Interestingly, chest x-rays, during each episode, depicted RUL pneumonia with an unresolved RUL atelectasis (Figure 1). A contrast enhanced CT of the chest revealed a questionable small indentation on the right main stem bronchus. Anatomical clarification was provided by CT luminogram and MRI images (Figures 2 and 3). Bronchoscopy revealed a soft tissue mass emanating from the branch point of the right upper lobe bronchus. This endobronchial tumor compressed the main stem bronchus causing a 10% obstruction and completely obliterated the right upper lobe bronchus.

Primary pulmonary or endobronchial tumors are not common in childhood, but when present display a wide spectrum of pathology and prognosis. In 1993 Hancock et al. reviewed the literature and found that of the 383 childhood primary pulmonary tumors reported 24% were benign and 76% were malignant.1 Of the benign tumors plasma cell granuloma (52.2%) and hamartoma (23.9%) comprised the majority. Metastases were not found with any of the benign tumors while the overall mortality rate was 8.7%. The most frequent malignant tumors were the bronchial adenomas followed by bronchogenic carcinoma. The overall mortality rate for the malignant tumors was 30.2%. Hancock et al. concluded this figure was misleading because patients with bronchial adenomas had an excellent prognosis irrespective of local invasion or tumor stage. With the bronchial adenomas excluded the mortality rate increased to 50.3%. Bronchogenic carcinoma carried the worst prognosis with most metastatic at diagnosis and a mortality rate approaching 90%.1 The biopsy of our patient’s mass was consistent with a type of bronchial adenoma, the typical carcinoid tumor. Bronchial carcinoids are the most common primary intrabronchial tumor in children. Pulmonary carcinoid tumors are neuroendocrine tumors arising from Kulchitsky neural crest cells found in the normal basal layer of bronchial epithelium. They are divided histologically into typical and atypical varieties, and both are capable of metastasizing to regional lymph nodes and to liver, bone, and/or brain. However, the atypical carcinoid is more likely to metastasize. Their propensity to induce paraneoplastic syndromes seems to be related to hepatic metastases. The treatment for pulmonary carcinoid tumors is complete surgical resection regardless of histology or node status. Five-year survival rates for typical and atypical carcinoid range from 88-92% and 60-75% respectively.2
Our patient underwent an extended right upper lobectomy with partial sleeve resection of the right mainstem bronchus. Lymph node resection and metastatic workup did not reveal tumor spread. She continues to be followed closely by pediatric hematology-oncology and pediatric pulmonology services.

1. Hancock BJ, Di Lorenzo M, Youssef S, Yazbeck S, Marcotte, JE, Collin PP: Childhood Primary Pulmonary Neoplasms. Journal of Pediatric Surgery 28(9):1133-1136, 1993.
2. Moraes TJ, Langer JC, Forte V., Shayan K, Sweezey N: Pediatric Pulmonary Carcinoid; A Case Report and Review of the Literature. Pediatric Pulmonology 35:318-322, 2003.
3. Van Way III CW, Salloum EJ: Adenoma, Bronchial. Emedicine.com: November 21, 2003.