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Appendix Cancers

Vermiform appendix and the Gastrointestinal SystemCancer of the Appendix (also known as appendiceal cancer) occurs when cells in the appendix become abnormal and multiply without control. There are a range of different types of tumours and cancers which can start in the appendix. About two thirds of appendix tumours are carcinoid tumours which develop in hormone-producing cells that are present in many parts of the body, including the appendix. Mucinous cystadenocarcinoma account for about a fifth of appendix tumours, these produces mucin (a jelly-like substance). Pseudomyxoma Peritonei (PMP) is a more common name given to the "jelly belly" syndrome caused by mucinous adenoma and mucinous adenocarcinoma. Less common tumours of the appendix include signet-ring cell adenocarcinoma and paraganglioma. Treatment varies according to the type and stage of tumour.

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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Seawell J, Sciarretta JD, Pahlkotter M, et al.
The Understated Malignancy Potential of Nonoperative Acute Appendicitis.
Am Surg. 2019; 85(7):712-716 [PubMed] Related Publications
Cancer of the appendix is rare and is most commonly found incidentally on pathology after an appendectomy for uncomplicated appendicitis (UA). The medical management alternative with antibiotics and observation remains an ongoing debate. The purpose of our study was to develop modern epidemiological data for adult patients completing an appendectomy for UA secondary to an appendiceal neoplasm (AN). ACS-NSQIP database was queried (2005-2016) to identify patients completing an appendectomy. Cohorts of patients who were diagnosed with UA and an AN were included in the study. Relevant perioperative clinical and outcomes data were collected. Type of AN, surgical procedure, and mortality were analyzed. A total of 239,615 UA patients were identified, of whom 2,773 (1.2%) met the inclusion criteria of AN. Patients with AN were predominantly white (79.5%), with a mean age of 54.5 ± 15.9 years, and 54.6 per cent were females. AN pathology findings included malignant neoplasm (64.5%), malignant carcinoid (17.3%), benign carcinoid (9.3%), and benign neoplasm (8.8%). The overall reported incidence was 1.2 per cent and the mortality rate was 0.7 per cent. Our study emphasizes surgical intervention in adult UA maintains a 1 per cent incidence of AN, and treatment with antibiotics alone will presumably lead to a delay in surgical treatment and progression of disease.

Lu P, McCarty JC, Fields AC, et al.
Risk of appendiceal cancer in patients undergoing appendectomy for appendicitis in the era of increasing nonoperative management.
J Surg Oncol. 2019; 120(3):452-459 [PubMed] Related Publications
BACKGROUND AND OBJECTIVES: Management practices for acute appendicitis are changing. In cases of nonoperative treatment, the risk of missed or delayed diagnosis of malignancy should be considered. We aimed to identify predictors associated with appendiceal cancer diagnosis after appendectomy for acute appendicitis.
MATERIALS AND METHODS: This retrospective cohort study was performed using the National Surgical Quality Improvement Program (NSQIP) appendectomy-targeted data set from 2016 to 2017. A total of 21 069 patients with imaging-confirmed or imaging indeterminate appendicitis who underwent appendectomy were included. Logistic regression was used to identify predictors of cancer diagnosis.
RESULTS: Increasing age had an increasing monotonic relationship with the odds of pathologic cancer diagnosis after appendectomy (age 50-59 odds ratio [OR], 2.08, 95% confidence interval [CI], 1.28-3.39, P = .003; age 60-69 OR, 2.89, 95% CI, 1.72-4.83, P < .001; age 70-79 OR, 3.85, 95% CI, 2.08-7.12, P < .001; age >80 OR, 5.32, 95% CI, 2.38-11.9, P < .001). Other significant predictors included obesity, morbid obesity, normal preoperative white blood cell count, and imaging indeterminate for appendicitis.
CONCLUSIONS: When counseling patients regarding operative vs nonoperative treatment options for management of acute appendicitis, the rising risk of a delayed or missed cancer diagnosis with increasing age must be discussed.

Lu P, Fields AC, Meyerhardt JA, et al.
Systemic chemotherapy and survival in patients with metastatic low-grade appendiceal mucinous adenocarcinoma.
J Surg Oncol. 2019; 120(3):446-451 [PubMed] Article available free on PMC after 01/09/2020 Related Publications
BACKGROUND: Appendiceal cancer is a rare malignancy that exhibits a wide range of histology and treatment response. Given the rarity and heterogeneous nature of the disease, it has been difficult to define optimal treatment strategies. Our goal is to examine the association between use of systemic chemotherapy and survival in patients with metastatic low-grade mucinous appendiceal adenocarcinoma.
METHODS: The National Cancer Database (2004-2015) was queried, and patients with mucinous, grade 1, stage IV appendiceal adenocarcinoma were identified. The Kaplan-Meier method was used to calculate survival, and a Cox regression model was used to identify predictors of survival.
RESULTS: Six hundred and thirty-nine patients were identified. Five-year overall survival (OS) for patients undergoing no chemotherapy vs chemotherapy was 52.9% and 61.3%, respectively. After adjusting with Cox proportional hazards model, chemotherapy was not associated with OS (HR:1.1, 95% CI:0.82-1.40, P = 0.61). Patients who underwent surgical resection (HR:0.40, 95% CI:0.28-0.57, P < .001) or were female (HR:0.61, 95% CI:0.5-0.8, P < .001) had improved survival in adjusted analysis.
CONCLUSIONS: There is no association between undergoing chemotherapy and OS in this cohort of patients with stage IV low-grade mucinous appendiceal adenocarcinoma. Development of national treatment guidelines is urgently needed for more consistency in the management of patients with appendiceal cancers.

Říha I, Třeška V, Nosek J, et al.
Rare causes of acute appendicitis.
Rozhl Chir. 2019; 98(5):189-193 [PubMed] Related Publications
INTRODUCTION: A clinical diagnosis of acute appendicitis does not necessarily imply only inflammatory changes found upon histopathological examination. Even though appendiceal tumors are very rare, accounting for less than 4% of all gastrointestinal tumors‚ they need to be taken into account and require the right choices regarding subsequent onco-surgical management.
METHODS: 1036 appendectomies were performed and 38 (3.7%) rare appendiceal tumor cases were identified at the department of Surgery in Pilsen from 1 January 2008 to 1 October 2018. The following text presents the difficult nature of the initial diagnosis, current therapy and principles of follow-up care in this relatively rare group of patients.
CONCLUSION: Appendiceal tumors are very rare. Depending on the histopathological type of tumor, it is necessary to choose adequate therapy and also provide proper follow up care.

Kim WS, Lee DG
Primary mixed adenocarcinoma and small-cell carcinoma of appendix: A case report (CARE-compliant).
Medicine (Baltimore). 2019; 98(19):e15614 [PubMed] Article available free on PMC after 01/09/2020 Related Publications
RATIONALE: Primary extrapulmonary small-cell carcinoma (SCC) of appendix is reported very rarely. We report herein a case of mixed SCC and adenocarcinoma of appendix.
PATIENT'S CONCERN: A 70-year-old female was consulted to our Emergency Department with the right lower abdominal pain and low-grade fever for 2 days.
DIAGNOSIS: Abdominal ultrasonography revealed the perforated appendicitis with periappendiceal abscess. Postoperative histology confirmed the diagnosis of mixed SCC and adenocarcinoma.
INTERVENTIONS: After laparoscopic appendectomy, she underwent right hemicolectomy for radical surgery.
OUTCOMES: Laparoscopic appendectomy was performed and histological examination showed mixed SCC and adenocarcinoma. After confirming that there was no other organ metastasis, right hemicolectomy was performed for radical surgery. Five months after surgery, the patient expired due to multiple organ metastases.
LESSONS: Further studies are required for better understanding of disease entities, and clinical trials are needed to define adequate treatment strategies for extrapulmonary SCC.

Ning S, Yang Y, Wang C, Luo F
Pseudomyxoma peritonei induced by low-grade appendiceal mucinous neoplasm accompanied by rectal cancer: a case report and literature review.
BMC Surg. 2019; 19(1):42 [PubMed] Article available free on PMC after 01/09/2020 Related Publications
BACKGROUND: Pseudomyxoma peritonei (PMP) is a disease involving the peritoneum characterized by the production of large quantities of mucinous ascites. PMP has a low incidence, is difficult to diagnose, and has a guarded prognosis. PMP induced by low-grade appendiceal mucinous neoplasm is extremely rare, and PMP accompanied by rectal cancer is even rarer.
CASE PRESENTATION: We present a unique case of a 70-year-old male with PMP induced by low-grade appendiceal mucinous neoplasm accompanied by rectal cancer. The patient's clinical, surgical, and histologic data were reviewed. The patient had persistent distended abdominal pain without radiating lower back pain, abdominal distension for 1 month, and no exhaustion or defecation for 4 days. A transabdominal ultrasound-guided biopsy was performed on the first day. The patient received an emergency exploratory laparotomy because of increased abdominal pressure. We performed cytoreductive surgery, enterolysis, intestinal decompression, special tumour treatment and radical resection of rectal carcinoma. The postoperative course was uneventful. The postoperative histological diagnoses were PMP, low-grade appendiceal mucinous neoplasm and rectal medium differentiated adenocarcinoma. At the 1-year follow-up visit, no tumour recurrence was observed by computed tomography (CT). We also performed a literature review.
CONCLUSIONS: We should be aware that PMP can rarely be accompanied by rectal cancer, which represents an easily missed diagnosis and increases the difficulty of diagnosis and treatment. Additionally, there are some typical characteristics of PMP with respect to diagnosis and treatment.

Xue P, Wang N, Mao Y, Zhu S
Anti-angiogenesis treatment in a patient with appendix metastasis of small cell lung cancer: A case report.
Medicine (Baltimore). 2019; 98(16):e15333 [PubMed] Article available free on PMC after 01/09/2020 Related Publications
RATIONALE: Small-cell lung cancer (SCLC) is a common pathological type of lung cancer, but appendiceal metastasis of SCLC was rare. At present, clinical studies on the maintenance therapy of SCLC have not reached a significant conclusion.
PATIENT CONCERNS: We reported on a 52-year-old man who diagnosed as extensive stage SCLC with abdominal pain for 2 months, aggravated for 2 days.
DIAGNOSES: The patient was diagnosed with extensive-stage SCLC, relapsed with appendix metastasis after treatment by emergency abdominal surgery.
INTERVENTIONS: The patient received systemic treatments, including surgery, bevacizumab in combination with chemotherapy and bevacizumab alone was continued as maintenance therapy.
OUTCOMES: The patient had an overall survival would more than 23 months, and he gained another 8 months of progression-free survival after first-line radiochemotherapy.
LESSONS: Although SCLC appendix metastasis is rare, continuous anti-angiogenic therapy combined with bevacizumab maintenance therapy after surgical treatment can prolong survival.

Yonemura Y, Canbay E, Wakama S, et al.
Analysis of Treatment Failure after Complete Cytoreductive Surgery for Peritoneal Metastasis from Appendiceal Mucinous Neoplasm at a Japanese High Volume Center for Peritoneal Surface Malignancy.
Gan To Kagaku Ryoho. 2019; 46(2):251-258 [PubMed] Related Publications
BACKGROUND AND OBJECTIVES: Treatment failure after complete cytoreduction for appendiceal mucinous carcinoma peritonei (AMCP)has not been fully investigated. The present study was performed to clarify the risk factor for recurrence after complete cytoreduction for AMCP.
METHODS: A total of 400 patients with AMCP who underwent complete cytoreductive surgery combined with perioperative chemotherapy were investigated.
RESULTS: Documented recurrence was developed in 135 (33.8%)patients. The 5- and 10-year progression-free survival was 51% and 49%, respectively. By multivariate analysis, histological subtype of peritoneal disease(high-grade AMCP[AMCP-H]and AMCP-H with signet ring cells), serum CA19- 9 level, and PCIB20 were significantly associated with reduced progression-free survival. In contrast, histologic subtype of mucin without epithelial cells(MWEC)showed the lowest risk for recurrence. Eighty-six patients had localized intra-abdominal recurrence, and 42 patients had diffuse peritoneal recurrence. Recurrence was found in the various peritoneal sectors. Eighty-one patients underwent complete cytoreduction for the recurrence, and the overall survival 5-year survival rate after secondary cytoreduction was 49%.
CONCLUSIONS: Risk factors for recurrence were histologic subtype, PCI cutoff level, and serum CA19-9 levels. Aggressive second attempt of cytoreduction in patients with localized recurrence improved the survival.

Eğin S, Kamalı G, Kamalı S, et al.
Neuroendocrine tumor of the appendix: Twelve years of results from a single institution.
Ulus Travma Acil Cerrahi Derg. 2019; 25(2):118-122 [PubMed] Related Publications
BACKGROUND: The aim of this study was to investigate the incidence of appendiceal neuroendocrine tumors (NET) in an acute appendicitis cohort, as well as to investigate the behavioral form of the tumor. Our secondary aim was to investigate survival in patients with appendiceal NET.
METHODS: Between February 2006 and June 2018, 6518 appendectomies were performed for acute appendicitis in the department of surgery, University of Health Sciences, Okmeydanı Education and Research Hospital, İstanbul, Turkey. The medical records of these consecutive 6518 appendectomy patients were evaluated retrospectively. After the histopathological analysis, a total of 22 patients were identified as cases of appendiceal NET. These patients were included in the study. A retrospective analysis of data including gender, age, intraoperative surgical findings, duration of postoperative follow-up and survival, tumor localization, the diameter of the tumor, tumor grade, invasion, surgical margin, and stage of the tumor was performed.
RESULTS: The incidence of appendiceal NET was 0.33%. Eleven patients were diagnosed as primary pathological stage pT1aN0M0 according to the European Neuroendocrine Tumor Society guidelines. One patient was diagnosed as primary pathological stage pT1bN0M0, and 10 patients were diagnosed as primary pathological stage pT2N0M0. The median tumor diameter was 7.6 mm. There was no patient with a tumor diameter greater than 20 mm.
CONCLUSION: The incidence of appendiceal NET in our study is consistent with that stated in the literature. The results of our research suggest that further surgical procedures for NETs that occur coincidentally in patients of the AA cohort are often unnecessary. In addition, the study revealed that disease-free survival (100%) was good over a mean follow-up of 59.2 months.

Masckauchan D, Trabulsi N, Dubé P, et al.
Long term survival analysis after hyperthermic intraperitoneal chemotherapy with oxaliplatin as a treatment for appendiceal peritoneal carcinomatosis.
Surg Oncol. 2019; 28:69-75 [PubMed] Related Publications
BACKGROUND AND OBJECTIVES: Complete cytoreductive surgery (CRS) followed by hyperthermic intraperitoneal chemotherapy (HIPEC) have been proven to lengthen survival in appendiceal peritoneal carcinomatosis (PC-A). The aim of this study was to analyze survival results of this therapy in our institution over the last 10 years.
METHODS: Data was retrospectively reviewed and analyzed. Treatment consisted of CRS plus HIPEC with oxaliplatin. Ronnett's histologic classification was used (peritoneal mucinous carcinomatosis (PMCA), PMCA with intermediate features (PMCA-I) and disseminated peritoneal adenomucinosis (DPAM)). Overall survival (OS) and disease-free survival (DFS) estimates were calculated using Kaplan-Meier survival curves.
RESULTS: 109 patients with PC-A underwent laparotomy with curative intent. Of those, 92 underwent CRS plus HIPEC. Median follow-up was 42 months. The 5 and 10-year OS rates for the HIPEC group were 82.2% and 76.5%. The 5 and 10-year OS estimates for DPAM and PMCA-I subgroups were 100% and 100%, 78.1% and 72.9%, respectively. For the PMCA subgroup, the 3 and 5-year OS were 61.4% and 40.1%, respectively. The 5 and 10-year DFS estimates were 71.9% and 42.7%.
CONCLUSION: CRS plus HIPEC with oxaliplatin represent an effective therapeutic approach for PC-A. Long term OS estimates for patients treated at our institution are encouraging.

Ion D, Serban MB, Păduraru DN, et al.
Appendiceal Mass - Dilemmas Regarding Extension of the Resection.
Chirurgia (Bucur). 2019 Jan-Feb; 114(1):126-130 [PubMed] Related Publications
Starting from a typical case, we try to highlight the main critical elements, both in the diagnostic algorithm and in the therapeutic one. We hereby present a 54-year old patient, known with hepatic steatosis and renal microlithiasis, which undergoes an ultrasonography in ambulatory, on the background of unsystematised abdominal discomfort; the ultrasound reveals the presence of a tumoral mass in the right iliac fossa.

Westfall KM, Brown R, Charles AG
Appendiceal Malignancy: The Hidden Risks of Nonoperative Management for Acute Appendicitis.
Am Surg. 2019; 85(2):223-225 [PubMed] Related Publications
One potential harm of nonoperative management for acute appendicitis is missed appendiceal cancer, a rare and often aggressive malignancy due to the frequency of late stage of diagnosis. Previous studies have reported an increasing incidence of appendiceal neoplasms in the population. This is a retrospective case-control study of 1007 adult patients, who presented to the University of North Carolina-Memorial Hospital (UNC-MH) between 2011 and 2015 with clinical signs and symptoms of appendicitis. We evaluated the incidence of primary appendiceal cancer in this population and determined factors that predict appendiceal cancer diagnosis using multivariate logistic regression analysis. The overall incidence of appendiceal neoplasm for adult patients presenting to UNC-MH with appendicitis from 2011 to 2015 was 2.3 per cent (23/1007). The incidence in patients without appendiceal perforation on pathology was 1.9 per cent (16/832). Age (odds ratio (OR) 1.03), number of days of abdominal pain (OR 1.16), self-reported fever (OR 2.08), appendiceal width (OR 1.95), and appendiceal wall thickness (OR 1.30) were predictors of appendiceal neoplasm diagnosis in patients that present with acute appendicitis. We recommend that an operative approach to acute appendicitis should remain the standard of care because operative management may not only be diagnostic but potentially therapeutic.

Sullivan BJ, Bolton N, Sarpel U, Magge D
A unique presentation of superinfected pseudomyxoma peritonei secondary to a low-grade appendiceal mucinous neoplasm.
World J Surg Oncol. 2019; 17(1):34 [PubMed] Article available free on PMC after 01/09/2020 Related Publications
BACKGROUND: Pseudomyxoma peritonei (PMP) is an uncommon condition characterized by diffuse mucinous material in the abdomen and pelvis, generally arising from a perforated epithelial neoplasm. Typically, the disease presents as suspected acute appendicitis, ovarian mass, abdominal distension, or ventral hernia. Our case represents a very rare presentation of superinfected PMP.
CASE PRESENTATION: A 46-year-old female with a past medical history notable for depression, asthma, and uterine leiomyomas presented to an urgent care with 5 days of progressive abdominal pain, bloating, nausea, and subjective fevers. The patient had a diffusely tender abdomen, without peritonitis, was mildly tachycardic, and had a white blood cell count of 15 K. A CT of the abdomen/pelvis was consistent with PMP with a ruptured appendiceal mucocele versus PMP secondary to an adnexal ovarian neoplastic pathology with an infectious component. The patient initially improved on antibiotics but ultimately required two surgeries, the first of which controlled intraabdominal sepsis while the second permitted definitive management of PMP with cytoreductive surgery (CRS) and HIPEC.
CONCLUSION: Superinfected PMP is a rare entity with very few documented cases. A staged approach that incorporates clearing the peritoneal infection, with or without resection of the primary tumor, followed by rehabilitation and definitive surgery appears to be a safe and effective management strategy.

Babcock B, Jabo B, Selleck M, et al.
Factors Predictive of Outcomes after Cytoreductive Surgery and Heated Intraperitoneal Chemotherapy for Colon and Appendiceal Carcinomatosis: A Single-Institution Experience.
Am Surg. 2018; 84(10):1575-1579 [PubMed] Related Publications
Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), although considered an acceptable treatment option in the management of selected patients with colon and appendiceal peritoneal carcinomatosis (PC), concerns about morbidity have limited its acceptance. Our objective was to evaluate the short- and long-term outcomes of CRS/HIPEC for appendix and colon PC performed at our institution and to elucidate factors predictive of patient outcomes. All patients who underwent CRS/HIPEC for appendix or colon PC from 2011 to 2017 were identified from our institution's prospective database. Postoperative outcomes, overall survival, and recurrence-free survival were assessed. Of 125 patients who underwent CRS/HIPEC during the study period, 45 patients were eligible (appendix n = 26; colon n = 19). The median postoperative length of stay was nine days (5-28 days). Grade III/IV complications occurred in 4/45 (8.8%) patients. There were no postoperative mortalities. Median DFS and overall survival have not yet been reached, in both the colon and appendix groups. As of the study conclusion date, 37/45 (82.2%) patients were alive with or without disease. Lymph node status was predictive of recurrence in appendix PC. In our experience, CRS/HIPEC can be safely performed with acceptable short- and long-term outcomes. Lymph node status is an important predictor of recurrence.

Aria AB, Chen L, Glass WF, et al.
Leukocytoclastic vasculitis with late-onset Henoch-Schönlein purpura after trifluridine/tipiracil treatment.
Dermatol Online J. 2018; 24(10) [PubMed] Related Publications
Trifluridine/tipiracil has been approved for the treatment of refractory metastatic colorectal cancer. Adverse effects of this drug combination include leukopenia, neutropenia, fatigue, diarrhea, and vomiting. We present a case of trifluridine/tipiracil-induced leukocytoclastic vasculitis (LCV) with late-onset Henoch-Schönlein purpura (HSP) in a 42-year-old man with metastatic appendiceal cancer. The patient's biopsy-proven LCV developed one month after he began trifluridine/tipiracil treatment and resolved after discontinuation of the drug. He presented to the emergency department two months after the appearance of his LCV with shortness of breath, elevated blood pressure, elevated creatinine, hematuria, and proteinuria. A kidney biopsy was performed and the presence of IgA deposits and cellular crescents indicated rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura (HSP). Neither LCV nor HSP have been reported as adverse effects of trifluridine/tipiracil treatment. Malignancy as a cause of our patient's HSP is another possibility. The delay between our patient's skin findings and acute renal failure indicates that suspected HSP should be monitored by urinalysis for a period of time owing to the risk of life-threatening renal disease.

Beane JD, Wilson GC, Sutton JM, et al.
Pleuropulmonary Recurrence Following Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemoperfusion for Appendiceal Pseudomyxoma Peritonei.
Ann Surg Oncol. 2019; 26(5):1429-1436 [PubMed] Related Publications
BACKGROUND: The aim of this study was to identify factors associated with pleuropulmonary disease recurrence following cytoreductive surgery with hyperthermic intraperitoneal chemoperfusion (CRS/HIPEC) for appendiceal pseudomyxoma peritonei (PMP) and to evaluate the oncologic impact of pleuropulmonary disease recurrence compared with isolated peritoneal recurrence.
METHODS: From a prospective database, we identified patients who developed pleuropulmonary recurrence, isolated peritoneal recurrence, or no recurrence following CRS/HIPEC for appendiceal PMP. Clinicopathologic, perioperative, and oncologic data associated with the index CRS/HIPEC procedure were reviewed. The Kaplan-Meier method was used to estimate survival. Multivariate analyses identified associations with recurrence and survival.
RESULTS: Of 382 patients undergoing CRS/HIPEC, 61 (16%) developed pleuropulmonary recurrence. Patients who developed a pleuropulmonary recurrence were more likely to have high-grade (American Joint Committee on Cancer [AJCC] grade 2/3) tumors (74% vs. 56%, p = 0.02) and increased operative blood loss (1651 vs. 1201 ml, p = 0.05) and were more likely to have undergone diaphragm stripping/resection (79% vs. 48%, p < 0.01) compared with patients with an abdominal recurrence. In a multivariate analysis, pleuropulmonary recurrence after CRS/HIPEC was associated with diaphragm stripping/resection, incomplete cytoreduction, and higher AJCC tumor grade. There was a trend towards reduced survival in patients with pleuropulmonary recurrence compared with patients with isolated peritoneal recurrence (median overall survival 45 vs. 53 months, p = 0.87).
CONCLUSION: Pleuropulmonary recurrence of appendiceal PMP following CRS/HIPEC is common and may negatively impact survival. Formal protocols for surveillance and therapeutic intervention need to be studied and implemented to improve oncologic outcomes.

Legué LM, Creemers GJ, de Hingh IHJT, et al.
Review: Pathology and Its Clinical Relevance of Mucinous Appendiceal Neoplasms and Pseudomyxoma Peritonei.
Clin Colorectal Cancer. 2019; 18(1):1-7 [PubMed] Related Publications
Until recently, many classifications existed for the terminology and histopathologic classification of appendiceal mucinous neoplasms, mucinous appendiceal adenocarcinomas, and pseudomyxoma peritonei (PMP). A major accomplishment was achieved by consensus-based histopathologic classifications on behalf of the Peritoneal Surface Oncology Group International regarding mucinous appendiceal tumours and PMP. As different classifications were used over the years and also owing to the rare nature of these tumors, many clinicians are not familiar with the terminology and the impact on patient management. Hence, an overview concerning mucinous appendiceal neoplasms, mucinous appendiceal adenocarcinomas, and PMP is provided to serve as an introduction into the basic morphology of these tumors with tentative recommendations for management.

Singh B, Singh G, Alzahrani N, et al.
Long term survival and perioperative propensity score matched outcomes in diaphragmatic interventions in cytoreductive surgery + intra-peritoneal chemotherapy.
Eur J Surg Oncol. 2019; 45(4):620-624 [PubMed] Related Publications
OBJECTIVES: To assess the impact of short and long term outcomes of diaphragmatic interventions in cyto-reductive surgery (CRS) and intra-peritoneal chemotherapy (IPC).
METHODS: 1230 consecutive CRS/IPC procedures were preformed between 1996 and 2018 in Sydney, Australia. Redo procedures and incomplete cyto-reductions were excluded. Among these, 599 underwent diaphragmatic intervention. Preoperative heterogeneity was assessed for in 6 parameters and addressed with propensity score matching. CRS/IPC requiring diaphragmatic interventions were compared to CRS/IPC without diaphragmatic involvement. Ten perioperative outcomes were measured. Overall survival was assessed based on diagnosis type.
RESULTS: Intraoperative results revealed a significant increase in operative hours (7.85 vs. 7.28, p = 0.033). Transfusion requirements were insignificantly different. Postoperatively, increased grade III and IV complications (36% vs. 26%, p = 0.052) were noted. There was no difference with regards to intensive care stay, hospital length of stay, hospital death and return to theatre. In terms of respiratory specific complications, an increased incidence of pneumothorax (13% vs. 3%, p = 0.001) and pleural effusions (24% vs. 16%, p = 0.043) were noted, whilst the differences in pneumonia were insignificant. Overall survival revealed diaphragm interventions; did not affect survival outcomes in colorectal cancers (p = 0.750, RR = 1.077, CI 0.683-1.697) and increased relative risk in low-grade appendiceal mucinous neoplasms (p = 0.025, RR = 2.437, CI 1.121-5.298).
CONCLUSION: After our three-tiered research strategy, we conclude that despite the marginal increase in short term morbidity; diaphragmatic interventions do not decrease survival in colorectal cancers and diaphragmatic disease in LAMNs maybe an independent prognosticator of disease aggression.

Shida D, Kanemitsu Y, Hamaguchi T, Shimada Y
Introducing the eighth edition of the tumor-node-metastasis classification as relevant to colorectal cancer, anal cancer and appendiceal cancer: a comparison study with the seventh edition of the tumor-node-metastasis and the Japanese Classification of Colorectal, Appendiceal, and Anal Carcinoma.
Jpn J Clin Oncol. 2019; 49(4):321-328 [PubMed] Related Publications
BACKGROUND: The eighth edition of the tumor-node-metastasis classification of malignant tumors updates cancer staging according to the evidence accumulated in the last 8 years since the release of the tumor-node-metastasis seventh edition. This review focuses on the new staging system.
METHODS: The eight edition was compared with the seventh edition as well as the Japanese Classification of Colorcetal, Appendiceal, and Anal carcinoma ninth edition.
RESULTS: Of colon and rectum, the tumor-node-metastasis eighth edition expands the M category. Specifically, colorectal cancer with peritoneal metastasis is newly categorized as M1c, distinguishing it from M1a (metastasis to one organ) and M1b (metastasis to more than one organ). In the ninth edition of Japanese Classification of Colorectal, Appendiceal, and Anal Carcinoma, M1c is further subdivided into M1c1 (metastasis to the peritoneum without other organ involvement) and M1c2 (metastasis to the peritoneum with other organ involvement). In the T category, the tumor-node-metastasis eighth edition excludes high-grade dysplasia (intraepithelial carcinoma) from Tis; this differs from both the tumor-node-metastasis seventh edition and the Japanese Classification of Colorectal, Appendiceal, and Anal Carcinoma ninth edition. In the N category, the tumor-node-metastasis eighth edition does not add the number of tumor deposits to the number of positive regional lymph nodes, whereas this number is added in the Japanese Classification of Colorectal, Appendiceal, and Anal Carcinoma ninth edition. The definition of anal cancer is also modified considerably in the tumor-node-metastasis eighth edition; specifically, tumors of perianal skin defined as within 5 cm of the anal margin are also classified as anal canal carcinoma, external iliac lymph nodes become regional lymph nodes, and both N2 and N3 are abolished in the N category. With regard to appendix, Tis (low-grade appendiceal mucinous neoplasma) and tumor deposit(s) are newly introduced. Finally, the tumor-node-metastasis eighth edition offers a new structure, labeled a 'prognostic factors grid', which consists of prognostic factors for survival in both colorectal and anal cancer.
CONCLUSIONS: Staging classification is updated regularly, which clinicians should always catch up with.

Zhang K, Meyerson C, Kassardjian A, et al.
Goblet Cell Carcinoid/Carcinoma: An Update.
Adv Anat Pathol. 2019; 26(2):75-83 [PubMed] Related Publications
Goblet cell carcinoid (GCC) or goblet cell carcinoma is a unique mixed endocrine-exocrine neoplasm that is almost exclusively seen in the appendix. The hallmark of GCC is the concentric infiltration of the appendiceal wall by small tight clusters, nests or cords of tumor cells that exhibit a goblet cell morphology with a small compressed nucleus and conspicuous intracytoplasmic mucin. The coexistence of high-grade adenocarcinoma with GCC has been increasingly recognized as a common finding, which has been called adenocarcinoma ex GCC or mixed GCC-adenocarcinoma. A number of studies have shown that it is the high-grade adenocarcinomatous component that dictates the prognosis. Several histologic classification/grading systems have been proposed, which correlate with overall patient survival. Treatment options are primarily based on tumor stage and the presence or absence of a high-grade adenocarcinomatous component.

Munoz-Zuluaga C, Sardi A, King MC, et al.
Outcomes in Peritoneal Dissemination from Signet Ring Cell Carcinoma of the Appendix Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy.
Ann Surg Oncol. 2019; 26(2):473-481 [PubMed] Related Publications
BACKGROUND: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is standard treatment for peritoneal dissemination from appendiceal cancer (AC); however, its role in high-grade histopathologic subtypes (high-grade mucinous carcinoma peritonei [HGMCP] and HGMCP with signet ring cells [HGMCP-S]) is controversial due to their aggressive behavior. This study analyzed clinical outcomes of high-grade AC after CRS/HIPEC.
METHODS: A prospective database of CRS/HIPEC procedures for HGMCP performed from 1998-2017 was reviewed. Perioperative variables and survival were analyzed.
RESULTS: Eighty-six HGMCP and 65 HGMCP-S were identified. HGMCP had more positive tumor markers (TM) (CEA/CA-125/CA-19-9) than HGMCP-S (63% vs 40%, p = 0.005). HGMCP had higher Peritoneal Cancer Index (32 vs 26, p = 0.097) and was less likely to have positive lymph nodes (LN) than HGMCP-S (28% vs 69%, p = < 0.001). Complete cytoreduction was achieved in 84% and 83%, respectively. PFS at 3- and 5-years was 59% and 48% for HGMCP vs 31% and 14% for HGMCP-S. Median PFS was 4.3 and 1.6 years, respectively (p < 0.001). OS at 3- and 5-years was 84% and 64% in HGMCP vs 38% and 25% in HGMCP-S. Median OS was 7.5 and 2.2 years, respectively (p < 0.001). LN negative HGMCP-S had longer median PFS and OS than LN positive HGMCP-S (PFS: 3.4 vs 1.5 years, p = 0.03; OS: 5.6 vs 2.1 months, p = 0.021).
CONCLUSIONS: The aggressive histology of HGMCP-S is associated with poor OS, has fewer abnormal TM, and is more likely to have positive LN. However, CRS/HIPEC can achieve a 5-year survival of 25%, which may improve to 51% with negative LN.

Feferman Y, Solomon D, Bhagwandin S, et al.
Sites of Recurrence After Complete Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Patients with Peritoneal Carcinomatosis from Colorectal and Appendiceal Adenocarcinoma: A Tertiary Center Experience.
Ann Surg Oncol. 2019; 26(2):482-489 [PubMed] Related Publications
BACKGROUND: This report describes patterns of disease recurrence after optimal cytoreduction (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for peritoneal carcinomatosis (PC) of colorectal (CRC) and appendiceal adenocarcinoma (AC) origin.
METHODS: Patients undergoing optimal CRS/HIPEC (2007-2016) at the authors' institution were retrospectively reviewed from a prospectively maintained database. Data regarding disease recurrence were analyzed.
RESULTS: Of 74 patients who underwent CRS/HIPEC for PC from CRC (n = 46) or AC (n = 28), 49 (66%) had recurrence during a median follow-up period of 39.5 months. The sites of recurrence were peritoneal-only (n = 34, 69%), hematogenous-only (n = 6, 12%), and combined peritoneal and hematogenous (n = 9, 19%) sites. No patients with AC had hematogenous-only recurrence. The median disease-free survival (DFS) time for all the patients was 15 months (95% confidence interval [CI] 12.5-17.5 months). The recurrence rate after CRS/HIPEC was 41% at 1 year, 73% at 3 years, and 76% at 5 years. All the patients with hematogenous-only metastases experienced recurrence within 12 months after CRS/HIPEC. Mucinous or signet ring features predicted peritoneal recurrence (p = 0.041), whereas a complete cytoreduction of 1 was a predictor of early recurrence (p = 0.040). Patients who underwent repeat cytoreduction survived longer than those who received systemic chemotherapy alone. The median survival time after peritoneal-only recurrence was 33 months (95% CI 27.8-38.9 months).
CONCLUSION: Recurrence for patients with PC is common, even after optimal CRS/HIPEC. Hematogenous-only recurrence occurs early after CRS/HIPEC, suggesting occult disease at the time of treatment and highlighting the need for methods to identify micro-metastases and improve patient selection. Patients experiencing peritoneal-only recurrence had long survival period after CRS/HIPEC, suggesting its effectiveness at controlling peritoneal disease for a time.

Zhang B, Zheng GL, Zhu HT, et al.
Clinicopathological characteristics and prognosis of primary appendiceal stromal tumors.
World J Surg Oncol. 2018; 16(1):225 [PubMed] Article available free on PMC after 01/09/2020 Related Publications
BACKGROUND: Gastrointestinal stromal tumors (GISTs) account for less than 1% of all gastrointestinal tumors. The biological behaviors of GISTs vary from benign to malignant. GISTs are common in the stomach (55.6%) and small intestine (31.8%), but rarely in the rectum, colon (6%), and other sites (5.5%). Currently, the majority of published reports of primary appendiceal stromal tumors (PASTs) are case reports or case series.
METHODS: The PASTs described in this study were identified from a literature review (23 cases) and our center (one case). The relationship between PAST gross types and clinicopathological factors was analyzed and summarized. At the same time, the study also analyzed the related risk factors and survival of PASTs and GISTs.
RESULTS: Twenty-four cases of PASTs were compared with 254 cases of GISTs from our center. The results showed that there was a significant difference between the two groups in tumor size (P < 0.001), histological type (P = 0.013), CD34 expression (P < 0.001), and DOG-1 expression (P < 0.001). Disease-free survival (DFS) analysis of 11 cases of PASTs and 227 cases of GISTs found that a comparison of 3-year and 5-year DFS was not statistically significant (P = 0.894 and P = 0.846, respectively). In the DFS multivariate analysis, tumor mucosal ulceration, tumor size, and NIH risk classification were independent prognostic factors in 3-year and 5-year DFS.
CONCLUSION: In this study, there was no significance in the survival of patients with appendix and gastric stromal tumors, which we hypothesized to be associated with the low sample size and incomplete follow-up records. Based on this, we conclude that the prognosis of primary appendiceal stromal tumors may be better than gastric tumors, but this needs to be confirmed in further prospective studies.

Roxburgh CS, Fenig YM, Cercek A, et al.
Outcomes of Low-Grade Appendiceal Mucinous Neoplasms with Remote Acellular Mucinous Peritoneal Deposits.
Ann Surg Oncol. 2019; 26(1):118-124 [PubMed] Article available free on PMC after 01/01/2020 Related Publications
BACKGROUND: Occasionally, low-grade appendiceal mucinous neoplasms (LAMN) present with mucinous peritoneal deposits (MPD) localized to periappendiceal tissue or diffused throughout the peritoneum.
OBJECTIVE: This study was aimed at evaluating the relevance of mucin cellularity for predicting outcomes of LAMN with remote MPD.
METHODS: The records of patients with LAMN and remote MPD who underwent initial assessment at a comprehensive cancer center from 1990 to 2015 were reviewed, and diagnostic procedures, treatments, and outcomes were analyzed.
RESULTS: Of 48 patients included in the analysis, 19 had cellular MPD (CMPD) and 29 had acellular MPD. Of 33 patients who underwent cytoreductive surgery, 30 had a complete cytoreduction; the 3 patients with an incomplete cytoreduction had CMPD. In the follow-up period (median, 4 years), 6 patients died of the disease, all of whom had CMPD. Of 11 patients who had progression of disease, 10 had CMPD.
CONCLUSION: Cellularity of remote MPD is an important determinant of disease outcome in LAMN. Approaches such as active surveillance may have a role in selected patients with LAMN and AMPD.

Votanopoulos KI, Mazzocchi A, Sivakumar H, et al.
Appendiceal Cancer Patient-Specific Tumor Organoid Model for Predicting Chemotherapy Efficacy Prior to Initiation of Treatment: A Feasibility Study.
Ann Surg Oncol. 2019; 26(1):139-147 [PubMed] Related Publications
INTRODUCTION: We have hypothesized that biofabrication of appendiceal tumor organoids allows for a more personalized clinical approach and facilitates research in a rare disease.
METHODS: Appendiceal cancer specimens obtained during cytoreduction with hyperthermic intraperitoneal chemotherapy procedures (CRS/HIPEC) were dissociated and incorporated into an extracellular matrix-based hydrogel system as three-dimensional (3D), patient-specific tumor organoids. Cells were not sorted, preserving tumor heterogeneity, including stroma and immune cell components. Following establishment of organoid sets, chemotherapy drugs were screened in parallel. Live/dead staining and quantitative metabolism assays recorded which chemotherapies were most effective in killing cancer cells for a specific patient. Maintenance of cancer phenotypes were confirmed by using immunohistochemistry.
RESULTS: Biospecimens from 12 patients were applied for organoid development between November 2016 and May 2018. Successful establishment rate of viable organoid sets was 75% (9/12). Average time from organoid development to chemotherapy testing was 7 days. These tumors included three high-grade appendiceal (HGA) and nine low-grade appendiceal (LGA) primaries obtained from sites of peritoneal metastasis. All tumor organoids were tested with chemotherapeutic agents exhibited responses that were either similar to the patient response or within the variability of the expected clinical response. More specifically, HGA tumor organoids derived from different patients demonstrated variable chemotherapy tumor-killing responses, whereas LGA organoids tested with the same regimens showed no response to chemotherapy. One LGA set of organoids was immune-enhanced with cells from a patient-matched lymph node to demonstrate feasibility of a symbiotic 3D reconstruction of a patient matched tumor and immune system component.
CONCLUSIONS: Development of 3D appendiceal tumor organoids is feasible even in low cellularity LGA tumors, allowing for individual patient tumors to remain viable for research and personalized drug screening.

Choudry MHA, Shuai Y, Jones HL, et al.
Postoperative Complications Independently Predict Cancer-Related Survival in Peritoneal Malignancies.
Ann Surg Oncol. 2018; 25(13):3950-3959 [PubMed] Related Publications
BACKGROUND: The authors hypothesized that postoperative complications after cytoreductive surgery-hyperthermic intraperitoneal chemoperfusion (CRS-HIPEC) have a negative impact on perioperative and oncologic outcomes and that the novel Comprehensive Comorbidity Index (CCI) would be a better predictor of such outcomes than the traditional Clavien-Dindo classification (CDC).
METHODS: The study used a prospective database of 1296 patients with peritoneal metastases (PM) undergoing CRS-HIPEC between 2001 and 2016. The Kaplan-Meier method was used to estimate survival. Multivariate analyses identified associations with perioperative and oncologic outcomes. The Akaike information criterion and the Schwarz (Bayesian information) criterion were used to compare model fitting for CCI versus CDC.
RESULTS: In this study, CRS-HIPEC was performed for malignant mesothelioma (12%) and PM from appendix (50%), colorectal (30%), and ovarian (8%) cancers. Major postoperative in-hospital complications (CDC grades 3-4) occurred for 24% of the patients. However, a range of CCI scores was calculated for each CDC grade because 36% of the patients experienced multiple complications. After a median follow-up period of 55 months, the median progression-free survival was 15 months, and the median overall survival was 39 months. In the multivariate Cox proportional hazards models, postoperative in-hospital complications (measured by CDC or CCI) were independent prognostic factors for 30-day post-discharge morbidity and readmission, as well as for survival. The CCI scores demonstrated higher prognostic sensitivity for these outcomes than CDC grades.
CONCLUSIONS: Reduction of postoperative complications after CRS-HIPEC is essential for optimal short- and long-term outcomes. For assessing total burden of postoperative complications per patient, CCI is superior to CDC and more sensitive for assessing surgery- and cancer-related outcomes after CRS-HIPEC.

Singh B, Singh G, Pourgholami M, et al.
Long-term Survival and Propensity Score Matched Outcomes of Bilateral
Anticancer Res. 2018; 38(10):5917-5921 [PubMed] Related Publications
BACKGROUND/AIM: To assess the impact of short- and long-term outcomes of bilateral vs. unilateral diaphragm interventions in cyto-reductive surgery (CRS) and intra-peritoneal chemotherapy (IPC).
PATIENTS AND METHODS: A total of 652 CRS/IPC procedures, between 1996 and 2018, required diaphragm interventions. Among these, 388 underwent bilateral intervention. Preoperative heterogeneity was assessed in 6 parameters and addressed with propensity score matching. The association of each respective analysis was assessed with 11 outcomes. Overall survival was assessed based on histology.
RESULTS: CRS/IPC requiring bilateral diaphragmatic interventions illustrated significantly increased operative hours (9.6 vs. 8.6 hours, p<0.001). Postoperatively, there was significantly increased red blood cell (RBC) transfusion (6.37 units vs. 4.47 units, p=0.007) and grade III and IV complications (57.3% vs. 40.6%, p=0.004). No difference was noted in ICU stay, total length of stay, hospital death and return to OT. In terms of respiratory complications, an increased incidence of pneumothorax (16.5% vs. 6.2%, p<0.001) was noted whilst pleural effusions and pneumonia occurrences were non-significant. Overall survival, revealed bilateral interventions in low-grade appendiceal mucinous neoplasm conferred an increased relative risk (p=0.037, RR=2.230, 95%CI=1.052-4.730). They did not have an effect on OS in colorectal cancer and mesothelioma.
CONCLUSION: Despite the increase in short-term morbidity, bilateral diaphragm interventions resulted in similar long-term survival to unilateral interventions.

Gündoğar Ö, Kımıloğlu E, Komut N, et al.
Evaluation of appendiceal mucinous neoplasms with a new classification system and literature review.
Turk J Gastroenterol. 2018; 29(5):533-542 [PubMed] Article available free on PMC after 01/01/2020 Related Publications
Appendiceal mucinous neoplasms constitute a diagnostic spectrum ranging from adenoma to mucinous adenocarcinoma. To date, many classification systems have been proposed to reflect the histomorphological diversity of neoplasms in this range and their clinical correspondence, and also to form a common terminology between the pathologist and clinicians. The aim of this review is to provide an updated perspective on the pathological features of appendiceal mucinous neoplasms. Using the 2016 Modified Delphi Consensus Protocol (Delphi) and the Eighth Edition of the American Joint Committee on Cancer (AJCC) Cancer Staging Manual, 19 cases presented from June 2011 to December 2016 were evaluated and diagnosed with appendiceal mucinous neoplasia. According to the Delphi, non-carcinoid epithelial tumours of the appendix were categorized in eight histomorphological architectural groups. These groups are adenoma, serrated polyp, low-grade appendiceal mucinous neoplasm, high-grade appendiceal mucinous neoplasm, mucinous adenocarcinoma, poorly-differentiated adenocarcinoma with signet-ring, signet-ring cell carcinoma and adenocarcinoma. The most common symptom was right lower quadrant pain. The median age of these cases was 60±15 years. There was a preponderance of females (F/M: 15/4). In our re-evaluation, six cases were diagnosed as serrated polyp. There were 11 cases in the LAMN group and two cases in the mucinous adenocarcinoma group. Using the Delphi and the AJCC manual, there were many changes in the classification, evaluation and treatment of appendiceal mucinous neoplasms. These classification systems have facilitated the compatibility and communication of clinicians and pathologists and have guided clinicians on treatment methods.

Akova F, Aydin E, Nur Eray Y, et al.
Long-term outcomes in pediatric appendiceal carcinoids: Turkey experience.
Eur J Pediatr. 2018; 177(12):1845-1850 [PubMed] Related Publications
The tendency of non-operative management of appendicitis let us explore the natural history of appendiceal carcinoids, compare them with appendicitis patients, and determine the possibility of deciding the extent of the surgery and post-operative follow-up on behalf of the intraoperative findings. A retrospective review was performed of patients with appendicitis between 2009 and 2017. Of 2781 patients, 10 (0.36%) were diagnosed with appendiceal carcinoids. Sixty percent were female with an average age of 13.10 ± 1.73. The mean tumor size was 0.97 ± 0.34 cm with 70% located at the tip. Majority had an insular pattern (n = 9), six had subserosal fat tissue invasion, one had extension to mesoappendix, one had vascular invasion, and two had lymphatic invasion. The average mitotic index was 3.20 ± 1.40/50HPF, and Ki 67 activity was 3 ± 1.7%. The mean follow-up period was 66.40 ± 25.92 months. Patients were further evaluated with ultrasonography (n = 10), CT (n = 3), and MRI (n = 10). Serum markers including chromogranin (n = 9), NSE (n = 6), and 5-HIAA (n = 6) were normal. None required further treatment and had any symptoms of carcinoid syndromes or recurrences post-operatively.Conclusion: Other than appendectomy, no additional surgery or follow-up is required in appendiceal carcinoids less than 1.5 cm in size, regardless of the lymphoid or vascular invasion. What is Known: • The treatment of patients with a 1-2-cm tumor is not clear in both the pediatric and adult populations, and additional resection is needed. • Patients are monitored post-operatively with radiological and/or biochemical testing. What is New: • Appendectomy is curative for tumors less than 2 cm. • No additional surgery or follow-up is required in appendiceal carcinoids less than 1.5 cm in size regardless of the lymphoid or vascular invasion.

Gilmore G, Jensen K, Saligram S, et al.
Goblet cell carcinoid of the appendix - diagnostic challenges and treatment updates: a case report and review of the literature.
J Med Case Rep. 2018; 12(1):275 [PubMed] Article available free on PMC after 01/01/2020 Related Publications
BACKGROUND: Goblet cell carcinoid is a rare but distinct entity of appendiceal tumors which is a hybrid or mixed tumor consisting of both epithelial (glandular) and neuroendocrine elements containing goblet cells. This entity is important to recognize and appropriately grade as it tends to be more aggressive than typical carcinoid tumors, often presenting with metastatic disease. As a result, the 5-year overall survival is 14-22% in stage III-IV disease. GCC therefore warrants more aggressive surgical and medical (chemotherapy) interventions than typical carcinoid tumors. Through this case report we give a brief update on GCC pathological features, staging, surgical management, and review the literature as a guide to indications for chemotherapy and choice of agents.
CASE PRESENTATION: We present the case of a 77-year-old Caucasian man with a history of stage I adenocarcinoma of transverse colon status post transverse colectomy who was incidentally found on surveillance colonoscopy to have an abnormal appendiceal orifice lesion. A biopsy revealed an appendiceal goblet cell carcinoid and he underwent a right hemicolectomy which revealed a pathologic stage III GCC for which he received eight cycles of adjuvant chemotherapy with capecitabine.
CONCLUSIONS: It is essential that patients who have tumors > 2 cm, are pT3 or pT4, have higher grade histology with signet ring (Tang grade B or grade C), locally advanced, or with positive surgical margins on appendectomy undergo a right hemicolectomy. Although there is no category 1 evidence, consensus recommendations are that patients with stage II (particularly Tang B and C) and stage III GCC be offered adjuvant chemotherapy with a regimen based on 5-fluorouracil, as these patients are known to have high rates of relapse.

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