Abstract Introduction A carcinoid tumor is a well-differentiated neuroendocrine neoplasm, rarely diagnosed as a primary pulmonary lesion in the pediatric population. Patients with bronchial tumors often present with nonspecific symptoms such as cough, wheeze and/or hemoptysis, which can complicate timely diagnosis. We report the case of a 16-year-old female who presented with worsening cough and hemoptysis, subsequently diagnosed with a primary transbronchial typical carcinoid tumor. Description A healthy 16-year-old experienced 6 episodes of mild mucoid hemoptysis over 2 months, initially attributed to epistaxis. She denied other symptoms including weight loss, wheeze, dyspnea, easy bruising, malaise, or fevers. The frequency of her symptoms increased, prompting further evaluation by her PCP who consulted our pediatric pulmonology team. We recommended adding a chest CT with contrast and serum inflammatory markers. The CT demonstrated a hyperenhancing lesion in the left hilum, measuring 20x13x25mm. The lesion appeared to be impinging on the posterior wall of the left lower lobe bronchus, extending to adjacent pulmonary venules (Figure1A). She was directed to Vanderbilt Children’s Emergency Department for admission. A bronchoscopy with BAL and endobronchial ultrasound with transbronchial needle aspiration (EBUS-TBNA) was performed within 24 hours, revealing a left lower lobe endobronchial tumor (Figure1B). Pathology was suggestive of a neuroendocrine tumor. Infectious workup was negative, including tuberculosis, histoplasmosis, and blastomycosis. Tumor staging included imaging for metastatic disease with CT of the abdomen, pelvis, and soft tissue neck, all of which were negative. A Ga-68 dodatate whole body somatostatin receptor-targeted (SSRT) PET/CT scan was also utilized to evaluate metastatic disease, demonstrating an isolated hyperintense signal in the left hilum (Figure1C). A lower sleeve lobectomy was performed, with pathology confirming a typical carcinoid low-grade (NET grade 1) tumor with negative margins and lymph nodes. Genetic testing for MEN1 and CDKN1B/MEN4 mutations was negative. As of 1-year follow-up, she remains asymptomatic without recurrent or metastatic disease. Discussion We present a rare case involving a pediatric primary bronchial typical carcinoid tumor, requiring a multidisciplinary approach and advanced diagnostics for swift and accurate diagnosis. Atypical carcinoid tumors have a significant decrease in survival rates compared to typical carcinoids; therefore, an accurate diagnosis is critical. Typical carcinoids usually express higher levels of somatostatin receptors, making SSRT PET scans particularly useful in the diagnostic evaluation. While histological evaluation is the gold-standard for definitive diagnosis, EBUS-TBNA and Ga-68 dodatate SSRT scan were helpful in evaluating the tumor type and metastatic potential, expediting surgical planning and treatment. This abstract is funded by: None
Kozak et al. (Fri,) studied this question.