Increased detection of non-recurrent inferior laryngeal nerve (NRLN) during thyroid surgery using systematic intraoperative neuromonitoring (IONM).

BACKGROUND: Non-recurrent inferior laryngeal nerve (NRLN) is a rare anatomical variant with a reported incidence of 0.6-1.3 %. It carries a higher risk of palsy during thyroid surgery. Its detection is mandatory in order to avoid such complication. METHODS: Systematic intraoperative neuromonitoring (IONM) was carried out for 806 consecutive nerve at risk (NAR) patients in two centers (Lille and Varese). In 402 patients surgery to the right side was performed with IONM. The IONM of the inferior laryngeal nerve (ILN) was conducted as recommended by international guidelines (V1/R1/V2/R2). RESULTS: A NRLN was detected in 11 of 402 NAR (2.7 %). In the first center (Lille) the incidence of NRLN was 6.0 % (7/117). No loss of signal (LOS) was reported in this group of patients. Postoperative laryngoscopy was normal in all patients with NRLN. CONCLUSIONS: The true incidence of NRLN may be higher than expected. Neurophysiology helps the surgeon to better understand the anatomy and function of nervous structures. Intraoperative neuromonitoring is a useful tool that should be systematically implemented in thyroid surgery to better understand the anatomy and physiology of the inferior laryngeal nerve. Its use may allow the surgeon to decrease the incidence of nerve palsy especially in case of NRLN. The IONM adjunct does not add significantly to the costs for thyroid surgery.

Pheochromocytoma revealed by acute heart failure. When should we operate? Presented at the ESES Congress, Gothenburg May 25-26, 2012.

PURPOSE: The aim of this study was to assess the safety and efficacy of adrenalectomy on patients with pheochromocytoma diagnosed at the time of an acute heart failure (AHF). METHODS: We reported cases of patients who presented an AHF secondary to a pheochromocytoma during a period of 10 years. The diagnosis of AHF was defined by a left ventricular ejection fraction of less than 30 % or the use of circulatory assistance. They had adrenalectomy as emergency surgery or later. Morbidity and mortality of surgery were studied. RESULTS: Thirteen patients required an adrenalectomy for AHF secondary to pheochromocytoma. Four patients (31 %) had an adrenalectomy in emergency. Nine patients (69 %) had a delayed surgery with a median delay of 25 days (7-180). Eight patients had circulatory assistance (61 %). Five of them had a circulatory assistance and a delayed surgery (38 %), two of them had a circulatory assistance followed by emergency surgery (at 1.5 and 3 days) and one had emergency surgery immediately followed by circulatory assistance. Emergency surgery was performed by laparotomy in all cases and delayed surgery by laparoscopy for seven patients (54 %). Perioperative complications consisted in: one circulatory arrest, two bleedings requiring transfusion, one intestinal ischaemia, one haemoperitoneum with re-operation (day 8). One patient died on day 5. Post-operative course of patients with delayed surgery was uneventful. CONCLUSIONS: AHF revealing a pheochromocytoma is a rare and serious event. Patients with emergency surgery have more complications than those with delayed surgery.

[CCAFU's contribution to the French National Cancer Institute's reference frame: Adrenal malignant tumors]. / Contribution 2013 du CCAFU au référentiel INCa : Tumeurs malignes de la surrénale.

INTRODUCTION: Malignant tumours of the adrenal gland are adrenocortical carcinomas (ACC), malignant phaeochromocytomas (MPC) or metastatic tumours. The objective of this article is to propose guidelines for the management of these tumours. MATERIAL AND METHODS: A review of the literature was performed by selecting articles on malignant tumours of the adrenal gland published in PUBMED. RESULTS: Abdominal computed tomography is the reference first-line examination. A diameter > 6 cm, a heterogeneous appearance, irregular margins, spontaneous high density (> 20 HU) and delayed wash-out are radiological signs of malignancy. MRI can be used to characterize the tumour with a sensitivity of 89% and a specificity of 99%. Hormone assays and an endocrinology consultation are recommended before any management. When ACC is suspected, (18)FDG-PET is the reference scintigraphic examination, while the isotope of choice for MPC is (18)F-DOPA, which is more sensitive than MIBG. These scintigraphic examinations have a sensitivity close to 100% and allow staging of distant metastases. Percutaneous biopsy has a limited place in the diagnostic work-up. It is only indicated in the case of suspected adrenal metastasis after having excluded phaeochromocytoma and must not be performed in the case of suspected adrenocortical carcinoma. Surgery is first-line treatment for localized and resectable tumours, but is rarely curative due to the high recurrence rate. For ACC, adjuvant therapy by mitotane or adjuvant radiotherapy can be proposed. Metabolic radiotherapy with (131)I-MIBG can be proposed for the treatment of MPC. First-line chemotherapy is indicated in the case of advanced disease or unresectable tumour. Surgical treatment of adrenal metastasis by adrenalectomy is recommended depending on the type and prognosis of the primary cancer. CONCLUSION: Preoperative laboratory, morphological and scintigraphic assessment is essential before any management. First-line treatment is surgical when the tumour is resectable, but must be completed by adjuvant therapy to limit the risk of recurrence.

[Pheochromocytomas and paragangliomas in pregnancy: About four cases and key messages on management]. / Phéochromocytomes et paragangliomes pendant la grossesse : à propos de quatre cas et messages clés sur la prise en charge.

OBJECTIFS: The diagnosis of a pheochromocytoma or paraganglioma secreting during pregnancy is a rare and serious situation, involving maternal-fetal prognosis. The purpose of this case series is to discuss the management of these patients. METHODS: This is a retrospective study of cases of pheochromocytoma (n=2) or paraganglioma (n=2) managed during pregnancy between 2013 and 2020 in one center (Lille, France). RESULTS: We report four cases of patients with a diagnosis of pheochromocytoma or paraganglioma during pregnancy, at respectively 4, 28, 31 and 34 weeks of amenorrhea (AS). Their pregnancies were affected by a sudden onset of hypertension sometimes associated with headaches, sweating, and palpitations. All patients delivered by Caesarean section after calcium channel blocker impregnation, with a good outcome. Tumor removal took place at a distance from delivery for each patient. CONCLUSIONS: The therapeutic strategy includes antihypertensive treatment with calcium channel blockers or alphablockers and surgical curative treatment linked to gestational age. Multidisciplinary management as well as early diagnosis can improve the maternal-fetal prognosis. The preferred way of delivery is Caesarean section, but vaginal delivery can also be considered. Removal should ideally take place at a distance from the birth. The analysis of these cases has led to the development of a protocol for monitoring and management of parturients with diagnosis of pheochromocytoma or paraganglioma during pregnancy.

Does the RET variant G691S influence the features of sporadic medullary thyroid carcinoma?

OBJECTIVE: The RET (rearranged during transfection) proto-oncogene G691S variant is over-represented in the germline of patients with sporadic medullary thyroid carcinoma (sMTC) vs. normal controls but so far is not associated with any medical or pathological features of the tumour. The aim of our study was to assess the influence of this variant on the age of onset, clinical, biological and pathological features of sMTC. DESIGN AND PATIENTS: One hundred patients with histologically proven MTC, for whom the germline genetic analysis of RET was negative and medical records were available, were included in the study. RESULTS: Patients with the heterozygous GS variant or the homozygous SS variant (n = 36) were on average 8.0 years younger than patients with the wild-type GG variant (n = 64, mean age 43.9 vs. 51.9 years, P < 0.01). The former group did not differ from the wild-type group in terms of MTC size, prevalence of C-cell hyperplasia (CCH) or papillary thyroid carcinoma (PTC). However, the prevalence of an increased preoperative basal calcitonin (bCT) level (> 1000 pg/ml) was 2.75-fold higher in the patients with the GS or SS variant than in those with the wild-type variant (P < 0.001). The proportion of patients with lymph node metastases was also higher in the former group (P < 0.05). Multivariate analysis confirmed that the presence of the RET variant is independently associated with higher preoperative bCT values (P = 0.011). CONCLUSIONS: Our data demonstrate that the RET G691S variant could modulate the age of onset of sMTC as demonstrated previously for familial tumours. Moreover, this variant is an independent predictor of a higher basal calcitonin synthesis rate in patients with sMTC.

Calcitonin levels do not decrease with weaning in chronic alcoholism.

OBJECTIVE: Alcohol might increase calcitonin but this assertion is mainly based on the acute effect of the drug in small animals and humans. The aim of this study was to investigate the effect of chronic alcoholic intoxication on plasma calcitonin (CT) levels. DESIGN: 20 smoking male subjects admitted to be weaned from chronic daily alcohol consumption >100 g were included after informed consent. Blood was sampled upon admission (T0) and after 5 (T5) and 21 (T21) days of alcohol weaning to measure mean erythrocyte volume, gamma-glutamyltransferase (GGT), calcium, gastrin, and CT levels. The control group consisted of 30 male subjects with daily alcohol consumption <20 g. MAIN OUTCOME: The characteristics of the alcohol group were as follows (mean +/- SD): age 41.2 +/- 13 years old; mean erythrocyte volume: 96.0 +/- 4.2 microm(3) (N: 85-95); calcium level: 94.7 +/- 3.7 mg/L (N: 85-105); gastrinemia: 59.3 +/- 14.9 ng/mL (N: <120). At T0 and T21, three alcoholic subjects had CT levels above 10 pg/mL, usually considered as the normal cut-off value. There was no correlation between CT and the different biochemical parameters at T0, T5, and T21. There was no difference between CT levels at the different stages in the alcohol group (T0: 6.4 +/- 3.6 pg/mL; T5: 6.5 +/- 5.3 pg/mL; T21: 8.4 +/- 5.6), although GGT significantly decreased with weaning duration (T0: 248 +/- 354 IU/L; T5: 211 +/- 290 IU/L; T21: 79 +/- 90 IU/L; ANOVA, p <0.05). But a significant difference was found between mean CT levels in the alcohol group and in the control group (3.1 +/- 0.7 pg/mL, p <0.0001). CONCLUSIONS: This study suggests that mean CT levels of chronically alcoholic smoking male subjects are higher than those of an age- and sex-matched control group. However, most alcoholic patients exhibited CT levels <10 pg/mL. No decrease in CT levels was noted over a short period of alcohol weaning. As CT measurement is currently recommended in thyroid nodule assessment, this finding may be important to know how to decipher borderline values of CT.

[Type I neurofibromatosis, pheochromocytoma and somatostatinoma of the ampulla. Literature review]. / Association neurofibromatose de type I, phéochromocytome et somatostatinome de l'ampoule de Vater. Revue de la littérature.

Gastrointestinal involvement in Von Recklinghausen's neurofibromatosis (autosomal dominant disease) is observed in 25% of patients and is frequently represented by duodenal carcinoids. We report a case of somatostatinoma of the ampulla in a 42-year-old female with neurofibromatosis and pheochromocytoma and show a literature review about the association of neurofibromatosis and somatostatinoma. This pathological association appears non hasardous and surgical ampullectomy offers a good alternative to pancreaticoduodenectomy in localised tumors treatment.

Contribution of genetic analysis in screening for MEN1 among patients with sporadic disease and one or more typical manifestation.

Multiple Endocrine Neoplasia type 1 (MEN1) is an autosomal dominant hereditary syndrome (OMIM 131100) due to MEN1 gene mutations, predisposing to the development of hyperplasic and tumoral lesions of neuroendocrine tissues. Since the identification of the gene in 1997, more than 400 different mutations of MEN1 have been registered. Genotypic analysis of MEN1 remains fastidious and must be reserved to targeted situations. If the lesions appear in a familial assessed context, there is a strong argument to search for MEN1 mutation. This is not the case in a sporadic context. With experience acquired in our laboratory, we evaluated the frequency of MEN1 mutations in patients with sporadic presentations. Our aim was to better define criteria for MEN1 genotypic analysis. One hundred and twenty four blood samples from unrelated patients, who gave their written informed consent, were analyzed. These patients exhibited 1 to 4 manifestations of MEN1 without any familial context. After DNA extraction, the analysis was undertaken by PCR-sequencing of all the MEN1 coding exons and exon/intron boundaries or by PCR of the pre-screened fragments alone, a technique made possible by indirect screening mutation methods. Mutations were identified by comparing the sequences to the reference MEN1 sequence available from GENBANK (U93237.1). Mutations were identified in 19 patients, with variable prevalence according to clinical manifestations: 100% for patients with 4 manifestations, 45.5% for patients with 3 manifestations, 19% for patients with 2 manifestations and 2% for patients with only one manifestation. Mutations were: 11 point variations (58%), including 2 splicing sites and 8 frameshift mutations (42%) including 5 deletions, 2 insertions and 1 insertion/deletion; one mutation was identified twice. We showed a relationship between clinical presentation and MEN1 mutation identification, especially with the number of clinical manifestations but also with the type of manifestation. Pancreatic manifestations were significantly linked with probability of mutation. In a sporadic context with at least two established manifestations of MEN1, the overall probability of identifying a mutation was 26%, warranting MEN1 genotypic analysis.

Iodine-123-Tyr-3-octreotide uptake in pancreatic endocrine tumors and in carcinoids in relation to hormonal inhibition by octreotide.

UNLABELLED: Uptake of 123I-Tyr-3-octreotide (TOCT) by hormone-secreting abdominal tumors was studied to compare scintigraphic observations with the reduction in hormone levels brought about by a brief therapeutic test. METHODS: A prospective study was conducted on 17 patients, totalizing 46 proven lesions, with endocrine tumors of the pancreas (10 patients, 20 lesions) and/or carcinoid metastases (8 patients, 26 lesions). Tumor hormonal hypersecretion was inhibited by octreotide. RESULTS: There was good agreement between the results of these examinations. CONCLUSIONS: The detection of abdominal tumors using this radiotracer is strongly related to its functional characteristics. Variations in the scintigraphic and test results according to different tumor types were in agreement with published data on the density of somatostatin receptors measured by in vitro studies or scintigraphy and by the therapeutic effects of octreotide.

Why do frozen sections have limited value in encapsulated or minimally invasive follicular carcinoma of the thyroid?

The diagnosis of encapsulated or minimally invasive follicular carcinoma of the thyroid requires the proof of vascular or capsular invasion. The aim of the present study was to evaluate the relationship between intraoperative diagnosis (benign, suggestive of carcinoma, or malignant) and the final histopathologic criteria for encapsulated or minimally invasive follicular carcinoma (tumor size, capsular invasion, vascular invasion, and differentiation). This was a retrospective study of 63 cases of encapsulated or minimally invasive carcinomas, with the final histopathologic diagnosis taken as the "gold standard." The sensitivity of frozen sections for the diagnosis of malignant neoplasm was 17%. The median number of vascular invasions was 1, identified with a mean number of 9 paraffin-blocks of the tumor. In most cases, intraoperative frozen sections are unable to establish the proof of malignant neoplasm. Intraoperative study of tumor differentiation is useful to select follicular tumors that require a rapid definitive diagnosis and a completion thyroidectomy within 48 to 72 hours (73% of the cases in our study).

Thoracoscopic excision of enlarged mediastinal parathyroid glands.

BACKGROUND: Most abnormal parathyroid glands can be removed through the neck, but those deep in the chest have required sternotomy or thoracotomy. To avoid the morbidity of these open procedures, ectopic parathyroid glands deep in the chest were removed with video-assisted thoracoscopy. METHODS: Two patients with persistent primary and two with persistent secondary hyperparathyroidism had technetium-thallium scintigraphy and computed tomography of the chest to localize their ectopic glands. Video-assisted thoracoscopy was used for operative removal of these glands in each patient. RESULTS: Parathyroid scans identified a mediastinal gland that was confirmed and localized precisely by the computed tomography scan. An enlarged ectopic gland weighing 1 to 2 gm was removed from each patient with normalization of serum calcium level. Glands were found by the main pulmonary artery, aortopulmonary window, ascending aorta, and aortic arch. One patient had recurrent disease 9 months later. CONCLUSIONS: Removal of parathyroid glands deep in the chest with video-assisted thoracoscopy is a safe and successful alternative to median sternotomy.

Scintiscans and carcinoid tumors.

BACKGROUND: The presence of somatostatin receptors on carcinoid tumors mediates imaging of tumor extent and inhibition of tumor growth and marker secretion. This prospective study aimed to evaluate radiolabeled somatostatin analog scans in the therapeutic workup of carcinoids. METHODS: Twenty-one patients with carcinoids underwent 26 scans with iodine octreotide or indium pentetreotide. The results for tumor and metastases imaging were analyzed and compared with those of a short inhibition test of marker secretion and with those of metaiodobenzylguanidine scan. RESULTS: The sensitivity for obtaining images of the overall 43 tumor sites was 72%. We had no false-positive results. Unknown tumor sites were discovered in three patients. The results were slightly better with indium pentetreotide and metastases imaging. A positive scan did not always preclude responsiveness to the functional effect of octreotide. Results of somatostatin analog scans were better than those with metaidobenzylguanidine. The two techniques were complementary in one patient. CONCLUSIONS: The choice of treatment for patients with carcinoid tumors should benefit from functional inhibition test with octreotide and from indium pentetreotide and metaidobenzylguanidine scans.

Exclusive use of calcium channel blockers in preoperative and intraoperative control of pheochromocytomas: hemodynamics and free catecholamine assays in ten consecutive patients.

This study investigates the role of nicardipine hydrochloride in preoperative and intraoperative blood pressure control and intraoperative catecholamines (norepinephrine and epinephrine) release in 10 patients undergoing pheochromocytoma resection. Nicardipine was used orally in the preoperative period for either 1 or 8 days (60 to 120 mg/24 hr) and then was infused during anesthesia until tumor removal, continuously at a rate of 2.5 to 7.5 micrograms/kg/min, depending on systolic arterial pressure level. All patients were successfully operated on. No severe hypertensive crisis occurred during tumor manipulation, although several patients had a 3- to 85-fold (norepinephrine) and 3- to 40-fold (epinephrine) increase of catecholamines from baseline levels. Hemodynamics data suggest that nicardipine caused significant inhibition of vascular smooth muscle contraction (42% decrease in systemic vascular resistance); calcium-dependent catecholamines release was not inhibited by nicardipine infused as mentioned. Use of nicardipine may be recommended for perioperative and intraoperative control of pheochromocytomas and might substitute for routine alpha-adrenergic blockade.

Multiglandular disease in seemingly sporadic primary hyperparathyroidism revisited: where are we in the early 1990s? A plea against unilateral parathyroid exploration.

BACKGROUND: Most of the available data on multiglandular disease (MGD) originate from long-term series. The spectrum of the disease has changed now because of earlier diagnosis. We decided to assess the current frequency of MGD in seemingly sporadic primary hyperparathyroidism. METHODS: MGD was defined as the finding that more than one grossly enlarged gland weighed more than 50 mg and the rim of normal parathyroid tissue was diagnostic of adenoma. Nine hundred eight consecutive neck explorations for hyperparathyroidism were performed, 624 before 1989 and 284 between 1989 and 1991, and studied retrospectively. Since 1989, oil Red O staining has been done to assess hyperfunction. RESULTS: When multiple endocrine neoplasia and non-multiple endocrine neoplasia familial cases of hyperparathyroidism are excluded, the frequency of MGD in seemingly sporadic primary hyperparathyroidism, 17.7% before 1989, is still 14% since then, including a 3% frequency of true multiple adenomas. With more restrictive criteria, which exclude the second enlarged gland if it weighed less than 100 mg, if it had no rim, and if oil Red O staining was negative, the frequency of MGD in sporadic hyperparathyroidism has remained 11% since 1989. Unilateral exploration had missed 78% of second enlarged glands, and preoperative imaging studies, when performed, showed more than one gland in only two of 22 cases. CONCLUSIONS: Routine bilateral neck exploration is recommended in primary hyperparathyroidism because of a current frequency of MGD of at least 11%.

Correlation of parathyroid scanning and anatomy in 261 unselected patients with sporadic primary hyperparathyroidism.

BACKGROUND: Despite abundant literature on parathyroid scanning with technetium 99m-labeled cationic complexes, comprehensive clinical reports that unequivocally correlate scanning findings with the anatomy of parathyroid glands in extensive and homogeneous cohorts of patients are lacking. METHODS: We analyzed the records of patients with sporadic primary hyperparathyroidism who had had a preoperative scan with either 99mTc-labeled sestamibi or 99mTc-labeled tetrofosmin at our institution and who were cured after a bilateral surgical neck exploration procedure. RESULTS: In 261 patients, 710 normal and 347 abnormal glands (1494 +/- 2626 mg), including 15 glands within the mediastinum, were identified. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of scanning were 82%, 98%, 91%, 94%, and 94%, respectively, in 197 patients with uniglandular disease and 53%, 98%, 98%, 60%, and 72%, respectively, in 64 patients with multiglandular disease. False-positive uptakes were encountered in 17 patients (7%), 3 false-positive uptakes being within the mediastinum. If the unilateral approach had been followed, guidance with preoperative scanning would have significantly increased the number of effective unilateral neck exploration procedures (164 patients (63%) vs 78 patients (30%); P < .001). One abnormal gland would also have been neglected in 28 patients (11%). CONCLUSIONS: Preoperative scanning would limit neck exploration procedures in two thirds of patients with sporadic primary hyperparathyroidism but may also increase the risk of failure in the most challenging cases.

Usefulness and limits of quick intraoperative measurements of intact (1-84) parathyroid hormone in the surgical management of hyperparathyroidism: sequential measurements in patients with multiglandular disease.

This study was designed to assess whether reliability of quick intraoperative assay of intact (1-84) immunoreactive parathyroid hormone (iPTH) could allow us to quit after removing one (or several) enlarged parathyroid gland(s) and obtaining a normal iPTH level. Intact iPTH was assayed during surgery before removal of enlarged parathyroid gland(s) and 5, 10, and 20 minutes afterward. Forty-seven patients entered the study: 40 with primary hyperparathyroidism (32 with uniglandular disease and eight with multiglandular disease) and seven with secondary hyperparathyroidism; all underwent bilateral neck exploration. Among 32 patients with uniglandular disease, five had normal basal intraoperative levels, 25 demonstrated a clear-cut drop from supranormal to normal levels, and two had elevated levels. Among the eight patients with multiglandular disease, two had undetectable levels and two had normal levels after removal of the first enlarged gland. The seven patients with secondary hyperparathyroidism demonstrated a decline in PTH levels, suggesting hormone clearance similar to that of patients with primary hyperparathyroidism. In conclusion, quick intraoperative assay with intact (1-84) iPTH (1) is not hampered by renal insufficiency, (2) may overlook a second enlarged gland after removal of a first adenoma and obtaining normal iPTH levels, and (3) should not be used as a substitute for bilateral neck exploration.

Safety of the posterior approach in adrenal surgery: experience in 105 cases.

BACKGROUND: At the advent of laparoscopic adrenalectomy when it was timely to reappreciate the results of time-honored procedures, we reviewed the cases of 105 patients who underwent adrenalectomy through the posterior approach. METHODS: Between 1970 and 1992 among 331 patients, 105 underwent adrenalectomy through the posterior approach (0 of 111 pheochromocytomas, 48 of 64 Conn's disease, 37 of 57 Cushing's disease, 2 of 20 virilizing-feminizing tumors, 13 of 61 nonsecreting adrenalomas, 3 of 12 metastases, 2 of 6 cysts). Adrenalectomy was bilateral in 20 cases. Among 86 tumors, 28 (32.6%) were larger than 5 cm in diameter, none exceeding 10 cm. Posterior approach, initially performed in the prone position, was used in the lateral position for the last 40 patients with tumors. A hockey-stick incision was made on the twelfth or eleventh rib, which was resected. RESULTS: During operation no patient died; one minimal caval tear and 13 pleural tears occurred and were sutured, with two pleural drainages; six patients received blood transfusion. Average operative time was 132 minutes (range, 45 to 290 minutes). After operation one patient died of iatrogenic sepsis, average time to ambulation was 1.5 days, and average in-hospital stay was 7.6 days (range, 1 to 21), which after the fourth day was mostly justified for nonsurgical reasons. From 1990 through 1992, 37 of 38 patients were walking the day after operation and average postoperative stay dropped to 4.5 days (range, 1 to 7 days). CONCLUSIONS: Adrenalectomy through the posterior approach is safe and allows early postoperative discharge.

Intraoperative radionuclear 125I-labeled metaiodobenzylguanidine scanning of pheochromocytomas and metastases.

We developed a technique to assess the feasibility of intraoperative radionuclear detection of pheochromocytomas and their metastases. Thirteen patients were entered into the study: five control subjects with nonchromaffin adrenal tumors, eight with pheochromocytomas, and one of these patients showing bone metastasis. Each subject received thyroid blockade and an intravenous injection of 500 microCi (37 megabecquerels) 125I-labeled metaiodobenzylguanidine (MIBG) 3 days before surgery. In the five control subjects, adrenal tumor uptake never exceeded the liver or spleen uptake. One patient with a negative preoperative MIBG scan demonstrated no intraoperative uptake. Five patients with pheochromocytoma had positive preoperative scans and in one other patient preoperative scanning was not done. In each of these six patients intraoperative count ratio of pheochromocytoma/liver from 14:2 to 250:16 and pheochromocytoma/contralateral adrenal ratio from 60:1.5 to 60:16 was demonstrated. An intraoperative scan in one of these patients detected two small metastatic tumor deposits previously overlooked by the surgeon after removing a larger mass that had been localized by a preoperative 131I-MIBG scan. A negative preoperative scan in one patient was followed by an intraoperative scan demonstrating a bone metastasis with a ratio of metastasis/normal bone of 10:0.5. Specimen studies demonstrated a significant MIBG uptake ratio of tumor/plasma ranging from 95 to 667 (average 404 +/- 242) greater than in control subjects (average 25 +/- 41); in the patient with metastasis the uptake ratio of metastasis/normal bone reached 98.4. We conclude that intraoperative 125I-MIBG scanning might detect pheochromocytoma deposits overlooked by preoperative 131I-MIBG scans.

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.