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

Both carcinoembryonic antigen and CA19-9 are considered as predictive markers of intestinal cancer recurrence and metastasis.In addition,CA19-9 elevation is considered as a predictive marker of connective tissue disease-related interstitial lung disease.The incidence of oxaliplatin and capecitabine-associated interstitial lung disease is low,and there is no report about CA19-9 as a predictive marker of oxaliplatin and capecitabine-associated interstitial lung disease.This paper reports a case of interstitial lung disease with CA19-9 elevation caused by oxaliplatin and capecitabine adjuvant therapy for ileocecal carcinoma.The change trend of serum carcinoembryonic antigen in this patient was consistent with tumor recurrence and metastasis,and that of serum CA19-9 was consistent with the severity of interstitial lung disease.Therefore,CA19-9 elevation after intestinal cancer surgery does not necessarily indicate the tumor recurrence and metastasis,and attention should be paid to the possibility of oxaliplatin and capecitabine-associated interstitial lung disease.
Humans ; CA-19-9 Antigen/blood* ; Capecitabine ; Cecal Neoplasms/drug therapy* ; Chemotherapy, Adjuvant ; Deoxycytidine/administration & dosage* ; Fluorouracil/administration & dosage* ; Lung Diseases, Interstitial/blood* ; Organoplatinum Compounds/administration & dosage* ; Oxaliplatin

Objective: To investigate the clinical features, morphological characteristics, immunophenotype, and differential diagnosis of goblet cell adenocarcinoma (GCA) in the digestive system. Methods: The clinicopathological data, morphological characteristics, immunophenotypes of 22 cases of GCA in the digestive system diagnosed from January 2010 to January 2021 were collected. Meanwhile, 25 cases of neuroendocrine neoplasm (NEN) and 24 cases of adenocarcinoma were used as controls. Relevant literature was also reviewed. Results: There were 16 males and 6 females, aged from 36 to 79 years with an average of 56 years. The anatomical sites of the 22 GCA were mostly appendix (17 cases) and occasionally extra-appendix (5 cases), including 3 cases in stomach, 1 case in duodenum and 1 case in anal. All 17 cases of appendiceal GCA were pure GCA. Among the 5 cases of extra-appendiceal GCA, One case of gastric GCA was pure, two cases of gastric GCA with NEN or adenocarcinoma, duodenal GCA with NEN and adenocarcinoma, anal GCA with NEN.Low-grade GCAs were composed of goblet, Paneth and neuroendocrine cells, which were arranged in intestinal crypt tubular or cluster structures and distributed in the wall of digestive system. The tubular and cluster structures lacked adhesion. Goblet cells were columnar, located in the base, with clear cytoplasm, small nuclei, inconspicuous atypia, and uncommon mitoses. Extracellular mucus and signet-ring cells with nuclear variations could be seen in some cases. Nerve fiber bundle invasion and tumor thrombus in vessels were often present. High-grade GCAs lacked tubular and cluster structures, and their histological structures were more complex. Tumor cells expressed mixed neuroendocrine and glandular epithelial markers. Similar to the expression patterns of synaptophysin and chromogranin A, CD200 and INSM1 were also dot-like or patch-positive in GCA. Conclusions: GCA is an infrequent tumor of the digestive system and shows the bi-directional differentiation characteristics of neuroendocrine and glandular epithelium. Accurate diagnosis and staging are related to its prognosis.
Adenocarcinoma/pathology* ; Appendiceal Neoplasms/surgery* ; Carcinoid Tumor/surgery* ; Chromogranin A ; Female ; Goblet Cells/pathology* ; Humans ; Male ; Neuroendocrine Tumors/pathology* ; Repressor Proteins ; Synaptophysin

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

Objective: To investigate the clinicopathological features, surgical methods and prognosis of primary appendiceal neoplasms. Methods: A descriptive case series study was performed. Clinical data of patients diagnosed with primary appendiceal neoplasms in Peking University First Hospital from 2006 to 2017 were retrospectively analyzed. Patients who underwent surgery and were confirmed as appendiceal neoplasms by postoperative pathology were included. Cases of cecal tumor invading the appendix and other organ tumors implanting in the appendix and cases of recurrent appendix tumors were excluded Pathological classification was based on the 4th edition of the WHO classification of digestive tract tumors (2010 edition), and the efficacy of operation methods of low grade appendiceal mucinous neoplasm (LAMN) were analyzed. Results: A total of 115 patients were enrolled, including 52 males and 63 females with a median age of 59 (51, 71) years. Clinical symptoms usually manifested as dormant pain in the right lower quadrant, migrating right lower abdominal pain, fever and bloating. Twenty-four cases were accidentally discovered during surgery, and 21 cases were found by physical examination. The preoperative diagnosis rate of CT and ultrasound was 40.2% (43/107) and 25.5% (24/94) respectively. The postoperative pathological types contained 83 cases of LAMN, 12 cases of mucinous adenocarcinoma, 9 cases of appendiceal neuroendocrine neoplasms (aNEN), 2 cases of mucinous adenocarcinoma with signet ring cells, 3 cases of serrated adenoma, 2 cases of goblet cell carcinoid, 2 cases of lymphoma, 1 case of leiomyoma and 1 case of schwannomas. All the patients underwent surgical resection, including 41 cases of appendectomy, 21 cases of partial cecectomy, 48 cases of right hemicolectomy, and 5 cases of combined organ resection due to appendiceal tumor infiltration or dissemination. Eighteen cases were diagnosed with pseudomyxoma peritonei (PMP) during operation. A total of 98 patients were enrolled for follow-up. The median follow-up time was 58 (5-172) months. The 5-year disease-free survival (DFS) rate was 84.5% and 5-year overall survival (OS) was 88.2%. Multivariate analysis revealed that high-grade malignancy tumors (HR=25.881, 95% CI: 2.827-236.935, P=0.004) and PMP formation (HR=42.166, 95% CI: 3.470-512.439,P=0.003) were independent risk factors for prognosis. Patients undergoing right hemicolectomy presented longer operation time, more blood loss and higher morbidity of complication as compared to those undergoing appendectomy and partial cecectomy (all P<0.05), while no significant differences in 5-year DFS (P=0.627) and 5-year OS (P=0.718) were found. Conclusions: Primary appendiceal neoplasms usually have no typical features, accompanied with low preoperative diagnosis rate. The common pathological types are LAMN, mucinous adenocarcinoma and aNEN. Appendectomy or partial cecectomy for LAMN may achieve satisfactory prognosis. High-grade malignancy tumors and PMP formation are independent risk factors for prognosis.
Adenocarcinoma, Mucinous/surgery* ; Appendectomy ; Appendiceal Neoplasms/surgery* ; Humans ; Male ; Neoplasm Recurrence, Local ; Retrospective Studies

Acute appendicitis (AA) is one of the most common causes of acute abdominal pain, which can progress to perforation of the appendix and peritonitis. Recently, AA has been classified into uncomplicated (nonperforated, no phlegmon) or complicated (abscess, perforation, phlegmon) appendicitis, for an appropriate initial treatment. With respect to surgical treatment of AA, laparoscopic surgery has been widely accepted worldwide as a safe and feasible first-line treatment. Over the last decade, non-operative treatment has been proposed as an alternative to surgery in uncomplicated AA, and has also played an important role in the management of complicated AA. AA is also the most common cause for abdominal surgery during pregnancy, though an accurate diagnosis of AA during pregnancy is challenging. In this review, the topics being discussed include: 1) Non-operative management for uncomplicated AA, 2) Management for AA in pregnancy, 3) Management for complicated appendicitis (especially immediate laparoscopic surgery for appendiceal abscess), 4) Appendiceal neoplasms related to complicated AA.
Abdominal Pain ; Appendiceal Neoplasms ; Appendicitis ; Appendix ; Diagnosis ; Laparoscopy ; Peritonitis ; Pregnancy

The da Vinci SP Surgical System (dVSP; Intuitive Surgical, Sunnyvale, CA, USA) was introduced to perform pure single-incision surgery in 2018. This new surgical platform demonstrated favorable performance compared with the positive aspect of single-incision laparoscopic surgery and robot surgery. To date, its use has mainly been in urological and gynecological procedures. We report a case of successful robotic single-incision right hemicolectomy for cecal cancer with the dVSP.
Cecal Neoplasms ; Laparoscopy

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

Lymph node metastasis in early colon cancer is relatively low. Furthermore, skip metastasis beyond principle lymph node is very rare. This is a case of early cecal cancer with skip metastasis to portocaval and retropancreatic space, without regional lymph node metastasis. A 69-year-old female diagnosed as cecal adenocarcinoma. The imaging study revealed as early cecal cancer without enlargement of regional lymph node. However, there is enlargement of portocaval lymph node and high fluorodeoxyglucose (FDG) uptake in positron emission tomography scan image. Right hemicolectomy with extended lymph node dissection was done including retropancreatic, portocaval and hepatoduodenal ligament lymph node. Though whole abdominal cavity exploration was done, there was no evidence of other synchronous cancer. The final pathologic findings revealed the poorly differentiated adenocarcinoma with invasion of submucosal layer and focally superficial layer of muscularis propria. Two of total 27 lymph nodes were involved by metastastatic adenocarcinoma which were from portocaval and retropancreatic space. The detailed preoperative imaging study could find unexpected lymph node metastasis beyond range of routine lymph node dissection. Even though the preoperative clinical stage is relatively early, the detailed and sufficient evaluation for clinical and imaging findings is important not to ignore skip metastasis.
Abdominal Cavity ; Adenocarcinoma ; Aged ; Cecal Neoplasms ; Colonic Neoplasms ; Female ; Humans ; Ligaments ; Lymph Node Excision ; Lymph Nodes ; Lymphatic Metastasis ; Neoplasm Metastasis ; Positron-Emission Tomography

PURPOSE: Appendiceal tumors are a heterogeneous group of diseases that include typical neuroendocrine tumors (TNET), goblet cell carcinoids (GCC), and atypical GCC. Atypical GCC are classified into signet-ring cell cancers (SRCC) and poorly differentiated appendiceal adenocarcinoids. The prognosis and management of these diseases is unclear because there are no prospective studies. The aim of this study is to assess the characteristics and outcome of appendiceal TNET, GCC, and SRCC patients. MATERIALS AND METHODS: Appendiceal TNET, GCC, and SRCC patients diagnosed between 1973 and 2011 were identified in the Surveillance Epidemiology and End Results (SEER) database. Demographics, type of surgery, and clinicopathologic characteristics were collected. Survival functions were estimated by the Kaplan-Meier method, and log-rank test was used to assess the difference in overall survival (OS) among the three histologies. RESULTS: The SEER database yielded 1,021 TNET patients, 1,582 with GCC, and 534 SRCC patients. TNET presented at a younger age (p < 0.001). Patients with SRCC presented with advanced stage disease (p < 0.001). The median OS (mOS) for GCC and TNET patients was not reached; mOS for SRCC was 24 months. Multivariate analysis stratified for stage revealed significantly longer survival for TNET and GCC than SRCC (p < 0.001). CONCLUSION: This is the largest report to date for appendiceal neuroendocrine tumor patients, suggesting a spectrum of diseases with different characteristics and outcomes. In this report, we present a treatment approach for this complex spectrum of disease, based on the experience of Ohio State and Emory Universities investigators.
Appendiceal Neoplasms ; Carcinoid Tumor ; Demography ; Disease Management ; Epidemiology ; Goblet Cells ; Humans ; Multivariate Analysis ; Neuroendocrine Tumors ; Ohio ; Prognosis ; Prospective Studies ; Research Personnel