Radiology of the neuroendocrine neoplasms of the gastrointestinal tract: a comprehensive review
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REVIEW
Radiology of the neuroendocrine neoplasms of the gastrointestinal tract: a comprehensive review Sundeep Malla1 · Pawan Kumar1 · Kumble Seetharama Madhusudhan1 Received: 20 June 2020 / Revised: 4 September 2020 / Accepted: 10 September 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Neuroendocrine neoplasms (NENs) are a group of neoplasms arising from the diffuse endocrine system (DES). The gastrointestinal tract (GIT) is the most common site of NEN. The WHO classification divides NEN into three broad categories viz. well-differentiated NENs, poorly differentiated NENs, and mixed neuroendocrine-non-neuroendocrine neoplasms. All GIT NEN have the potential to synthesize and secrete various bioactive substances which may lead to various clinical syndromes. The NEN may occur anywhere in the GIT and exhibit varying clinical presentation, prognosis, and metastatic potential. Further, some tumors show association with familial syndromes like multiple endocrine neoplasia type 1 and neurofibromatosis type 1. Ultrasonography, computed tomography (CT), magnetic resonance imaging, and positron emission tomography-CT are the imaging modalities useful in the diagnosis, localization, and staging of GIT NEN. Management depends on the site, size, grade, and stage of the tumor with interventional radiology playing a significant role in some cases. This imaging review describes the role of a radiologist in the management of GIT NEN. Keywords Neuroendocrine neoplasm · Carcinoid tumor · Gastrointestinal tract carcinoid · Neuroendocrine carcinoma · Gastrinoma
Introduction Neuroendocrine neoplasms (NENs) are defined as the epithelial neoplasms with predominant neuroendocrine differentiation. They include a myriad of tumors arising from the cells of the diffuse endocrine system (DES). The neoplasms of this system have neural crest as well as endocrinal differentiation which confers the ability to synthesize and secrete a variety of pharmacologically active substances (peptides, amines, etc.) [1, 2]. As a result, there may be specific clinical manifestations depending on the predominant hormonal activity which aids in their distinction from other site-specific lesions. Furthermore, hormonal synthesis designates immunoreactivity for various neuroendocrine markers which facilitates their detection on immunohistochemistry and functional imaging [2, 3]. NEN may arise anywhere in the neuroendocrine system; however, their most common location is the gastrointestinal tract (GIT; 67%) followed by the * Kumble Seetharama Madhusudhan [email protected] 1
Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
tracheobronchial tree (~ 25%) and pancreas [4]. Within the GIT, the rectum is the most common site (34%) followed by small bowel (26%), stomach (12%), colon (8%), duodenum (8%), caecum (6%), and appendix (6%) [5]. Further, based on their embryological location within the GIT, these may be classified into foregut (stomach, duodenum, pancreas,
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