Late stage diagnosis of mucinous adenocarcinoma of the appendix: a case report of an unusual tumor with a rare presentat
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CASE REPORT
Open Access
Late stage diagnosis of mucinous adenocarcinoma of the appendix: a case report of an unusual tumor with a rare presentation Katerina Roma1* , Mark Baldwin1, Daniel Sedmak5, Matthew Silva4, William Stellar2 and Gina Many3
Abstract Background: The incidence of mucinous appendiceal adenocarcinomas (MAA) has increased over the past three decades. Advanced stage tumor diagnosis is likely attributable to non-specific findings. Here we describe advanced stage appendiceal MAA presenting as inguinal ulcers, scrotal abscesses, and other nonspecific symptoms. To our knowledge, this is the first report of MAA presenting as inguinal pain with inflamed phlegmonous tissue and scrotal abscess. Case presentation: A 67-year-old male presented to a rural facility complaining of weight-loss, fatigue, hematuria, dysuria, painful right inguinal ulceration, and right scrotal abscess drainage. Computed tomography of the abdomen and pelvis revealed a distended appendix (> 1.3 cm) and a fistula between the appendix, urinary bladder, right scrotum, and right groin. Laparoscopic appendectomy was performed and diagnosed as MAA. After a right hemicolectomy, the MAA was staged as pT3b pN0 M0 G2. Conclusion: This case highlights a unique presentation of late stage appendiceal MAA. Due to the increased incidence of appendiceal MAAs, reports of unique clinical features are needed to facilitate early diagnosis and intervention, especially in rural settings with limited access to specialists. Keywords: Case report, Appendiceal cancer, Inguinal pain, Inguinal ulcer, Scrotal abscess, Hematuria, Bladder mass
Background Appendiceal neoplasms are rare, accounting for less than 1% of intestinal neoplasms [1]. There are three different types of appendiceal neoplasms, which are defined by the World Health Organization as mucinous adenoma, low-grade appendiceal mucinous neoplasm (LAMN), and mucinous appendiceal adenocarcinoma (MAA) [2]. Among primary malignant neoplasms of the appendix, MAA presents most frequently (37% of all appendiceal neoplasms) and its incidence in the United States is * Correspondence: [email protected] 1 Pacific Northwest University of Health Sciences, Yakima, WA 98901, USA Full list of author information is available at the end of the article
rising [3, 4]. Mucinous adenomas are characterized as benign masses confined to the mucosa with intact muscularis. LAMN and mucinous adenocarcinoma have the potential to cause pseudomyxoma peritonei (PMP) due to excessive mucin production. PMP is a spread of gelatinous material (mucin) from the lumen of the appendix into the abdominal cavity, morphologically referred to as mucoceles [5]. The median time to PMP development after MAA onset is ~ 2 years [6]. With peritoneal metastasis, the five-year survival rate for mucinous adenocarcinoma is reported as low as 25% [6]. In addition, mucinous adenocarcinomas have a relatively higher risk for hematogenous and nodal metastasis [6]. Therefore,
© The Author(s). 2020 Open Access This article is licensed under a Creative Commons
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