2,773 results match your criteria Wermer Syndrome MEN Type 1


Less Than Subtotal Parathyroidectomy for Multiple Endocrine Neoplasia Type 1 Primary Hyperparathyroidism: A Systematic Review and Meta-Analysis.

World J Surg 2022 Jun 29. Epub 2022 Jun 29.

Digestive and Endocrine Surgery Department, Magellan Center, Bordeaux University Hospital, University of Bordeaux, Bordeaux, France.

Background: Multiple endocrine neoplasia type 1 (MEN1)-associated primary hyperparathyroidism (pHPT) is classically associated with an asymmetric and asynchronous parathyroid involvement. Subtotal parathyroidectomy (STP), which is currently the recommended surgical treatment, carries a high risk of permanent hypoparathyroidism. The results of less than subtotal parathyroidectomy (LSTP) are conflicting, and its place in this setting is still a matter of debate. Read More

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Comparison Between Sporadic and Multiple Endocrine Neoplasia Type 1-Associated Insulinoma.

J Am Coll Surg 2022 Jun 24. Epub 2022 Jun 24.

Department of Surgery, Mayo Clinic, Rochester, MN.

Background: The differences between sporadic and MEN-1 associated insulinoma are not well described. Herein, we compared demographics, neoplasm characteristics, presentation, and survival in patients with sporadic vs MEN-1 insulinomas including benign and malignant disease.

Methods: A retrospective study identified insulinoma patients. Read More

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Recent advances in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms.

World J Gastrointest Surg 2022 May;14(5):383-396

Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, 18057 Rostock, Germany.

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are a rare group of tumors originating from neuroendocrine cells of the digestive system. Their incidence has increased over the last decades. The specific pathogenetic mechanisms underlying GEP-NEN development have not been completely revealed. Read More

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Clinical Features of Pancreatic Neuroendocrine Microadenoma: A Single-Center Experience and Literature Review.

Pancreas 2022 Jun 15. Epub 2022 Jun 15.

From the Department of Gastroenterological Surgery II, Hokkaido University Faculty of Medicine.

Objectives: Pancreatic neuroendocrine microadenoma (NEMA) is a nonfunctioning neuroendocrine tumor of less than 5 mm. Most studies of NEMA were based on autopsies, and few reports have revealed the clinical frequency of NEMA. We investigated the clinicopathological features of NEMA. Read More

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Analysis of 55 patients with multiple endocrine neoplasia type 1-associated insulinoma from a single center in China.

Orphanet J Rare Dis 2022 06 13;17(1):219. Epub 2022 Jun 13.

Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, 100730, China.

Objective: To investigate the clinical characteristics of patients with multiple endocrine neoplasia type 1 (MEN1)-related insulinoma and their relationship with specific biochemical changes and to summarize the features of treatment options for the Chinese population with this disease and the impact on long-term prognosis.

Methods: "MEN1" and "insulinoma" were used when searching the Peking Union Medical College Hospital (PUMCH) medical record retrieval system to obtain clinical information about patients. We identified patients diagnosed with MEN1-associated insulinoma based on endocrinological, radiological, and pathological examinations, and subsequently analyzed their clinical data. Read More

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Thymic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1.

Endocrine 2022 Jun 13. Epub 2022 Jun 13.

Medical Research Center Oulu, Oulu University Hospital, Research Unit of Internal Medicine, University of Oulu, Oulu, Finland.

Objective: MEN1 is associated with an increased risk of developing tumors in different endocrine organs. Neuroendocrine tumors of the thymus (TNETs) are very rare but often have an aggressive nature. We evaluated patients with MEN1 and TNET in three university hospitals in Finland. Read More

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Dichotomous Roles of in Macrophages and Fibroblasts in Bleomycin-Induced Pulmonary Fibrosis.

Int J Mol Sci 2022 May 11;23(10). Epub 2022 May 11.

School of Pharmaceutical Sciences, Jilin University, Changchun 130021, China.

Pulmonary fibrosis therapy is limited by the unclear mechanism of its pathogenesis. C57BL/6 mice were used to construct the pulmonary fibrosis model in this study. The results showed that , which encodes menin protein, was significantly downregulated in bleomycin (BLM)-induced pulmonary fibrosis. Read More

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Multiple endocrine neoplasia type 1 with ectopic parathyroid adenoma.

Arch Med Sci 2022 14;18(3):829-832. Epub 2022 Apr 14.

Department of Pathology, the Second Xiangya Hospital, Central South University, Changsha, Hunan, China.

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Impact of the parathyroidectomy on quality of life in multiple endocrine neoplasia type 1.

Endocr Connect 2022 May 1. Epub 2022 May 1.

D Lourenço Junior, Endocrine Genetics Unit (LIM-25), Endocrinology Division (Hospital das Clinicas) and Endocrine Oncology Division, Institute of Cancer of the State of São Paulo (ICESP), Universidade de São Paulo Hospital das Clínicas, Sao Paulo, Brazil.

Background: Potential influences of the parathyroidectomy (PTx) on quality of life (QoL) in multiple endocrine neoplasia type 1-related primary hyperparathyroidism (HPT/MEN1) are unknown.

Method: Short Form 36 Health Survey Questionnaire was prospectively applied in 30 HPT/MEN1 patients submitted to PTx (20, subtotal; 10, total with autograft), before, 6 and 12 months (mo.) after surgery. Read More

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Oral health aspects in sporadic and familial primary hyperparathyroidism.

J Clin Exp Dent 2022 May 1;14(5):e396-e403. Epub 2022 May 1.

Department of Physiology and Pharmacology, Drug Research and Development Center (NPDM), Faculty of Medicine, Federal University of Ceará, Fortaleza, Brazil.

Background: Primary hyperparathyroidism (pHPT) is the third most common endocrinopathy, affecting 1-3% of postmenopausal women, with a total incidence of 21.6 cases per 100,000 people in the adult population. This study aimed to analyze the oral health and related aspects of individuals with pHPT. Read More

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Clinical features and postoperative survival in patients with sporadic versus multiple endocrine neoplasia type 1-related pancreatic neuroendocrine tumors: An international cohort study.

Surgery 2022 May 13. Epub 2022 May 13.

Department of Surgery, Stanford University, CA. Electronic address:

Background: The optimal surgical management of pancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1 is controversial. This study sought to compare clinicopathologic characteristics and outcomes of multiple endocrine neoplasia type 1-associated and sporadic pancreatic neuroendocrine tumors from a large multi-national database.

Methods: A multi-institutional, international database of patients with surgically resected pancreatic neuroendocrine tumors was analyzed. Read More

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[Ga] Ga-DOTA-TOC PET/CT uptake by parathyroid adenoma in the context of multiple endocrine neoplasia type 1 (MEN1).

Rev Esp Med Nucl Imagen Mol (Engl Ed) 2022 May 9. Epub 2022 May 9.

Servicio de Medicina Nuclear, Hospital Universitario y Politécnico La Fe, Valencia, Spain.

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

World J Surg Oncol 2022 May 10;20(1):151. Epub 2022 May 10.

Shengli Clinical Medical College of Fujian Medical University, Fuzhou, China.

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. Read More

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Multiple endocrine neoplasia type 1 caused by mosaic mutation: clinical follow-up and genetic counseling?

Eur J Endocrinol 2022 May 24;187(1):K1-K6. Epub 2022 May 24.

Aix Marseille Univ, APHM, INSERM, MMG, Laboratory of Molecular Biology Hospital La Conception, Marseille, France.

MEN1 is an autosomal dominant hereditary syndrome characterized by several endocrine tumors, in most cases affecting the parathyroid glands, pancreas, and anterior pituitary. It is the result of inactivating mutations in the tumor suppressor gene MEN1. More than 1300 different mutations have been identified in this gene. Read More

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Convergence between germline and somatic mutations in pancreatic neuroendocrine tumors.

Eur J Endocrinol 2022 May 19;187(1):85-90. Epub 2022 May 19.

Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.

Objectives: The pancreatic neuroendocrine tumors (PanNETs) are a group of clinically heterogeneous neoplasms. Although previous studies illustrated the somatic mutation pattern for PanNETs, the germline mutation pattern is still unclear. Here, we comprehensively screened the underlying germline mutations in a cohort of multiple endocrine neoplasia type 1 (MEN1)-related and sporadic PanNETs to reveal the characteristics of germline mutation in PanNET patients. Read More

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Genetic Deletion of Menin in Mouse Mesenchymal Stem Cells: An Experimental and Computational Analysis.

JBMR Plus 2022 May 7;6(5):e10622. Epub 2022 Apr 7.

Department of Medicine McGill University and McGill University Health Centre Montreal Canada.

Loss-of-function mutations in the tumor-suppressor gene cause the multiple endocrine neoplasia type 1 syndrome. Menin, the gene product, is expressed in many tissues, including bone, where its function remains elusive. We conditionally inactivated menin in mesenchymal stem cells (MSCs) using paired-related homeobox 1 (Prx1)-Cre and compared resultant skeletal phenotypes of ; menin-knockout mice (KO) and wild-type controls using in vivo and in vitro experimental approaches and mechanics simulation. Read More

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Single-incision port robot-assisted surgery for thymic carcinoid tumor resection.

J Cardiothorac Surg 2022 May 3;17(1):90. Epub 2022 May 3.

Department of Thoracic Surgery, School of Medicine, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Background: Multiple endocrine neoplasia (MEN) is divided into MEN type 1 (MEN-1) and MEN type 2 (MEN-2). MEN-1 may be associated with thymic carcinoid tumors. We present a case of the surgical removal of a thymic carcinoid associated with MEN-1 via a single-incision port RATS. Read More

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The Research Landscape of Multiple Endocrine Neoplasia Type 1 (2000-2021): A Bibliometric Analysis.

Front Med (Lausanne) 2022 8;9:832662. Epub 2022 Apr 8.

Department of General Surgery, Xiangya Hospital of Central South University, Changsha, China.

Introduction: This study aimed to investigate the landscape of Multiple Endocrine Neoplasia Type 1 research during the last 22 years using machine learning and text analysis.

Method: In December 2021, all publications indexed under the MeSH term "Multiple Endocrine Neoplasia Type 1" were obtained from PubMed. The whole set of search results was downloaded in XML format, and metadata such as title, abstract, keywords, mesh words, and year of publication were extracted from the original XML files for bibliometric evaluation. Read More

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Pancreaticoduodenectomy Is the Best Surgical Procedure for Zollinger-Ellison Syndrome Associated with Multiple Endocrine Neoplasia Type 1.

Cancers (Basel) 2022 Apr 11;14(8). Epub 2022 Apr 11.

Department of Surgery, Philipps-University, 35041 Marburg, Germany.

Aim: The aim of this research was to evaluate the long-term outcome of pancreaticoduodenectomy (PD) versus other duodenopancreatic resections (non-PD) for the surgical treatment of the Zollinger-Ellison syndrome (ZES) in patients with multiple endocrine neoplasia type 1 (MEN1).

Methods: Prospectively recorded patients with biochemically confirmed MEN1-ZES who underwent duodenopancreatic surgery were retrospectively analyzed in terms of clinical characteristics, complications, cure rate, and long-term morbidity, including quality of life assessment (EORTC QLQ-C30).

Results: 35 patients (16 female, 19 male) with MEN1-ZES due to duodenopancreatic gastrinomas with a median age of 42 (range 30-74) years were included. Read More

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Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience.

J Clin Med 2022 Apr 1;11(7). Epub 2022 Apr 1.

Endocrinology Unit, First Department of Propaedeutic and Internal Medicine, Centre of Expertise for Rare Endocrine Disease (C.E.R.E.D.), Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece.

Background: Primary hyperparathyroidism (PHPT) in the most common and earliest manifestation of multiple endocrine neoplasia type-1 (MEN1). Epidemiological data have been reported in MEN1 patients but data on long-term follow-up focusing on PHPT are scarce.

Methods: In this retrospective cohort study, we included patients diagnosed with MEN1-related PHPT that were under regular follow-up in our institution. Read More

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[Comparison of clinical features between sporadic pancreatic neuroendocrine tumors and those associated with multiple endocrine neoplasia type 1].

Zhonghua Yi Xue Za Zhi 2022 Apr;102(14):1014-1019

Department of Geriatric Gastroenterology,the First Affiliated Hospital of Nanjing Medical University, Nanjing 210000, China.

To compare the clinical features of multiple endocrine adenoma type 1 (MEN-1) associated pancreatic neuroendocrine neoplasms (pNENs) as well as sporadic pNENs. The clinical data of 28 sporadic pNENs patients and 10 MEN-1-related pNENs patients admitted to the First Affiliated Hospital of Nanjing Medical University from January 2010 to June 2021 were collected. Meanwhile, by searching PubMed database and reviewing the clinical data of 20 foreign patients with MEN-1-related pNENs which were reported at the same time. Read More

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Pancreatic Carcinoid Tumor in a Pediatric Patient.

Am J Case Rep 2022 Apr 8;23:e936029. Epub 2022 Apr 8.

Department of Surgery, King Abdulaziz Medical City, Jeddah, Saudi Arabia.

BACKGROUND Carcinoid tumors are well-differentiated tumors that develop from neuroendocrine cells. They are rare tumors and occur most commonly in the gastrointestinal tract, followed by the pulmonary system. They usually present with abdominal pain or cough or persistent pneumonia. Read More

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A MEN1 Patient Presenting With Multiple Parathyroid Adenomas and Transient Hypercortisolism: A Case Report and Literature Review.

Front Endocrinol (Lausanne) 2022 8;13:802453. Epub 2022 Mar 8.

Division of Endocrinology, Tongji Hospital, Huazhong University of Science & Technology, Wuhan, China.

Background: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary endocrine syndrome caused by mutations in MEN1 tumor suppressor gene.

Case Presentation: A 53-year-old Chinese female was admitted to Division of Endocrinology, Tongji Hospital, for hypercalcemic crisis. Increased level of parathyroid hormone (PTH) was confirmed by laboratory tests, and imaging examination showed multiple parathyroid adenomas. Read More

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Risk factors for complications after surgery for pancreatic neuroendocrine tumors.

Surgery 2022 Jul 24;172(1):127-136. Epub 2022 Mar 24.

Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Surgery, Regional Academic Cancer Center Utrecht, University Medical Center Utrecht and St. Antonius Hospital Nieuwegein, Utrecht, The Netherlands. Electronic address:

Background: Surgical resection is the only potentially curative treatment for pancreatic neuroendocrine tumors. The choice for the type of procedure is influenced by the expected oncological benefit and the anticipated risk of procedure-specific complications. Few studies have focused on complications in these patients. Read More

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Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database.

Endocr Connect 2022 May 11;11(5). Epub 2022 May 11.

F.I.R.M.O. Italian Foundation for the Research on Bone Diseases, Florence, Italy.

Multiple endocrine neoplasia type 1 (MEN1) is a rare, inherited cancer syndrome characterized by the development of multiple endocrine and non-endocrine tumors. MEN1 patients show a reduction of bone mass and a higher prevalence of early onset osteoporosis, compared to healthy population of the same age, gender, and ethnicity. During the monitoring and follow-up of MEN1 patients, the attention of clinicians is primarily focused on the diagnosis and therapy of tumors, while the assessment of bone health and mineral metabolism is, in many cases, marginally considered. Read More

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Update on the clinical management of multiple endocrine neoplasia type 1.

Clin Endocrinol (Oxf) 2022 Mar 23. Epub 2022 Mar 23.

Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.

This review provides an overview of novel insights in the clinical management of patients with Multiple Endocrine Neoplasia Type 1, focusing on the last decade since the last update of the MEN1 guidelines. With regard to Diagnosis: Mutation-negative patients with 2/3 main manifestations have a different clinical course compared to mutation-positive patients. As for primary hyperparathyroidism: subtotal parathyroidectomy is the initial procedure of choice. Read More

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Adrenocortical carcinoma: Pediatric aspects (Review).

Exp Ther Med 2022 Apr 16;23(4):287. Epub 2022 Feb 16.

Department of Endocrinology, 'Carol Davila' University of Medicine and Pharmacy, 050474 Bucharest, Romania.

Adrenocortical carcinoma (adrenal cortex-derived cancer), an orphan malignancy, is a very aggressive disease that affects both adults and children with an annual incidence of 1-2 adult and 0.2-0.38 pediatric cases/million (in the pediatric population it represents 0. Read More

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Role of 68Ga-DOTATOC PET/CT in Insulinoma According to 3 Different Contexts: A Retrospective Study.

Clin Nucl Med 2022 May;47(5):394-401

From the Service de Médecine Nucléaire, Hôpital Tenon AP-HP and Sorbonne University.

Objective: The aim of this study was to assess the performance of 68Ga-DOTATOC PET/CT in the detection and extension of insulinomas according to 3 different contexts: sporadic benign, sporadic metastatic, and multiple endocrine neoplasia type 1 (MEN1).

Patients And Methods: The data of 71 adult patients who underwent 68Ga-DOTATOC PET/CT for suspected or confirmed sporadic insulinoma, suspicion of insulinoma in the context of MEN1, follow-up of metastatic insulinoma, or suspicion of recurrence of insulinoma were retrospectively analyzed. Pathological examination or strong clinical and biological findings were used as standards of truth. Read More

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Occult insulinoma with treatment refractory, severe hypoglycaemia in multiple endocrine neoplasia type 1 syndrome; difficulties faced during diagnosis, localization and management; a case report.

BMC Endocr Disord 2022 Mar 16;22(1):68. Epub 2022 Mar 16.

Registrar in General Medicine, National Hospital of Sri Lanka, Ward 45/46, Colombo, Sri Lanka.

Background: Multiple endocrine neoplasia type 1 (MEN 1) syndrome is a rare, complex genetic disorder characterized by increased predisposition to tumorigenesis in multiple endocrine and non-endocrine tissues. Diagnosis and management of MEN 1 syndrome is challenging due to its vast heterogeneity in clinical presentation.

Case Presentation: A 23-year-old female, previously diagnosed with Polycystic Ovarian Syndrome (PCOS) and pituitary microprolactinoma presented with drowsiness,confusion and profuse sweating developing over a period of one day. Read More

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