Combination of CT imaging and endoscopy in diagnosis of appendicovesical fistula caused by appendiceal adenocarcinoma.

The appendiceal diseases, particularly appendicitis, are the most common disorders in the digestive system localized at the right lower quadrant area. However, appendiceal carcinoma with vesico-appendiceal fistula is a rare clinical phenomenon. Lacking specific symptoms, appendiceal carcinomas with fistula formations are often misdiagnosed as acute appendicitis cases. The purpose of this study is to increase awareness of appendiceal neoplasms and appendicovesical fistulas. We reported our experiences in three complex cases related to digestive and urological systems, and reviewed the literature on diagnosis with various X-ray imaging techniques for this lesion. In this report, the first case failed to be diagnosed. The other two patients with appendicovesical fistulas secondary to appendiceal adenocarcinomas were successfully detected with computed tomography (CT) and cystoscopy. The patients recovered after right hemicolectomies and en bloc partial cystectomies and survived without tumor metastasis up to 7-year follow-up. In conclusion, a combined use of CT imaging and endoscopy techniques provides an accurate diagnostic alternative for appendicovesical fistula secondary to appendiceal adenocarcinoma.

Impact of molecular alterations and targeted therapy in appendiceal adenocarcinomas.

UNLABELLED: Appendiceal adenocarcinomas (AAs) are rare and this has limited their molecular understanding. The purpose of our study was to characterize the molecular profile of AA and explore the role of targeted therapy against cyclooxygenase-2 (COX-2) and epidermal growth factor receptor (EGFR). PATIENTS AND METHODS: We performed a retrospective review of 607 patients with AA at a single institution. A total of 149 patients underwent molecular testing for at least one of the following: activating mutations in KRAS, BRAF, cKIT, EGFR, or PI3K; protein expression of c-KIT or COX-2; or microsatellite instability (MSI) status by immunohistochemistry. Kaplan-Meier product limit method and log-rank test were used to estimate overall survival (OS) and to determine associations among OS, COX-2 expression, KRAS mutations, and other characteristics. RESULTS: Age, grade, stage, signet ring cells, mucinous histology, and completeness of cytoreduction score correlated with survival outcomes. COX-2 expression, KRAS, PI3K, and BRAF mutations were seen in 61%, 55%, 17%, and 4% of patients, respectively. High MSI was seen in 6% of patients. KRAS mutation was strongly associated with well differentiated or moderately differentiated AA (p < .01). COX-2 expression (p = .33) and the presence of KRAS mutation (p = .91) had no impact on OS. The use of celecoxib in patients whose tumors expressed COX-2 (p = .84) and the use of cetuximab or panitumumab in patients with KRAS wild-type tumors (p = .83) also had no impact on OS. CONCLUSION: In this cohort, we demonstrated that COX-2 expression and KRAS mutations were frequently seen in AA, although neither exhibited any prognostic significance. MSI was infrequent in AA. Targeted therapy against COX-2 and EGFR appeared to provide no clinical benefit. Well and moderately differentiated AA were molecularly distinct from poorly differentiated AA.

Defining a role for systemic chemotherapy in local and advanced appendix adenocarcinoma.

BACKGROUND: Appendix adenocarcinomas (AAs) are rare tumours that often present late, with a propensity for peritoneal metastases (PMs). This study aimed to evaluate outcomes of AA patients undergoing cytoreductive surgery (CRS) with curative intent and determine the role of systemic chemotherapy. MATERIALS AND METHODS: Data were collected from a prospective database and classified according to World Health Organization (WHO) 2019 classification. Tumour clearance from CRS was described using a completeness of cytoreduction (CC) score ranging from 0 [no residual disease (RD)] to 3 (>2.5 cm RD). Patients with CC0-2 CRS received hyperthermic intraperitoneal chemotherapy (HIPEC). Systemic chemotherapy was categorised as 'prior' (>6 months before), 'neoadjuvant' (<6 months before), 'adjuvant' (<6 months after CC0-1 CRS) or 'palliative' (after CC2-3 CRS). Analyses used Kaplan-Meier and Cox regression methods. RESULTS: Between January 2005 and August 2021, 216 AA patients were identified for inclusion. Median age was 59 years (21-81 years). CRS/HIPEC was carried out in 182 (84%) patients, of whom 164/182 (76%) had mitomycin C HIPEC. CC0-1 was achieved in 172 (80%) patients. Systemic chemotherapy was given to 97 (45%) patients from the whole cohort and to 37/46 (80%) patients with positive nodes. Median overall survival (OS) was 122 months (95% confidence interval 61-182 months). After multivariate analysis, patients with acellular and lower-grade PM had similar OS to those with localised (M0) disease (P = 0.59 and P = 0.19). For patients with positive nodes, systemic chemotherapy was associated with reduced risk of death compared to no chemotherapy (P < 0.0019). CONCLUSION: This study identifies AA patients with positive lymph nodes derive the most benefit from systemic chemotherapy. We confirm the prognostic importance of stage and peritoneal grade, with excellent outcomes in patients with acellular mucin and lower-grade PM.

A case of appendix adenocarcinoma associated with ulcerative colitis.

Ulcerative colitis (UC) is a chronic relapsing inflammatory disorder of the colon. Patients with UC have an increased risk of developing colorectal cancer. However, appendix adenocarcinoma associated with UC is extremely rare.

Impact of high-risk features for stage II adenocarcinoma of the appendix.

BACKGROUND: Clinico-pathological high-risk features are frequently utilized in adjuvant chemotherapy (AC) decisions in stage II colorectal cancer and their utility in stage II appendiceal adenocarcinoma (AA) is not established. The aim of this study is to determine the impact of high-risk features in clinical outcomes and whether high risk features are predictive of AC benefit in stage II AA. METHODS: Patients with pathological stage II AA between 2010 and 2015 were identified from the National Cancer Database (NCDB) using ICD-O-3 morphology and topography codes: 8140, 8480 and C18.1. High risk stage II AA was defined as having at least one of the following clinicopathological features: T4 tumor, <12 lymph nodes examined, poorly differentiated histology, positive margins, or lymphovascular invasion. Patients with none of these features were defined as low-risk. RESULTS: A total of 1040 patients with pathological stage II AA were identified. 51.0% males, 84.5% Caucasian; median age 61 (range, 19-90). 46.4% were determined to have high-risk stage II AA. High-risk status was associated with worse OS compared to low-risk in univariate (HR 1.55; 95% CI 1.18-2.02; p = 0.001) and multivariable analyses (HR 1.36; 95% CI 1.03-1.79; p = 0.028). High-risk stage II AA patients had significantly worse 5-year OS compared to low-risk patients (67.1% vs. 74.5%, p = 0.0013). AC was administered in 34.4% (n = 166) of high-risk patients and in 36.5% (n = 203) of low-risk patients. Among high-risk patients, AC was not associated with better OS in univariate (HR 0.86; 95% CI 0.59-1.26; p = 0.448) and multivariable analyses (HR 1.35; 95% CI 0.90-2.04; p = 0.151) compared to no AC. Similarly, among low-risk patients, AC was not associated with better OS in univariate (HR 0.92; 95% CI 0.60-1.39; p = 0.679) and multivariable analyses (HR 1.27; 95% CI 0.81-2.02; p = 0.299) compared to no AC. For high-risk patients, 5-year OS was 68.3% in patients that received AC vs. 66.5% in patients that did not (p = 0.722). For low-risk patients, 5-year OS was 74.0% in patients that received AC vs. 76.3% in patients that did not (p = 0.813). CONCLUSION: High-risk stage II AA patients had significantly worse 5-year OS compared to low-risk patients. AC did not improve survival regardless of high-risk features in stage II AA in this retrospective study. A prospective randomized clinical trial would be required to determine the impact of high-risk features on AC in stage II AA.

Adenocarcinoma pobremente diferenciado de apéndice cecal: presentación de un caso clínico y revisión de la literatura / Poorly differentiated cecal appendix adenocarcinoma: presentation of clinical case and the review of literature

Presentamos un caso infrecuente de adenocarcinoma de apéndice cecal, la evolución de esta enfermedad, sus complicaciones y las expectativas que genera. Enfatizamos sobre el seguimiento en estos casos así como la importancia de contar con un equipo multidisciplinario formado por cirujanos, clínico y radioterapeutas oncólogos.

We present an uncommon case of cecal appendix adenocarcinoma, the evolution of the disease, along with its complications and the expenses that will follow this mortal disease. We will also emphasize which is the conduct to follow in these type of cases as well as the importance of counting with a multidiscliplinary specialized oncological team formed by surgeons, clinicians and oncological radiotherapists.

Adenocarcinoma de apêndice: relato de dois casos / Appendicecal carcinoma: report of two cases

The authors report two cases of patients with appendix adenocarcinoma, manifested as a syndrome of abdominal tumor of unknown origin. It was not possible to perform etiological diagnosis in the preoperative period for any of them. Literature data show that large locoregional tumor is a manifestation of appendix adenocarcinoma, although acute appendicites is the most frequent clinical manifestation. Preoperative diagnosis is rare and usually performed during laparotomy or through histopathological examination of the specimen. In large tumors, total mass resection including hemicolectomy should be carried out whenever possible. Whenever diagnosis of appendix adenocarcinoma is performed by the histopathological examination of the acute appendicites specimen, re-intervention is indicated for a right hemicolectomy.