Epithelial tumors of the appendix refer to neoplastic lesions originating from the epithelial tissue of the appendix mucosa. These neoplasms exhibit highly heterogeneous pathological features and biological behavior, which contribute to their strong propensity for peritoneal metastasis. Currently, evidence-based medicine regarding appendiceal epithelial neoplasms and the management of their peritoneal metastasis is limited, leading to a lack of standardized clinical practices. To address this, the Professional Committee of Integrated Rehabilitation for Peritoneal Tumors of the Chinese Anti-Cancer Association has organized multidisciplinary experts to focus on key aspects such as the pathological classification of epithelial tumors of the appendix, clinical staging of tumors,the indications for extended resection after local resection, the surgical treatment strategies for concurrent peritoneal metastasis, perioperative rehabilitation, and individualized treatment, while integrating the technical capabilities of relevant specialties. At the same time, it has standardized the perioperative management of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), as well as the clinical application of the pre-rehabilitation system, to enhance the practical operability. Ultimately, the Expert Consensus on the Comprehensive Management of Peritoneal Metastasis from Appendiceal Epithelial Neoplasms (2025 Edition) was developed. This consensus is aimed at further standardizing the systematic diagnosis and treatment process of epithelial tumors of the appendix, thereby reducing the risk of recurrence, improving patient prognosis, and promoting the standardization and homogenization of the diagnosis and treatment of peritoneal metastasis from such tumors.
Humans ; Appendiceal Neoplasms/therapy* ; Peritoneal Neoplasms/therapy* ; Cytoreduction Surgical Procedures ; Consensus ; Hyperthermic Intraperitoneal Chemotherapy

Objective: To analyzed perioperative safety of cytoreductive surgery (CRS) for patients with colorectal cancer peritoneal metastasis (CRPM) and to construct a predictive model for serious advese events (SAE). Methods: A descriptive case-series study was conducted to retrospectively collect the clinicopathological data and treatment status (operation time, number of organ resection, number of peritoneal resection, and blood loss, etc.) of 100 patients with peritoneal metastases from colorectal cancer or appendix mucinous adenocarcinoma who underwent CRS at the Sixth Affiliated Hospital of Sun Yat-sen University from January 2019 to August 2021. There were 53 males and 47 females. The median age was 52.0 (39.0-61.8) years old. Fifty-two patients had synchronous peritoneal metastasis and 48 had metachronous peritoneal metastasis. Fifty-two patients received preoperative neoadjuvant therapy. Primary tumor was located in the left colon, the right colon and the rectum in 43, 28 and 14 cases, respectively. Fifteen patients had appendix mucinous adenocarcinoma. Measures of skewed distribution are expressed as M (range). Perioperative safety was analyzed, perioperative grade III or higher was defined as SAE. Risk factors associated with the occurrence of SAEs were analyzed using multivariate logistic regression. A nomogram was plotted by R software to predict SAE, the efficacy of which was evaluated using the area under the ROC curve (AUC) and correction curves. Results: The median peritoneal cancer index (PCI) score was 16 (1-39). Sixty-eight (68.0%) patients achieved complete tumor reduction (tumor reduction score: 0-1). Sixty-two patients were treated with intraperitoneal hyperthermic perfusion chemotherapy (HIPEC). Twenty-one (21.0%) patients developed 37 SAEs of grade III-IV, including 2 cases of ureteral injury, 6 cases of perioperative massive hemorrhage or anemia, 7 cases of digestive system, 15 cases of respiratory system, 4 cases of cardiovascular system, 1 case of skin incision dehiscence, and 2 cases of abdominal infection. No grade V SAE was found. Multivariate logistic regression analysis showed that CEA (OR: 8.980, 95%CI: 1.428-56.457, P=0.019), PCI score (OR: 7.924, 95%CI: 1.486-42.259, P=0.015), intraoperative albumin infusion (OR: 48.959, 95%CI: 2.115-1133.289, P=0.015) and total volume of infusion (OR: 24.729, 95%CI: 3.956-154.562, P=0.001) were independent risk factors for perioperative SAE in CRS (all P<0.05). Based on the result of multivariate regression models, a predictive nomogram was constructed. Internal verification showed that the AUC of the nomogram was 0.926 (95%CI: 0.872-0.980), indicating good prediction accuracy and consistency. Conclusions: CRS is a safe and effective method to treat CRPM. Strict screening of patients and perioperative fluid management are important guarantees for reducing the morbidity of SAE.
Adenocarcinoma, Mucinous/therapy* ; Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Appendiceal Neoplasms/surgery* ; Colorectal Neoplasms/pathology* ; Combined Modality Therapy ; Cytoreduction Surgical Procedures/methods* ; Female ; Humans ; Hyperthermia, Induced/methods* ; Male ; Middle Aged ; Peritoneal Neoplasms/secondary* ; Retrospective Studies ; Survival Rate

OBJECTIVETo investigate clinicopathological features and prognostic factors of appendiceal neuroendocrine neoplasms(a-NEN).

METHODSClinical data of 20 patients diagnosed with a-NEN at Zhongshan Hospital of Fudan University between January 2000 and December 2016 were retrospectively analyzed. Pathological diagnosis was based on the WHO classification criteria of digestive system tumors (2010 edition). Based on the mitotic count and Ki-67 index, a-NENs were divided into grade 1 neuroendocrine tumor (NET G1), grade 2(G2) NET G2) and grade 3 (neuroendocrine carcinoma, NEC). Some special types of a-NEN (e.g. goblet cell carcinoid) and mixed adenoneuroendocrine neoplasms were classified as mixed adenoneuroendocrine carcinoma (MANEC). Follow-up was conducted by telephone or return visits. Univariate analysis was carried out using the Kaplan-Meier method, and the log-rank test was used to draw survival curves.

RESULTSOf 20 patients, 14 were male and 6 were female with median age of 54 years. Seventeen cases presented acute right lower quadrant abdominal pain, 1 chronic right lower quadrant abdominal pain, 1 persistent abdominal discomfort with outburst whole abdominal pain and 1 was found during body check without symptoms. Twenty cases comprised 8 G1 patients, 4 G2 patients, 3 G3 patients, and 5 MANEC patients. When diagnosed, there was 1 patient with liver metastasis, 1 patient with abdominal and pelvic metastases, and 2 patients with postoperative pathological findings of lymph node metastasis. Six patients underwent appendectomy, 12 underwent right hemicolectomy, 1 underwent right hemicolectomy plus small intestine resection, and 1 underwent partial hepatectomy plus right hemicolectomy. The follow-up time was 7-187 months(average, 36 months). The total 1- and 3-year survival rates were 94.7% and 60.2%, respectively. Univariate analysis showed that age >50 years (χ=7.036, P=0.008), pathology grade as MANEC (χ=5.297, P=0.021), and metastasis (χ=6.558, P=0.010) indicated lower 5-year survival rate.

CONCLUSIONSMost a-NEN patients have no typical symptoms, and the main complaint at consultation is acute right lower quadrant abdominal pain. Prognosis is poor for patients with age >50 years, MANEC pathology grade and metastasis.

Appendiceal Neoplasms ; complications ; diagnosis ; surgery ; Carcinoma, Neuroendocrine ; complications ; diagnosis ; therapy ; Female ; Gastrointestinal Neoplasms ; Humans ; Male ; Middle Aged ; Neuroendocrine Tumors ; complications ; diagnosis ; surgery ; Prognosis ; Retrospective Studies

OBJECTIVETo explore the clinicopathological characteristics and prognostic factors of primary appendiceal adenocarcinoma.

METHODSThe clinicopathological data of 42 patients with primary appendiceal adenocarcinoma treated in the Cancer Hospital of Chinese Academy of Medical Sciences between March 1994 and October 2009 were retrospectively analyzed. The survival analysis was conducted using Kaplan-Meier method. The factors influencing survival were analyzed using univariate (Log-rank) and multivariate (Cox) models.

RESULTSA total of 42 patients (29 female and 13 males, median age 56 years) with appendiceal adenocarcinoma were included in this study. Of them, 26 (61.9%) were mucinous adenocarcinoma, 12 (28.6%) were intestinal-type adenocarcinoma and 4 (9.5%) were signet cell carcinoma. 18 patients underwent curative resection, 20 patients received cytoreductive surgery, and 4 patients underwent biopsy only. Thirty patients received systemic chemotherapy (5-Fu-based regimens). One patient who died of postoperative pulmonary embolism on day 8 was excluded from the survival analysis. The overall 1-, 3-, and 5-year survival rate was 80.3%, 46.0% and 38.3%, respectively. Univariate analysis revealed that presence of symptoms of acute appendicitis, curative resection, histological grade, histological subtype, preoperative CEA level, systematic chemotherapy, and stage were all significant factors affecting the survival. Multivariate analysis showed that the preoperative CEA level (P = 0.01), histological grade (P = 0.001), and stage (P = 0.001) were independent prognostic factors.

CONCLUSIONSHigh level of CEA, G2/3 grade, and advanced stage are associated with poor prognosis in patients with primary appendiceal adenocarcinoma.

Adenocarcinoma ; drug therapy ; metabolism ; pathology ; surgery ; Adenocarcinoma, Mucinous ; drug therapy ; metabolism ; pathology ; surgery ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Appendectomy ; methods ; Appendiceal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Carcinoembryonic Antigen ; metabolism ; Carcinoma, Signet Ring Cell ; drug therapy ; metabolism ; pathology ; surgery ; Female ; Fluorouracil ; administration & dosage ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Neoplasm Grading ; Neoplasm Staging ; Proportional Hazards Models ; Retrospective Studies ; Survival Rate ; Young Adult

Pseudomyxoma peritonei is a rare disorder chracterized by abdominal distension resulting from the accumulation of a mucinous, gelatinous, translucent material which arises from rupture of pre-existing mucinous cystadenomas and cystadenocarcinomas of the ovary and mucocele of the appendix. We report 11 cases of pseudomyxoma peritonei which were diagnosed by surgical and pathological findings. The results were as follows. 1) The mean age of 11 cases was 51 years and 5 cases were male and 6 cases were female and male to female ratio was 1 to 1.2. 2) Clinical manifestations in 11 cases were abdominal distension in 5 cases (45.5%), palpable mass in 4 cases(36.6%), abdominal pain in 4 cases(36.6%), nausea in 3 cases(27.3%), weight loss in 3 cases(27.3%), diarrhea in 2 cases(18.2%), constipation in 1 case(9.0%), anal bleeding in 1 case(9.0%) and defecation difficulty in 1 case(9.0%). The duration of symptoms was 2 days to 1 year. 3) The primary sites of pseudomyxoma peritonei were ovarian cancer in 3 cases(27.2%), appendiceal cancer in 2 cases(18.2%), gastric cancer in 2 cases(18.2%), rectal cancer in 1 case(9.1%), cecal cancer in 1 case(9.1%) and unknown origin in 2 cases(18.2%). 4) Following surgical interventions were performed; right hemicolectomy in 2 cases, palliative gastrojejunostomy in 2 cases, oophorectomy in 1 case, omentectomy in 1 case and right hemicolectomy with gastrojejunostomy in 1 case. 5) The adjunctive anti-cancer chemotherapy was done in 4 cases(36.4%) by combination of actinomycin D, adriamycin, CCNU, cisplatin, cytoxan and fluorouracil, And chemotherapy only was done in 2 cases and hyperthermia was done in 1 case.
Abdominal Pain ; Appendiceal Neoplasms ; Appendix ; Cecal Neoplasms ; Cisplatin ; Constipation ; Cyclophosphamide ; Cystadenocarcinoma ; Cystadenoma, Mucinous ; Dactinomycin ; Defecation ; Diarrhea ; Doxorubicin ; Drug Therapy ; Female ; Fever ; Fluorouracil ; Gastric Bypass ; Gelatin ; Hemorrhage ; Humans ; Lomustine ; Male ; Mucins ; Mucocele ; Nausea ; Ovarian Neoplasms ; Ovariectomy ; Ovary ; Pseudomyxoma Peritonei* ; Rectal Neoplasms ; Rupture ; Stomach Neoplasms ; Weight Loss