Thompson, Geoffrey B.

Abstract Background Management of patients with bilateral adrenal masses and corticotropin (ACTH)‐independent Cushing syndrome (CS) or subclinical CS is problematic. We report our experience with adrenal venous sampling (AVS) in the evaluation of 10 patients with bilateral masses who had ACTH‐independent CS or subclinical CS. Patients and Methods Ten patients (9 women, 1 man, mean age 56.4 years) with bilateral adrenal masses and ACTH‐independent CS ( n = 3) or subclinical CS ( n = 7) underwent AVS. Autonomous cortisol secretion was documented in all cases with suppressed serum ACTH concentrations and lack of cortisol suppression with dexamethasone administration. Adrenal venous sampling was performed on the second day of dexamethasone administration. Cortisol and epinephrine levels were measured from each adrenal vein (AV) and from a peripheral vein (PV). Results Mean (± SD) maximal diameter of the adrenal masses on computed tomography was 3.3 ± 1.3 cm (range: 1.2–6.0 cm). Successful catheterization was confirmed with AV:PV epinephrine gradients. A cortisol AV:PV gradient >6.5 was consistent with a cortisol‐secreting adenoma in 11 adrenal glands; 5 patients had clinically important bilateral autonomous cortisol hypersecretion, 3 had bilateral cortisol‐secreting adenomas, and 2 had ACTH‐independent macronodular adrenal hyperplasia. Adrenal venous sampling‐guided adrenalectomy was completed in all 10 patients—2 patients had total bilateral adrenalectomy and 2 others had subtotal bilateral adrenalectomy. During a mean follow‐up of 36.1 months (range: 0.7–123 months), CS or clinically important cortisol secretory autonomy did not recur. Conclusions Adrenal venous sampling contributed to the localization of autonomous hypercortisolism in the setting of ACTH‐independent CS or subclinical CS in patients with bilateral adrenal masses.

AbstractBackgroundRepair of complex incisional hernias poses a major challenge.AimThe aim of this study was to review the outcomes of the modified Rives‐Stoppa repair of complex incisional hernias using a synthetic prosthesis.MethodsWe reviewed patients undergoing a modified Rives‐Stoppa repair of complex incisional hernias from 1990 to 2003. Patients were followed through clinic visits and mailed questionnaires. Follow‐up data were complete in all patients (mean 70 months, range 24–177 months), and 87% of patients completed a mailed questionnaire. Primary outcome included mortality, morbidity, and hernia recurrence. Secondary outcome measures were duration of hospital stay, long‐term abdominal wall pain, and self‐reported patient satisfaction.ResultsAltogether, 254 patients underwent a modified Rives‐Stoppa repair. Among them, 60% had a significant co‐morbidity, and 30% had one or more previously failed hernia repairs. Mortality was zero, and overall morbidity was 13% (wound infection 4%, prosthetic infection 3%, seroma/hematoma 4%). The overall hernia recurrence rate was 5%, including explantation of mesh because of infection. Wound/prosthetic infection was predictive for hernia recurrence (31% vs. 4%, p = 0.003). Among the respondents, 89% reported overall satisfaction with their repair.ConclusionThe Rives‐Stoppa repair of complex incisional hernias using synthetic prosthetic materials is safe with a low recurrence rate (5%) and high patient satisfaction. Postoperative wound infection is a risk factor for hernia recurrence.

Abstract A policy of supportive treatment is frequently adopted for patients with metastatic disease to the adrenal glands. This study reports an experience with adrenalectomy for adrenal metastasis. Between 1983 and 1993, adrenalectomy was performed in 52 patients for metastasis to the adrenal glands. Survival was calculated by the Kaplan- Meier method and compared with the log rank test. Primary tumour sites included kidney (n = 15), lung (n = 11), colon (n = 7), unknown (n = 5), stomach (n = 3), melanoma (n = 3) and other (n = 8). Adenocarcinoma (69 per cent) was the most common histological cell type. Thirty-two patients were asymptomatic on initial evaluation. Symptomatic adrenal pain relief was achieved in 11 of 13 patients. Overall survival rates were 73 per cent at 1 year and 40 per cent at 2 years. Patients with potentially curative resection had better survival than those who had a palliative procedure. Patients with adrenal metastases due to adenocarcinoma had improved survival compared with that in those with other histological cell types. Although long-term survival is generally poor, highly selected patients with adrenal metastasis (symptomatic disease or adenocarcinoma) may benefit from surgical resection.

OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children.METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients <18 years of age with histologically confirmed pheochromocytoma or paraganglioma.RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of >6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 ± 32 months.CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of >6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.

AbstractBackgroundSolid pseudopapillary neoplasms of the pancreas are rare malignant lesions of the pancreas that typically occur in young women. Large series from any one centre are notably absent in the literature. The aim of this study was to determine long-term outcomes of operative therapy.MethodsThe records of all 14 patients diagnosed with pseudopapillary neoplasms of the pancreas over 17 years were reviewed.ResultsThirteen of the 14 patients were female and the mean age at diagnosis was 30 years. Solid pseudopapillary neoplasm was suspected in only half of these patients before operation. On computed tomography, ultrasonography and/or magnetic resonance imaging, three lesions were solid, three were largely cystic, and five had solid and cystic components. All 14 patients underwent surgical exploration and curative resections were possible in 13, including distal pancreatectomy in nine, pancreaticoduodenectomy in three and resection of a local intraperitoneal recurrence in one patient. After follow-up ranging from 3 months to 20 years, 12 patients were alive, including one who had undergone re-exploration and resection of local and subcutaneous recurrences 9 years previously.ConclusionSolid pseudopapillary neoplasm of the pancreas should be considered in the differential diagnosis of any solid or partly cystic pancreatic mass in women aged less than 35 years. An attempt at en bloc resection without formal lymphadenectomy should be undertaken, including resection of synchronous or metachronous distant metastases.

Surgically correctable causes of hypertension are uncommon. Simultaneous occurrence of 2 such causes in the same individual is extremely rare. The authors describe a 25-year-old woman with congenital erythrocytosis, renal artery stenosis, and a paraganglioma. The possible mechanisms of renal artery stenosis in the presence of a catecholamine-secreting tumor are discussed.

AbstractChildhood papillary thyroid carcinoma is associated with more locally aggressive and more frequent distant disease than its adult counterpart. Recurrence rates tend to be higher in children, but cause‐specific mortality remains low. Optimal initial treatment of childhood papillary thyroid carcinoma should include total or near‐total thyroidectomy and central compartment node clearance. Modified neck dissections should be performed for biopsy‐proven lateral neck disease. Every effort should be made to maintain parathyroid and laryngeal nerve function. Radical neck dissections are to be avoided. Radioiodine remnant ablation (RRA), appropriate thyroid hormone suppressive therapy (THST), and judicious use of therapeutic doses of 131I are applied to achieve a disease‐free status, which is most often confirmed by negative neck ultrasonography, negative whole‐body scan (either withdrawal or recombinant human thyroid‐stimulating hormone‐stimulated), and extremely low levels of serum thyroglobulin. Appropriate utilization of 131I, THST, repeat surgery, external beam radiotherapy, and rarely chemotherapy may provide long‐term palliation and some cures in patients with recurrent/persistent disease. Follow‐up should be lifelong, and the care of children after age 17 should subsequently be transferred to adult‐care endocrinologists with expertise in managing thyroid neoplasia. Optimal surgical management can be achieved if adequate operations are routinely carried out by “high‐volume” thyroid surgeons with expertise in the care of children. Nowhere is a multidisciplinary approach (endocrinologists, surgeons, nuclear medicine physicians, pediatricians, pathologists, oncologists) more critical than in the long‐term management of papillary thyroid carcinoma that presents during childhood.

AbstractThe etiology, prognosis, and optimal management of primary gastric carcinoids remain controversial. Records of 36 consecutive patients with gastric carcinoid (15 men) were reviewed retrospectively between 1975 and 1990. Follow‐up was complete in 97% of cases. Mean age at diagnosis was 58.4 years (range 24–82 years). The clinical presentations included anemia (72%), pain (69%), and carcinoid syndrome (11%). Associated autoimmune and endocrine abnormalities were common and included atrophic gastritis (67%), pernicious anemia (58%), hypothyroidism (39%), diabetes (19%), Addison's disease (6%), and hyperparathyroidism (6%). Lesions were nonantral in 78%, involving only the corpus in 42%, the fundus in 28%, and only the antrum in 8%; 42% were multiple. Urinary 5‐hydroxyindoleacetic acid (5‐HIAA) and serum gastrin levels were elevated in 17% and 50% of those tested, respectively. Histologic examination revealed that 28% of lesions were ≥2 cm, and 33% had liver metastases on presentation or developed them during follow‐up. Eight patients (22%) died of tumor with a median survival of 39 months. The presence of metastases, atypical histology, serosal involvement, and size > 2 cm were adverse prognostic factors. In patients without hypergastrinemia (n=6), 66% developed metastases, 60% had elevated 5‐HIAA, and 50% died of carcinoid tumor. In sharp contrast, those patients with hypergastrinemia and “typical” gastric carcinoids (n=15), metastases and death did not occur (p<0.003 and p < 0.005, respectively, compared with eugastrinemic patients). In the latter group (hypergastrinemia and typical histology), pernicious anemia was common (73%); solitary lesions and serosal penetration were seen comparatively less often (33% versus 100%, p<0.005 and 0% versus 33%, p<0.05, respectively); and no lesion measured > 2.0 cm compared to 66% in the group without elevated gastrin (p<0.002). No patient with hypergastrinaemia had elevated levels of 5‐HIAA. Overall survival at 5 and 10 years was 58% and 28%, respectively. Survival was 80% at 5 and 10 years in the hypergastrinemic group. Gastric carcinoid tumors that are histologically atypical, large (>2 cm), or present without elevated gastrin are potentially lethal and require aggressive therapy. In contrast, gastric carcinoids with typical histology and hypergastrinemia are rarely lethal, and conservative management with local excision and close observation is appropriate.

AbstractAmiodarone‐associated thyrotoxicosis (AAT) is often poorly tolerated owing to underlying cardiac disease, and it is frequently refractory to conventional medical treatment. The goal of this study was to describe the patient characteristics, management, and outcomes of all the patients treated surgically for AAT at a single institution. We conducted a retrospective chart review of all patients managed surgically for AAT (April 1985 through November 2002) at the Mayo Clinic in Rochester, Minnesota. Altogether, 29 men and 5 women, ages 39 to 85 years (median 60 years), treated with amiodarone for 3 to 108 months underwent near‐total or total thyroidectomy. Frequent symptoms were worsening heart failure, fatigue, weight loss, and tremor. Altogether, 12 patients failed medical management of their AAT, and 21 received no preoperative medical therapy. One patient had been successfully managed medically but required definitive treatment. Common indications for operation were the need to remain on amiodarone, cardiac decompensation, medically refractory disease, and severe symptoms, both hyperthyroid and cardiac, necessitating prompt resolution. The median ± SD American Society of Anesthesiologists (ASA) classification (1 = healthy through 5 = moribund) was 3.00 ± 0.58. A total of 27 specimens had histology consistent with AAT. Complications included death (n = 3), rehospitalization (n = 3), symptomatic hypocalcemia (n = 2), pneumonia (n = 2), cervical hematoma (n = 1), prolonged ventilatory wean (n = 1), and stroke (n = 1); one patient developed hypotension, adult respiratory distress syndrome, and sepsis. Of the 31 surviving patients, 25 (80%) remained on amiodarone postoperatively. The median follow‐up was 29 months, at which time all surviving patients were free of hyperthyroid symptoms. Thyroidectomy is an effective treatment for AAT but has a high incidence of perioperative morbidity and mortality. The cardiovascular co‐morbidities and high operative risk in this group of patients may account for the increased complication rate.