Pituitary adenomas in the setting of multiple endocrine neoplasia type 1: a single-institution experience
| العنوان: | Pituitary adenomas in the setting of multiple endocrine neoplasia type 1: a single-institution experience |
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| المؤلفون: | Lydia P. Wheeler, Jamie J. Van Gompel, Irina Bancos, John L.D. Atkinson, Michael W. Ruff, Naykky Singh Ospina, Benjamin T. Himes, Fredric B. Meyer, William F. Young, Desmond A. Brown, Brittny Major, Salomon Cohen-Cohen |
| المصدر: | Journal of Neurosurgery. :1-7 |
| بيانات النشر: | Journal of Neurosurgery Publishing Group (JNSPG), 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Adenoma, Adult, Male, medicine.medical_specialty, Adolescent, Databases, Factual, endocrine system diseases, medicine.medical_treatment, Carcinoid tumors, Asymptomatic, Gastroenterology, Neurosurgical Procedures, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Sphenoid Bone, Multiple Endocrine Neoplasia Type 1, Humans, Medicine, Pituitary Neoplasms, Prolactinoma, MEN1, Child, Multiple endocrine neoplasia, Early Detection of Cancer, Aged, Retrospective Studies, Aged, 80 and over, Transsphenoidal surgery, business.industry, Pituitary tumors, Infant, General Medicine, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Parathyroid Neoplasms, Treatment Outcome, medicine.anatomical_structure, Child, Preschool, 030220 oncology & carcinogenesis, Endocrine neoplasm, Disease Progression, Female, Parathyroid gland, medicine.symptom, business, 030217 neurology & neurosurgery |
| الوصف: | OBJECTIVEMultiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal-dominant tumor disorder characterized by the development of pituitary tumors and other endocrine neoplasms. Diagnosis is made clinically based on the development of 2 or more canonical lesions (parathyroid gland, anterior pituitary, and enteropancreatic tumors) or in family members of a patient with a clinical diagnosis of MEN1 and the occurrence of one of the MEN1-associated tumors. The goal of this study was to characterize pituitary tumors arising in the setting of MEN1 at a single institution. The probability of tumor progression and the likelihood of surgical intervention in patients with asymptomatic nonfunctional pituitary adenomas were also analyzed.METHODSA retrospective review of a prospectively maintained institutional database was performed for patients with MEN1 diagnosed from 1970 to 2017. Data included patient demographics, tumor characteristics, treatment strategies, and outcomes.RESULTSA review of the database identified 268 patients diagnosed with MEN1, of whom 158 (59%) were female. Among the 268 patients, 139 (51.8%) had pituitary adenomas. There was a higher prevalence in women than in men (65% vs 35%, p < 0.005). Functional adenomas (57%) were more common. Prolactin-secreting adenomas were the most common functional tumors. Macroadenomas were seen in 27% of patients and were more likely to be symptomatic and locally aggressive (p < 0.001). Forty-nine patients (35%) underwent transsphenoidal resection at some point during their disease course. In 52 patients who were initially observed with MEN1 asymptomatic nonfunctional adenomas, only 5 (10%) progressed to need surgery. In MEN1 patients, an initial parathyroid lesion is most likely followed in order by pituitary, pancreatic, adrenal, and, finally, rare carcinoid tumors.CONCLUSIONSAsymptomatic nonfunctional pituitary adenomas in patients with MEN1 may be followed safely with MRI. In this series, parathyroid tumors developed at the lowest median age of all cardinal tumors, and development of additional cardinal MEN1 lesions followed a predictable pattern. This pattern of disease progression could have significant implications for disease surveillance in clinical practice and may help to target clinical resources to the lesions most likely to develop next. This may aid with early detection and treatment and warrants further study. |
| تدمد: | 1933-0693 0022-3085 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a7f905681e938d9a0e6db1bc0a301344Test https://doi.org/10.3171/2020.1.jns193538Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....a7f905681e938d9a0e6db1bc0a301344 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 19330693 00223085 |
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