ESPE Abstracts

Pulmonary carcinoid tumors represent well-differentiated pulmonary neuroendocrine tumors, which include typical and atypical carcinoids. This type of lung tumors is rare, but represents the most common primary malignant lung tumor in children and adolescents, especially the typical carcinoids. They can be asymptomatic at the time of diagnosis or can present with nonspecific findings like recurrent pneumonia, cough, and hemoptysis. We report the case of a 15-year-old girl reffered for MEN1 screening due to recent diagnosis of typical pulmonary carcinoid. Her personal history revealed the debut of events two years ago with an episode of pneumonia and hemoptysis which was followed by gradually visual impairment. She was referred for more investigations which led to the CT scan describing the right pulmonary tumor and multiple disseminated liver nodules and the MRI showing an increased pituitary volume (14 mm diameter) and bilateral ocular tumors. Biochemical screening and genetic testing excluded MEN1 diagnosis. Cg A, urinary 5HIIA, NSE tumor markers were normal, except Serotonin which was two times the upper limit of normal values. Octreotide scan was performed and showed the octreoscan-positive right pulmonary tumor and octreoscan-positive choroidal, pituitary and liver metastases. Thus, the patient started systemic treatment with somatostatin analogs and was referred to the oncology department for systemic chimiotherapy regimen. In conclusion, typical pulmonary carcinoids represent a small percentage of pediatric tumors and their diagnosis is difficult to establish due to their nonspecific symptoms. Typical lung carcinoids have an excellent prognosis following surgical resection. They rarely metastasise, which emphasizes the particularity of this case and the need for an increased awareness of this rare form of lung cancer. There isn’t a consensus between societies and their management guidelines regarding optimal treatment scheme in the metastatic lung NET, so there is a need for more studies. Therefore, an early and accurate diagnosis may lead to an appropriate treatment scheme with a substantial impact on prognosis.