Understanding the clinical course of genotype-negative MEN1 patients can inform management strategies

Pieterman, Carolina R. C.; Hyde, Samuel M.; Wu, Seng-Tang; Landry, Jace P.; Chiang, Yi Ju; Christakis, Ioannis; Grubbs, Elizabeth G.; Fisher, Sarah B.; Graham, Paul H.; Waguespack, Steven G.; Perrier, Nancy D.

doi:10.1016/j.surg.2020.04.067

Cited by 20 publications

(20 citation statements)

References 18 publications

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“…Once the gene test is positive, close clinical follow up observation should be carried out according to the guidelines in order to achieve early detection, early diagnosis, early treatment and improve survival time. First degree relatives of patients are also recommended for MEN1 gene screening to identify which family members need to enter the clinical observation process, early detection and treatment of complications caused by endocrine hormones, and more importantly, early detection of highly malignant lesions will increase tumor R0 resection rate, so that patients have a longer survival time [7,9,10]. One patient with the same MEN1 gene mutation as the proband was found in this pedigree, and there was no clinical phenotype at present.…”

Section: Discussionmentioning

confidence: 99%

Multiple Endocrine Neoplasia Type 1 Involving Both Liver and Lung: A Case Report

Lai

Huang

et al. 2021

Preprint

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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 parathyroid, anterior pituitary and enteropancreas. In recent years, several new insights on the clinical features of MEN1 have been reported in the literature. However, it is not clear whether MEN1-associated primary tumors may occur in the liver. Case presentation: we report the case of a 52-year-old man with multiple endocrine neoplasia type 1 diagnosed by next-generation sequencing. After Single-hole thoracoscopic right middle lobectomy, Laparoscopic radical resection of liver tumor and Radiofrequency ablation of parathyroid space, the parathyroid hormone decreased from 177pg/ml to normal(20pg/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 who underwent radical resection of the tumors, and we propose the possibility that liver and other non-endocrine organs may also develop diseases associated with MEN1，though this view needs further verification. Gene detection has crucial clinical instructive significance for diagnosis and treatment.

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Section: Discussionmentioning

confidence: 99%

Multiple Endocrine Neoplasia Type 1 Involving Both Liver and Lung: A Case Report

Lai

Huang

et al. 2021

Preprint

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“…His screening imaging identified a suspected but not proven nonfunctioning PA that is being followed. Figure 4 outlines the basic considerations when managing a patient suspected to have MEN1 1,10,11,15,32,36,65 …”

Section: Specific Case Considerationsmentioning

confidence: 99%

“…Framework for Initial Management and Surveillance of the Patient With Multiple Endocrine Neoplasia Type 1 (MEN1) 1,10,11,15,32,36,65 . (A) General flowchart, (B) parathyroid gland management, (C) duodenopancreatic neuroendocrine tumor (dpNET) management, and (D) pituitary adenoma (PA) management are illustrated.…”

Section: Specific Case Considerationsmentioning

confidence: 99%

“…We must reject the sophistry of manageable toxicity and think carefully about the iatrogenic burdens we place on patients, especially those for whom we have the privilege to offer longitudinal care in the setting of germline tumor syndromes like MEN1. 1,10,11,15,32,36,65 (A) General flowchart, (B) parathyroid gland management, (C) duodenopancreatic neuroendocrine tumor (dpNET) management, and (D) pituitary adenoma (PA) management are illustrated. CgA indicates chromogranin A; CT, computed tomography; DXA, dual energy x-ray absorptiometry; EGD, esophagogastroduodenoscopy; EUS, endoscopic ultrasound; IGF-1, insulin-like growth factor-1; iPTH, intact parathyroid hormone; MRI, magnetic resonance imaging; NET, neuroendocrine tumor; PHPT, primary hyperparathyroidism; PP, pancreatic polypeptide; PPI, proton pump inhibitor; VIP, vasoactive intestinal peptide.…”

Section: Patient Perspectivementioning

confidence: 99%

“…Figure 4 outlines the basic considerations when managing a patient suspected to have MEN1. 1,10,11,15,32,36,65…”

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confidence: 99%

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

Shirali

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Lewis

et al. 2021

CA A Cancer J Clinicians

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Besonderheiten in Diagnostik und Therapie des hereditären primären Hyperparathyreoidismus

Mogl

Goretzki

2023

Chirurgie

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Understanding the clinical course of genotype-negative MEN1 patients can inform management strategies

Cited by 20 publications

References 18 publications

Multiple Endocrine Neoplasia Type 1 Involving Both Liver and Lung: A Case Report

Multiple Endocrine Neoplasia Type 1 Involving Both Liver and Lung: A Case Report

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

Besonderheiten in Diagnostik und Therapie des hereditären primären Hyperparathyreoidismus

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