Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline

"OBJECTIVE: The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL). PARTICIPANTS: The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), seven experts in the field, and a methodologist. The authors received no corporate funding or remuneration. EVIDENCE: This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews. CONSENSUS PROCESS: One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society, European Society of Endocrinology, and Americal Association for Clinical Chemistry reviewed drafts of the guidelines. CONCLUSIONS: The Task Force recommends that initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. Consideration should be given to preanalytical factors leading to false-positive or false-negative results. All positive results require follow-up. Computed tomography is suggested for initial imaging, but magnetic resonance is a better option in patients with metastatic disease or when radiation exposure must be limited. (123)I-metaiodobenzylguanidine scintigraphy is a useful imaging modality for metastatic PPGLs. We recommend consideration of genetic testing in all patients, with testing by accredited laboratories. Patients with paraganglioma should be tested for SDHx mutations, and those with metastatic disease for SDHB mutations. All patients with functional PPGLs should undergo preoperative blockade to prevent perioperative complications. Preparation should include a high-sodium diet and fluid intake to prevent postoperative hypotension. We recommend minimally invasive adrenalectomy for most pheochromocytomas with open resection for most paragangliomas. Partial adrenalectomy is an option for selected patients. Lifelong follow-up is suggested to detect recurrent or metastatic disease. We suggest personalized management with evaluation and treatment by multidisciplinary teams with appropriate expertise to ensure favorable outcomes."

Laparoscopic bilateral adrenalectomy: results for 30 consecutive cases.

BACKGROUND: Most patients requiring bilateral adrenalectomy have adrenocorticotropin hormone (ACTH)-dependent Cushing's syndrome. Some of these patients are severely debilitated from the chronic effects of cortisol overproduction. This study aimed to analyze the indications, safety, efficacy, and outcomes for laparoscopic bilateral adrenalectomy from the authors' experience. METHODS: A retrospective review was conducted at a university tertiary referral center. Between March 1996 and August 2006, 30 consecutive patients underwent simultaneous laparoscopic bilateral adrenalectomy. The patient records were analyzed to obtain patient demographics, disease etiology, surgical approach, operating room information, postoperative complications (30 days), hospital length of stay (LOS), and follow-up information. RESULTS: The 30 participants (22 women and 8 men) had a mean age of 44 years. The indications for bilateral adrenalectomy were refractory Cushing's disease (n = 16), occult ectopic ACTH syndrome (n = 9), and bilateral pheochromocytoma (n = 5). A mean of 53 months elapsed between onset of symptoms and adrenalectomy. Laparoscopic bilateral adrenalectomy was completed for all the patients with no intraoperative complications. Four patients (13%) experienced six complications. The mean postoperative LOS was 3.5 days (range, 1-12 days). Seven patients required a preoperative LOS, for a mean of 7.1 days (range, 1-20 days), and a postoperative LOS, for a mean of 5 days (range, 2-12 days). The 23 patients who did not require preoperative hospitalization had a mean postoperative LOS of 3 days (range, 1-7 days). All the patients received postoperative steroid replacement and appropriate follow-up assessment with an endocrinologist. At this writing, the patients with Cushing's syndrome available for follow-up evaluation continue to receive steroid replacement, and all the pheochromocytoma patients have experienced a documented postoperative biochemical cure. CONCLUSIONS: Laparoscopic bilateral adrenalectomy is safe and effective for this high-risk patient population. Although patients should be monitored closely in the postoperative period, most are discharged with glucocorticoid and mineralocorticoid replacement in a short time without complications.

Good blood pressure control on antihypertensives, not only response to spironolactone, predicts improved outcome after adrenalectomy for aldosteronoma.

BACKGROUND: Spironolactone is frequently used before adrenalectomy for hyperaldosteronism to control blood pressure. Response to spironolactone has been suggested to predict a better outcome. However, whether using other antihypertensive medications to control blood pressure predicts outcome remains unknown. We sought to determine the relationship between preoperative normalization of blood pressure with antihypertensive medications and response to adrenalectomy for hyperaldosteronism. METHOD: A retrospective cohort study of 102 patients who underwent adrenalectomy for hyperaldosteronism at a tertiary medical center were included. Blood pressures were measured at first clinical presentation, preoperatively, postoperatively, and at 1 and 6 months postoperatively. The primary outcome measure was complete resolution of hypertension (blood pressure <140/90) without antihypertensive medications, versus incomplete resolution requiring antihypertensive medications. RESULTS: Hypertension resolved and medications were discontinued in 39% of patients. Hypertension resolved without medications in 53% of patients who were normotensive preoperatively versus 24% of patients who were hypertensive (P = .006). In contrast, hypertension resolved without medications in only 45% of patients who were normotensive on spironolactone preoperatively versus 34% of patients who were hypertensive (P = .38). CONCLUSIONS: Patients with good preoperative control of hypertension on antihypertensive medications, irrespective of response to spironolactone, are more likely to have complete resolution.

Accuracy of fine-needle aspiration cytology in patients with radiation-induced thyroid neoplasms.

BACKGROUND: Fine-needle aspiration cytology (FNAC) is useful for selecting patients with thyroid nodules for thyroidectomy. Its value in patients who have been exposed to low-dose therapeutic radiation is questionable because these patients have an increased risk of multifocal benign and malignant tumours, and thyroid cancer is common in such patients. METHODS: Between 1960 and 1999, 171 patients with one or more thyroid nodules who had a history of exposure to radiation underwent operation; 49 of these patients had preoperative FNAC. The cytology results in these 49 patients were compared with those of an age- and sex-matched control group of patients with thyroid nodules who did not have a history of radiation exposure. RESULTS: Of those who had been exposed to radiation, six of 20 patients with 'benign' cytology by FNAC and six of 16 patients with 'suspicious' cytology had thyroid cancer. All 13 specimens considered to be malignant on FNAC were indeed malignant. There was a higher rate of false-negative cytological examinations among patients with a history of irradiation that in those without. CONCLUSION: FNAC of thyroid nodules in patients with a history of irradiation is not as accurate as that in non-irradiated patients, primarily because of coexisting occult thyroid cancers.

A critical analysis of intraoperative time utilization in laparoscopic adrenalectomy.

BACKGROUND: Time and efficiency analysis is a technique common in industry that is being applied to surgical procedures. The aim of this study is to analyze the time spent performing the component parts of laparoscopic adrenalectomy by both the lateral transabdominal and the posterior retroperitoneal approaches. METHODS: Operational videotapes of 33 patients undergoing laparoscopic adrenalectomy (12 lateral, 21 posterior) were reviewed. The operation was divided into six steps: trocar entry, laparoscopic ultrasonography, exposure of the adrenal gland, dissection of the adrenal, extraction of specimen, and irrigation-aspiration. Time spent for each step and the relation with age, gender, body mass index (BMI), tumor size, side, and histology were assessed using Student's t-test, Pearson correlation, and regression analysis. RESULTS: Although tumor size was larger in the lateral compared to the posterior approach (5.5 vs 2.5 cm, p < 0.001), there was no difference between the groups regarding total operating time (116.1 vs 112.8 min). Most of the operating time was spent on dissection of the adrenal gland with both techniques (lateral, 60%; posterior, 66%). Exposure of the adrenal gland was longer in the lateral compared to the posterior approach (15.1 vs 5.8 min, respectively; p < 0.05). In the transabdominal technique, this step was longer on the right side than on the left (18.9 vs 11.4 min, respectively; p < 0.05). In the lateral approach, dissection time was dependent on tumor size (r = 0.90, p < 0.05) but not on BMI, whereas in the posterior approach both tumor size and BMI were positively correlated (r = 0.56 and r = 0.64, respectively). CONCLUSIONS: To our knowledge, this is the first study to apply time analysis techniques to laparoscopic adrenal surgery. Understanding the variables that affect operative time may influence the choice of the surgical approach in a given patient. This study also suggests that efforts to improve operative efficiency are best directed at the dissection of the adrenal.