In order to provide the best patient care health professionals should invest in educational opportunities that give them up to date knowledge and skills. In this resource section, you can find directories with various educational resources, self-training modules, information about advanced degree courses, research and fellowship opportunities, and links to various conferences and meetings around the world. Each of these tools for continuing education has its own advantages and disadvantages. For example while online tools provide the benefits of a self-paced environment for learning, conferences allow discussion and generation of new ideas.
The World Health Organizations Human Genetics encourages health professionals to use these resources for personal and professional growth. We aim to support an educated and informed health workforce to provide the best health services to populations worldwide and to achieve the best possible health for all. Higher educational resources Distance learning Online self-training tools Advanced degree courses Continuing education for health professionals Continuing education for nurses Research and fellowship opportunities Conferences and meetings.
Studies have shown that anterior pituitary tumors in MEN1 patients are more aggressive and less likely to respond to medical management; thus requiring surgical management more often. Radiation treatment is used for patients with the residual unresectable tumor. Surgical management is done in patients with large pituitary tumors with compression of optic nerve or tumors more than 1 cm or tumors which do not improve with medical management.
Common sites of involvement are the gastrointestinal GI tract, bronchi, pancreas, and thymus. Somatostatin analogs such as octreotide or lanreotide have been shown to cause regression of gastric carcinoid. Thymic carcinoids are aggressive in MEN1 patients. Goudet et al. Screening for these patients is done with radiologic imaging like CT scan and MRI every 1 to 2 years.
Surgical removal is the treatment of choice. Thymic carcinoid has been seen even in patients who underwent prophylactic thymectomy; therefore, surveillance with imaging is required. Radiotherapy and chemotherapy can be used in patients with an unresectable tumor or metastatic disease. Compared to adrenal incidentalomas, there is more likelihood of hyperaldosteronism and adrenocortical cancer but less likelihood of pheochromocytoma in MEN1 patients.
There is no consensus about management of nonfunctioning adrenal tumors in MEN1 patients, but because of an increased risk of adrenocortical cancer, surgery is recommended if the tumor size is larger than 4 cm or tumor size 1 to 4 cm if there is a significant measurable growth over 6 months with suspicious radiological features.
LeBodic et al. Breast cancer is more common in female MEN1 patients and occurs at an early age. Rachel et al. The prognosis of the MEN1 patient is variable. Tumors associated with MEN1 are more aggressive and challenging to treat. Neuroendocrine tumors can metastasize quickly and can cause premature death. Studies have shown an increased risk of premature death and decreased average life expectancy in MEN1 patients. Parathyroid tumors can cause metabolic bone disease and kidney stones.
Tumors of gastro-entero-pancreatic tissue can cause complication by excessive secretion of gastrin, insulin, glucagon, or vasoactive intestinal peptide or by metastatic disease. Patient education is vital. A genetic counselor should also encourage the rest of the family to get genetic testing done. Complete knowledge of the disease can help the patient to make an appropriate and informed decision. Because of the rarity of the MEN1 syndrome and the complexity of the disease, it is difficult to manage these patients.
Majority of recommendation regarding management of MEN1 patients lack a high level of evidence suggesting the need for more research in this field. Al-Salameh et al. MEN1 is a complex syndrome that increases morbidity and mortality by causing multiple aggressive tumors. These patients should be managed in a tertiary care center by an interprofessional team including an endocrinologist, endocrine surgeon, gastroenterologist, neurosurgeon, genetic counselor, nurse, pharmacist, and patient navigator or advocate for a better outcome.
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Phillip Hynes. Tehmina Warsi. Introduction Multiple endocrine neoplasia type 1 MEN1 is a rare endocrine tumor syndrome with high penetrance. It is also known as Wermer syndrome.
Multiple Endocrine Neoplasia Type 1 (MEN I, Wermer Syndrome)
History and Physical A comprehensive history should be obtained during an initial visit as MEN1 can affect multiple organs. Evaluation Evaluation of hyperparathyroidism involves measurement of serum calcium and PTH. Prognosis The prognosis of the MEN1 patient is variable.
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Complications The complications in MEN1 can occur because of the disease itself or due to surgery. Pituitary tumors can cause complications like pituitary insufficiency and visual field defects. Deterrence and Patient Education Patient education is vital. Pearls and Other Issues Because of the rarity of the MEN1 syndrome and the complexity of the disease, it is difficult to manage these patients. Enhancing Healthcare Team Outcomes MEN1 is a complex syndrome that increases morbidity and mortality by causing multiple aggressive tumors. See References. Interested in Participating? Take a quiz of the questions on this article.
Take Quiz Which is not part of multiple endocrine neoplasia 1? Click Your Answer Below. Parathyroid adenoma. Pituitary adenoma. Would you like to access teaching points and more information on this topic? A patient complains of nausea without vomiting, constipation, weight loss, fatigue, malaise, decreased libido, and depression. Physical exam is normal except for guaiac-positive stool.
Laboratories include a calcium CT scan shows a 3 by 3 cm mass in the head of the pancreas. Select the most likely diagnosis.