Well-Differentiated Bronchopulmonary Neuroendocrine Tumors: More Than One Entity

Broek, Medard F M van den; Levy, Sonja; Buikhuisen, Wieneke A.; Dijke, Kim; Hartemink, Koen J.; Leeuwaarde, Rachel S van; Vriens, Menno R.; Tesselaar, Margot E.T.; Herder, Wouter W. de; Dekkers, Olaf M.; Ven, Annenienke van de; Havekes, Bas; Drent, Madeleine L.; Bisschop, Peter H.; Zandee, Wouter T; Valk, Gerlof D.

doi:10.1016/j.jtho.2021.07.020

Cited by 4 publications

(4 citation statements)

References 30 publications

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“…Carcinoids in patients with DIPNECH in our study had more indolent behavior, with slow growth rates and no metastatic disease observed in 6 DIPNECH patients. The DIPNECH cohort was small, but the observed indolent behavior is consistent with that previously noted in a larger case series 39 . At least one previous study has looked at VDTs in hamartomas.…”

Section: Discussionsupporting

confidence: 85%

“…The DIPNECH cohort was small, but the observed indolent behavior is consistent with that previously noted in a larger case series. 39 At least one previous study has looked at VDTs in hamartomas. Notably, one group calculated the VDT for 9 hamartomas, which, similar to our data set, showed a wide range of doubling times with one particularly fast-growing outlier.…”

Section: Discussionmentioning

confidence: 99%

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Growth Rates of Pulmonary Carcinoid Tumors and Hamartomas

Ryan

Hammer

2023

J Comput Assist Tomogr

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BackgroundPulmonary nodule growth is often measured by volume doubling time (VDT), which may guide management. Most malignant nodules have a VDT of 20 to 400 days, with longer VDTs typically observed in indolent nodules. We assessed the utility of VDT in differentiating pulmonary carcinoids and hamartomas.MethodsA review was performed from January 2012 to October 2021 to identify patients with pathologic diagnoses and at least 2 chest computed tomography scans obtained 6 or more months apart. Visualization software was used to segment nodules and calculate diameter and volume. Volume doubling time was calculated for scans with 1-mm slices. For the remainder, estimated nodule volume doubling time (eVDT) was calculated using nodule diameter. Volume doubling times/eVDTs were placed into growth categories: less than 400 days; 400–600 days; and more than 600 days.ResultsSixty nodules were identified, 35 carcinoids and 25 hamartomas. Carcinoids were larger than hamartomas (median diameter, 13.5 vs 11.5 mm; P = 0.05). For carcinoid tumors, median VDT (n = 15) was 1485 days, and median eVDT (n = 32) was 1309 days; for hamartomas, median VDT (n = 8) was 2040 days and median eVDT (n = 25) was 2253 days. Carcinoid tumor eVDT was significantly shorter than hamartomas (P = 0.03). By growth category, 1 of 25 hamartomas and 5 of 35 carcinoids had eVDT less than 400 days and 24 of 25 hamartomas and 27 of 35 carcinoids had eVDT more than 600 days. Of 4 carcinoid tumors with metastases, 2 had eVDT less than 400 days and 2 had eVDT more than 600 days.ConclusionsGrowth rate was not a reliable differentiator of pulmonary hamartomas and carcinoids. Slow growing carcinoids can metastasize. Radiologists should be cautious when discontinuing computed tomography follow-up based on growth rates alone.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

Growth Rates of Pulmonary Carcinoid Tumors and Hamartomas

Ryan

Hammer

2023

J Comput Assist Tomogr

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“…In addition, some treatment recommendations are extrapolated from their sporadic counterpart tumors, which may be unreliable. For example, recent studies indicate that MEN1-associated BP NETs have a better prognosis than the equivalent sporadic tumors, unexplained by differences in baseline characteristics [ 4 ]. Thus, the lack of high-quality evidence guiding treatment decisions for several MEN1-associated tumors calls into question the value of intensive screening, limitations that were acknowledged in the 2012 guidelines.…”

Section: What Makes a Good Tumor Screening/surveillance Program?mentioning

confidence: 99%

“…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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