Role of 68Ga-DOTATATE PET/CT in patients with multiple endocrine neoplasia type 1 (MEN1)

Lastoria, Secondo; Marciello, Francesca; Faggiano, Antongiulio; Aloj, Luigi; Caracò, Corradina; Aurilio, Michela; D'Ambrosio, Laura; Gennaro, Francesca Di; Ramundo, Valeria; Camera, Luigi; Luca, Leonardo De; Fonti, Rosa; Napolitano, Vincenzo; Colao, Annamaria

doi:10.1007/s12020-015-0702-y

Cited by 54 publications

(102 citation statements)

References 23 publications

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“…This later scenario would be downgraded to weakly supportive (a tentative diagnosis) of the diagnosis of ZES in patients with MEN1 (Table 4.B.2). This occurs because these patients very frequently have other nongastrinoma NETs including NF-pNETs, carcinoids (gastric, lung, thymus), pituitary and adrenal NETs, which may all be positive on somatostatin receptor scintigraphy imaging (SRI) with either 68 Ga-DOTATATE PET/CT or 111 In-DTPA-octreotide with SPECT/CT imaging, and thus a positive SRI result provides minimal support for the diagnosis of ZES [33, 163–165]. …”

Section: Discussion and Proposal Of New Criteria To Support Thementioning

confidence: 99%

Diagnosis of Zollinger–Ellison Syndrome in the Era of Ppis, Faulty Gastrin Assays, Sensitive Imaging and Limited Access to Acid Secretory Testing

Metz

Cadiot

Poitras

et al. 2017

Int. J. Endo. Oncol.

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In recent years the diagnosis of Zollinger-Ellison syndrome (ZES) has become increasingly controversial with several new approaches and criteria proposed, differing from the classical biochemical criterion of inappropriate hypergastrinemia (i.e., hypergastrinemia in the presence of hyperchlorhydria) (Table 1). These changes have come about because of the difficulty and potential dangers of stopping proton pump inhibitors (PPIs) for gastric acid analysis; the recognition than many of the current assays used to assess gastrin concentrations are unreliable; the development of sensitive imaging modalities that detect neuroendocrine tumors (NETs) including an increasing number of the primary gastrinomas; the increased use of percutaneous or endoscopic ultrasound (EUS)-directed biopsies/cytology and the general lack of availability of acid secretory testing. In this article we will discuss the basis for these controversies, review the proposed changes in diagnostic approaches and make recommendations for supporting the diagnosis of ZES in the modern era.

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Section: Discussion and Proposal Of New Criteria To Support Thementioning

confidence: 99%

Diagnosis of Zollinger–Ellison Syndrome in the Era of Ppis, Faulty Gastrin Assays, Sensitive Imaging and Limited Access to Acid Secretory Testing

Metz

Cadiot

Poitras

et al. 2017

Int. J. Endo. Oncol.

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“…Moreover, 68 Ga-PET/CT is another accurate technique for detecting NF-PNEN, and it seems to have value in the prognosis of MEN1-related PNEN. 68 Ga-PET/CT could then be a promising tool helping in the management of these neoplasms [24,25]. …”

Section: Discussionmentioning

confidence: 99%

Active Surveillance versus Surgery of Nonfunctioning Pancreatic Neuroendocrine Neoplasms ≤2 cm in MEN1 Patients

Partelli¹,

Tamburrino²,

Lopez

et al. 2016

Neuroendocrinology

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Background: The aim of this study was to evaluate the efficacy of conservative treatment for nonfunctioning pancreatic neuroendocrine neoplasms (NF-PNEN) ≤2 cm in multiple endocrine neoplasia type 1 (MEN1)-affected patients compared with surgical treatment. Methods: The databases of 4 tertiary referral institutions (San Raffaele Scientific Institute, Milan; Philipps-Universität Marburg, Marburg; University of Padua, Padua; Royal Free Hospital, London) were analyzed. A comparison of conservative management and surgery at initial diagnosis of NF-PNEN ≤2 cm between 1997 and 2013 was performed. Results: Overall, 27 patients (45%) underwent up-front surgery and 33 patients (55%) were followed up after the initial diagnosis. A higher proportion of patients in the surgery group were female (70 vs. 33%, p = 0.004). Patients were mainly operated on in the period 1997-2007 as compared with the period 2008-2013 (n = 17; 63 vs. 37%; p = 0.040). The rate of multifocal tumors was higher in the surgery group (n = 24; 89%) than in the ‘no surgery' group (n = 22; 67%; p = 0.043). After a median follow-up of 126 months, 1 patient deceased due to postoperative complications within 30 days after surgery. The 5-, 10-, and 15-year progression-free survival (PFS) rates were 63, 39, and 10%, respectively. The median PFS was similar in the two groups. Overall, 13 patients (32.5%) were operated on after initial surgical or conservative treatment. The majority of the surgically treated patients had stage 1 (77.5%), T1 (77.5%), and G1 (85%) tumors. Conclusions: NF-PNEN ≤2 cm in MEN1 patients are indolent neoplasms posing a low oncological risk. Surgical treatment of these tumors at initial diagnosis is rarely justified in favor of conservative treatment.

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“…68 Ga-DOTA PET-CT was evaluated in three prospective studies [33, 35, 45] and one study reported the diagnostic value in a retrospective case series [39]. Albers et al [33] compared the diagnostic value of combined conventional imaging (EUS/MRI) with 68 Ga-DOTATOC-PET-CT for the diagnosis of dpNETs in routine follow-up in 33 MEN1 patients.…”

Section: Resultsmentioning

confidence: 99%

Diagnosing Nonfunctional Pancreatic NETs in MEN1: The Evidence Base

Treijen

Beek

Leeuwaarde

et al. 2018

Journal of the Endocrine Society

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In multiple endocrine neoplasia type 1 (MEN1), nonfunctional pancreatic neuroendocrine tumors (NF-pNETs) are the most frequently diagnosed NETs and a leading cause of MEN1-related death. The high prevalence and malignant potential of NF-pNETs outline the need for an evidence-based screening program, as early diagnosis and timely intervention could reduce morbidity and mortality. Controversies exist regarding the value of several diagnostic tests. This systematic review aims to evaluate current literature and amplify an up-to-date evidence-based approach to NF-pNET diagnosis in MEN1. Three databases were systematically searched on the diagnostic value of biomarkers and imaging modalities. Twenty-seven studies were included and critically appraised (modified Quality Assessment of Diagnostic Accuracy Studies). Another 12 studies, providing data on age-related penetrance and tumor growth, were included to assess the optimal frequency and timing of screening. Based on current literature, biomarkers should no longer play a role in the diagnostic process for NF-pNETs, as accuracies are too low. Studies evaluating the diagnostic value of imaging modalities are heterogeneous with varying risks of bias. For the detection of NF-pNETs, endoscopic ultrasound (EUS) has the highest sensitivity. A combined strategy of EUS and MRI seems to be the most useful. Gallium 68 octreotate-DOTA positron emission tomography-CT could be added if NF-pNETs are diagnosed to identify metastasis. Reported growth rates were generally low, and two distinct phenotypes were observed. Surveillance programs should focus on and be adapted to the presence of substantial growth in NF-pNETs. The optimal age to start screening must yet be determined, as insufficient evidence for an evidence-based recommendation was available.

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Role of 68Ga-DOTATATE PET/CT in patients with multiple endocrine neoplasia type 1 (MEN1)

Cited by 54 publications

References 23 publications

Diagnosis of Zollinger–Ellison Syndrome in the Era of Ppis, Faulty Gastrin Assays, Sensitive Imaging and Limited Access to Acid Secretory Testing

Diagnosis of Zollinger–Ellison Syndrome in the Era of Ppis, Faulty Gastrin Assays, Sensitive Imaging and Limited Access to Acid Secretory Testing

Active Surveillance versus Surgery of Nonfunctioning Pancreatic Neuroendocrine Neoplasms ≤2 cm in MEN1 Patients

Diagnosing Nonfunctional Pancreatic NETs in MEN1: The Evidence Base

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