ideation, and HRQOL in patients with pHPT and compared these variables with a control group with nontoxic thyroid nodules. PATIENTS: The study included 194 patients with pHPT and 186 control subjects. MAIN OUTCOME MEASURES: Depression was evaluated with the Hospital Anxiety and Depression Scale (HADS) and the Patient Health Questionnaire-9, which also assessed suicidal ideation. Anxiety was evaluated with the HADS. Health-related quality of life was measured with the 36-Item Short Form survey. RESULTS: Parathyroidectomy achieved a 98% cure rate. Preoperatively, severe depression (HADS score ≥ 11) was seen in 20% of the pHPT group and 9% of the control group. The Patient Health Questionnaire-9 detected moderate to severe depression in 17% of the patients with pHPT and 7% of the control subjects. Patients with pHPT had higher HADS anxiety scores (mean, 7.7) than control subjects (P < .01) or the German normative sample (P < .001). Compared with control subjects, patients with pHPT had significantly lower 36-Item Short Form survey preoperative physical and mental health summary scores (42.7 vs 49.5 and 41.2 vs 46.8, respectively; P = .001 for both comparisons). At 12 months follow-up, depression and anxiety decreased significantly in patients with pHPT; the prevalence of suicidal ideation was more than halved from the baseline (10.7% vs 22%; P = .008). Both physical and mental health scores (45.7 and 47.7, respectively) improved in patients with pHPT (P < .001 each) but not in control subjects. CONCLUSIONS: Depression, anxiety, and decreased HRQOL appear to be related to pHPT. Successful parathyroidectomy seems to reduce psychopathologic symptoms and improve HRQOL in this setting.

German Association of Endocrine Surgeons practice guideline for the surgical management of malignant thyroid tumors.

INTRODUCTION: Over the past years, the incidence of thyroid cancer has surged not only in Germany but also in other countries of the Western hemisphere. This surge was first and foremost due to an increase of prognostically favorable ("low risk") papillary thyroid microcarcinomas, for which limited surgical procedures are often sufficient without loss of oncological benefit. These developments called for an update of the previous practice guideline to detail the surgical treatment options that are available for the various disease entities and tumor stages. METHODS: The present German Association of Endocrine Surgeons practice guideline was developed on the basis of clinical evidence considering current national and international treatment recommendations through a formal expert consensus process in collaboration with the German Societies of General and Visceral Surgery, Endocrinology, Nuclear Medicine, Pathology, Radiooncology, Oncological Hematology, and a German thyroid cancer patient support organization. RESULTS: The practice guideline for the surgical management of malignant thyroid tumors includes recommendations regarding preoperative workup; classification of locoregional nodes and terminology of surgical procedures; frequency, clinical, and histopathological features of occult and clinically apparent papillary, follicular, poorly differentiated, undifferentiated, and sporadic and hereditary medullary thyroid cancers, thyroid lymphoma and thyroid metastases from primaries outside the thyroid gland; extent of thyroidectomy; extent of lymph node dissection; aerodigestive tract resection; postoperative follow-up and surgery for recurrence and distant metastases. CONCLUSION: These evidence-based recommendations for surgical therapy reflect various "treatment corridors" that are best discussed within multidisciplinary teams and the patient considering tumor type, stage, progression, and inherent surgical risk.

Total versus near-total thyroidectomy in Graves' disease and their outcome on postoperative transient hypoparathyroidism: study protocol for a randomized controlled trial?

BACKGROUND: Graves' disease is an independent risk factor for transient postoperative hypoparathyroidism. Besides the disease itself, preparation techniques are influential. Transient postoperative hypoparathyroidism has severe consequences for patients' physical and psychological state. It can be life threatening during the acute phase and may impair patients' health, psyche and quality of life thereafter. For the surgical therapy of Graves' disease, total thyroidectomy is recommended according to the national S2-guideline. The evidence- based on a metaanalysis- is criticized by the Cochrane diagnostic review commentary for substantial methodological deficits. Two randomized controlled trials lead to the hypothesis that a near-total resection with bilateral remnants of ≤ 1g on each side compared to total thyroidectomy will significantly reduce the occurrence of transient postoperative hypoparathyroidism with equal therapeutic safety. METHODS/DESIGN: Patients with Graves' disease indicated for definite surgery are eligible for the trial. Trial-specific exclusion criteria are: conservative treatment, malignancy, previous thyroid surgery and coincident hypoparathyroidism. The trial is created for therapeutic purpose through process innovation. It is designed as a prospective randomized controlled patient and observer blinded multicentered trial in a parallel design including an active comparator and an intervention group. The intervention addresses the surgical procedure: near-total thyroidectomy leaving bilateral remnants of ≤ 1g on each side in the intervention group and total thyroidectomy in the control group. The occurrence of transient postoperative hypoparathyroidism is defined as primary endpoint. Secondary endpoints are: reoperations due to bleeding, recurrent laryngeal nerve palsy, permanent hypoparathyroidism, recurrent disease, changes of endocrine orbitopathy and quality of life within a one-year follow-up period. The primary efficacy analysis follows the intention-to-treat principle. A binary logistic regression model will be applied. Complications and serious adverse events will be descriptively analyzed. DISCUSSION: The trail is expected to balance out the shortcomings of the current evidence. It will define the surgical gold standard for the surgical therapy of Graves' disease. Patients' safety and quality of life are assumed to be enhanced. Therapy costs are likely to be reduced and health care optimized. The conduction of the trial is feasible through the engagement and commitment of the German association of endocrine surgeons and the National Network for Surgical Trials. TRIAL REGISTRATION: German clinical trials register (DRKS) DRKS00004161.

Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma.

OBJECTIVE: Adrenocortical carcinoma (ACC) is a rare malignancy with an unfavorable prognosis. The impact of a locoregional lymph node dissection (LND) has never been defined in this disease. We report the disease-specific outcome of patients treated with or without LND during primary adrenalectomy. METHODS: The medical records of patients followed by the German ACC Registry were retrospectively reviewed. Patients with incomplete resection or distant metastases were excluded. Only if the histologic analysis retrieved 5 or more lymph nodes, an intended LND was assumed (LND group). The predefined primary end point of the study was disease-specific survival. RESULTS: Of 283 included patients, 47 patients (16.6%) were treated with LND, whereas 236 patients (83.4%) underwent surgery without LND. Patients who underwent LND had a larger median tumor size (12.0 cm, range: 2.3-30 cm vs 10.0 cm, range: 4.0-39 cm, P = 0.007) and were more often treated by multivisceral resection (LND: 47.8% vs no-LND: 18.1%; P < 0.001). The other baseline characteristics (age, sex, endocrine activity, Weiss score, Ki-67 index, and adjuvant treatment) did not differ significantly. Median follow-up of all patients still alive was 40 months (range: 6-326). Multivariate analysis adjusted for age, tumor stage, multivisceral resection, adjuvant treatment, and lymph nodes status on preoperative imaging demonstrated a significantly reduced risk for tumor recurrence (hazard ratio: 0.65; 95% confidence interval: 0.43-0.98; P = 0.042) and for disease-related death (hazard ratio: 0.54; 95% confidence interval: 0.29-0.99; P = 0.049) in LND patients when compared with no-LND patients. CONCLUSIONS: Our retrospective data indicate that locoregional LND improves tumor staging and leads to a favorable oncologic outcome in patients with localized ACC.

German Association of Endocrine Surgeons practice guidelines for the surgical treatment of benign thyroid disease.

INTRODUCTION: Benign thyroid disorders are among the most common diseases in Germany, affecting around 15 million people and leading to more than 100,000 thyroid surgeries per year. Since the first German guidelines for the surgical treatment of benign goiter were published in 1998, abundant new information has become available, significantly shifting surgical strategy towards more radical interventions. Additionally, minimally invasive techniques have been developed and gained wide usage. These circumstances demanded a revision of the guidelines. METHODS: Based on a review of relevant recent guidelines from other groups and additional literature, unpublished data, and clinical experience, the German Association of Endocrine Surgeons formulated new recommendations on the surgical treatment of benign thyroid diseases. These guidelines were developed through a formal expert consensus process and in collaboration with the German societies of Nuclear Medicine, Endocrinology, Pathology, and Phoniatrics & Pedaudiology as well as two patient organizations. Consensus was achieved through several moderated conferences of surgical experts and representatives of the collaborating medical societies and patient organizations. RESULTS: The revised guidelines for the surgical treatment of benign thyroid diseases include recommendations regarding the preoperative assessment necessary to determine when surgery is indicated. Recommendations regarding the extent of resection, surgical techniques, and perioperative management are also given in order to optimize patient outcomes. CONCLUSIONS: Evidence-based recommendations for the surgical treatment of benign thyroid diseases have been created to aid the surgeon and to support optimal patient care, based on current knowledge. These recommendations comply with the Association of the Scientific Medical Societies in Germany requirements for S2k guidelines.

Laparoscopic versus open adrenalectomy for adrenocortical carcinoma: surgical and oncologic outcome in 152 patients.

BACKGROUND: The role of laparoscopic adrenalectomy in the treatment of patients with adrenocortical carcinoma (ACC) is controversial. OBJECTIVE: Our aim was to compare oncologic outcome in patients with ACC who underwent either open adrenalectomy (OA) or laparoscopic adrenalectomy (LA) for localised disease. DESIGN, SETTING, AND PARTICIPANTS: We conducted a retrospective analysis of 152 patients with stage I-III ACC with a tumour < or =10 cm registered with the German ACC Registry. INTERVENTION: Patients were stratified into two groups according to the surgical procedure (LA or OA). For comparison, we used both a matched pairs approach by selecting for each patient from the LA group (n=35) one corresponding patient from the OA group (n=117) and multivariate analysis in all 152 patients. MEASUREMENTS: Disease-specific survival was chosen as the predefined primary end point. Secondary end points were recurrence-free survival, frequency of tumour capsule violation and postoperative peritoneal carcinomatosis, and incidence and reasons for conversion from LA to OA. RESULTS AND LIMITATIONS: LA and OA did not differ with regard to the primary end point using either the matched pairs approach (hazard ratio [HR] for death: 0.79; 95% confidence interval [CI], 0.36-1.72; p=0.55) or multivariate analysis (HR for death: 0.98; 95% CI, 0.51-1.92; p=0.92). Similarly, adjusted recurrence-free survival was not different between LA and OA (HR: 0.91; 95% CI, 0.56-1.47; p=0.69). Frequency of tumour capsule violation and peritoneal carcinomatosis were comparable between groups. In 12 of 35 patients of the LA group, surgery was converted to open surgery with no impact on the clinical outcome. CONCLUSIONS: For localised ACC with a diameter of < or =10 cm, LA by an experienced surgeon is not inferior to OA with regard to oncologic outcome.

Use of cinacalcet HCl to achieve the recommended targets of bone metabolism in a patient with therapy-resistant renal hyperparathyroidism.

We report on a patient with end-stage renal disease and severe progressive secondary hyperparathyroidism, whose condition failed to respond to conventional pharmacologic or surgical interventions. Although immunotherapy produced a partial response, it failed to decrease serum parathyroid hormone to the levels recommended by the National Kidney Foundation Kidney Disease Outcomes Quality Initiative clinical practice guidelines. Treatment with a new calcimimetic agent, cinacalcet HCl (Mimpara, Amgen, Munich, Germany), resulted in a rapid decline in elevated parathyroid hormone levels, near normalization of other laboratory markers of bone metabolism, improvement in mobility and skeletal pain caused by renal osteodystrophy, and an increase in body weight.

Intraoperative haemodynamic stability in patients with phaeochromocytoma--minimally invasive vs conventional open surgery.

OBJECTIVE: There is conflicting evidence, whether or not minimally invasive adrenalectomy (MA) is associated with an increased perioperative cardiovascular instability in phaeochromocytomas compared to conventional open adrenalectomy (CA). DESIGN AND PATIENTS: In a retrospective analysis of 49 patients with phaeochromocytoma we compared 27 cases of MA to 22 cases of CA by assessing intraoperative haemodynamic parameters and perioperative complications. Patients undergoing MA for adrenocortical adenomas (aldosteronomas n = 15, inactive adenomas n = 13) served as controls. Additionally, we investigated the effect of phenoxybenzamine (POB) pretreatment on intraoperative cardiovascular stability in 42 patients (ranked by maximum daily POB-dose) by comparing the highest (n = 10) with the lowest (n = 10) POB dose quartile (0.32 +/- 0.2 and 2.17 +/- 0.6 mg/kg/day, P < 0.001). RESULTS: In phaeochromocytomas we found no significant difference in intraoperative haemodynamic parameters or complications when comparing MA with CA. In comparison to adrenocortical adenomas, MA in phaeochromocytomas was associated with a significantly higher maximum systolic BP (188 +/- 29 vs 154 +/- 22 mmHg, P < 0.001), more frequent hypertensive episodes (1[0-4]vs 0[0-1], P < 0.001), more episodes of systolic BP > 200 mmHg (0[0-4]vs 0[0-1], P = 0.03) and a higher demand for intraoperative fluids (3194 ml vs 1750 ml, P < 0.001). Most haemodynamic parameters did not differ significantly between high-dose POB pretreatment and low-dose POB pretreatment, but high-dose POB pretreatment was associated with a significantly higher intraoperative heart rate (120 +/- 19.5 vs 94 +/- 15.2 min(-1), P < 0.01). CONCLUSION: There is no significant difference in haemodynamic stability between MA and CA in phaeochromocytomas, but it is significantly inferior when compared to MA for cortical adenomas. We could not detect a beneficial effect of high-dose compared to low-dose POB pretreatment on intraoperative cardiovascular stability.

Evaluation of the McPeek postoperative outcome score in three trials.

BACKGROUND: Postoperative outcome of patients is determined by recovery characteristics and self-reported quality of life. The first can be assessed with the McPeek score which values three aspects of recovery: mortality, postoperative critical care and duration of hospitalization. MATERIALS AND METHODS: We calculated the McPeek score of 669 patients in three trials: (1) colorectal cancer surgery, (2) antihistamine/volume loading in various operations, and (3) cholecystectomy. Beforehand, the average of intensive care unit treatment and duration of hospitalization were determined for the different operations to define McPeek score points. The score was tested on reliability, validity, and sensitivity. In addition, clinical applicability was assessed in a survey. RESULTS: The score was reliable with similarly distributed score points in the three trials at different institutions. Inter-rater reliability was high (97% overlap). Validity was proven by moderate high correlation to convergent criteria such as complications (trial I to III r=0.43, r=0.38, r=0.60), preoperative American Society of Anesthesiologists class (ASA) (r=0.24, r=0.28, r=0.57), and age (r=0.23, r=0.32, r=0.31). The score was different between patients with and without neoplasms (P<0.001, trial II) and between elective or emergency patients (P<0.001, trial III). In a survey, investigators reported that the score was easy to assess and more comprehensive than four other scores. CONCLUSIONS: The McPeek score values the postoperative outcome on a nonlinear scale. A priori, the average duration of hospitalization and critical care for a specific operation has to be defined. Our validation suggests that it is a reliable, valid, sensitive, and practical instrument for outcome analysis after anesthesia and surgery.

Clinical value of parathyroid scintigraphy with technetium-99m methoxyisobutylisonitrile: discrepancies in clinical data and a systematic metaanalysis of the literature.

There is a considerable discrepancy in the literature concerning the sensitivity of parathyroid scintigraphy (PS) with 99mTc-MIBI. We therefore analyzed our own data and compared them to the literature in a metaanalysis. All patients who received 99mTc -MIBI scintigraphy and subsequent surgery in our department for the detection of enlarged parathyroid glands in primary (pHPT) or secondary (sHPT) hyperparathyroidism between 1991 and 1999 were included in our retrospective analysis. The results of surgery served as the gold standard. For a true positive result, the scintigraphy had to predict the exact location of parathyroid adenoma (PA) or parathyroid hyperplasia (PH). We then compared these data to the results of a nonstatistical systematic metaanalysis of the literature. Patients (178) underwent PS between 1991 and 1999; 139 were operated on and included in this study. Of these, 109 had pHPT and 30 had sHPT. The sensitivity and specificity of the PS were found to be 45%/94% for pHPT and 39%/40% for sHPT. Fifty-two studies concerning PS were included in the metaanalysis. Sensitivities reported varied from 39% to >90%. Consideration of the different possible techniques used for PS could not explain these discrepancies. Our data show that the sensitivity of PS in clinical routine may be lower than expected from the literature. Our data are consistent with other studies and with partially unpublished clinical observations from other university hospitals. We believe that a well-designed and properly conducted prospective study is necessary to evaluate the reasons for the differences observed.

Quality of life and patient satisfaction after reoperation for primary hyperparathyroidism: analysis of long-term results.

Morbidity after reoperation for persistent or recurrent primary hyperparathyroidism (pHPT) is higher than after primary surgery. According to our experience, there is a contrast between postoperative normalization of laboratory parameters and the quality of life/patient satisfaction after reoperation. Therefore the aim of the study was to analyze the outcomes of reoperations in comparison to primary surgery. We evaluated the patients' reported quality of life using the SF-36 (an accepted health status assessment tool) and complete prospectively documented perioperative and follow-up data including postoperative complications. Additionally, we searched for reasons why primary surgical intervention did not succeed. In a prospective cohort study the perioperative data of 653 consecutive patients with pHPT, including 75 reoperated patients (11.5%) who underwent parathyroidectomy between 1987 and 1999, were evaluated by uni- and multivariate analysis. At a median 78 months (6-156 months) postoperatively, all patients underwent a planned follow-up that included the SF-36, physical examination, and laboratory investigations. A total of 51 reoperated patients were available for follow-up. Postoperative alleviation of symptoms or being symptom-free was reported by 70.6%. Patients after reoperation had lower SF-36 scores in all health domains postoperatively than patients after a primary operation. Of the reoperated patients, 19.6% stated that after evaluating the development of their complaints they would not consent to reoperation again. Subgroup analysis showed that 80% of patients with postoperatively persistent pHPT, 60% of those who did not observe symptom alleviation, and 44% of those after sternotomy were in the group of dissatisfied patients. Surprisingly, none of the patients with more than one reoperation, only two of the five patients with permanent recurrent laryngeal nerve injury, and only one of the four patients with persistent hypoparathyroidism were dissatisfied overall. Parathyroidectomy resulted in normocalcemia in 90.2% of the reoperated patients, with an operative morbidity of 27.4% and no mortality. After an unsuccessful operation for pHPT, patients should be treated at an expert center to avoid persistent hypercalcemia. Reoperations necessitating sternotomy should be restricted to patients with severe symptoms and signs.

Adrenal involvement in multiple endocrine neoplasia type 1.

Adrenal lesions belong to the spectrum of multiple endocrine neoplasia type 1 (MEN-1) syndrome. However, the prevalence of adrenal involvement, the characteristics, and the clinical management of adrenal lesions have not yet been clearly defined. A total of 66 patients with confirmed MEN1 germline mutations and 1 additional patient with typical manifestations in three organ systems were monitored in a regular screening program that included evaluation of the adrenals (median follow-up 96 months; range 12 to 300 months). Age at the diagnosis of MEN-1 and of adrenal tumors and the clinical characteristics, genotype, treatment, and follow-up of adrenal disease were analyzed. Adrenal lesions were identified in 18 of 67 (26.8%) MEN-1 patients and were diagnosed 5 years later than MEN-1. The median tumor diameter at diagnosis was 3.0 cm (range 1.2-15.0 cm), with most tumors being 3 cm or smaller. Eight patients had bilateral tumors. Ten patients had nonfunctional benign tumors, three had benign adrenal Cushing syndrome, and one patient had a pheochromocytoma. Four patients developed adrenocortical carcinomas (ACCs), three of which were functional. Nine adrenalectomies and one subtotal adrenalectomy were performed in six patients. Three patients with ACC died owing to the tumor. Patients with mutations in exons 2 and 10 developed adrenal tumors significantly more often than patients with other mutations (p <0.01). Adrenal tumors are a common feature of MEN-1 but occur later in the course of the disease. The lesions are often small and nonfunctional and can therefore be managed by close surveillance; others have significant malignant potential and should be considered for surgery when they are 3 cm or larger.