The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group

Broek, Medard F M van den; Laat, Joanne M. de; Leeuwaarde, Rachel S van; Ven, Annenienke C van de; Herder, Wouter W. de; Dekkers, Olaf M.; Drent, Madeleine L.; Kerstens, Michiel N.; Bisschop, Peter H.; Havekes, Bas; Hackeng, Wenzel M.; Brosens, Lodewijk A.A.; Vriens, Menno R.; Buikhuisen, Wieneke A.; Valk, Gerlof D.

doi:10.1210/clinem/dgaa800

Cited by 15 publications

(15 citation statements)

References 30 publications

(28 reference statements)

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“…Current MEN1 guidelines suggest imaging for BP and thymic NETs every 1 to 2 years with CT or MRI but acknowledges the uncertainty of this approach (ie, weak recommendation, very low-quality evidence) [ 1 ]. Recent studies report radiologic evidence of BP NETs in approximately 25% of MEN1 patients, but that such tumors are typically associated with low growth rates and an excellent overall survival with little excess mortality [ 3 , 4 , 9 ]. Although occasional tumors display a more aggressive disease course, the current evidence calls into question the value of frequent screening in asymptomatic patients, particularly because there is little evidence to support intervention for small, stable lesions.…”

Section: Multiple Endocrine Neoplasia Type 1 Screening: Areas Of Controversymentioning

confidence: 99%

MEN1 Surveillance Guidelines: Time to (Re)Think?

Newey

Newell‐Price

2022

Journal of the Endocrine Society

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Clinical Practice Guidelines for patients with Multiple Endocrine Neoplasia type 1 (MEN1) recommend a variety of surveillance options. Given progress over the past decade in this area, it is timely to evaluate their ongoing utility. MEN1 is characterized by the development of synchronous or asynchronous tumors affecting a multitude of endocrine and non-endocrine tissues, resulting in premature morbidity and mortality, such that the rationale for undertaking surveillance screening in at-risk individuals appears robust. Current guidelines recommend an intensive regimen of clinical, biochemical and radiological surveillance commencing in early childhood for those with a clinical or genetic diagnosis of MEN1, with the aim of early tumor detection and treatment. Although it is tempting to assume that such screening results in patient benefits and improved outcomes, the lack of a strong evidence base for several aspects of MEN1 care, and the potential for iatrogenic harms related to screening tests or interventions of unproven benefit, make such assumptions potentially unsound. Furthermore, the psychological, as well as economic burdens of intensive screening remain largely unstudied. Although screening undoubtedly constitutes an important component of MEN1 patient care, this perspective aims to highlight some of the current uncertainties and challenges related to existing MEN1 guidelines with a particular focus on the role of screening for pre-symptomatic tumors. Looking forward, a screening approach that acknowledges these limitations and uncertainties and places the patient at the heart of the decision-making process, is advocated.

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Section: Multiple Endocrine Neoplasia Type 1 Screening: Areas Of Controversymentioning

confidence: 99%

MEN1 Surveillance Guidelines: Time to (Re)Think?

Newey

Newell‐Price

2022

Journal of the Endocrine Society

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“…8 Next to other manifestations as gastricand thymic NET, adrenal tumors and breast cancer, patients are also at risk of developing bpNET with a prevalence of 4.7% to 6.6% of MEN1 patients. [9][10][11][12][13][14] Clinical practice guidelines advise frequent thoracic imaging to detect and monitor these tumors. However, more recent studies have shown that MEN1-associated bpNET seem to have an indolent behavior and do not decrease overall survival in MEN1 patients, although a few aggressive cases with fatal outcome have been described.…”

Section: Introductionmentioning

confidence: 99%

“…[19][20][21][22][23][24] Until now, bpNET of any type are considered the same disease, which is also reflected in the recently updated international guidelines. 25,26 However, on the basis of clinical experience and earlier reports on the natural course of sp-bpNET, MEN1-related bpNET and DIPNECH-related bpNET, the question arises whether these subtypes are in fact different entities; MEN1and DIPNECH-related bpNET rarely metastasize or lead to bpNET-related death, [9][10][11][12][13][19][20][21][22][23][24] while the prognosis of sp-bpNET seems more heterogeneousand perhaps worse than nonsporadic forms of bpNET. [3][4][5][6][7] To our knowledge, head-to-head comparisons between sp-bpNET, MEN1-related bpNET and DIPNECHrelated bpNET are lacking to date.…”

Section: Introductionmentioning

confidence: 99%

Well-Differentiated Bronchopulmonary Neuroendocrine Tumors: More Than One Entity

Broek

Levy

Buikhuisen

et al. 2021

Journal of Thoracic Oncology

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Introduction: Until now, well-differentiated bronchopulmonary neuroendocrine tumors (bpNET) occurring either sporadically (sp-bpNET) or in the context of multiple endocrine neoplasia type 1 (MEN1) and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are regarded as similar entities. However, in contrast to sp-bpNET: MEN1-related and DIPNECH-related bpNET rarely metastasize or lead to bpNET-related death. We aimed to describe and compare the course of the disease of sp-bpNET, DIPNECH-and MEN1-related bpNET.Methods: All patients with histologically confirmed MEN1-related bpNET from the DutchMEN Study Group database , patients with resected sp-bpNET and DIPNECH patients referred to a Dutch European Neuroendocrine Tumor Society center between 2000 and 2018 were included. Fisher's exact test was used for comparison between groups. The primary end point was disease-specific mortality (DSM). Kaplan-Meier and logrank test were used to compare survival. Cox regression was used to identify risk factors for DSM in the sp-bpNET subgroup.Results: We included 112 sp-bpNET, 29 MEN1, and 27 DIPNECH patients. Tumor classification was similar across subgroups. A total of 20 patients (18%) with sp-bpNET died because of bpNET, compared with none in the MEN1 group and DIPNECH group. Median disease-specific survival was 12.3 (confidence interval: 6.3-18.3) years for patients with sp-bpNET, and not estimable for the other subgroups (p < 0.001). Differences in baseline characteristics did not explain worse survival in sp-bpNET. Tumor classification and age at diagnosis were independent risk factors for DSM in sp-bpNET.Conclusions: Patients with sp-bpNET have a significantly higher DSM compared with MEN1 or DIPNECH-related bpNET, unexplained by differences in baseline characteristics. This implies that not all bpNET are similar entities.

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“…No scholars have suggested that MEN1-related primary tumors might occur in the liver. Although liver metastasis may occur in MEN1-related duodenopancreatic tumors [ 14 ] and thymic tumors [ 15 ], metachronous liver metastasis has been reported in only MEN1-related lung tumors [ 16 ]. No liver metastasis has been reported in the pituitary tumor or parathyroid tumor.…”

Section: Discussionmentioning

confidence: 99%

Multiple endocrine neoplasia type 1 involving both the liver and lung: a case report

Lai

Huang

et al. 2022

World J Surg Onc

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Background Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant tumor syndrome with a high degree of heterogeneity in clinical phenotypes, generally involving the parathyroid, anterior pituitary, and enteropancreas. In recent years, several new insights into the clinical features of MEN1 have been reported in the literature. However, it is not clear whether MEN1-associated primary tumors can occur in the liver. Case presentation We report the case of a 52-year-old man with multiple endocrine neoplasia type 1 diagnosed by genetic sequencing. After uniportal thoracoscopic right middle lobectomy, laparoscopic radical resection of the liver tumors, and radiofrequency ablation of the parathyroid space, the parathyroid hormone level decreased from 177 pg/ml to a normal level (20 pg/ml). No local tumor recurrence was observed during a follow-up of 5 months. Conclusion We report the first case of MEN1 with simultaneous liver and lung involvement in which the patient underwent radical resection of the tumors, and we propose the possibility that the liver and other nonendocrine organs may also develop diseases associated with MEN1; although, this view needs further verification. Gene detection has crucial clinical significance for guiding diagnosis and treatment.

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The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group

Cited by 15 publications

References 30 publications

MEN1 Surveillance Guidelines: Time to (Re)Think?

MEN1 Surveillance Guidelines: Time to (Re)Think?

Well-Differentiated Bronchopulmonary Neuroendocrine Tumors: More Than One Entity

Multiple endocrine neoplasia type 1 involving both the liver and lung: a case report

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