A Unique Association of Right-Sided Aortic Arch With Kommerell’s Diverticulum, Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia, and Typical Carcinoid: A Case Report

Our case presents a unique occurrence marking the first documentation of a connection between a typical carcinoid in the context of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) and vascular anomalies, including a right-sided aortic arch with Kommerell's diverticulum. Kommerell's diverticulum is a rare congenital anomaly. The lusoria subclavian artery, another developmental anomaly, arises from the right aortic arch instead of the typical left side. Neuroendocrine cells may contribute to lung disease pathogenesis by altering their physiology before clinical symptoms appear. A 56-year-old woman with an unyielding chronic cough underwent diagnostic evaluation, unveiling rare vascular anomalies alongside a pulmonary nodule. Radiological investigations disclosed a solid nodule in the middle lobe, accompanied by proximal right-sided aortic arch ectasia and an aberrant left subclavian artery. Following multidisciplinary deliberation, thoracic and vascular surgeons elected for surgical nodule resection. Utilizing uniportal video-assisted thoracoscopic surgery, the procedure revealed the anomaly of the right-sided aortic arch. Preliminary histological examination indicated a low-grade pulmonary carcinoid, obviating the need for further lymphadenectomy due to its low malignancy potential. Subsequent histological analysis confirmed a well-differentiated neuroendocrine tumor G1 consistent with typical carcinoid within a DIPNECH framework. Currently, the patient is in follow-up. This case underscores the importance of multidisciplinary evaluation and tailored surgical approaches for managing patients with rare vascular anomalies and pulmonary nodules, emphasizing the requisite comprehensive preoperative assessment and collaborative efforts among diverse medical specialties to optimize outcomes.