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.
Addison's disease presenting with idiopathic intracranial hypertension (IIH) is rare but well reported in the literature. IIH has also been reported to occur with other endocrine conditions. We explore some interesting diagnostic and management challenges of a young female that presented with IIH and Addison's disease. We discuss the features of this unifying neuroendocrine diagnosis.A previously well 17-year-old female presented to the Emergency Department after a syncopal episode. She had been suffering from worsening and increasing headaches over the last eight months, with vomiting once or twice per day. She had papilledema and reduced visual fields bilaterally. CT head and venogram were normal. Lumbar puncture (LP) opening pressure was raised. She was noted to be hypotensive and hyponatremic. Investigations for hyponatremia revealed random cortisol of <28 nmol/L. She was treated for adrenal crisis. Further investigations were performed and she was diagnosed with IIH associated with Addison's disease.Addison's disease should always be considered in a patient presenting with IIH and hyponatremia. While the mechanism for this association is not completely clear, treating the underlying adrenal insufficiency with steroid replacement alone is an effective treatment and provides symptomatic relief.
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