NEUROENDOCRINE TUMOR OF THE LUNGS SECONDARY TO DIFFUSE IDIOPATHIC PULMONARY NEUROENDOCRINE CELL HYPERPLASIA (DIPNECH)

Abstract

CASE
 A 66-year-old female, known diabetic and hypertensive, with diagnosis of invasive ductal carcinoma of the right breast presented with a chest x-ray finding of a round lung density in the right paravertebral region at the level of T10-11. Chest CT scan showed multiple non-calcified nodules in the right middle and both lower lobes. CT guided biopsy of the dominant lung nodule was positive for Synaptophysin, Chromogranin, CD-56 with Ki-67 of 1-3%, compatible with Typical Carcinoid Tumor. Bone scan was negative. Video Assisted Thoracic Surgery (VATS) with wedge resection of the right lung and middle lobe confirmed the diagnosis of well differentiated pulmonary endocrine tumor, with nolymphovascular invasion. The background lung tissue from the right middle lobe revealed multiple foci of neuroendocrine tumor (0.1 cm to 0.4 cm); the right lower lobe tissue (0.1 cm to 0.3 cm) with areas of focal neuroendocrine cell hyperplasia. She was generally asymptomatic except for occasional cramps of the lower extremities, frequent watery stools with 4 kg weight loss and intermittent facial flushing. A multidisciplinary team confirmed the diagnosis of a rare case of lung carcinoid, non-functional, arising from Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH). Consensus plan consisted of presevation of lung parenchyma and close follow-up. She underwent total right mastectomy and was maintained on Tamoxifen. No further surgery was planned at this time and she continues to be asymptomatic.