First Description of an Enteric-Type Adenocarcinoma with a High-Grade Neuroendocrine Component of the Lung

Irene Moya-Horno, Rodolfo Posada, Clara Mayo, Miguel Ángel Molina, María Teresa Fernández-Figueras, Manel Esteller and Eva Musulén

Department of Medical Oncology, Instituto Oncológico Dr Rosell, Hospital Universitari General de Catalunya Grupo Quironsalud, Barcelona, Spain
Department of Pathology, Hospital Universitari General de Catalunya-Grupo Quironsalud, Sant Cugat del Vallès, Barcelona, Spain
Laboratory of Oncology, Pangaea Oncology, Hospital Universitari Quirón Dexeus, Barcelona, Spain
Universitat Internacional de Catalunya (UIC), Sant Cugat del Vallès, Barcelona, Spain
Josep Carreras Leukaemia Research Institute (IJC), Badalona, Barcelona, Spain
Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain
Physiological Sciences Department, School of Medicine and Health Sciences, University of Barcelona (UB), Barcelona, Spain
Institució Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Spain

^*Correspondance to: Eva Musulén 

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Abstract:

Background: Pulmonary Enteric-type Adenocarcinoma (PEAC) is a rare variant of Non-Small Cell Lung Cancer (NSCLC) characterized by similar morphology and immunohistochemical expression to primary colon adenocarcinoma.
Case Report: A 58-year-old smoker female was submitted to a complete lobectomy due to a mass without lymph node  involvement. The resected tumor was a PEAC with a biphasic histologic morphology with one component of glands with a cribriform and acinar pattern with luminal necrosis, and the other component with cells with eosinophilic or clear cytoplasm and round nuclei with prominent nucleoli appearing in a solid growth pattern. The gland cells expressed markers of lung and enteric differentiation such as TTF-1, Cytokeratin 20 (CK20), and CDX2, while the solid component cells showed expression of CK20 and synaptophysin with a Ki-67 proliferation index of 65% defining them as a high-grade Neuroendocrine Carcinoma (NEC). The neoplasm metastasized first to the left clavicle and rapidly spread to multiple bones, cerebellum and pancreas at the expense
of the NEC component resulting in the patient's death twelve months after diagnosis of the lung neoplasm. Both PEAC and clavicle tumor shared KRAS p.G12V and TP53 c.375+1G>C mutations but CDK6 amplification, a frequent genetic alteration of neuroendocrine differentiation, was only found at the metastasis.
Conclusion: We describe in this study the first case of Pulmonary Enteric-type Mixed Adenoneuroendocrine Carcinoma (PEMANEC) in analogy to colon neoplasms, where an adenocarcinoma with a high-grade neuroendocrine component is defined as a Mixed Adenoneuroendocrine Carcinoma (MANEC).

Keywords:

Pulmonary enteric-type adenocarcinoma; Neuroendocrine carcinoma; Pulmonary enteric-type mixed adenoneuroendocrine carcinoma

Cite the Article:

Moya-Horno I, Posada R, Mayo C, Molina MÁ, Fernández-Figueras MT, Esteller M, et al. First Description of an Enteric-Type Adenocarcinoma with a High-Grade Neuroendocrine Component of the Lung. Clin Oncol. 2021;6:1876..