A Teenager With Chronic Cough and Wheeze

Abstract

SESSION TITLE: Lung Cancer 2 SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Tuesday, October 31, 2017 at 07:30 AM - 08:30 AM INTRODUCTION: Carcinoid is the most common primary lung malignancy in children and diagnosis is often delayed. CASE PRESENTATION: A previously healthy 13 year old female developed a dry cough and hemoptysis which resolved with antibiotics. Her cough quickly returned along with an audible wheeze, dyspnea, orthopnea, and fatigue. Albuterol did not relieve her symptoms. A chest radiograph demonstrated a right middle lobe opacity concerning for atelectasis or pneumonia so she was referred to pediatric pulmonology. The patient presented with monophonic wheeze over her right middle lobe. A CT chest with contrast demonstrated a mass in the right bronchus intermedius (Fig. 1), and bronchoscopy revealed an obstructive mass (figure 2). Following manipulation and biopsy of the mass, the patient developed a hypertensive crisis, and prolonged bleeding at the biopsy site. She was treated for presumed carcinoid crisis. After 36 hours, her blood pressure normalized and she was extubated. Histology revealed spindle cell proliferation with squamous epithelial lining and IHC stain for chromogranin was negative. It was determined that the tissue obtained from the biopsy was a reactive process overlying an unsampled neoplasm. Serum markers for carcinoid were negative. However, because of the mass location/appearance and intraoperative hypertensive crisis/bleeding, carcinoid was highest on the differential. The patient underwent right pneumonectomy with nodal dissection. A lung sparing procedure was unable to be performed due to invasion of the mass outside of the bronchial wall with involvement of major vessels. IHC staining of the mass demonstrated positivity for chromogranin, CD56, and synaptophysin, with no lymph node involvement, confirming primary pulmonary carcinoid. DISCUSSION: Pulmonary carcinoid is a well differentiated malignant tumor, classified by the WHO as a subtype of lung neuroendocrine tumor. Although rare, they are the most common primary pulmonary malignancy in children. Carcinoid crisis results from an abrupt release of neuroendocrine mediators, typically perioperatively, and can result in hypo/hyper tension, flushing, bronchospasm, or complete vasomotor collapse(1). CONCLUSIONS: This case is the first reported carcinoid crisis resulting from manipulation of a primary pulmonary carcinoid in a child. Reference #1: (1) Varela, Patricio et. al. Primary tracheobronchial tumors in children. Sem. Ped. Surg., 2016-06-01, Vol 25;3;p15-155 DISCLOSURE: The following authors have nothing to disclose: Adam Van Mason, Alvin Singh No Product/Research Disclosure Information