Patient characteristics and clinical outcomes following initial surgical intervention for MEN1 associated pancreatic neuroendocrine tumours: A systematic review and exploratory meta-analysis of the literature

Ratnayake, Chathura B B; Loveday, Benjamin; Windsor, John A.; Lawrence, Benjamin; Pandanaboyana, Sanjay

doi:10.1016/j.pan.2019.03.002

Cited by 12 publications

(12 citation statements)

References 49 publications

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“…Prevention of long-term complications is important, because pancreatic insufficiency decreases quality of life in this young and otherwise unaffected population. Insulinoma is often the first manifestation of MEN1 and a common surgical indication in children and adolescents with MEN1, which underscores the importance of long-term pancreatic function 32 -35 . None of the patients in this cohort who underwent enucleation developed endocrine or exocrine insufficiency, which is in line with other studies 36,37 . New-onset diabetes, on the contrary, was observed after distal pancreatectomies with or without enucleations in 26 per cent of patients.…”

Section: Discussionsupporting

confidence: 92%

Surgery for multiple endocrine neoplasia type 1-related insulinoma: long-term outcomes in a large international cohort

Beek¹,

Nell²,

Verkooijen

et al. 2020

British Journal of Surgery

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Background Insulinomas are found in 10–15 per cent of patients with multiple endocrine neoplasia type 1 (MEN1) and lead to life‐threatening hypoglycaemia. Surgical outcome and the optimal surgical strategy for MEN1‐related insulinoma are unknown. Methods Patients with MEN1‐related insulinomas were identified in 46 centres in Europe and North America between 1990 and 2016. Insulinomas were considered localized if the lesion was in the pancreatic head or body/tail. Patients with pancreatic neuroendocrine tumours throughout the pancreas were suspected of having multifocal insulinoma. The primary outcome was postoperative hypoglycaemia, defined as persistent hypoglycaemia, or recurrent hypoglycaemia caused by a new insulinoma or insulin‐producing liver metastases. Hypoglycaemia‐free survival was estimated by the Kaplan–Meier method. Results Ninety‐six patients underwent resection for MEN1‐related insulinoma. Sixty‐three and 33 patients had localized and multifocal insulinomas respectively. After a median follow‐up of 8 (range 1–22) years, one patient (1 per cent) had persistent disease and six (6 per cent) had developed recurrent disease, of whom four had a new insulinoma. The 10‐year hypoglycaemia‐free survival rate was 91 (95 per cent c.i. 80 to 96) per cent. Of those with localized disease, 46 patients underwent pancreatic resection and 17 enucleation. One of these patients had persistent disease and one developed recurrent insulinoma. Among patients with multifocal disease, three developed new insulinomas and two developed insulin‐producing liver metastases. Conclusion Surgery for MEN1‐related insulinoma is more successful than previously thought.

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Section: Discussionsupporting

confidence: 92%

Surgery for multiple endocrine neoplasia type 1-related insulinoma: long-term outcomes in a large international cohort

Beek¹,

Nell²,

Verkooijen

et al. 2020

British Journal of Surgery

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“…32,38 Major pancreatic resections have been associated with higher cure rates and improved overall survival compared with enucleation, yet they also have higher rates of postoperative complications and long-term morbidity. [39][40][41] Minimally invasive distal pancreatectomy has become a widely accepted surgical intervention, and large retrospective analyses have demonstrated less blood loss and shorter hospital stays with comparable morbidity and oncologic outcome. 42,43 Techniques are continually evolving, 44,45 most significantly with the emergence of the robotic surgery platform.…”

Section: Duodenopancreatic Netmentioning

confidence: 99%

It's not a mystery, it's in the history: Multidisciplinary management of multiple endocrine neoplasia type 1

Shirali

Pieterman

Lewis

et al. 2021

CA A Cancer J Clinicians

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“…Still, liver metastases from small, apparently stable nf-PanNETs can occur (Pieterman et al 2017, Triponez et al 2018. Extent of surgical resection is also debated, with a recent systematic review concluding that major pancreatic resections yield lower recurrence rates but more frequent postoperative endocrine insufficiency, while no difference was observed between reoperations and survival (Ratnayake et al 2019). These findings need cautious interpretation as only 13/27 studies included the indications for surgery, thus confounding by indication cannot be excluded (Ratnayake et al 2019).…”

Section: Clinical Case Continuedmentioning

confidence: 99%

“…Extent of surgical resection is also debated, with a recent systematic review concluding that major pancreatic resections yield lower recurrence rates but more frequent postoperative endocrine insufficiency, while no difference was observed between reoperations and survival (Ratnayake et al 2019). These findings need cautious interpretation as only 13/27 studies included the indications for surgery, thus confounding by indication cannot be excluded (Ratnayake et al 2019). Additionally, several of the studies were heterogeneous and of low quality, as assessed by the authors' predefined criteria (Ratnayake et al 2019).…”

Section: Clinical Case Continuedmentioning

confidence: 99%

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HEREDITARY ENDOCRINE TUMOURS: CURRENT STATE-OF-THE-ART AND RESEARCH OPPORTUNITIES: MEN1-related pancreatic NETs: identification of unmet clinical needs and future directives

Pieterman

Sadowski

Maxwell

et al. 2020

Endocrine-Related Cancer

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The PanNET Working Group of the 16th International Multiple Endocrine Neoplasia Workshop (MEN2019) convened in Houston, TX, USA, 27–29 March 2019 to discuss key unmet clinical needs related to PanNET in the context of MEN1, with a special focus on non-functioning (nf)-PanNETs. The participants represented a broad range of medical scientists as well as representatives from patient organizations, pharmaceutical industry and research societies. In a case-based approach, participants addressed early detection, surveillance, prognostic factors and management of localized and advanced disease. For each topic, after a review of current evidence, key unmet clinical needs and future research directives to make meaningful progress for MEN1 patients with nf-PanNETs were identified. International multi-institutional collaboration is needed for adequately sized studies and validation of findings in independent datasets. Collaboration between basic, translational and clinical scientists is paramount to establishing a translational science approach. In addition, bringing clinicians, scientists and patients together improves the prioritization of research goals, assures a patient-centered approach and maximizes patient involvement. It was concluded that collaboration, research infrastructure, methodologic and reporting rigor are essential to any translational science effort. The highest priority for nf-PanNETs in MEN1 syndrome are (1) the development of a data and biospecimen collection architecture that is uniform across all MEN1 centers, (2) unified strategies for diagnosis and follow-up of incident and prevalent nf-PanNETs, (3) non-invasive detection of individual nf-PanNETs that have an increased risk of metastasis, (4) chemoprevention clinical trials driven by basic research studies and (5) therapeutic targets for advanced disease based on biologically plausible mechanisms.

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Patient characteristics and clinical outcomes following initial surgical intervention for MEN1 associated pancreatic neuroendocrine tumours: A systematic review and exploratory meta-analysis of the literature

Cited by 12 publications

References 49 publications

Surgery for multiple endocrine neoplasia type 1-related insulinoma: long-term outcomes in a large international cohort

Surgery for multiple endocrine neoplasia type 1-related insulinoma: long-term outcomes in a large international cohort

It's not a mystery, it's in the history: Multidisciplinary management of multiple endocrine neoplasia type 1

HEREDITARY ENDOCRINE TUMOURS: CURRENT STATE-OF-THE-ART AND RESEARCH OPPORTUNITIES: MEN1-related pancreatic NETs: identification of unmet clinical needs and future directives

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