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Background: Malignant thymic carcinoids are uncommon tumours among patients with multiple endocrine neoplasia type 1 (MEN-1). Current guidelines advise performance of a preventative, routine transcervical thymectomy (TCT) during parathyroidectomy, although data on the yield is scarce. In this review, we aimed to revisit available literature to investigate and summarize the efficacy of routine TCT for the prevention of thymic carcinoid tumours among MEN-1 patients after searching four databases (PubMed, Embase, Medline, and Cochrane Library). Summary: Seven eligible studies were identified: retrospective observational studies: 3 and case reports describing one or more patients: 4. A total of 122 patients were included, 56 males (45.9%) and a pooled mean age of 40 years (±10). All underwent a routine TCT as part of parathyroidectomy; no details on the extent of TCT were available. Overall, only one (0.9%) incidental carcinoid was found. However, although all other patients underwent prophylactic TCT at the time of parathyroidectomy, an additional nine (7.4%) developed a thymic carcinoid during follow-up, after a median time of 36 months (range: 8–226). Key Messages: There is currently not enough evidence to support the efficacy of a routine TCT to prevent the development of thymic carcinoid among MEN-1 patients. As this is a rare but potentially highly aggressive tumour, attention should be shifted towards improving follow-up programs and developing specific imaging-screening protocols. This enables early detection of thymic carcinoids in a timely manner and improves outcomes, even after performance of a routine TCT. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to evaluate the efficacy of conservative treatment for nonfunctioning pancreatic neuroendocrine neoplasms (NF-PNEN) ⩽ 2 cm in multiple endocrine neoplasia type 1 (MEN1)-affected patients compared with surgical treatment. Methods: The databases of 4 tertiary referral institutions (San Raffaele Scientific Institute, Milan; Philipps-Universität Marburg, Marburg; University of Padua, Padua; Royal Free Hospital, London) were analyzed. A comparison of conservative management and surgery at initial diagnosis of NF-PNEN ⩽ 2 cm between 1997 and 2013 was performed. Results: Overall, 27 patients (45%) underwent up-front surgery and 33 patients (55%) were followed up after the initial diagnosis. A higher proportion of patients in the surgery group were female (70 vs. 33%, p =0.004). Patients were mainly operated on in the period 1997-2007 as compared with the period 2008-2013 (n = 17; 63 vs. 37%; p = 0.040). The rate of multifocal tumors was higher in the surgery group (n = 24; 89%) than in the 'no surgery' group (n = 22; 67%; p = 0.043). After a median follow-up of 126 months, 1 patient deceased due to postoperative complications within 30 days after surgery. The 5-, 10-, and 15- year progression-free survival (PFS) rates were 63, 39, and 10%, respectively. The median PFS was similar in the two groups. Overall, 13 patients (32.5%) were operated on after initial surgical or conservative treatment. The majority of the surgically treated patients had stage 1 (77.5%), T1 (77.5%), and G1 (85%) tumors. Conclusions: NF-PNEN ⩽ 2 cm in MEN1 patients are indolent neoplasms posing a low oncological risk. Surgical treatment of these tumors at initial diagnosis is rarely justified in favor of conservative treatment. [ABSTRACT FROM AUTHOR]

Pituitary adenomas are a common feature of a subset of endocrine neoplasia syndromes, which have otherwise highly variable disease manifestations. We provide here a review of the clinical features and human molecular genetics of multiple endocrine neoplasia (MEN) type 1 and 4 (MEN1 and MEN4, respectively) and Carney complex (CNC). MEN1, MEN4, and CNC are hereditary autosomal dominant syndromes that can present with pituitary adenomas. MEN1 is caused by inactivating mutations in the MEN1 gene, whose product menin is involved in multiple intracellular pathways contributing to transcriptional control and cell proliferation. MEN1 clinical features include primary hyperparathyroidism, pancreatic neuroendocrine tumours and prolactinomas as well as other pituitary adenomas. A subset of patients with pituitary adenomas and other MEN1 features have mutations in the CDKN1B gene; their disease has been called MEN4. Inactivating mutations in the type 1α regulatory subunit of protein kinase A (PKA; the PRKAR1A gene), that lead to dysregulation and activation of the PKA pathway, are the main genetic cause of CNC, which is clinically characterised by primary pigmented nodular adrenocortical disease, spotty skin pigmentation (lentigines), cardiac and other myxomas and acromegaly due to somatotropinomas or somatotrope hyperplasia. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Neuroendocrine tumors are uncommon tumors that are histopathologically and biologically heterogeneous and include the multiple endocrine neoplasia (MEN) 1 and 2 syndromes. The morbidity of MEN-1 and MEN-2 is often due to the symptomatology of the endocrine hormones produced, and the mortality mainly occurs from hepatic dysfunction incurred by liver metastases. At present, there is essentially no effective cure once the tumor has metastasized to the liver. Patient: We present a patient with progressive, metastatic MEN-1 with the classic '3 P's' triad of neuroendocrine tumor of the pancreas, parathyroid adenoma and a pituitary adenoma. Results: After progression on high-dose Sandostatin LAR (60 mg/month) and multiple surgeries, the patient had a partial response (40% decrease) to a novel regimen of capecitabine and temozolomide (CAPTEM) and progression-free survival of 18 months. He had minor grade 1 toxicities and no grade 2, 3 or 4 toxicities. Discussion: The history and treatment options for MEN-1/2 cancers are reviewed, as well as the data behind our novel regimen, CAPTEM. Conclusion: The CAPTEM regimen is a tolerable, safe, easy to administer oral regimen with possible efficacy for MEN-1 tumors. Copyright © 2012 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]