دورية أكاديمية
Multiple endocrine neoplasia type 1 with Zollinger-Ellison syndrome: clinicopathological analysis of a Japanese family with focus on menin immunohistochemistry.
| العنوان: | Multiple endocrine neoplasia type 1 with Zollinger-Ellison syndrome: clinicopathological analysis of a Japanese family with focus on menin immunohistochemistry. |
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| المؤلفون: | Kimura N; Department of Clinical Research, National Hospital Organization Hakodate Hospital, Hakodate, Hokkaido, Japan.; Department of Diagnostic Pathology, National Hospital Organization Hakodate Hospital, Hakodate, Hokkaido, Japan., Hirata Y; Department of Hematology and Oncology, National Hospital Organization Hakodate Hospital, Hakodate, Hokkaido, Japan., Iwashiro N; Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate, Hokkaido, Japan., Kijima H; Department of Pathology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori, Japan., Takayasu S; Department of Endocrinology and Metabolism, Hirosaki University Graduate School of Medicine and Hospital, Hirosaki, Aomori, Japan., Yamagata S; Department of Endocrinology and Metabolism, Hirosaki University Graduate School of Medicine and Hospital, Hirosaki, Aomori, Japan.; Institute of Human Nutrition, Columbia University Irving Medical Center, New York, NY, United States., Sakihara S; Department of Endocrinology and Metabolism, Hirosaki University Graduate School of Medicine and Hospital, Hirosaki, Aomori, Japan.; Division of Diabetes and Endocrinology, Aomori Rosai Hospital, Aomori, Japan., Uchino S; Department of Endocrine Surgery, Noguchi Thyroid Clinic and Hospital Foundation, Beppu, Oita, Japan., Ohara M; Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate, Hokkaido, Japan. |
| المصدر: | Frontiers in endocrinology [Front Endocrinol (Lausanne)] 2023 Oct 06; Vol. 14, pp. 1221514. Date of Electronic Publication: 2023 Oct 06 (Print Publication: 2023). |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Frontiers Research Foundation] Country of Publication: Switzerland NLM ID: 101555782 Publication Model: eCollection Cited Medium: Print ISSN: 1664-2392 (Print) Linking ISSN: 16642392 NLM ISO Abbreviation: Front Endocrinol (Lausanne) Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: [Lausanne : Frontiers Research Foundation] |
| مواضيع طبية MeSH: | Multiple Endocrine Neoplasia Type 1*/genetics , Multiple Endocrine Neoplasia Type 1*/surgery , Multiple Endocrine Neoplasia Type 1*/diagnosis , Neuroendocrine Tumors* , Zollinger-Ellison Syndrome*/diagnosis , Zollinger-Ellison Syndrome*/genetics , Zollinger-Ellison Syndrome*/pathology, Humans ; East Asian People ; Immunohistochemistry ; Transcription Factors |
| مستخلص: | Background: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of multiple epithelial neuroendocrine tumors (NETs) and non-NETs in various organs. MEN1 encodes a 610-amino acid-long tumor suppressor protein, menin. The optimal treatment for multiple tumors, identification of the most critical tumors for patient prognosis, and menin immunohistochemistry findings remain controversial. Therefore, we aimed to elucidate these issues through a histological analysis of tumors and tumor-like lesions in a Japanese family, comprising a father and his two sons, who had MEN1 with Zollinger-Ellison syndrome (ZES). Patients and Methods: All family members had a germline alteration in exon 10, c.1714-1715 del TC of MEN1 , and exhibited multiple synchronous and metachronous tumors. The patients had pulmonary NETs, hyperparathyroidism, hypergastrinemia, pituitary adenomas, pancreaticoduodenal NETs, adrenocortical adenoma with myelolipoma, nodular goiter of the thyroid, lipomas, and angiofibroma. Most tumors were resected and histologically examined. We compared their clinical courses and tumor histology, and conducted menin immunohistochemistry (IHC). Results: Two patients died of pulmonary NET G2. One patient who underwent pancreaticoduodenectomy was cured of ZES; however, the two other patients who did not undergo pancreaticoduodenectomy suffered persistent ZES despite treatment with octreotide. Menin IHC revealed varying NET intensities, ranging from positive to negative stains. Conclusion: Pancreaticoduodenectomy is the most effective treatment for ZES. Long-term follow-up is essential for pulmonary NET G2 owing to the risk of distant metastasis and/or multiplicity. Moreover, the variability of menin IHC in MEN1-related tumors may indicate the pattern of tumor formation rather than the diagnostic utility of menin in MEN1. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2023 Kimura, Hirata, Iwashiro, Kijima, Takayasu, Yamagata, Sakihara, Uchino and Ohara.) |
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| فهرسة مساهمة: | Keywords: Zollinger-Ellison syndrome; immunohistochemistry; menin; metastasis; multiple endocrine neoplasia type 1; neuroendocrine tumor; prognosis; pulmonary atypical carcinoid |
| المشرفين على المادة: | 0 (Transcription Factors) 0 (MEN1 protein, human) |
| تواريخ الأحداث: | Date Created: 20231023 Date Completed: 20231206 Latest Revision: 20231206 |
| رمز التحديث: | 20231215 |
| مُعرف محوري في PubMed: | PMC10588651 |
| DOI: | 10.3389/fendo.2023.1221514 |
| PMID: | 37867522 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 1664-2392 |
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| DOI: | 10.3389/fendo.2023.1221514 |