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Bronchial carcinoid tumors in pediatric patients
L T Wang1, E W Wilkins, H H Bode
1Children's Service Massachusetts General Hospital, Boston 02114.
Insights
Bronchial carcinoid tumors (BCT) are rare in children but present with distinct symptoms like wheezing and atelectasis. Surgical resection offers a high chance of symptom-free recovery for pediatric BCT patients.
Area of Science:
- Pediatric Oncology
- Pulmonary Medicine
- Surgical Pathology
Background:
- Bronchial carcinoid tumors (BCT) are the most common primary lung neoplasms in children.
- Pediatric BCT cases are infrequent, necessitating a review of clinical presentations and outcomes.
Purpose of the Study:
- To characterize the clinical presentation, diagnosis, and treatment outcomes of pediatric bronchial carcinoid tumors.
- To compare pediatric BCT characteristics with those reported in adult populations.
Main Methods:
- Retrospective review of 25 pediatric BCT cases (17 from Massachusetts General Hospital, 8 from other pediatric centers).
- Comparison of findings with seven large adult BCT series.
- Analysis of patient demographics, symptom duration, presenting symptoms, and post-surgical outcomes.
Main Results:
- The average age at diagnosis was 17 years, with symptom duration ranging from 2 weeks to 2.6 years.
- Pediatric patients commonly presented with wheezing and atelectasis, unlike asymptomatic adults.
- Five patients had weight loss, one had hoarseness, and one presented with Cushing's syndrome.
- Twelve of 14 patients were disease-free for up to 34 years after surgical resection.
Conclusions:
- BCT should be suspected in children with persistent pneumonitis, atelectasis, wheezing, or hemoptysis.
- Surgical resection is effective, leading to symptom-free recovery in most pediatric BCT cases despite the tumor's low-grade malignancy.
Abstract:
Bronchial carcinoid tumors (BCT) are the most frequent primary pulmonary neoplasms of childhood. Seventeen of 208 patients diagnosed as having BCT at the Massachusetts General Hospital were between 10 and 21 years of age. We reviewed our records of the 17 patients and 8 other pediatric cases and compared their findings with those of seven large series of adults. Distribution was equal between the sexes. The average age at diagnosis was 17 years; 4 patients were < or = 15 years old. The duration of symptoms prior to diagnosis varied from 2 weeks to 2.6 years, with a median duration of 8.5 months. In contrast to adults, no child was asymptomatic. The majority of children presented with wheezing and atelectasis in addition to the characteristic adult triad of cough, hemoptysis, and pneumonitis. Five patients presented with weight loss and one patient presented with hoarseness. One of the 17 pediatric patients presented with Cushing's syndrome and a functional BCT. Twelve of 14 patients were disease free for 9 months to 34 years after surgical resection. We conclude that BCT should be suspected in children with pneumonitis resistant to therapy, atelectasis, wheezing, and hemoptysis. Surgical resection will result in symptom-free recovery in the majority of cases in spite of low-grade malignancy.
