P0120 - Primary Appendiceal Cancers: A Systematic Review

Dawood Findakly, MD
Creighton University School of Medicine Phoenix Program, Phoenix, AZ

Introduction: Primary appendiceal Cancers (PAC) are rare, incidentally found in in approximately 1% of appendectomies, and have a unique profile with diverse histology. This review aimed to assess the clinicopathologic findings, treatment, and outcomes of PAC cases reported in the literature.

Methods: We performed PubMed, Medline, EMBASE, Web of Science, and Scopus literature search for "primary appendix cancer" between 2010 to 2020 pooled with a case from our institution.

Results: A total of 108 patients were included. Male: female ratio of 1:1.17 with a mean age (±SD) of 54.9 (±16.2) years. The most common presenting complaints were abdominal pain, progressive nausea with emesis, and bloating in 31.1%, 14.6%, 10.2% of cases, respectively, with a median duration of symptoms of 30 (range 1-240) days. In terms of size, 83.3% were more than 2.0 cm. The most common subtypes were mucinous adenocarcinomas (34.7%), low-grade appendiceal mucinous neoplasm (14.7%), carcinoid/neuroendocrine carcinoma (14.7%), mucinous neoplasm with signet ring cell features (10.5%), crypt cell adenocarcinoma (5.3%), colonic type adenocarcinomas (4.2%), and poorly-differentiated adenocarcinoma of the appendix (4.2%). Localized tumor, regional tumor, and distant metastasis were found in 31.3%, 8.3%, and 60.4% of cases, respectively. The most common treatment modalities were surgery, surgery plus chemotherapy, and chemotherapy alone, in 49.5%, 46.3%, and 2.1%, respectively. The median duration of follow-up was 12 (range 1-170) months. In terms of outcomes, the mortality rate in patients with metastatic cancer was 40%. Of the reported data, Patients were alive and disease-free (16.7%), alive with disease (10.0%), alive with recurrence or metastasis (6.7%), alive in remission (48.3%), died of the disease (15%), and lost to follow-up (3.3%).

Discussion: Most patients with PAC had metastatic disease at the time of initial presentation which is responsible for 40% of patient deaths. The most common observed overall treatment modality was surgery followed by surgery with chemotherapy.

Given their rarity, heterogeneity of clinical behaviors, diverse treatment modalities, and variable outcomes, a high index of clinical suspicion and a multidisciplinary approach is needed. This review aims to shed light on the inherent complexity induced by PAC and highlights a key consideration in future endeavors to help promote early detection, management, and eventually improve outcomes of this rare entity.



Disclosures:


Dawood Findakly, MD. P0120 - Primary Appendiceal Cancers: A Systematic Review, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.