A Case of Spontaneous Splenic Rupture After a Hemicolectomy on Anticoagulation.

Spontaneous or atraumatic splenic rupture (ASR) is a rare but life-threatening condition that requires swift recognition and intervention. We report the case of a 66-year-old female with a history of hypothyroidism, appendiceal goblet cell adenocarcinoma, and new-onset atrial fibrillation (Afib) requiring anticoagulation. She initially presented with right upper quadrant abdominal pain. She had previously undergone an appendectomy followed by a right hemicolectomy to achieve clear surgical margins after the appendiceal carcinoma diagnosis. In the post-anesthesia care unit, she developed Afib and was started on therapeutic anticoagulation. Cardiac catheterization later revealed three-vessel coronary artery disease, prompting a transition from heparin to apixaban. Three days later, the patient suddenly experienced left shoulder pain and was found to be diaphoretic and hypotensive. Three days post-catheterization, the patient developed sudden left shoulder pain, along with diaphoresis and hypotension. An initial concern for post-catheterization myocardial infarction was ruled out. A subsequent CT of the abdomen and pelvis revealed a large splenic hematoma with rupture and hemoperitoneum, necessitating emergent open splenectomy. Post-operatively, the patient required intensive care monitoring and transfusion support before being discharged to a long-term acute care facility. ASR is typically associated with identifiable pathological conditions; however, this case highlights the complexity of multifactorial etiologies. It emphasizes the need to consider ASR in patients presenting with sudden left shoulder pain and hemodynamic instability, particularly when anticoagulation therapy or recent abdominal surgery are factors. This case underscores the importance of a high index of suspicion and timely intervention to prevent fatal outcomes. Further research is warranted to explore the relationship between anticoagulation therapy and ASR.

[Appendiceal goblet cell adenocarcinoma: Has the controversy come to an end?] / Adénocarcinome à cellules caliciformes appendiculaire : la fin de la controverse ?

In 2019, the 5th edition of the WHO classification of digestive tumours has retained the terminology "goblet cell adenocarcinoma" (GCA) to designate a tumour whose amphicrine nature owed it more than ten denominations since its initial description among which the most tenacious "goblet cell carcinoid" is no longer recommended today. This rare tumour represents 15-19% of appendicular tumours. Its incidence is rising. The positive diagnosis is based on morphological examination and mandatory identification of a low-grade component of glands comprising goblet cells stained by PAS and Alcian blue. The appendix must be entirely examined. Global tumour grade (low, intermediate, high) is based on the proportions of low-grade and high-grade components. This tumour's immunohistochemical profile is particular because of expression of CK20 and often CK7 as well as neuroendocrine markers. It is often an incidental finding on a surgical specimen, among individuals aged 50 or more years, presenting with a locally advanced stage with vascular and perineural invasion. Lymph node metastases are present in a third of cases. Non-specific mutations of ARID1A and genes of the Wnt pathway may be identified. GCA is not associated with microsatellite instability or Lynch syndrome. Its prognosis is intermediate. Surgery is the reference therapy based on the stage. The main differential diagnoses are colorectal adenocarcinoma NOS, mucinous adenocarcinoma and signet ring cell adenocarcinoma. Patients are referred to the RENAPE expert network.

Goblet cell adenocarcinoma.

Appendix, considered a vestigial and disposable organ, has been long neglected as a source of abdominal tumors. Among the appendiceal tumors, goblet cell adenocarcinoma (GCA) is a rare primary epithelial neoplasm which has undergone multiple name changes and classifications in recent years, adding to confusion surrounding this unique amphicrine tumor. This entity was previously known as goblet cell carcinoid and adenocarcinoma ex goblet cell carcinoid. This review article provides an update on pathology, nomenclature, and recent classification systems with emphasis on 2019 World Health Organization Classification of Tumors, 3-tiered grading system.1.

Appendiceal goblet cell adenocarcinoma with perineural invasion extending into the ileocecal lesion.

BACKGROUND: Appendiceal goblet cell adenocarcinoma (GCA) is a rare subtype of primary appendiceal adenocarcinoma with an incidence of 1-5 per 10,000,000 people per year. Appendiceal tumors are often diagnosed after appendectomy for acute appendicitis. Notably, however, there is currently no standard treatment strategy for GCA, including additional resection. We report a case of appendiceal GCA with perineural extension into the cecum, in which ileal resection was considered effective. CASE PRESENTATION: A 41-year-old man was diagnosed with acute appendicitis and underwent appendectomy. Histopathological findings revealed GCA (T3, Pn1). He was referred to our hospital for additional resection. Preoperative examination indicated a diagnosis of GCA cT3N0M0. Laparoscopic ileocecal resection and D3 lymph node dissection were performed 2 months after initial appendectomy. The patient had a good postoperative course and was discharged 8 days after surgery. Histopathological findings showed a GCA invading the cecum, despite an intact appendiceal stump, no lymph node metastasis, no vascular invasion, and no horizontal extension into the submucosa. Direct invasion of the tumor through the serosa was not observed, but perineural extension was conspicuous in the cecum, suggesting that the GCA extended into the cecum via perineural invasion. The resection margins were negative. The patient has survived free of recurrence for a year after ileocecal resection. CONCLUSIONS: The current patient was diagnosed with appendiceal GCA following appendectomy for acute appendicitis. Despite intact of appendiceal stump and no evidence of lymph node or distant metastasis, he underwent laparoscopic ileocecal resection and D3 lymph node dissection 2 months after initial appendectomy, with a favorable outcome. Despite the detection of perineural invasion, the patient declined adjuvant therapy. This case suggests that extensive resection may be required in patients with appendiceal GCA, but the role of adjuvant therapy remains unclear.

Clinicopathological and molecular characterization of extra-appendix goblet cell adenocarcinomas.

Goblet cell adenocarcinoma (GCA) is a distinctive type of endocrine-exocrine mixed tumor, exhibiting intermediate morphological features between neuroendocrine tumor and adenocarcinoma. It predominantly arises in the appendix, but primary extra-appendiceal GCA is extremely rare. Here, we presented six cases of primary extra-appendiceal GCA from 2016 to 2022. Notably, one case was originating in the bladder which was the first report of primary GCA to occur outside the digestive tract. The tumors frequently displayed variable goblet cell morphology, characterized by cytoplasmic mucin accumulation and basally located nucleus. Low-grade components typically exhibited glandular or clustered patterns without prominent fibrotic responses. High-grade components demonstrated cribriform, cluster and single-file arrangement accompanied by marked fibrous reactions. Immunohistochemically, the tumors showed positivity for both neuroendocrine markers（synaptophysin, chromogranin A, CD56 ）and adenoids markers（CDX-2, CK20）. Next-generation sequencing revealed the most prevalent mutated genes within GCAs were TP53. Due to their morphological and immunohistochemical similarities to primary appendiceal GCA counterparts, we propose a distinct category for extra-appendiceal Goblet cell adenocarcinoma.

Right Hemicolectomy and Appendicectomy as Treatments for Goblet Cell Adenocarcinoma: A Comparative Analysis of Two Large National Databases.

INTRODUCTION: Right hemicolectomy (RHC) remains the treatment standard for goblet cell adenocarcinoma (GCA), despite limited evidence supporting survival benefit. This study aims to explore factors influencing surgical management and survival outcomes among patients treated with RHC or appendicectomy using NCRAS (UK) and SEER (USA) data. METHODS: A retrospective analysis was conducted using 998 (NCRAS) and 1703 (SEER) cases. Factors influencing procedure type were explored using logistic regression analyses. Overall survival (OS) probabilities and Kaplan-Meier (KM) plots were generated using KM analysis and the log-rank test compared survival between groups. Cox regression analyses were performed to assess hazard ratios. RESULTS: The NCRAS analysis revealed that age and regional stage disease were determinants of undergoing RHC, with all age groups showing similar odds of receiving RHC, excluding the 75+ age group. The SEER analysis revealed tumour size > 2 cm, and receipt of chemotherapy were determinants of undergoing RHC, unlike the distant stage, which was associated with appendicectomy. Surgery type was not a significant predictor of OS in both analyses. In NCRAS, age and stage were significant predictors of OS. In SEER, age, stage, and Black race were significant predictors of worse OS. CONCLUSIONS: The study shows variations in the surgical management of GCA, with limited evidence to support a widespread recommendation for RHC.

Appendiceal goblet cell adenocarcinoma newly classified by WHO 5th edition: a case report (a secondary publication).

BACKGROUND: Appendiceal goblet cell adenocarcinoma (AGCA) is a newly proposed cancer type in the 5th edition of the WHO Classification of Tumours in 2019. We experienced this rare form of appendiceal primary neoplasm. CASE PRESENTATION: An 85-year-old male presented a positive fecal occult blood test. A series of imagings revealed a type 1 tumor, located on the appendiceal orifice. The subsequent biopsy made the diagnosis of signet-ring cell carcinoma. Consequently, he underwent the laparoscopic-assisted ileocecal resection. Initially, the tumor was suspected to be a Goblet cell carcinoid (GCC). There was a discrepancy between the histological and immunostaining findings: the tumor cells exhibited morphological similarities to GCCs, however displayed limited staining upon immunostaining. Ultimately, we concluded that the tumor should be classified as AGCA, by following WHO 5th Edition. AGCA represents a newly categorized subtype of adenocarcinomas. Because of our preoperative suspicion of malignancy, we performed tumor resection with regional lymph node dissection, despite the fact that most appendiceal malignant tumors are typically identified after an appendectomy. CONCLUSION: We experienced a case that provides valuable insights into the comprehension of AGCA, a recently established pathological entity in the WHO 5th Edition. This article is an acceptable secondary publication of a case report that appeared in Azuma et al. (J Jpn Surg Assoc 83:1103-1108, 2022).

Bowel pseudo-obstruction caused by goblet cell adenocarcinoma of the appendix: A case report.

INTRODUCTION: Goblet cell adenocarcinoma of the appendix is a rare diagnosis with features of both adenocarcinomas and carcinoid tumors. Commonly presenting with chronic abdominal pain, appendicitis, or abdominal distention, it can also be incidentally discovered during appendectomies. CASE PRESENTATION: A 50-year-old man with right lower abdominal pain was admitted to our hospital, which is a critical care center. A computed tomography(CT) scan showed ileal narrowing, but endoscopy found no strictures. He was admitted with suspected bowel obstruction and improved with an ileal tube. Laparoscopic surgery revealed a tumor of the appendix. Histologically, he was diagnosed goblet cell adenocarcinoma, suggesting tumor infiltration of nerve fibers impairing peristalsis. DISCUSSION: Goblet cell adenocarcinoma of the appendix has unique histology and a poor prognosis. Treatment typically involves surgery and chemotherapy. This case highlights challenges in preoperative diagnosis, with the tumor causing bowel pseudo-obstruction by invading the intestinal wall and nerve plexus. Extensive infiltration of Auerbach's plexus was observed, consistent with the length of intestinal stenosis. CONCLUSION: This case describes goblet cell adenocarcinoma of the appendix leading to bowel pseudo-obstruction due to ileal end stenosis. It emphasizes the importance of considering this diagnosis in cases of bowel obstruction without an obvious mass.

Goblet Cell Adenocarcinoma in the Stomach: A Case Report.

Goblet cell adenocarcinoma (GCA) is known as an amphicrine tumor often seen in the appendix. Here, we report a rare case of GCA in the stomach. An 80-year-old man underwent gastroscopy due to epigastric pain and was diagnosed with gastric cancer. He received total gastrectomy and histology showed a mixture of a moderately-differentiated tubular adenocarcinoma, a mucinous adenocarcinoma, and a tumor composed of goblet-like mucinous cells with neuroendocrine differentiation. The tumor volume ratio was about 4:1:5, respectively, and a final diagnosis of GCA was made. The metastasis of the regional lymph node was occupied by only the component of goblet-like cells. GCA should be recognized as a rare histologic subtype of gastric cancer.

Case report: A rare case of coexistence of low-grade appendiceal mucinous neoplasia and goblet cell adenocarcinoma in the appendix.

Background: Primary appendiceal tumors are rare. Low-grade appendiceal mucinous neoplasia (LAMN) and goblet cell adenocarcinoma (GCA) account for 20% and 14% of primary appendiceal tumors, respectively. The coexistence of LAMN and GCA is an extremely rare event. This report presents a case of an elderly male patient with an appendiceal tumor composed of LAMN and GCA in the same appendix. Case presentation: A 72-year-old male patient was admitted to our institution presenting with a history of abdominal pain localized to the right lower quadrant for two months. Abdominal computed tomography (CT) showed a large dilated thickened cystic mass in the appendix, along with a small duodenal diverticulum. Laboratory tests indicated elevated levels of serum carcinoembryonic antigen (CEA) and cancer antigen 199 (CA19-9) markers. The patient underwent a laparoscopic right hemicolectomy and exploration of the duodenal diverticulum, and there was no finding of perforation of the duodenal diverticulum. Focal positivity for chromogranin A (CgA) and synaptophysin (Syn) was observed in the tumor cells of GCA. The final pathological diagnosis revealed the coexistence of LAMN staged pT4a and grade 1 GCA staged pT3 in the appendix. Unfortunately, the patient died due to severe septic shock and circulatory failure secondary to a perforated duodenal diverticulum. Conclusions: The coexistence of LAMN and GCA are extremely rare in the appendix and may result from the proliferation of two independent cellular lines. The coexistence of distinct neoplasms poses diagnostic and management challenges. Multidisciplinary team discussion may be essential in the effective management of these patients.

Peritoneal dissemination of appendiceal goblet cell adenocarcinoma mimicking white pus caused by peritonitis following appendicitis: an instructive case report.

BACKGROUND: Goblet cell adenocarcinoma is an extremely rare tumor in which the same cells exhibit both mucinous and neuroendocrine differentiation. It is considered more aggressive compared to conventional carcinoids and more likely to cause metastasis. CASE PRESENTATION: We report a case of goblet cell adenocarcinoma with peritoneal metastases. A 62-year-old man underwent appendectomy for acute appendicitis. Intraoperatively, inflammatory white pus and a small amount of dirty ascites were observed in the lower abdomen with severely inflamed appendix. Histopathological examination of the specimen collected during appendectomy revealed goblet cell adenocarcinoma with a positive surgical margin. One month later, additional ileal resection was planned. Laparoscopic examination revealed disseminated nodules throughout the abdominal cavity. Therefore, the patient underwent resection of the peritoneal nodules. The peritoneal specimens confirmed the histopathological findings. Thus we diagnosed the patient with peritoneal dissemination of appendiceal goblet cell adenocarcinoma. CONCLUSIONS: In cases wherein white pus is observed during surgery for acute appendicitis, considering the possibility of dissemination, collecting samples for histopathological examination, and initiating early treatment are crucial.

A rare goblet cell adenocarcinoma arising from Barrett's esophagus: the first reported case in the esophagus.

Goblet cell adenocarcinoma (GCA) is a rare and distinctive amphicrine tumor comprised of goblet-like mucinous cells and neuroendocrine cells. It is believed to originate from pluripotent stem cells located at the base of crypts. GCA predominantly arises from the appendix, with a few reported cases in extra-appendiceal locations such as the colorectum, small intestine, and stomach. In this case report, we present a unique instance of a 64-year-old male who initially received a diagnosis of neuroendocrine carcinoma in the distal esophagus based on biopsy but, following resection, was subsequently re-diagnosed with GCA arising from Barrett's esophagus.

Goblet cell adenocarcinoma: a case report and update regarding investigation and management.

This article describes the case of a patient who presented with an acute abdomen. Histopathology of the ruptured appendix identified Goblet Cell Adenocarcinoma. The biology of this rare tumour is now better understood, leading to updated best practise regarding investigation, staging and management.

Goblet cell adenocarcinoma of the appendix: A case report of three cases.

INTRODUCTION AND IMPORTANCE: Appendiceal goblet cell adenocarcinoma is in 0.3-0.9 % of appendectomy specimens. There is still controversy regarding whether surgery with dissection or additional resection is necessary for goblet cell adenocarcinoma and whether adjuvant chemotherapy is practical. We present three cases of goblet cell adenocarcinomas. CASE PRESENTATION: Case 1: A 30-year-old woman was diagnosed with appendicitis and underwent appendicectomy. Histopathological evaluation revealed a malignant neoplasm with goblet-like cells and tumour infiltration into the subserosa. The patient underwent laparoscopic ileocecal resection, and the main lymph nodes at the root of the feeding vessels were removed. Case 2: A 50-year-old man was diagnosed with appendicitis and underwent appendicectomy. Histopathological evaluation revealed a malignant neoplasm with goblet-like cells; malignant cells were found at the surgical resection margins. The patient underwent laparoscopic ileocolic resection. Case 3: A 60-year-old man undergoing treatment for malignant melanoma. He was diagnosed with appendicitis associated with an appendiceal tumour, and emergency laparoscopic caecal resection was performed and diagnosed as goblet cell adenocarcinoma. We decided to prioritize treatment for malignant melanoma, and the patient is under follow-up for goblet cell adenocarcinoma and no metastasis was detected. CLINICAL DISCUSSION: We performed additional resection in two case of goblet cell adenocarcinoma. Diagnosing appendiceal goblet cell adenocarcinoma is difficult, and the prognosis of patients with positive lymph nodes is poor. Surgical treatment should be considered for the advanced stages of this disease. CONCLUSION: Goblet cell adenocarcinoma, diagnosed after appendectomy, additional resection including lymph node dissection may provide a long-term prognosis.

Molecular Landscape and Association With Crohn Disease of Poorly Cohesive Carcinomas of the Nonampullary Small Bowel.

OBJECTIVES: Poorly cohesive carcinomas (PCCs) are neoplasms defined by a predominantly dyshesive growth pattern with single cell or cord-like stromal infiltration. The -distinctive clinicopathologic and prognostic features of small bowel PCCs (SB-PCCs) in comparison with conventional-type small intestinal adenocarcinomas have only recently been characterized. However, as SB-PCCs' genetic profile is still unknown, we aimed to analyze the molecular landscape of SB-PCCs. METHODS: A next-generation sequencing analysis through Trusight Oncology 500 on a series of 15 nonampullary SB-PCCs was performed. RESULTS: The most frequently found gene alterations were TP53 (53%) and RHOA (13%) mutations and KRAS amplification (13%), whereas KRAS, BRAF, and PIK3CA mutations were not identified. Most SB-PCCs (80%) were associated with Crohn disease, including both RHOA-mutated SB-PCCs, which featured a non-SRC-type histology, and showed a peculiar appendiceal-type, low-grade goblet cell adenocarcinoma (GCA)-like component. Rarely, SB-PCCs showed high microsatellite instability, mutations in IDH1 and ERBB2 genes, or FGFR2 amplification (one case each), which are established or promising therapeutic targets in such aggressive cancers. CONCLUSIONS: SB-PCCs may harbor RHOA mutations, which are reminiscent of the diffuse subtype of gastric cancers or appendiceal GCAs, while KRAS and PIK3CA mutations, commonly involved in colorectal and small bowel adenocarcinomas, are not typical of such cancers.

Goblet cell adenocarcinoma of the ascending colon: An underrecognized diagnostic pitfall.

Goblet cell adenocarcinomas (GCA) are infrequent neoplasms of the digestive system that exhibit both mucinous and neuroendocrine differentiation. They predominate in the appendix and rarely involve the colon. Herein, the authors report a case of GCA involving the ascending colon in a 60-year-old woman who presented with severe anemia.

Clinicopathological features of appendiceal goblet cell adenocarcinoma in Japan: a multicenter retrospective study.

PURPOSE: In the 5th edition of the World Health Organization classification, appendiceal goblet cell adenocarcinoma (GCA) is categorized separately from neuroendocrine tumors and other appendiceal adenocarcinomas. We clarified the clinicopathological characteristics of Japanese appendiceal GCA. METHODS: We designed a retrospective multicenter cohort study and retrieved the data of patients with appendiceal neoplasms and histologically diagnosed appendiceal goblet cell carcinoid (GCC) treated from January 2000 to December 2017 in Japan. The available GCC slides were reviewed and diagnosed with a new grading system of GCA. RESULTS: A total of 922 patients from 43 institutions were enrolled; of these, 32 cases were patients with GCC (3.5%), and 20 cases were ultimately analyzed. The 5-year survival rate was 61.4% (95% confidence interval: 27.4-83.2), and the median survival time was 93.1 months. For peritoneal metastasis, regional lymph node metastasis was a significant factor (p = 0.04), and Grade 3 was a potential factor (p = 0.07). No peritoneal metastasis was observed in either T1/2 patients (n = 2) or Grade 1 patients (n = 4). We were unable to detect any significant factors associated with regional lymph node metastasis. CONCLUSION: For peritoneal metastasis, regional lymph node metastasis was a significant factor, and Grade 3 was a potential factor.

Goblet cell adenocarcinoma of the appendix: an update and practical approach to diagnosis and grading.

Goblet cell adenocarcinoma is a rare appendiceal tumour with amphicrine differentiation that has distinct morphologic and clinical features compared to carcinomas seen elsewhere in the gastrointestinal tract. These tumors have engendered considerable confusion in the literature regarding their classification, and they have been described under several different names including goblet cell carcinoid, adenocarcinoid, and adenocarcinoma, among others. In the recent fifth edition of the World Health Organization Classification of Digestive System Tumors, goblet cell adenocarcinoma is the preferred diagnosis because of the increasing recognition of a frequent co-existing high-grade adenocarcinoma component. This review will present the clinicopathologic, molecular, and immunohistochemical features of goblet cell adenocarcinoma and discuss the current challenges in diagnosis, grading, and clinical management.

Clinicopathological spectrums and prognosis of primary appendiceal adenocarcinoma, goblet cell adenocarcinoma, and low-grade appendiceal mucinous neoplasms.

Primary appendiceal adenocarcinoma (APCA), goblet cell adenocarcinoma (GCA), and low/high-grade appendiceal mucinous neoplasms (LAMN/HAMN) are distinct entities with overlapping clinical presentation and histomorphology, leading to diagnostic challenges. We retrospectively reviewed our archived cases between 2010 and 2018 for diagnosis reappraisal and comparative analysis using updated terminology and modern parameters. A total of 87 cases (22 APCA, 40 GCA, and 25 LAMN pT≥3) were included. The entire cohort had 49 women and 38 men with a median age of 59.9 (range 26-88) years. There were no statistically significant differences in age and sex among the three groups. Clinically, patients with GCA were more likely to present with acute appendicitis (65%) and more likely to have appendectomy as initial surgery (68%). Both APCA and GCA were more likely to involve the proximal appendix while LAMN was more likely to involve the distal appendix (p<0.05). All APCAs were associated with mucosal precursor lesions, most commonly tubular, tubulovillous, or villous adenoma, flat LAMN/HAMN-pTis mucinous epithelium, or mixed, which correlated with distinct histomorphology, tumour differentiation, and stage. Although polypoid precursor lesions were rare in GCA, a significant proportion of GCA showed crypt atypia associated with neoplastic cells. Immunohistochemically, APCA had more frequent ß-catenin nuclear positivity and loss of SATB2 expression (p<0.05). KRAS mutation was more common in APCA than in GCA (8/11 vs 1/7, p<0.01). We further validated the three-tiered grading system (G1, G2, G3) in GCA, which correlated well with tumour stage and patient survival. APCA had worse progression-free and disease-specific survivals than GCA and LAMN (pT≥3) with the latter being relatively indolent even when perforated with peritoneal spread. Our study is the first comprehensive comparison between all three appendiceal neoplasms. We also describe a spectrum of previously under-recognised crypt atypia in GCA, which should trigger a diligent search for GCA if present.

Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry.

Background: Goblet cell adenocarcinoma (GCA) of the appendix is a rare and aggressive tumour with varying nomenclature and classification systems. This has led to heterogeneity in published data, and there is a lack of consensus on incidence, survival, and management. Methods: We provide an overview of GCA with a comprehensive systematic review using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology and a retrospective analysis of all cases recorded in the English National Cancer Registration and Analysis Service database between 1995 and 2018. The Kaplan-Meier estimator was used to calculate overall survival, and Cox proportional hazards regression was used to identify prognostic factors. Results: The systematic review demonstrated an incidence of 0.05-0.3 per 100,000 per year among North American registry studies. The 1-, 3-, and 5-year survival rate was 95.5%, 85.9%-87.6%, and 76.0%-80.6%, respectively. Age, stage, and grade were identified as prognostic factors for survival. Our analysis included 1,225 cases. Age-standardised incidence was 0.0335 per year in 1995 and gradually rose to 0.158 per year in 2018. The 1-, 3-, and 5-year survival rate was 90.0% [95% confidence interval (95% CI): 85.4-94.0], 76.0% (95% CI: 73.8-80.9), and 68.6% (95% CI: 65.9-72.2), respectively. On univariate Cox regression analyses, female sex, stage, and grade were associated with worse overall survival. On multivariate analysis, only stage remained a statistically significant prognostic factor. Conclusions: GCA of the appendix is rare, but incidence is increasing. We report a lower incidence and survival than North American registry studies. Higher stage was associated with decreased survival. Further prospective studies are required to establish optimal management.