Bronchial Carcinoid Tumor in a Young Man with Neurofibromatosis. Case Report and Review of the Literature

Abstract

Neurofibromatosis 1 is a relatively common autosomal dominant disease characterized by the occurrence of distinctive skin pigmentation (cafe-au-lait spots and axillary/groin freckling) and multiple neuroectodermal and mesenchymal lesions (e.g. neurofibroma). Patients with NF1 also have a predilection for other types of tumors, such as carcinoid tumors in the duodenum, particularly in the periampullary region. Case presentation: An 18-year-old man with neurofibromatosis 1 presented with obstructive pneumonia. Bronchoscopy showed an endobronchial tumor obstructing the superior segment of the left lower lobe. Pathologic studies showed a carcinoid tumor. Two weeks later, the patient underwent left lower lobe lobectomy with lymph node dissection. Metastatic carcinoid tumor was present in subaortic lymph node.