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Biomedical subjects

B Carnaille

Publications and source records attributed to B Carnaille.

At least 37 records · Page 2Linked to original sources

Late outcome of 304 consecutive patients with multiple gland enlargement in primary hyperparathyroidism treated by conservative surgery.

The aim of this study was to assess the late outcome of patients with primary hyperparathyroidism and multiple gland enlargement (MGE) treated by conservative surgery. MGE in primary hyperparathyroidism is the presence of two or more enlarged glands weighing more than 50 mg. Conservative surgery consists in resecting the grossly enlarged glands without biopsying the normal glands. Some authors have suggested that this approach overlooks minute hyperplasia, leading to late recurrences of hyperparathyroidism; conversely, it may result in the unnecessary resection of grossly enlarged, but not hyperfunctioning, glands. Altogether 1231 patients were operated on for primary hyperparathyroidism between 1966 and 1995. Of these patients, 304 (24.9%) had MGE, including 42 cases of multiple endocrine neoplasia (MEN), 12 familial cases, and 250 seemingly sporadic cases. Two, three, or four glands (or more) were involved in 61.8%, 21.4%, and 16.4% of cases, respectively. During the early postoperative period one patient died and ten were reoperated for persistent hypercalcemia. The pathologic diagnoses were double adenomas (13.5%), hyperplasia (35.8%), association of the two (39.8%), and a normal second gland (10.8%) on light microscopy findings. None of the 30 deaths that occurred during follow-up was related to hyperparathyroidism. Altogether 190 patients (79%) were available for follow-up (average 89.3 months): 90% were normocalcemic, 4.7% hypocalcemic, and 5.2% hypercalcemic. A late iPTH assay was done in 147. PTH was appropriate to the serum calcium level in 84.3% and appropriate to normal calcemia in 91.6% of 132 cases. Conservative surgery is thus an acceptable treatment for MGE in patients with hyperparathyroidism. Few late recurrences occur, for which there are no individual predictive criteria.

Adenoma↗

Intraoperative gastrin measurements during surgical management of patients with gastrinomas: experience with 20 cases.

Despite recent advances in imaging techniques for endocrine tumors of the duodenum and the pancreas, preoperative localization of gastrinomas is inconsistent. Successful surgical management of patients with Zollinger-Ellison syndrome (ZES) and removal of all gastrin-secreting tumors remains a difficult task. The aim of the study was to evaluate the predictive value of intraoperative gastrin measurements for successful surgical treatment in patients with gastrinomas. Intraoperative gastrin measurements were performed in 20 patients with ZES who underwent resection of gastrin-secreting tumors. Gastrin was measured with a radioimmunologic assay in blood samples obtained from a peripheral vein and from the portal vein at the beginning of the operation (T0) and 20 minutes after removal of the lesion(s) (T1). In 16 patients gastrin was also measured 4 minutes after injection of secretin 3 U/kg (T2). Thirteen patients (65%) were cured by surgery. In two of them, peripheral and portal gastrin levels were normal at T0, precluding any further interpretation of the test. Completeness of surgery was confirmed by normalization of gastrin levels at T1 or the absence of stimulation at T2 (or both) in 10 patients. In only one case did the gastrin levels remain elevated at T1 despite a favorable outcome after surgery. In each of the seven patients (35%) who had persisting disease at 1 year, failure of the surgical procedure was predicted by persistence of high levels of gastrin at T1. In patients with hypergastrinemia, the positive predictive value of intraoperative gastrin measurement for completeness of surgery and the specificity were 100%. The negative predictive value was 88% and the sensitivity 91%. The overall accuracy of the test was 94%. In patients with ZES the normalization of systemic hypergastrinemia during surgery affirms the successful removal of all gastrin-secreting tumors. We conclude that intraoperative gastrin measurement is a valuable addendum for optimizing the surgical management of gastrinoma.

Gastrins↗

Hypocalcemia following thyroid surgery: incidence and prediction of outcome.

Postoperative hypocalcemia is a common and most often transient event after extensive thyroid surgery. It may reveal iatrogenic injury to the parathyroid glands and permanent hypoparathyroidism. We prospectively evaluated the incidence of hypocalcemia and permanent hypoparathyroidism following total or subtotal thyroidectomy in 1071 consecutive patients operated during 1990-1991. We then determined in a cross-sectional study which early clinical and biochemical characteristics of patients experiencing postoperative hypocalcemia correlated with the long-term outcome. Postoperative calcemia under 2 mmol/l was observed in 58 patients (5. 4%). In 40 patients hypocalcemia was considered severe (confirmed for more than 2 days, symptomatic or both). At 1 year after surgery five patients (0.5%) had persistent hypocalcemia. We found that patients carried a high risk for permanent hypoparathyroidism if fewer than three parathyroid glands were preserved in situ during surgery or the early serum parathyroid hormone level was </= 12 pg/ml, the delayed serum calcium levels </= 8 mg/dl, or the delayed serum phosphorus level >/= 4 mg/dl under oral calcium therapy. When one or more of these criteria are present, long-term follow-up should be enforced to check for chronic hypocalcemia and to avoid its severe complications by appropriate supplement therapy.

Cross-Sectional Studies↗

Intraoperative insulin measurement during surgical management of insulinomas.

Intraoperative hormonal measurements have been used successfully to guide the surgical treatment of various endocrine diseases. In this study, we report the results of intraoperative insulin measurement (IIM) in patients with organic hypoglycemia. IIMs were performed during 52 operations in 51 patients. Hyperinsulinism was secondary to a sporadic insulinoma (M = 40), a type I multiple endocrine neoplasia (MEN-I) (M = 8), an insulin-secreting carcinoma (M = 1), or pancreatic nesidioblastosis (M = 2). The insulin was measured with a radioimmunologic assay in blood samples simultaneously drawn from a peripheral vein and the portal vein at the beginning of the operation (T1) and 20 minutes after tumor removal (T2). Normoglycemia was achieved after surgery in 50 cases (96%). Systemic and portal insulin levels were normal at T1 in eight patients, precluding any further interpretation of the test. Completeness of surgery was confirmed by normalization of both systemic and portal insulin levels at T2 in 36 patients. In seven cases the systemic or portal insulin levels (or both) remained elevated at T2 despite a favorable outcome after surgery. Failure of the surgical procedure was predicted in two patients by the persistence of high levels of insulin at T2. In patients with initially elevated serum insulin levels, the positive predictive value and the specificity of intraoperative insulin measurement for completeness of surgery were both 100%. The sensitivity was 84%, the negative predictive value 22%, and the accuracy of the test 84%. We concluded that IIM is a simple, highly reliable tool for predicting the completeness of surgery in patients with organic hypoglycemia. IIM appears to be a valuable addendum to the surgical armamentarium against insulinoma especially for patients with atypical causes, such as MEN, insulin-secreting carcinoma, or pancreatic nesidioblastosis.

Adolescent↗

Reoperation for persistent or recurrent primary hyperparathyroidism.

AIM: To analyse the causes and outcome of reoperations for persistent or recurrent primary hyperparathyroidism (HPT). MATERIALS AND METHODS: We reviewed the medical records of 38 patients who underwent reoperation between December 1965 and April 1997 for persistent or recurrent primary HPT. During this period of time, 1448 patients underwent operation for primary HPT, 294 for renal HPT and 58 for questionable disease, i. e. a total of 1800. In the 1448 cases of HPT, 22 patients were reoperated after a first cervicotomy in the institution, i. e. a first reoperation rate of 1.5%. Sixteen patients were referred after unsuccessful parathyroid surgery. Six patients were reoperated on twice and one patient seven times for a graft-dependent recurrence. RESULTS: Reasons for failed parathyroid operations included tumour tissue in ectopic location (75%) or tumour tissue that had not been seen in normal position (19%), parathyreomatosis (2%), parathyroid cancer (2%) and graft-dependent hypercalcaemia (2%). Of the ectopic glands, 45 % were intrathymic, 12.5% intrathyroidal, 7.5% retro-oesophageal, 7.5% in the carotid sheath, 5% interthyrotracheal, 5% in the mediastinum (extrathymic), 5% in the aorto-pulmonary window, 2.5% undescended, 2.5% overdescended and 2.5% intertracheooesophageal. Of the patients, 38% had uniglandular lesions, 60% multiglandular lesions, and 2% parathyroid cancer. The histologically confirmed cause of HPT was adenoma in 50% of cases, hyperplasia in 38% and "normal" but overweight glands in 9.5%. Of the 38 patients studied, 15 presented one or more supernumerary glands (fifth to eighth gland). Reoperation was performed through a cervical incision in 82% of cases and using a mediastinal approach in 18% (8 median sternotomies, 1 thoracoscopy). The success rate of the reoperations was 92%. A total of 8% of patients suffered permanent unilateral vocal cord paralysis; 5% are definitely hypocalcaemic. The sensitivities of preoperative localization studies ranged from 69% for sestamibi scan and 63% for selective venous catheterization to 16% for computed tomography. CONCLUSION: Repeated parathyroidectomy can be avoided in more than 98% of patients if an experienced surgeon performs bilateral cervical exploration during the initial parathyroid operation. For patients with persistent or recurrent primary HPT, preoperative localization studies and a focused surgical approach can result in a 92% success rate with a minimal complication rate.

Adenoma↗

Experience gained from operation of 103 adrenal incidentalomas.

BACKGROUND: Adrenal incidentaloma poses a perplexing problem for physicians. The challenge is to identify the rare functioning or malignant adrenal tumor that should be removed. PATIENTS AND METHODS: From 1970 until December 1996, we managed 579 patients with adrenal hyperplasia or neoplasm. Of these, 110 were followed without any operation and 469 were operated on. The histology from the operated patients revealed 145 pheochromocytomas, 72 Cushing's syndrome, 98 aldosteronomas, 21 virilizing tumors, 3 feminizing tumors, 98 non-functioning adrenalomas, 17 metastases, 7 cysts, and 15 miscellaneous tumors. RESULTS: Among the 98 non-functioning adrenal tumors, 79 were incidentalomas. Histological examination of 103 operated incidentalomas found 55 adenomas (5 functioning=aldosteronomas), 12 benign pheochromocytomas, 3 malignant or suspicious pheochromocytomas, 8 cystic lymphangiomas, 4 metastases, 4 angiomas, 3 ganglioneuromas, 4 myelolipomas, 1 hyperplasia, 2 hematomas, 2 cysts, and 5 adreno-cortical carcinomas. Among the non-operated incidentalomas, the most frequent findings were adenomas, metastases, hematomas, cysts and pseudotumoral hyperplasia; three disappeared spontaneously. No patients died of unrecognized adrenocortical carcinoma. CONCLUSION: Our experience confirms that in case of incidentaloma, the first concern is a nonfunctional pheochromocytoma (15%), and the less common occurrence is adreno-cortical carcinoma (2%). We recommend a restrictive surgical approach in the setting of adrenal incidentalomas.

Adenoma↗

Localization of abnormal parathyroid glands with jugular sampling for parathyroid hormone, and subtraction scanning with sestamibi or tetrofosmine.

BACKGROUND: Accurate pre-operative localization of abnormal parathyroid glands is an essential prerequisite for elective surgical approaches such as the unilateral approach for treatment of primary hyperparathyroidism. METHODS: In 175 patients undergoing a bilateral neck exploration for primary hyperparathyroidism, localization of abnormal parathyroid glands was performed with jugular venous sampling for parathyroid hormone (PTH, n = 75), subtraction scan with iodine 123 and technetium-99m-sestamibi (MIBI, n = 50), or tetrofosmine (TTF, n = 50). The results of these localization studies were compared with operative findings. RESULTS: The sensitivity of PTH, MIBI, and TTF was 41, 63, and 71%, respectively. The specificity was 90, 98, and 98% and the positive predictive value was 85%, 82%, and 87%, respectively. The smallest abnormal parathyroid that was correctly detected weighed 110 mg with PTH, 118 mg with MIBI, and 70 mg with TTF. Subtraction scan (MIBI and TTF) detected mediastinal glands in five cases out of six. In patients with multiglandular disease, the results of all three studies were severely impaired. CONCLUSIONS: Subtraction scanning with MIBI or TTF, but not jugular sampling for PTH, appeared to be useful for localization of abnormal parathyroid glands in patients with primary hyperparathyroidism. Nevertheless, an elective unilateral approach guided by the localization studies would not have allowed the excision of all abnormal parathyroid glands in patients with multiglandular disease.

Female↗

Improvements in parathyroid surgery in the intact 1-84 PTH assay era.

BACKGROUND: Measurement of intact 1-84 parathormone (PTH) level by immunometric assays has been an important tool in the medical management of hypercalcaemia. The aim of the present study was to evaluate its practical contribution in the diagnosis and surgical treatment of primary hyperparathyroidism. METHODS: The results of surgery were compared (number of failed and inadequate cervicotomies, number of overlooked cervical glands) in two groups of primary hyperparathyroid patients operated on without (group I, n = 624) and with (group II, n = 360) intact PTH evaluation. The postoperative intact PTH level was measured 1-50 months after surgery in 109 unselected normocalcaemic patients. The benefit of intact PTH assay was studied in borderline patients. We tried to settle a correlation between parathyroid resected weight and intact PTH pre-operative level in different subsets of the disease. RESULTS: The sensitivity for the diagnosis of primary hyperparathyroidism was 86.6% for intact PTH level alone, and 95.9% when plotted with synchronous serum total calcium. Hyperfunctional adenoma was incidentally discovered in 14 asymptomatic normocalcemic normo-PTH patients. The predictive positive value of intact PTH assay for the diagnosis of primary hyperparathyroidism was 99.3%. Intact PTH assay often affirmed the diagnosis of primary hyperparathyroidism in borderline cases. The postoperative intact PTH measurement was not a good indicator for evaluating the late outcome in seemingly cured patients. Sharp but statistically significant correlation was found between the resected weight and the intact PTH level. The discovery of a small adenoma in a patient with high intact PTH level, bone disease and low vitamin D level should raise suspicion of a second hyperfunctioning gland. CONCLUSIONS: Intact PTH assay was a main contributor to the surgical management of primary hyperparathyroidism, reducing the number of unnecessary cervicotomies and enabling the cure of all cases of primary hyperparathyroidism except those due to mediastinal glands. It raised unanswered problems in the late postoperative course.

Calcium↗

Pancreatitis and primary hyperparathyroidism: forty cases.

BACKGROUND: Pancreatitis is associated with primary hyperparathyroidism (PHPT) in 1.5-7% of cases. The relationship of cause and effect between the two diseases has been debated. METHODS: To evaluate this relationship, the clinical, biochemical and pathological data on 1435 patients operated on for hyperparathyroidism (HPT) over the past 30 years were retrospectively reviewed. A total of 1224 of these patients had biologically proven and cured PHPT and 211 patients had renal HPT (RHPT). The diagnosis of pancreatitis (PTS) was based on a high serum amylase level and/or abnormalities on ultrasound or computed tomography (CT) scan explorations. Only patients without biliary stones were included in the PTS group associated with HPT. RESULTS: A total of 3.2% (n = 40) of patients with PHPT had PTS, which was acute in 18 cases, subacute in 8 cases and chronic in 14 cases. This rate of PTS is higher than in a random hospital population. Surgical cure of HPT was followed by the spontaneous healing of 17/18 acute PTS, whereas six of the 22 patients with subacute or chronic PTS developed complications due to the evolution of their disease (diabetes, pancreatic duct stenosis treated by surgery). A single diseased gland was found in 27 patients with PTS, which is in favour of primary parathyroid disease, being responsible for, and not a consequence of, PTS. Only the serum calcium (13.0 vs 12.1 g/dL) level was significantly increased in PHPT patients with PTS, when compared to those without PTS. The calcium level is probably of major importance in the development of PTS, which was never encountered in 211 patients with RHPT, who had low calcium and high PTH levels. CONCLUSIONS: The data suggest that (i) the PTS-PHPT association is not incidental; (ii) PTS is the consequence and not the cause of PHPT; (iii) hypercalcaemia seems to be a major factor in the development of PTS in PHPT patients; and (iv) cure of PHPT leads to the healing of acute PTS, whereas it does not affect the evolution of subacute and chronic PTS.

Acute Disease↗

[Exclusive use of calcium channel blockers and cardioselective beta-blockers in the pre- and per-operative management of pheochromocytomas. 70 cases].

The pre and intraoperative use of calcium channel blockers (CCB) has been suggested for the management of either eutopic or ectopic pheochromocytomas. We report our experience of 70 pheochromocytomas, operated between 1988 and 1996 and managed with CCB, especially nicardipine. 59 were hypertensive (84.2%). Preparation consisted of nicardipine in 61 patients or another CCB in 9 cases with duration ranging from 24 hours to several weeks depending on plasma volume and blood pressure control. Intraoperatively, nicardipine infusion was started after intubation, adjusted according to systolic blood pressure (SBP) and stopped before ligation of the tumor venous drainage. Increases in SBP greater than 200 mmHg were observed in 10 patients and were effectively controlled by nicardipine in all cases. In 16 patients, the S > BP remained less than 150 mmHg throughout anesthesia. Heart rate greater than 100 b p m occureed in 51 patients and was easily controlled with esmolol whenever used (n = 27). Arythmias were unfrequent (n = 4) and required treatment in only one case. This study confirms the ability to adequately manage pheochromocytomas with the use of nicardipine as sole vasodilating agent.

Adolescent↗

[Limits of parathyroid scintigraphy before surgery for hyperparathyroidism of renal origin].

AIM OF THE STUDY: To evaluate the results of parathyroid scinti scans (sestamibi or tetrofosmin) for detection of hyperplastic parathyroid glands responsible for renal hyperparathyroidism. METHODS: Injection of 15 mCi sestamibi or tetrofosmin and gammacamera acquisition of images focused on neck and mediastinum, 20 minutes and 2 hours thereafter. Injection of 150 mCi Iodine 123, acquisition of images 2 hours afterwards and visual subtraction. PATIENTS: 51 patients with renal insufficency or renal transplant were referred for surgical treatment of hyperparathyroidism. 52 scintiscans (sestamibi n = 19, tetrofosmin n = 33) were performed before operation (subtotal parathyroidectomy, bilateral thymectomy and parathyroid tissue cryopreservation). RESULTS: 180 hyperplastic parathyroid glands were resected, 71 of which had been detected by scintiscan. The factors modifying the results were the weight of the resected lesion and reoperation. All hyperplastic glands were detected in only 1 out of 41 scintiscans performed before first hand operations, whereas all missed glands were imaged in 8 out of the 10 explorations performed before reoperation for persistent renal hyperparathyroidism. The radionuclide, the type of hyperparathyroidism, the parathyroid location, patient's age and gender did not influence the results. No false-positive result was observed. CONCLUSION: Parathyroid scintiscan should not be routinely performed before the first neck exploration for renal hyperparathyroidism. It is mandatory in those cases needing reoperation for recurrent disease.

Adolescent↗

[Morbidity from total thyroidectomy in subjects less than 15 years of age. A multicenter study of 107 cases].

In the MEN II (Multiple Endocrine Neoplasia) setting, genetic screening is currently available with a 98% reliability. Therefore, total thyroidectomy has been recommended as early as at 3 years of age for gene-carriers. A nationwide retrospective survey has been conducted over 5 years, dealing with 107 cases of total thyroidectomy in patients less than 15 years of age to assess the morbidity of this kind of surgery. Currently, permanent morbidity (recurrent nerve palsy and hypocalcemia) amounts to 1 and 1% of cases respectively. It is observed only when node clearance is deemed to be mandatory. Mortality was zero. In published series (4 overall, from 1972 through 1995), rates seemed to be more important: 11 and 13% respectively, with 1% mortality. These numbers relate to surgery with advanced cancers, often locally invasive. Prophylactic total thyroidectomy morbidity draws near the results of our study. These numbers can argue for the interest of a "prophylactic" total thyroidectomy, before advanced thyroid disease, as a treatment for MTC gene-carriers children and teenagers.

Adolescent↗

[Loco-regional persistence and recurrences of operated differentiated thyroid cancers. 22 cases on 589 treated patients from 1964 to 1990, follow-up from 5 to 31 years].

AIM OF THE STUDY: Evaluation of frequency and outcome of loco-regional persistance and recurrences of differentiated thyroid carcinomas after surgery. MATERIAL AND METHODS: from 1964 to December 1990, we operated and followed up more than 5 years (from 5 to 31 years): 589 thyroid cancers (309 papillary, 262 follicullar et 18 Hürthle cells); 145 patients were lost to follow-up (24%), 33% of them were microcarcinomas. Surgery consisted ultimately in 411 total thyroidectomies, 51 sub-total thyroidectomies, 113 lobectomies with isthmusectomies, 9 isthmusectomies or tumorectomies and 4 tracheotomies. Neck dissection was not routinely performed but rather selectively (n = 137). All Patients were put under suppressive hormone therapy. After total thyroidectomy, ablative 131 iodine was almost routinely done. All patients have been controlled by clinical examination, radio-iodine scanning and, since 1983, by sequential thyroglobulin assays. We have analysed the course of patients according to L. J. Degroot's staging (Stage I: Patient with intrathyroidal disease. Stage II: Patient with cervical node involvement. Stage III: Patient with extra-thyroidal neck invasive disease. Stage IV: Distant metastasis). RESULTS: Stage I, n = 383, Stage II, n = 96. Total = 479. Local recurrences in the bed of total thyroidectomy were exceptional: n = 2/323 (0.6%). Recurrences in the thyroid remnant after non total thyroidectomy were rare: n = 3/156 (2%). Cervical nodal recurrences were also rare: n = 7/479 (1.5%). Stage III (n = 73): 34/306 papillary, 9/78, well differentiated follicular, 25/175 poorly differentiated follicular. 2/9 follicular of other type and 3/18 hurtle cell. In 67 patients, the loco-regional sterilization was achieved, 5 of which were reoperated for nodal cervical metastasis. 6 patients died from continuing loco-regional disease and 10 presented distant metastases. 4 of which died from. Among opered patients are still alive (52/73 = 71,2%) no loco-regional recurrence persisted. CONCLUSION: The true problem is not recurrence but the persistance after surgery in stage III patients, despite the application of 1131. The determining factor of the persistance and recurrence is the initial stage of the carninomatous disease at the time of treatment.

Adenocarcinoma, Follicular↗

[Preoperative localization of parathyroid lesions with Tc-99m-MIBI/iodine 123].

OBJECTIVE: Assess the sensitivity of MIBI-Tc for the detection of parathyroid lesions and define the threshold of detection by evaluating contrast uptake at 20 minutes and 2 hours. METHODS: Fifty-five operated and cured patients were studied. Image acquisitions were made 20 minutes and 2 hours after injection of 555 MBq MIBI-Tc then 2 hours after injection of 5.55 MBq iodine 123. RESULTS: A total of 87 pathological glands were found including 47 adenomas (80 to 8820 mg) and 40 hyperplasias (44 to 2175 mg). Detection sensitivity was 81% for adenomas and 30% for hyperplasia, but the cell type did not appear to be a determining factor. CONCLUSION: Scintigraphic visualization of parathyroid lesions is more frequent when there is a single lesion. At least one lesion was seen in 80% of the patients. The threshold of detection was 225 mg. Enhancement of parathyroid uptake with MIBI-Tc compared with the thyroid between 20 minutes and 2 hours was not a constant and of unknown origin.

Adenoma↗

Comparison of somatostatin analogue and metaiodobenzylguanidine scintigraphy for the detection of carcinoid tumours.

The purpose of this prospective study was to compare the ability of radiolabelled somatostatin analogue (RSA) and metaiodobenzylguanidine (MIBG) scintigraphy to display carcinoid tumours. Forty patients were studied after radiological assessment based on clinical symptomatology. These patients had radiologically demonstrated tumours (n=28), resected tumours discovered to be of the carcinoid type (n=5) or clinically and biologically suspected carcinoid tumours (n=7). They underwent indium-111 DTPA-pentetreotide or iodine-123-Tyr-3-octreotide and 131I-MIBG scintigraphy. The results were compared with those of complementary surgical or morphological examinations and analysed according to the site of the tumour and the symptomatology. In the case of 31 patients with a total of 55 tumoral sites, the sensitivity of the initial radiological assessment, of RSA and of MIBG was 96%, 86% and 64%, respectively, for the detection of at least one tumour per patient, but 51%, 85% and 51%, respectively, for the total number of sites. No site was detected solely by MIBG. The concordance between RSA and MIBG was better when all sites were considered (kappa index+0.44) than for only extrahepatic abdominal tumoral sites (kappa index+0.095). Abdominal, thoracic or bone marrow tumours were more easily detected with RSA than with MIBG. Hepatic invasion (21 cases) was more easily detected by radiology (sensitivity 100%) than by RSA and MIBG, both of which displayed a sensitivity of 80%, but with differences in uptake intensity. Tumour detection using MIBG was more significantly linked with flush (P<0.01) than with diarrhoea (P>0.10). In the assessment of carcinoid tumours, RSA scintigraphy should be carried out initially (just after hepatic ultrasonography) and supplemented by MIBG, as comparison of the studies serves to guide therapeutic options and might be valuable for prognosis.

3-Iodobenzylguanidine↗

Primary hyperparathyroidism in multiple endocrine neoplasia type IIa: retrospective French multicentric study. Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group), French Association of Endocrine Surgeons.

Primary hyperparathyroidism (PHPT) in multiple endocrine neoplasia (MEN) type IIa is rare, occurring in 20% to 30% of the patients. The aim of this study was to evaluate clinical findings, surgical therapy, and outcome for 56 patients affected by PHPT among 249 MEN-IIa patients collected from 84 families assembled by the Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group). This retrospective study was based on cases registered by the GETC (20 participating centers) from 1969 to 1994. Characteristics of PHPT in 56 patients (31 women, 25 men) with MEN-IIa were reviewed. All but two underwent cervicotomy. The median age at diagnosis was 37.6 years. PHPT was found concomitantly with medullary thyroid carcinoma (MTC) or pheochromocytoma in 43 patients (77%). PHPT was asymptomatic in 68% of the patients. Serum calcium levels ranged from 2.20 to 3.70 mmol/L (median 2.82 mmol/L; normal 2. 10-2.60 mmol/L). The number of parathyroid glands removed at surgery was 0 (n = 2), 1 (n = 24), 2 (n = 5), > 2 (n = 12), 4 (n = 11). Pathology (initial surgery) consisted of 24 adenomas, 4 double adenomas, and 25 hyperplasia. Cure after initial surgery was obtained in 89%, including a 22% incidence of hypoparathyroidism. There were 6 cases (11%) with persistent PHPT. With a mean follow-up of 6.4 years, five patients (9%) had recurrent PHPT. The results indicate that MEN-IIa-related PHPT is generally associated with mild, often asymptomatic hypercalcemia. Despite recurrences encountered 5 to 15 years after the first cervicotomy, resection of only macroscopically enlarged glands generally appears sufficient. Subtotal or total parathyroidectomy with autotransplantation is associated with a high rate of hypoparathyroidism.

Adenoma↗