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To determine the role of reoperation in patients with persistent or recurrent Zollinger-Ellison Syndrome (ZES).Approximately, 0% to 60% of ZES patients are disease-free (DF) after an initial operation, but the tumor may recur.A prospective database was queried.A total of 223 patients had an initial operation for possible cure of ZES and then were subsequently evaluated serially with cross sectional imaging-computed tomography, magnetic resonance imaging, ultrasound, more recently octreoscan-and functional studies for ZES activity. The mean age at first surgery was 49 years and with an 11-year mean follow-up 52 patients (23%) underwent reoperation when ZES recurred with imageable disease. Results in this group are analyzed in the current report. Reoperation occurred on a mean of 6 years after the initial surgery with a mean number of reoperations of 1 (range 1-5). After reoperation 18/52 patients were initially DF (35%); and after a mean follow-up of 8 years, 13/52 remained DF (25%). During follow-up, 9/52 reoperated patients (17%) died, of whom 7 patients died a disease-related death (13%). The overall survival from first surgery was 84% at 20 years and 68% at 30 years. Multiple endocrine neoplasia type 1 status did not affect survival, but DF interval and liver metastases did.These results demonstrate that a significant proportion of patients with ZES will develop resectable persistent or recurrent disease after an initial operation. These patients generally have prolonged survival after reoperation and 25% can be cured with repeat surgery, suggesting all ZES patients postresection should have systematic imaging, and if tumor recurs, advise repeat operation.

To address the value of surgery in patients with sporadic Zollinger-Ellison syndrome (ZES) with negative imaging studies.Medical control of acid hypersecretion in patients with sporadic ZES is highly effective. This has led to these patients frequently not being sent to surgery, especially if preoperative imaging studies are negative, due, in large part, to existence of almost no data on the success of surgery in this group.Fifty-eight prospectively studied patients with sporadic ZES (17% of total studied) had negative imaging studies, and their surgical outcome was compared with 117 patients with positive imaging results.Thirty-five patients had negative imaging studies in the pre-somatostatin receptor scintigraphy (SRS) era, and 23 patients in the post-SRS era. Patients with negative imaging studies had long disease histories before surgery [mean ± SEM (from onset) = 7.9 ± 1 [range, -0.25 to 35 years]) and 25% were followed for 2 or more years from diagnosis. At surgery, gastrinoma was found in 57 of 58 patients (98%). Tumors were small (mean = 0.8 cm, 60%1 cm). The most common primary sites were duodenal 64%, pancreatic 17%, and lymph node (10%). Fifty percent had a primary-only, 41% primary + lymph node, and 7% had liver metastases. Thirty-five of 58 patients (60%) were cured immediately postoperatively, and at last follow-up [mean = -9.4 years; range, 0.2-22 years], 27 patients (46%) remained cured. During follow-up, 3 patients died, each had liver metastases at surgery. In comparison to positive imaging patients, those with negative imaging studies had lower preoperative fasting gastrin levels; had a longer delay before surgery; more frequently had a small duodenal tumor; less frequently had a pancreatic tumor, multiple tumors, or developed a new lesion postoperatively; and had a longer survival.Sporadic ZES patients with negative imaging studies are not rare even in the post-SRS period. An experienced surgeon can find gastrinoma in almost every patient (98%) and nearly one half (46%) are cured, a rate similar to patients with positive imaging findings. Because liver metastases were found in 7%, which may have been caused by a long delay in surgery and all the disease-related deaths occurred in this group, surgery should be routinely undertaken early in ZES patients despite negative imaging studies.

To determine whether routine use of duodenotomy (DUODX) alters cure rate, survival, or development of liver metastases in 143 patients (162 operations) with Zollinger-Ellison syndrome (ZES) without MEN1.DUODX has been shown to increase the detection of duodenal gastrinomas, but it is unknown if it alters rate of cure, liver metastases, or survival. Data from our prospective studies of surgery in ZES allow us to address this issue because DUODX was not performed before 1987, whereas it was routinely done after 1987.All patients with sporadic ZES (non-MEN1) undergoing surgery for possible cure without a prior DUODX from November 1980 to June 2003 were included. Patients had preoperative computed tomography (CT), magnetic resonance imaging (MRI), or ultrasound; if unclear, angiography and somatostatin receptor scintigraphy since 1994. At surgery, all had the same standard ZES operation and were assessed immediately postoperatively, at 3 to 6 months, and yearly for cure (fasting gastrin, secretin test. and imaging studies).A DUODX was performed in 79 patients (94 operations), and no DUODX was performed in 64 patients (68 operations), with 10 patients having both (no DUODX, then a DUODX later). Gastrinoma was found in 98% with DUODX compared with 76% with no DUODX (P0.00001). Duodenal gastrinomas were found more frequently with DUODX (62% vs. 18%; P0.00001), whereas pancreatic, lymph node, and other primary gastrinomas occurred similarly. Six of the 10 patients with 2 operations had a duodenal tumor found with DUODX during a second operation that was missed in the first operation without DUODX. Both the immediate postoperative cure rate (65% vs. 44%; P = 0.010) and long-term cure rate at last follow-up (8.8 +/- 0.4 years; range, 0.1 to 21.5) (52% vs. 26%; P = 0.0012) were significantly greater with a DUODX than without. In patients without pancreatic tumors or liver metastases at surgery, both the rate of developing liver metastases (6% vs. 9.5%) and the disease-related death rate (0% vs. 2%) were low and not significantly different in patients with or without a DUODX.These results demonstrate that routine use of DUODX increases the short-term and long-term cure rate due to the detection of more duodenal gastrinomas. The rate of development of hepatic metastases and/or disease-related mortality in patients without pancreatic tumors is low, and no effect of DUODX on these parameters was seen. Duodenotomy (DUODX) should be routinely performed during all operations for cure of sporadic ZES.

To analyze the results of a prospective study of 176 patients with Zollinger-Ellison syndrome (ZES) (138 sporadic, 38 MEN1) undergoing 207 operations over a 17-year period.The existence of lymph node (LN) primary gastrinoma causing ZES is controversial.Three groups of patients were compared: LN only resected, cured, and no relapse (likely LN primary); same criteria but relapse (unlikely LN primary); and duodenal primary and LN metastases (Duo-LN).Forty-five (26%) had only LN(s) as the initial tumor found. Twenty-six of the 45 (58%) fit the definition of a likely LN primary because they were apparently cured postresection. At 10.4 +/- 1.2 years, 69% of the 26 patients with likely LN primary tumors have remained cured and have LN primaries. In the 8 of 26 with recurrent ZES, it occurred at 5 +/- 1 years, and 3 had duodenal gastrinoma that had been missed. Ten percent (13/138) of all patients with sporadic ZES and 0% (0/38) with ZES and MEN1 remained cured with only a LN tumor removed. In patients with sporadic gastrinomas no clinical, laboratory, or radiographic localization feature differed among patients with likely LN primary (n = 16) and those with unlikely LN primary (n = 6) or those with Duo-LN (n = 37). In the likely LN primary group, the largest LN was 2.2 +/- 0.2 cm, the number of LNs removed was 1.3 +/- 0.1 (25%or =1 LN), and 78% were in the gastrinoma triangle, which also did not differ from the other 2 groups. Disease-free survival was similar in the likely LN primary group, patients with Duo-LN, and those with pancreatic primaries.These results support the conclusion that primary LN gastrinomas occur and are not rare (approximately 10% of sporadic cases). These results suggest that a proportion (25%) of these tumors are either multiple or malignant. Because no clinical, laboratory, or tumoral characteristic distinguishes patients with LN primary tumors, all patients with ZES undergoing surgery should have an extensive exploration to exclude duodenal or pancreatic tumors and routine removal of lymph nodes in the gastrinoma triangle.

BACKGROUND: Fasting hypoglycemia with neuroglycopenic symptoms corrected by administration of glucose are the hallmarks for the diagnosis of insulinoma. Surgical resection is the treatment of choice for insulinomas, but localization of these lesions can be challenging. Blind distal pancreatectomy has been advocated for occult insulinomas not detected on imaging studies or during abdominal exploration. With the advent of newer localization techniques, we challenge the wisdom of this approach. STUDY DESIGN: The records of patients (multiple endocrine neoplasia excluded) with pathologically proved insulinoma who were screened at our institution or referred to us after a failed blind distal pancreatectomy were reviewed. All records included patient history and results of physical examination and routine blood and urine tests. The diagnosis of insulinoma was confirmed during a supervised fast. Patients with biochemically proved insulinoma underwent localization studies and operation. Studies included CT scans, MRI, transabdominal ultrasound, intraoperative ultrasonography, angiography (more recently, Ca ++ -stimulated arteriography), and venous sampling. RESULTS: From 1970 to 2000, 99 patients (34 men, 65 women; mean age 43 years) underwent operation. All patients with benign tumors (92) were cured after operation. Seventeen patients were referred to the NIH after a failed blind distal pancreatectomy. Of these, 5 were diagnosed as having factitious hypoglycemia. In the other 12 patients a tumor was localized in the pancreatic head. Two patients incorrectly diagnosed with nesidioblastosis after initial surgery were subsequently cured by resection of an insulinoma. CONCLUSIONS: The use of preoperative imaging studies, most notably Ca ++ -stimulated arteriography, and intraoperative ultrasonography permits detection of virtually all insulinomas, including reoperated cases. When a tumor is not detected, the procedure should be terminated and the patient referred to a center capable of performing advanced preoperative and intraoperative localization techniques. With the preoperative and intraoperative imaging strategies currently available, the use of blind distal pancreatectomy for occult insulinoma should be abolished.

Peritoneal carcinomatosis occurs as an advanced stage of certain malignancies and is often considered incurable. 1 No good standard treatment options exist, so new and innovative treatments must be sought and investigated to combat this stage of disease. These treatments may be used as an adjuvant after surgical resection or to palliate symptoms such as ascites in patients with bulky disease. Regional intraperitoneal chemotherapy provides prolonged high concentration of a cytotoxic agent directly to intraperitoneal tumor cells while limiting systemic exposure of the patient to the drug. It has been shown to improve results compared with systemic chemotherapy alone in patients with ovarian cancer. 2 Continuous hyperthermic peritoneal perfusion (CHPP) is a method by which heated cytotoxic chemotherapy is recirculated at high flow rates through the peritoneal cavity. 3 This technique is designed to improve distribution of the chemotherapy in the peritoneal cavity and take advantage of known synergy between chemotherapy and hyperthermia. Although this has been previously performed after open laparotomy and complete lysis of adhesions, we describe here a minimally invasive approach that may be more appropriate for many clinical settings.

Background: For a variety of histologies, the liver represents the only or the dominant site of metastatic disease. Regional treatment of the liver has the theoretic advantage of maximizing drug delivery while minimizing systemic toxicity. This article describes the technique of isolated hepatic perfusion using tumor necrosis factor and melphalan under conditions of moderate hyperthermia for the treatment of unresectable liver tumors. Study Design: Fifty patients with biopsy-proved unresectable primary or metastatic cancer to the liver were treated. Isolated hepatic perfusion was performed for 60 minutes under conditions of moderate hyperthermia during a laparotomy with inflow through the gastroduodenal artery and outflow through an isolated segment of inferior vena cava. During isolated hepatic perfusion portal and infrahepatic blood flow were shunted externally by a centrifugal pump to the axillary vein. Complete vascular isolation was confirmed intraoperatively using a continuous 131 I-labeled serum albumin leak monitoring system. Operative and perfusion parameters were recorded. Results: By using a standard operative technique to achieve complete vascular isolation of the liver during perfusion, there was no leak of perfusate detected in 48 of 50 patients as determined by the continuous leak monitoring system and absence of detectable systemic tumor necrosis factor levels. Operating time, transfusion requirements, and blood loss were within the range expected for a major operative procedure. Stable hemodynamic and perfusion parameters were achieved consistently and all patients successfully completed the 60-minute perfusion. Two patients (4%) died as a result of treatment and significant tumor regression was observed in 75%. Conclusions: Isolated hepatic perfusion is a technique that can be used to deliver high doses of chemotherapy or biologic therapy regionally and without systemic exposure. By using a standard operative technique, continuous intraoperative leak monitoring, and an external veno-veno bypass circuit, this procedure can be done safely and with acceptable morbidity and mortality. This article demonstrates that sustained and complete vascular isolation of the liver can be achieved and indicates further study is warranted to better define the role of isolated hepatic perfusion in the treatment of unresectable liver tumors.

PURPOSE Isolated hepatic perfusion (IHP) is a new treatment for patients with isolated unresectable liver metastases, which can result in a partial or complete response in approximately 75% of patients. Preoperative knowledge of hepatic arterial anatomy is important to adequately perfuse the liver. Digital subtraction angiography (DSA) is currently used to identify the hepatic arterial anatomy. The purpose of this study was to determine if MR angiography (MRA) could replace DSA prior to IHP. METHOD Twenty-seven patients scheduled to undergo IHP underwent MRA with a contrast-enhanced 3D time-of-flight gradient echo sequence. Both maximal intensity projections (MIPs) and source coronal images were used to evaluate the images. The results of the MRA were interpreted by two readers who were blinded to the surgical results. The first 17 patients also underwent DSA, and a separate comparison was made with those results. Anatomy was characterized as either normal hepatic arteries (NHAs), normal vasculature with an accessory left hepatic artery (aLHA), or a replaced right hepatic artery (rRHA). RESULTS MRA correctly detected all 22 patients with NHAs but also identified 6 aLHAs, of which only 2 were confirmed surgically. MRA correctly detected all five rRHAs. MIP images alone accurately depicted the hepatic arterial anatomy in only 9 of 27 (33%), usually because significant vessels were not visualized or their origin could not be determined. Source coronal images were required to accurately determine the anatomy in all patients. Among the 17 patients who underwent DSA, MRA detected 14 of 14 with NHA and 3 of 3 with rRHA. Six aLHAs were identified by MRA and five were confirmed by DSA. CONCLUSION Enhanced 3D MRA is an accurate method of depicting the hepatic arterial supply. In comparison to surgery, MRA overestimates the number of aLHAs, but this may be because these small vessels are not detected at surgery. Based on the results of this study, DSA has been replaced by MRA in the planning of IHP at our institution. A better display of MRA images is needed as MIP images were usually insensitive for the small caliber arteries supplying the liver.

Background: The success rate for bilateral exploration in patients with primary hyperparathyroidism approaches 95%. Multiglandular parathyroid hyperplasia accounts for approximately 10% to 30% of primary hyperparathyroidism. The incidence of recurrent or persistent hyperparathyroidism is highest in familial forms of the disease, in which multiglandular disease is more common; this may be due to asymmetric enlargement of parathyroid glands. Because of improvements in tumor-imaging capability, some surgeons are now advocating unilateral exploration for primary hyperparathyroidism, but there is limited experience concerning how often these imaging methods fail. Study Design: The outcomes of 7 patients who had sporadic primary hyperparathyroidism with multigland hyperplasia were reviewed. We gathered demographic data and laboratory values and reviewed radiologic tests, surgical findings, pathologic findings, and postoperative followup. Results: All patients underwent preoperative localization with ultrasonography and technetium/sestamibi scans. The sensitivity of these two tests for the dominantly enlarged gland was 100% for both, but dropped to 0% and 5%, respectively, for all other enlarged glands. The sensitivity of CT and MRI for the dominant tumor was 67% (2 of 3) and 50% (1 of 2), respectively. Six of 7 patients underwent subtotal (312 gland) parathyroidectomy. The mean volume of all glands was 1.51 ± 5.89 cm 3 compared with a mean of 5.66 ± 11.4 cm 3 for all dominant glands and 0.123 ± 0.1 cm 3 for all nondominant hyperplastic glands. There was a large amount of variability between the volumes of dominant and other glands as demonstrated by large SDs from the mean. Conclusions: There is a marked heterogeneity in gland size in patients with sporadic multigland hyperplasia, which is similar to that found in multiple endocrine neoplasia type I. This heterogeneity may result in failure to recognize multigland disease if a unilateral neck exploration is performed. Intraoperative parathyroid hormone assay may prove to be an important adjunct in this population of patients who have unsuspected multigland disease.

Objectives The authors evaluate the results of preoperative imaging protocols and surgical re-exploration in a series of patients with missed parathyroid adenomas after failed initial procedures for primary hyperparathyroidism. Background The success rate is lower and the complication rate is increased in patients undergoing reoperation for primary hyperparathyroidism compared with initial procedures. Scarring and distortion of tissue planes plus the potential for ectopic gland location leads to this worsened outcome. Methods Two hundred eighty-eight consecutive patients with persistent/recurrent hyperparathyroidism were treated at a single institution after a failed procedure or procedures at outside institutions. Two hundred twenty-two of these patients (77%) were believed to have a missed single adenoma, and these patients underwent 228 operations and 227 preoperative work-ups. Preoperative evaluation consisted of a combination of four noninvasive imaging studies-neck ultrasound, nuclear medicine scan, neck and mediastinal computed tomography scan, and neck and mediastinal magnetic resonance imaging. Based on the noninvasive testing alone, 27% patients underwent surgery whereas the other patients underwent invasive studies, including selective angiography (58%), selective venous sampling for parathyroid hormone (43%), or percutaneous aspiration of suspicious lesions (15%). Results Abnormal parathyroid adenomas were found in 209 of 222 initial procedures and 6 of 6 second procedures, with an overall success rate in terms of resolution of hypercalcemia in 97% (215/222) of patients. The single most common site of missed adenoma glands was in the tracheal-esophageal groove in the posterior superior mediastinum (27%). The most common ectopic sites for parathyroid adenomas are thymus (17%), intrathyroidal (10%), undescended glands (8.6%), carotid sheath (3.6%), and the retroesophageal space (3.2%). The most sensitive and specific noninvasive imaging test is the sestamibi subtraction scan, with 67% true-positive and no false-positive results. The rate of true-positive and false-positive results for ultrasound, computed tomography, magnetic resonance imaging, and technetium thallium scans were 48%/21%, 52%/16%, 48%/14%, and 42%/8%, respectively. The incidence of injury to the recurrent laryngeal nerve was 1.3%. Conclusions A single missed parathyroid adenoma is the most common cause for a failed initial parathyroid operation. Appropriate use of preoperative imaging tests and knowledge of the potential location of parathyroid adenomas can lead to very high cure rates with minimal morbidity.