Zollinger-Ellison syndrome: technique, results, and complications of portal venous sampling.

Miller, Donald L.; Doppman, John L.; Metz, DC; Maton, Paul N.; Norton, J A; Jensen, R. T.

doi:10.1148/radiology.182.1.1727289

Cited by 40 publications

(8 citation statements)

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“…To assess for the presence of a functional PET in addition to fasting gastrin levels, plasma insulin, proinsulin, glucose, ACTH, glucagon, pancreatic polypeptide, serotonin, calcitonin and urinary 5-hydroxyindolacetic acid, N -methyl histamine excretion and cortisol excretion were determined 24,139,141,267 . Prior to surgery in patients with insulinomas and selected patients with gastrinomas, functional localization studies were performed assessing hormonal gradients either using selective venous sampling or hepatic vein sampling after intra-arterial injections of either calcium or secretin as described previously 64,91,92,293,422 . The presence of a PET was also assessed by tumor imaging studies using cross sectional imaging (ultrasound, CT scan, MRI and, if results unclear, angiography) and SRS as described above.…”

Section: Methodsmentioning

confidence: 99%

Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

et al. 2013

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Multiple endocrine neoplasia type 1 (MEN1) is classically characterized by the development of functional or nonfunctional hyperplasia or tumors in endocrine tissues (parathyroid, pancreas, pituitary, adrenal). Because effective treatments have been developed for the hormone excess state, which was a major cause of death in these patients in the past, coupled with the recognition that nonendocrine tumors increasingly develop late in the disease course, the natural history of the disease has changed. An understanding of the current causes of death is important to tailor treatment for these patients and to help identify prognostic factors; however, it is generally lacking.To add to our understanding, we conducted a detailed analysis of the causes of death and prognostic factors from a prospective long-term National Institutes of Health (NIH) study of 106 MEN1 patients with pancreatic endocrine tumors with Zollinger-Ellison syndrome (MEN1/ZES patients) and compared our results to those from the pooled literature data of 227 patients with MEN1 with pancreatic endocrine tumors (MEN1/PET patients) reported in case reports or small series, and to 1386 patients reported in large MEN1 literature series. In the NIH series over a mean follow-up of 24.5 years, 24 (23%) patients died (14 MEN1-related and 10 non-MEN1-related deaths). Comparing the causes of death with the results from the 227 patients in the pooled literature series, we found that no patients died of acute complications due to acid hypersecretion, and 8%–14% died of other hormone excess causes, which is similar to the results in 10 large MEN1 literature series published since 1995. In the 2 series (the NIH and pooled literature series), two-thirds of patients died from an MEN1-related cause and one-third from a non-MEN1-related cause, which agrees with the mean values reported in 10 large MEN1 series in the literature, although in the literature the causes of death varied widely. In the NIH and pooled literature series, the main causes of MEN1-related deaths were due to the malignant nature of the PETs, followed by the malignant nature of thymic carcinoid tumors. These results differ from the results of a number of the literature series, especially those reported before the 1990s. The causes of non-MEN1-related death for the 2 series, in decreasing frequency, were cardiovascular disease, other nonendocrine tumors > lung diseases, cerebrovascular diseases. The most frequent non-MEN1-related tumor deaths were colorectal, renal > lung > breast, oropharyngeal. Although both overall and disease-related survival are better than in the past (30-yr survival of NIH series: 82% overall, 88% disease-related), the mean age at death was 55 years, which is younger than expected for the general population.Detailed analysis of causes of death correlated with clinical, laboratory, and tumor characteristics of patients in the 2 series allowed identification of a number of prognostic factors. Poor prognostic factors included higher fasting gastrin levels, presence of othe...

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Section: Methodsmentioning

confidence: 99%

Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

et al. 2013

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“…Originally, selective sampling for hormonal gradients was performed by portal-venous-sampling (PVS) 139, 142. This method was largely replaced by selective-arterial injection of secretin (gastrinomas) or calcium (other functional PETs) with assessment of hepatic venous hormone concentrations, because it can be performed at the time of angiography, has less complications and requires less expertise, but is similarly sensitive to PVS139, 140, 143, 144.…”

Section: Tumor Localization/stagingmentioning

confidence: 99%

Gastrointestinal Neuroendocrine Tumors: Pancreatic Endocrine Tumors

2008

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Pancreatic endocrine tumors (PETs) have long fascinated clinicians and investigators despite their relative rarity. Their clinical presentation varies depending upon whether the tumor is functional or not and also according to the specific hormonal syndrome produced. Tumors may be sporadic or inherited but little is known about their molecular pathology, especially the sporadic forms. Chromogranin A appears to be the most useful serum marker for diagnosis, staging and monitoring. Initially, therapy should be directed at the hormonal syndrome as this has the major initial impact on the patient's health. Most PETs are relatively indolent but ultimately malignant, except for insulinomas which are predominantly benign. Surgery is the only modality that offers the possibility of cure although it is generally noncurative in patients with Zollinger-Ellison syndrome or nonfunctional PETs with MEN1. Preoperative staging of disease extent is necessary to determine the likelihood of complete resection though debulking surgery is often felt to be useful in unresectable patients. Once metastatic, biotherapy is usually the first modality employed because it is generally well tolerated. Systemic or regional therapies are generally reserved until symptoms occur or tumor growth is rapid. Recently a number of newer agents, as well as receptor-directed radiotherapy, are being evalulated for patients with advanced disease. This review addresses a number of recent advances regarding the molecular pathology, diagnosis, localization and management of PETs including discussion of peptide receptor radionuclide therapy and other novel antitumor approaches. We conclude with a discussion of future directions and unsettled problems in the field.

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“…However, severe complications, which mainly related to catheterizing the portal vein, are not infrequent. Miller and colleagues reported a rate of 6% severe complications (Miller et al, 1992). For this reason and the development of other less invasive modality, i.e.…”

Section: Percutaneous Transhepatic Portal Venous Sampling (Ptpvs)mentioning

confidence: 99%