Multidisciplinary management of newly diagnosed pediatric large cell neuroendocrine carcinoma of the lung causing hemoptysis

Abstract

To the Editor: The management of acute hemoptysis in a child with lung cancer is challenging, particularly in the setting of newly diagnosed disease where the goal is cure. Our experience highlights the importance of multidisciplinary management for emergency management in order to provide acute stabilization that will also allow for optimal definitive treatment. An 18-year-old boy with history of stage II Wilms tumor treated with nephrectomy and chemotherapy at age 6 presented with 24 h of acute, active hemoptysis. He had no breath sounds in the left lower lung (LLL) and anemia. Chest radiograph demonstrated a LLL mass (Figure 1A). Contrast-enhanced thoracic computed tomography (CT) scan confirmed the mass, with contrast blush suggesting active arterial extravasation (Figure 1B and C). An additional nodule and subcarinal adenopathy were identified. Findings suggested primary lungmalignancy. He was hospitalized and required intubation for airway protection. Bronchoscopy with potential argon beam ablation of endobronchial invasion, ultrasound-guided transbronchial biopsy of the subcarinal mass, and placement of a left mainstem bronchial blocker were recommended. Bronchoscopy allowed direct visualization of the mass and confirmed pulsatile hemorrhage from the lingular and superior segmental bronchi with bronchial erosion and invasion identified, and endobronchial argon ablation was performed using argon gas to induce coagulation. After achieving hemostasis with cold saline and epinephrine, endobronchial ultrasound with transbronchial needle aspiration (EBUS-TBNA) of the subcarinal hilar lymph nodes was performed, and an endobronchial blocker was placed in the left main bronchus, which could be inflated in the setting of active submassive hemoptysis to safely isolate the hemorrhage and prevent aspiration and asphyxiation. Given the tumor neovascularity and invasion of the bronchus, bronchial and pulmonary artery angiography was performed to look for active contrast extravasation, which could then be targeted for embolization. Evaluation with bronchial and pulmonary arteriography revealed 2 abnormal left bronchial arteries with neovascularity but no shunting (Figure S1). Bronchial artery embolization (BAE) of both abnormal branches resulted in complete cessationof vascular flow. Left pulmonary arteriogram confirmed the mass but no thrombus or tumor source, with elevated left PA pressure measuring 30–33 mm Hg. The patient remained intubatedwith the left mainstem blocker in place but uninflated. On the day following embolization, when sufficiently stable, emergent radiotherapy (RT)was initiated to control hemoptysis and prevent further bleeding. He received 12Gy in six fractions andwas extubated. His respiratory status improved considerably. A thorascopic subcarinal biopsywas required for diagnosis, confirming a large cell neuroendocrine carcinoma (LCNEC). PET/MRI imaging showed a hypermetabolic LLLmasswith a satellite lesion, and left hilar, subcarinal, and contralateral right paratracheal lymph nodes, consistent with stage T4N3M0 disease. Being unresectable, chemotherapy with cisplatin/etoposide for a total of five cycles and concurrent RT (56 Gy in 28 fractions) followed by consolidation with durvalumabwas initiated. Three months after chemoradiation and while on durvalumab, the LLL mass had decreased to 4.8 × 4 cm; one year after chemoradiation, the necrotic LLL mass has further decreased in size and is no longer FDG-avid. The patient continues with durvalumab treatment. LCNEC is a rare pediatric lung cancer. Treatment recommendations follow the adult experience, including a single-arm study of 29 patients evaluated cisplatin/etoposide in advanced LCNEC with median progression-free survival (PFS) of 5.2 months and overall survival (OS) of 7.7 months, and in the PACIFIC trial of patients with stage III NSCLC, durvalumab after chemoradiation prolonged the 24-month median OS (66.3% vs. 55.6%).1,2 For conventionally fractionated RT in NSCLC, doses of 60–70 Gy are generally used.3 Given the patient’s emergency dose of 12 Gy to prevent potentially fatal hemoptysis, the RT dose selected for definitive treatment was 56 Gy. The emergency RT dose was determined with a goal of hemostatic control, and with the consideration of future definitive RT, it was necessary to keep the emergency dose sufficiently low. This avoided precluding the optimal definitive treatment regimen, while still providing adequate dose to control bleeding. Optimization of therapy was especially challenging in this boy, as LCNEC is a highly aggressive, high-grade malignancy, and as he presented with extensive locoregional disease, but without distant metastatic disease, the patient was considered potentially curable. Given the patient’s presentation with hemoptysis, BAE was essential for initial local tumor control and cessation of massive pulmonary hemorrhage. The effectiveness of BAE in the management