Spectrum of Pulmonary Neuroendocrine Proliferations and Neoplasms

Authors: Ryo E. C. Benson, MD, , Melissa L. Rosado-de-Christenson, MD, , Santiago Martínez-Jiménez, MD, , Jeffrey R. Kunin, MD, and , Paul P. Pettavel, MD

Neuroendocrine neoplasms are ubiquitous tumors found throughout the body, most commonly in the gastrointestinal tract followed by the thorax. Neuroendocrine cells occur normally in the bronchial and bronchiolar epithelium and may be solitary or may occur in clusters. Although neuroendocrine cell proliferations may be found in association with chronic lung disease, a broad range of neuroendocrine proliferations and neoplasms may occur and exhibit variable biologic behavior. Diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) is a diffuse idiopathic form of neuroendocrine cell hyperplasia and is considered a preinvasive lesion that may give rise to carcinoid tumors. Patients with DIPNECH are typically older women who may be asymptomatic or may present with chronic respiratory symptoms. DIPNECH manifests as multifocal bilateral pulmonary micronodules on expiratory high-resolution computed tomographic (CT) images; the air trapping is secondary to constrictive bronchiolitis. Carcinoid tumors are low-grade malignant neoplasms that typically affect symptomatic children and young adults. Carcinoids manifest as well-defined pulmonary nodules or masses that are often closely related to central bronchi. They may exhibit intrinsic calcification and contrast material enhancement at CT, and patients with carcinoids may have postobstructive atelectasis and pneumonia. Although typical carcinoids are indolent neoplasms and patients have a good prognosis, atypical carcinoids are aggressive malignancies with a propensity for metastasis. Both are optimally treated with complete surgical excision. Large cell neuroendocrine carcinoma and small cell lung cancer are highly aggressive neuroendocrine malignancies that usually affect elderly smokers. These tumors manifest with large peripheral or central pulmonary masses. Local invasion, intrathoracic lymphadenopathy, and distant metastases are frequent at presentation. As a result, affected patients may not be candidates for surgical resection, are often treated with chemotherapy with or without radiation, and have a poor prognosis.