Clinical practice guidelines for patients with multiple endocrine neoplasia type 1 (MEN1) recommend a variety of surveillance options. Given progress over the past decade in this area, it is timely to evaluate their ongoing utility. MEN1 is characterized by the development of synchronous or asynchronous tumors affecting a multitude of endocrine and nonendocrine tissues, resulting in premature morbidity and mortality, such that the rationale for undertaking surveillance screening in at-risk individuals appears robust. Current guidelines recommend an intensive regimen of clinical, biochemical, and radiological surveillance commencing in early childhood for those with a clinical or genetic diagnosis of MEN1, with the aim of early tumor detection and treatment. Although it is tempting to assume that such screening results in patient benefits and improved outcomes, the lack of a strong evidence base for several aspects of MEN1 care, and the potential for iatrogenic harms related to screening tests or interventions of unproven benefit, make such assumptions potentially unsound. Furthermore, the psychological as well as economic burdens of intensive screening remain largely unstudied. Although screening undoubtedly constitutes an important component of MEN1 patient care, this perspective aims to highlight some of the current uncertainties and challenges related to existing MEN1 guidelines with a particular focus on the role of screening for presymptomatic tumors. Looking forward, a screening approach that acknowledges these limitations and uncertainties and places the patient at the heart of the decision-making process is advocated.

Intracranial spread of a systemic malignancy is common in advanced staged cancers; however, metastasis specifically to the pineal gland is a relatively rare occurrence. A number of primary lesions have been reported to metastasize to the pineal gland, the most common of which is lung. However, metastasis of a bronchial neuroendocrine tumor to the pineal gland is a seldom-reported entity. Here, we present a 53-year-old female who presented with worsening headaches and drowsiness. MRI brain revealed a heterogeneously enhancing partially cystic mass in the pineal region. The patient had an extensive oncologic history consisting of remote stage IIA invasive breast ductal carcinoma as well as a more recently diagnosed atypical bronchopulmonary neuroendocrine tumor with lymph node metastases. She underwent microsurgical volumetric resection of the large pineal mass and a gross total removal of the tumor was achieved. Histopathology confirmed a metastatic tumor of neuroendocrine origin and the immunohistochemical profile was identical to the primary bronchopulmonary carcinoid tumor. Eight weeks after surgery, she underwent stereotactic radiosurgical treatment to the resection cavity. At 1-year follow-up, the patient remains clinically stable without any new focal neurological deficits and without any evidence of residual or recurrent disease on postoperative MRI. Metastatic neuroendocrine tumors should be considered in the differential diagnosis of pineal region tumors and aggressive surgical resection should be considered in selected patients. Gross total tumor resection may afford excellent local disease control. We discuss the relevant literature on neuroendocrine tumors and current treatment strategies for intracranial metastases of neuroendocrine origin.

Bronchial neuroendocrine tumors (NET) are classified into well-differentiated typical carcinoids (TC), atypical carcinoids (AC), large cell neuroendocrine carcinomas (LCNEC), and small cell lung carcinomas (SCLC). We retrospectively reviewed and analyzed the diagnostic and therapeutic aspects, follow-up data, and outcomes of all patients diagnosed with a bronchial NET from 1995 to 2015 at our institution. Patients with LCNEC or SCLC were excluded due to the biological and clinical differences from the other bronchial NET. The clinical, laboratory, imaging, treatment, and follow-up data were collected and analyzed keeping in mind the recently published international recommendations. Forty-six patients were included in the study. Of these, 37 had a TC and 5 an AC. In 4 patients, the histological characterization was inadequate. Forty-four patients underwent surgery. Four patients developed metastatic disease. Interestingly, 14 patients had one or more other tumors diagnosed at some stage and 3 of them had three different tumors. A total of 7 patients died. The analysis of the laboratory and pathology assessment identified some inconsistencies when compared to the international recommendations. Although the treatment of bronchial NET at our institution was consistent with the successively published recommendations, it appears that the diagnostic process and the follow-up surveillance were not. We think that a systematic multidisciplinary approach might improve bronchial NET patient care. A relatively high rate of occurrence of a second, or also a third, non-NET tumor was observed, though the statistical value of such observation could not be exhaustively elucidated in this numerically limited patient population. In our opinion, the observed high rate of second malignancies in this patient cohort highlights the necessity of optimizing the follow-up of the bronchial NET patients, also considering the very good survival rate achieved with regard to the bronchial NET.