A 6-Year-Old Boy With an Atypical Cause of Chest Pain and Hemoptysis

Abstract

INTRODUCTION: Carcinoid tumors are neuroendocrine neoplasms that are extremely rare in young children. We present a case of an endobronchial atypical carcinoid tumor in a 6-year old child who presented with chest pain and hemoptysis. CASE PRESENTATION: A 6-year-old Caucasian male with history of moderate persistent asthma presented with 2-week history of cough, chest pain and 5-day history of hemoptysis. He also had weight loss and frequent intermittent loose stools noted in the last 2 months. His initial work up showed an increased total white cell count (27.3 X 103/microL), and normal results for ESR, ANA, p-ANCA, c-ANCA, and a negative PPD and sweat test. Respiratory cultures showed no growth. Since he continued to have further episodes of hemoptysis, he was transferred to our institution. His examination was significant for diminished air entry on left side, with tracheal deviation to the left. Chest xray (Figure 1A) showed volume loss on left side with mediastinal shift. A chest CT showed bronchial obstruction in left main bronchus due to an endobronchial mass (Figure 1B and 1C) and bronchoscopy revealed a fleshy pedunculated mass lesion in left main bronchus (Figure 1E). A biopsy of the mass revealed nests and trabeculae of neoplastic cells with 5 to 10 mitotic figures per high power field, suggestive of an atypical carcinoid tumor (Figure 1G). An Octreotide-indium111 labeled Positron Emission Tomography (PET) scan revealed no significant tumor activity in any other body regions (Figure 1D). He underwent left main bronchus sleeve resection with end-to-end anastomosis, following which he recovered well without any complications. At 6 months follow up post-resection, his symptoms have completely resolved and he has normal lung function. DISCUSSION: Pulmonary carcinoid tumors are neuroendocrine tumors (NET) that can range from low-grade malignant typical carcinoids to intermediate atypical carcinoids to high-grade small cell lung cancer. Malignant NETs of the lung in pediatric age group are generally associated with a good outcome for ages >5 years[1]. Our patient did not have any features of Cushing syndrome or MEN1 syndrome, which can also be associated with carcinoid tumors. His weight loss and diarrhea do suggest the likelihood of carcinoid syndrome that is caused by increased secretion of serotonin and kallikrein from these tumors. CONCLUSIONS: Atypical carcinoid tumors are rare neoplasms in children that can present in the form of an endobronchial mass lesion. In the presence of other features of carcinoid syndrome, they should be considered in the differential diagnosis.