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Pulmonary enteric adenocarcinoma: an overview

Published online by Cambridge University Press:  28 April 2020

Raffaele Palmirotta*
Affiliation:
Department of Biomedical Sciences and Human Oncology, University of Bari ‘Aldo Moro’, P.zza G. Cesare, 11 – 70124Bari, Italy
Domenica Lovero
Affiliation:
Department of Biomedical Sciences and Human Oncology, University of Bari ‘Aldo Moro’, P.zza G. Cesare, 11 – 70124Bari, Italy
Stella D'Oronzo
Affiliation:
Department of Biomedical Sciences and Human Oncology, University of Bari ‘Aldo Moro’, P.zza G. Cesare, 11 – 70124Bari, Italy IRCCS-Istituto Tumori ‘Giovanni Paolo II’, Viale Orazio Flacco, 65, 70124Bari, Italy
Annalisa Todisco
Affiliation:
Department of Biomedical Sciences and Human Oncology, University of Bari ‘Aldo Moro’, P.zza G. Cesare, 11 – 70124Bari, Italy
Valeria Internò
Affiliation:
Department of Biomedical Sciences and Human Oncology, University of Bari ‘Aldo Moro’, P.zza G. Cesare, 11 – 70124Bari, Italy
Fabio Mele
Affiliation:
IRCCS-Istituto Tumori ‘Giovanni Paolo II’, Viale Orazio Flacco, 65, 70124Bari, Italy
Luigia Stefania Stucci
Affiliation:
Department of Biomedical Sciences and Human Oncology, University of Bari ‘Aldo Moro’, P.zza G. Cesare, 11 – 70124Bari, Italy
Franco Silvestris
Affiliation:
Department of Biomedical Sciences and Human Oncology, University of Bari ‘Aldo Moro’, P.zza G. Cesare, 11 – 70124Bari, Italy
*
Author for correspondence: Raffaele Palmirotta, E-mail: raffaelepalmirotta@gmail.com

Abstract

Most commonly described as sporadic, pulmonary adenocarcinoma with enteric differentiation (PAED) is a rare variant of invasive lung cancer recently established and recognised by the World Health Organization. This tumour is highly heterogeneous and shares several morphological features with pulmonary and colorectal adenocarcinomas. Our objective is to summarise current research on PAED, focusing on its immunohistochemical and molecular features as potential tools for differential diagnosis from colorectal cancer, as well as prognosis definition and therapeutic choice. PAED exhibits an ‘entero-like’ pathological morphology in more than half cases, expressing at least one of the typical immunohistochemical markers of enteric differentiation, namely CDX2, CK20 or MUC2. For this reason, this malignancy appears often indistinguishable from a colorectal cancer metastasis, making the differential diagnosis laborious. Although standard diagnostic criteria have not been established yet, in the past few years, a number of approaches have been addressed, aimed at defining specific immunohistochemical and molecular signatures. Based on previously published literature, we have collected and analysed molecular and immunohistochemical data on this rare neoplasm, and have described the state of the art on diagnostic criteria as well as major clinical and therapeutic implications.

The analysis of data from 295 patients from 58 published articles allowed us to identify the most represented immunohistochemical and molecular markers, as well as major differences between Asian PAEDs and those diagnosed in European/North American countries. The innovative molecular approaches, exploring driver mutations or new gene alterations, could help to identify rare prognostic factors and guide future tailored therapeutic approaches to this rare neoplasm.

Type
Review
Copyright
Copyright © The Author(s), 2020. Published by Cambridge University Press

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