Tumour size: the only predictive factor for 131I MIBG uptake in phaeochromocytoma and paraganglioma.

BACKGROUND: 131I Meta-iodobenzylguanidine (131I MIBG) scintigraphy can detect chromaffin tumours with a high specificity but its sensitivity remains limited. In this study, the influence of clinical features and tumour pathology on the results of 131I MIBG of patients with phaeochromocytomas and paragangliomas was examined. METHODS: The records of 104 patients operated on for chromaffin tumours who had pre-operative 131I MIBG were reviewed. Demographic data, clinical features, biochemical results and pathology of tumours were analysed. The size of the tumour was assessed by the three measured diameters of the specimen and its calculated volume. Univariate relationship between tumours' characteristics and the results of 131I MIBG were examined. RESULTS: Out of 119 tumours, 104 (87%) were detected by 131I MIBG. Mean +/- SD largest diameter and volume of the tumours were 6.0 +/- 2.4 cm (range 0.8-11.5 cm) and 68 +/- 74 cm3 (range 0.2-421 cm3). Results of 131I MIBG were significantly correlated with both the largest diameter of the tumour (P < 0.01) and by its volume (P < 0.001). 131I MIBG was negative in 35.5% of tumours < 20 cm3 and in 2.6% of those < 20 cm3. No other criteria were correlated with positive 131I MIBG, including aetiology, pathology or catecholamine secretion pattern. CONCLUSIONS: The result of 131I MIBG scanning in patients with chromaffin tumours is significantly correlated with the size of the tumour.

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.

[Exclusive use of calcium channel blockers and cardioselective beta-blockers in the pre- and per-operative management of pheochromocytomas. 70 cases]. / Utilisation exclusive des inhibiteurs des canaux calciques et des beta-bloquants cardio-sélectifs dans la prise en charge péri et per-opératoire des phéochromocytomes. A propos de 70 cas.

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.

[Limits of parathyroid scintigraphy before surgery for hyperparathyroidism of renal origin]. / Limites de la scintigraphie parathyroïdienne avant chirurgie pour hyperparathyroïdisme d'origine rénale (51 cas).

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.

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 /= 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.

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.

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.

Effectiveness and limits of preoperative imaging studies for the localisation of pheochromocytomas and paragangliomas: a review of 282 cases. French Association of Surgery (AFC), and The French Association of Endocrine Surgeons (AFCE).

OBJECTIVE: To find out the optimal strategy for the preoperative location of pheochromocytomas and paragangliomas. DESIGN: Retrospective study. PATIENTS: 282 patients operated on for histologically confirmed pheochromocytoma in France between 1980 and 1991, the past decade. MAIN OUTCOME MEASURES: The results of imaging procedures, i.e. computed tomography (CT), (131)I meta-iodobenzylguanidine scintigraphy (MIBG) and magnetic resonance imaging (MRI) were reviewed. RESULTS: Pheochromocytomas were sporadic in 206 (73%). They were unilateral in 189 (67%), bilateral in 54 (19%) and extra-adrenal in 39 (14%). Overall sensitivity of the studies was 89% for CT, 98% for MRI, and 81% for (131)I-MIBG. In unilateral adrenal lesions sensitivity were 100% for CT and MRI, and 88% for (131)I-MIBG; in bilateral lesions 66% for CT, 100% for MRI, and 62% for (131)I-MIBG; in extra-adrenal lesions 64% for CT, 88% for MRI, and 64% for (131)I-MIBG. CONCLUSION: The accuracy with which pheochromocytomas and paragangliomas can be visualized questions nowadays the routine use of abdominal approach. In selected cases of sporadic unilateral chromaffin tumours, a posterior, lateral, or even laparoscopic approach should be considered.

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.

[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]. / Persistance et récidives loco-régionales des cancers thyroïdiens différenciés opérés. 22 cas sur 589 patients traités de 1964 à 1990, suivis de 5 à 31 ans.

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.

Questionability of the benefits of routine laparotomy as the surgical approach for pheochromocytomas and abdominal paragangliomas.

BACKGROUND: Improvement of preoperative imaging of pheochromocytomas and abdominal paragangliomas may render routine laparotomy questionable as the surgical approach of choice for these lesions. METHODS: We studied the records of 100 patients with chromaffin tumors who underwent abdominal exploration. The disease was familial in 28 patients and was malignant in 19. Seventy-five patients had intraadrenal disease (bilateral in 13). Computed tomography (CT), metaiodobenzylguanidine (MIBG) scintigraphy, and magnetic resonance imaging (MRI) were performed since 1979, 1984, and 1987 in 97, 73, and 43 patients, respectively. False-positive and false-negative results were defined as any discrepancy between imaging results and surgical findings. RESULTS: Overall accuracy of preoperative localization was 85% with CT scan, 77% with MIBG scintigraphy, and 86% with MRI. In unilateral pheochromocytoma, accuracy was 94% with CT scan, 80% with MIBG scintigraphy, and 96% with MRI. When all three studies were performed (n = 38), overall accuracy was 97% and only one extraadrenal tumor in a patient with familial pheochromocytoma was overlooked. CONCLUSIONS: The outstanding accuracy of available imaging techniques questions the strategy of routine laparotomy for sporadic and seemingly benign pheochromocytomas, favoring more elective approaches such as the posterior approach or laparoscopy.