Early and Late Complications After Surgery for MEN1-related Nonfunctioning Pancreatic Neuroendocrine Tumors

Nell, Sjoerd; Rinkes, Inne H.M. Borel; Verkooijen, Helena M.; Bonsing, Bert A.; Eijck, Casper H. van; Goor, Harry van; Kleine, Ruben H J de; Kazemier, Geert; Dijkum, E. J. Nieveen van; Dejong, Cornelis H. C.; Valk, Gerlof D.; Vriens, Menno R.

doi:10.1097/sla.0000000000002050

Cited by 50 publications

(57 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Merging the need for pancreaticoduodenal resections to achieve biochemical cure on the one hand and the scarcity of data regarding postoperative complications and long-term oncological outcomes after pancreaticoduodenal resections on the other, future studies should address these topics to come to meaningful advice. 28 In conclusion, life expectancy in patients with MEN1 having gastrinomas is reduced compared with other studies. OS was associated with initial FSG levels ≥20x ULN, a pNET ≥2.0 cm on conventional imaging, synchronous liver metastases, multiple concurrent NETs, and gastroduodenoscopy suspicious for gastric NET in patients with MEN1.…”

Section: Prognostic Factors For Osmentioning

confidence: 72%

“…Especially, in the coexistence of hypergastrinemia and pNETs ≥2.0 cm, the optimal surgical strategy is hard to establish. Merging the need for pancreaticoduodenal resections to achieve biochemical cure on the one hand and the scarcity of data regarding postoperative complications and long‐term oncological outcomes after pancreaticoduodenal resections on the other, future studies should address these topics to come to meaningful advice …”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

Beek

Nell

Pieterman

et al. 2019

Journal of Surgical Oncology

Self Cite

View full text Add to dashboard Cite

Background and objectivesGastrinomas are the most prevalent functioning neuroendocrine tumors (NET) in multiple endocrine neoplasia type 1 (MEN1). Guidelines suggest medical therapy in most patients, but surgery may be considered in a subgroup. Currently, factors to guide management are necessary. This population‐based cohort study assessed prognostic factors of survival in patients with MEN1‐related gastrinomas.MethodsPatients with MEN1 having gastrinomas were identified in the Dutch MEN1 database from 1990 to 2014 based on fasting serum gastrin (FSG) levels and/or pathology. Predictors of overall survival were assessed using Cox regression.ResultsSixty‐three patients with gastrinoma (16% of the MEN1 population) were identified. Five‐ and 10‐year overall survival rates were 83% and 65%, respectively. Prognostic factors associated with overall survival were initial FSG levels ≥20x upper limit of normal (ULN) (hazard ratio [HR], 6.2 [95% confidence interval, 1.7‐23.0]), pancreatic NET ≥2 cm (HR 4.5; [1.5‐13.1]), synchronous liver metastases (HR 8.9; [2.1‐36.7]), gastroduodenoscopy suspicious for gastric NETs (HR 12.7; [1.4‐115.6]), and multiple concurrent NETs (HR 5.9; [1.2‐27.7]).ConclusionLife expectancy of patients with MEN1 gastrinoma is reduced. FSG levels and pancreatic NETs ≥2 cm are prognostic factors. FSG levels might guide surveillance intensity, step‐up to additional diagnostics, or provide arguments in selecting patients who might benefit from surgery.

show abstract

Section: Prognostic Factors For Osmentioning

confidence: 72%

Section: Discussionmentioning

confidence: 99%

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

Beek

Nell

Pieterman

et al. 2019

Journal of Surgical Oncology

Self Cite

View full text Add to dashboard Cite

show abstract

“…Despite the aggressive protocol, 2 patients died of metastatic pNETs (Hausman et al 2004, Gauger et al 2009). Nell and coworkers (Nell et al 2016) also reported 33% of major early complications and 23% of endocrine and/or exocrine insufficiency among 61 patients with MEN1 who underwent pancreatic surgery in a Dutch academic center. These patients were young (median 41 years old) and in good general health (ASA score I or II for 89% of the patients), further emphasizing the negative impact of complications in this population.…”

Section: Non-functional Pancreatic Netsmentioning

confidence: 93%

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

Sadowski¹,

Cadiot²,

Dansin³

et al. 2017

Endocrine-Related Cancer

View full text Add to dashboard Cite

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.

show abstract

“…47 Collectively, these data highlight the risk of significant post-operative morbidity associated with pancreatic surgery in MEN1. 47 Collectively, these data highlight the risk of significant post-operative morbidity associated with pancreatic surgery in MEN1.…”

Section: What Are the Tre Atment Op Ti On S For Nonme Ta S Tati C Mmentioning

confidence: 95%

What is the appropriate management of nonfunctioning pancreatic neuroendocrine tumours disclosed on screening in adult patients with multiple endocrine neoplasia type 1?

Challis

Casey

Grossman

et al. 2019

Clinical Endocrinology

View full text Add to dashboard Cite

Multiple endocrine neoplasia type 1 (MEN1) is an inherited tumour syndrome characterised by a predisposition to the development of endocrine tumours of the parathyroid glands, pituitary and pancreas: 30%-80% of patients with MEN1 develop pancreatic neuroendocrine tumours (pNETs), with metastatic tumours and/or their sequelae contributing to increased morbidity and early mortality. The optimal management of nonfunctioning (NF) pNETs in MEN1 remains controversial. Whilst pancreatic resection is widely recommended for tumours >2 cm, for smaller tumours (≤2 cm) a well-established consensus guiding the indications for surgical intervention does not exist. Although total pancreatectomy may be curative for some patients, both short-and long-term complications make this an unsatisfactory option for many patients. For small (<2 cm) MEN1 NF-pNETs, some clinicians advocate surveillance based largely on retrospective data that suggest 50%-80% of these lesions are stable over time and infrequently exhibit accelerated growth rates. It is increasingly recognised, however, that NF-pNETs exhibit unpredictable malignant behaviour that is not determined by tumour size alone, thereby prompting other clinicians to advocate surgery for all MEN1 NF-pNETs, irrespective of size. Such uncertainty poses clinical management challenges with regards to the timing and extent of surgery, which is further hindered by the inability to stratify patients based on predicted tumour behaviour. It is therefore critical that future MEN1 research initiatives include: (a) the discovery of biomarkers that better predict tumour behaviour; (b) the evaluation of medical therapies that may delay, or even prevent, the need for pancreatic surgery;and, ultimately, (c) improvement in the quality of life for individuals with MEN1. Here, based on the published literature, we address the Clinical Question, 'What is the management of NF-pNETs disclosed on screening in adult patients with MEN1?'.

show abstract

Early and Late Complications After Surgery for MEN1-related Nonfunctioning Pancreatic Neuroendocrine Tumors

Abstract: MEN1 NF-pNET surgery is associated with high rates of major short and long-term complications. Current findings should be taken into account in the shared decision-making process when MEN1 NF-pNET surgery is considered.

Cited by 50 publications

References 29 publications

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

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

What is the appropriate management of nonfunctioning pancreatic neuroendocrine tumours disclosed on screening in adult patients with multiple endocrine neoplasia type 1?

Contact Info

Product

Resources

About