Prospective Endoscopic Ultrasonographic Evaluation of the Frequency of Nonfunctioning Pancreaticoduodenal Endocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1

Thomas-Marques, Laurence; Murat, A.; Delemer, Brigitte; Penfornis, A.; Cardot-Bauters, Catherine; Baudin, Éric; Niccoli-Sire, Patricia; Levoir, D; Choplin, H.; Chabre, Olivier; Jovenin, Nicolas; Cadiot, Guillaume; Endocrines, Groupe des Tumeurs

doi:10.1111/j.1572-0241.2006.00367.x

Cited by 154 publications

(129 citation statements)

References 34 publications

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“…EUS as a harmless procedure improves non-surgical pancreatic imaging and enables to detect and localize symptomatic neuroendocrine pancreatic tumours better and earlier and may be helpful in planning therapeutical strategy (Bansal et al 1999, Kann et al 2001, 2003, 2006, Wamsteker et al 2003, Hellman et al 2005, McLean & Fairclough 2005, Thomas-Marques et al 2006. It also has been introduced into the diagnostic repertoire in MEN1-disease, however also raising new and specific questions.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, EUS enables to visualize small neuroendocrine tumours down to a diameter of 1-2 mm can be visualized (Kann et al 2001, 2003, Wamsteker et al 2003, Hellman et al 2005, Thomas-Marques et al 2006. Therefore, its use in endocrinology for imaging of the endocrine pancreas and also the adrenal glands is becoming more frequent (Kann et al 1998a, 1998b, 1999, 2004, Kann 2005.…”

Section: Introductionmentioning

confidence: 99%

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Natural course of small, asymptomatic neuroendocrine pancreatic tumours in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study

Kann¹,

Balakina²,

Ivan³

et al. 2006

Endocr Relat Cancer

107

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Endoscopic ultrasound (EUS) enables detection and localization of pancreatic neuroendocrine tumours. Even small tumours down to a diameter of 1-2 mm can be visualized. Since such small tumours usually cannot be detected by computed tomography (ct), magnetic resonance imaging (mri) and somatostatin receptor scintigraphy (srs), and experience with EUS imaging is limited, there is no clear evidence for clinical management in multiple endocrine neoplasia type 1 (MEN1). Knowledge about the natural course of growth and metastatic distribution is mandatory to come to appropriate clinical decisions and guidelines. This prospective study was aimed to assess the natural course of small (!15 mm) neuroendocrine pancreatic tumours without clinical symptoms due to endocrine activity or mechanical problems and without clear indication for surgical therapy in MEN1 by EUS.A total of 82 asymptomatic tumours !15 mm (5.9G3.2 mm diameter at baseline) in 20 patients with MEN1-disease (8 female/12 male, 43G13 years) were studied over a period of 20G12 months (33.8 patient years, 106.7 tumour years) by EUS. Change in largest diameter of each tumour and annual tumour incidence rate in the patients' cohort were calculated.Increase of largest tumour diameter was found to be 1.3G3.2% per month, annual tumour incidence rate 0.62 new tumours per patient year. In one patient, rapid progressive pancreatic manifestation of MEN1 was observed. There was no evidence in ct and/or srs and/or mri for metastatic disease in all patients. Only 4/84 (4.8%) pancreatic tumours could be visualized by computed tomography, 5/79 (6.3%) by somatostatin receptor imaging and 4/39 (10.3%) by magnetic resonance imaging.Small asymptomatic neuroendocrine pancreatic tumours in MEN1 usually seem to grow slowly. Annual tumour incidence rate is low. However, faster growing tumours and patients with rapidly progressive disease can be observed. Risk for obvious metastatic disease from asymptomatic neuroendocrine pancreatic tumours !15 mm in MEN1 seems to be low.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Natural course of small, asymptomatic neuroendocrine pancreatic tumours in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study

Kann¹,

Balakina²,

Ivan³

et al. 2006

Endocr Relat Cancer

107

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“…The gold standard modalities for pancreatic gland analysis in patients with MEN1 are EUS and MRI, which are complementary (Barbe et al 2012). Although the sensitivity of EUS is much higher than that of MRI for the detection of pNETs (Thomas-Marques et al 2006, Barbe et al 2012, Kappelle et al 2017, larger tumors (>2 cm) can be found using MRI, especially in the pancreatic tail (Barbe et al 2012). Liver MRI is the reference imaging modality for the detection of liver metastases (d 'Assignies et al 2013).…”

Section: Tumor Localizationmentioning

confidence: 99%

The future: surgical advances in MEN1 therapeutic approaches and management strategies

Sadowski¹,

Cadiot²,

Dansin³

et al. 2017

Endocrine-Related Cancer

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Multiple endocrine neoplasia type 1 (MEN1) is a hereditary autosomal dominant disorder associated with numerous neuroendocrine tumors (NETs). Recent advances in the management of MEN1 have led to a decrease in mortality due to excess hormones; however, they have also led to an increase in mortality from malignancy, particularly NETs. The main challenges are to localize these tumors, to select those that need therapy because of the risk of aggressive behavior and to select the appropriate therapy associated with minimal morbidity. This must be applied to a hereditary disease with a high risk of recurrence. The overall aim of management in MEN1 is to ensure that the patient remains disease-and symptom-free for as long as possible and maintains a good quality of life. Herein, we review the changes that occurred in the last 20 years in the surgical management of MEN1-associated functional and non-functional pancreaticoduodenal NETs and thymic and bronchial NETs.

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“…A recent prospective study showed that the rate of NFPETs in MEN1 patients is higher than 55% when endoscopic ultrasound (EUS) is performed to detect them [7]. Since the number and size of the NFPETs tend to increase in time and the affected population is relatively young (median age is 39 years), this rate is certainly going to grow as time passes by.…”

Section: Discussionmentioning

confidence: 99%

“…The revision of the NFPETs in MEN1 has clearly shown an association between large primary tumor size, the presence of distant metastases and the mortality due to tumoral progression [7][8][9]. EPTs have been recently stratified, on the basis of the WHO classification, in 3 categories: benign tumors, well-differentiated carcinomas with low-grade malignant behaviour and poorly-differentiated carcinomas with high grade malignant behaviour [10].…”

Section: Discussionmentioning

confidence: 99%

Feasibility of Mesenteric Vein Reconstruction with PTFE Prosthesis for Non Functioning Endocrine Pancreatic Tumor Surgery

Fratini¹,

Francesco²,

Francesco³

2012

Pancreatic Dis Ther

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Endocrine pancreatic tumors (EPTs) are rare entities with a low incidence (3-10 per million). A relatively frequent feature (15-53%) among this group of tumors is represented by the non-functioning endocrine pancreatic tumors (NFEPTs) whose peculiarity is due to the absent secrection of mature or active hormones, leaving the patient free from clinically evident hypersecrection syndromes, generally discovered when the mass effect becomes evident, the adjacent pancreatic structures (splenic, superior mesenteric and portal vein, celiac or superior mesenteric arteries, common bile duct, duodenum, etc.) are infiltrated or hepatic metastases are growing. A potentially malignant attitude is high and well related to the dimension of the tumor with inexorably fatal outcome if appropriate surgery is delayed.The size of the mass and an evident involvement of nearer vascular structures might rise some doubts about the decision to radically remove the tumor. An aggressive surgery should be balanced with the risk/benefit ratio for generally young patients with a reasonable long life expectancy.We parallel two clinical cases among the patients we observed through the years at our institution and operated on by the same surgeon, who were displaying the same tumoral histology and loco-regional invasiveness of portomesenteric axis, but differing one each other for the presence of metastatic disease to the liver the first case. Further aim of the present report is to support the evidence of the feasibility and safeness of extensive surgical demolition with prosthetic reconstruction of the porto-mesenteric axis.

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Prospective Endoscopic Ultrasonographic Evaluation of the Frequency of Nonfunctioning Pancreaticoduodenal Endocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1

Abstract: The frequency of nonfunctioning pancreatic endocrine tumors is higher (54.9%) than previously thought. The size and number of these tumors can increase over time. Pancreatic EUS should be performed once MEN1 is diagnosed to monitor disease progression.

Cited by 154 publications

References 34 publications

Natural course of small, asymptomatic neuroendocrine pancreatic tumours in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study

Natural course of small, asymptomatic neuroendocrine pancreatic tumours in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study

The future: surgical advances in MEN1 therapeutic approaches and management strategies

Feasibility of Mesenteric Vein Reconstruction with PTFE Prosthesis for Non Functioning Endocrine Pancreatic Tumor Surgery

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