Current Status of Treatment among Patients with Appendiceal Tumors—Old Challenges and New Solutions?

Author:

Chawrylak Katarzyna1ORCID,Leśniewska Magdalena1ORCID,Mielniczek Katarzyna1,Sędłak Katarzyna1ORCID,Pelc Zuzanna1ORCID,Kobiałka Sebastian1,Pawlik Timothy M.2ORCID,Polkowski Wojciech P.1ORCID,Rawicz-Pruszyński Karol1

Affiliation:

1. Department of Surgical Oncology, Medical University of Lublin, Radziwiłłowska 13 St., 20-080 Lublin, Poland

2. Department of Surgery, The Ohio State University Wexner Medical Center and James Comprehensive Cancer Center, Columbus, OH 43210, USA

Abstract

The 5th edition of the World Health Organization (WHO) classification of tumors of the digestive system distinguishes four categories of appendiceal tumors (ATs): serrated lesions and polyps, mucinous neoplasms, adenocarcinomas, and neuroendocrine neoplasms (NENs). The differential diagnosis of ATs can be challenging in medical practice, due to their rarity and lack of data from randomized controlled trials on a large, diverse group of patients. ATs are usually noted in specimens obtained during appendectomies due to clinical acute appendicitis. In the European population, most ATs (65%) occur over the age of 50 and among women (56.8%). According to histological type, 54.6% are neuroendocrine tumors (NETs); 26.8% cystic, mucinous, and serous neoplasms; and 18.6% adenocarcinoma not otherwise specified (NOS). On pathologic analysis, most AT findings are benign lesions or small NENs that do not require further therapeutic measures. The presence of appendiceal mucinous neoplasm (AMN) can lead to pseudomyxoma peritonei (PMP). While the multimodal treatment for abdominal malignancies has evolved over the past several decades, the clinical workup and treatment of ATs remain a challenge. Therefore, this review aims to describe the diagnostic possibilities, molecular-based diagnosis, staging, differences in the treatment process, and prognostic factors associated with ATs.

Publisher

MDPI AG

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