A Case of Non-Small-cell Lung Cancer in a Patient With Neurofibromatosis Type 1

Neurofibromatosis type 1 (NF-1) is an autosomal dominant condition that classically presents with cafe au lait spots, cutaneous neurofibromas, axillary and inguinal freckling, and iris Lisch nodules. Patients with a diagnosis of NF-1 have an increased risk of malignancy owing to mutations associated with the tumor suppressor gene p53; however, primary pulmonary malignancies are relatively uncommon. A 39-year-old female patient with a previous diagnosis of NF-1 had presented with progressive dyspnea and chest pain. Chest radiography and computed tomography (CT) demonstrated a left apical lung mass with mediastinal and pericardial infiltration; metastatic deposits were identified in the brain, adrenal glands, and thoracic spine on brain, chest, and abdominal CT scans. Echocardiography demonstrated a pericardial effusion and a left ventricular mass suggestive of a cardiac fibroma. Biopsy of a supraclavicular lymph node revealed a diagnosis of nonesmall-cell lung adenocarcinoma. The present case report details a rare case of none small-cell lung cancer in a patient with NF-1 and highlights the need to consider the diagnosis of lung cancer in patients with NF-1 presenting with symptoms suggestive of pulmonary malignancy. (C) 2020 Elsevier Inc. All rights reserved.