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

Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a mass-like swelling of the appendix, in order to avoid unnecessary surgery.
Appendiceal Neoplasms/*diagnosis ; Appendicitis/*diagnosis ; Appendix/immunology/*pathology ; Autoimmune Diseases/*diagnosis/immunology ; Diagnosis, Differential ; Humans ; Immunoglobulin G/*immunology ; Male ; Middle Aged ; Neoplasms

Neuroendocrine tumor (NET) is a cancer-like tumor that occurs mostly in the gastrointestinal system. Within the gastrointestinal tract, NET most commonly occurs in the rectum whereas appendix is very rarely involved. In most cases of appendiceal NET, it is found at a relatively early stage compared to other NETs because appendiceal NET frequently presents with acute appendicitis because appendiceal NET frequently presents with acute appendicitis even when the size is smaller than 1 cm. Therefore, it is very rare for lymph node metastasis to occur in a young adult. Herein, we report a rare case of grade 1 appendiceal NET with lymph node metastasis which developed in a teenage male.
Adolescent ; Appendiceal Neoplasms/*diagnosis/pathology/surgery ; Carcinoid Tumor/diagnosis/pathology ; Colectomy ; Colonoscopy ; Humans ; Lymphatic Metastasis ; Male ; Neuroendocrine Tumors/*diagnosis/pathology ; Tomography, X-Ray Computed

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Appendectomy ; Appendiceal Neoplasms ; complications ; metabolism ; pathology ; surgery ; Appendicitis ; etiology ; surgery ; Diagnosis, Differential ; Female ; Granular Cell Tumor ; complications ; metabolism ; pathology ; surgery ; Humans ; Paraganglioma ; metabolism ; pathology ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism

OBJECTIVETo investigate the clinicopathologic characteristics, prognosis and histologic origin of the mucinous tumor of the peritoneum.

METHODSAccording to 2010 WHO classification of tumours of the digestive system, 34 cases diagnosed as "pseudomyxoma peritonei (PMP) " were reevaluated and divided into low grade and high grade. Immunohistochemistry was applied to investigate the expression of SATB2 and the histologic origin of the mucinous tumor of the peritoneum, using antibodies against SATB2, CK7, CK20 and CDX-2. The relationship between clinicopathologic characteristics and prognosis of the low grade and high grade tumors were analyzed.

RESULTSTwenty five patients had low grade mucinous tumors (two of them were no cell type), nine patients had high grade mucinous tumors. There was no significant difference between low grade and high grade mucinous tumors in age, sex, recurrence and organs involvement (P>0.05). Thirty patients were followed up, the overall survival rates of patients with low grade and high grade mucinous tumors were 13/21 (61.9%) and 3/9, respectively. The median survival time was 74 and 24 months in low and high grade patients, and the difference was statistically significant (P=0.002).Immunohistochemistry showed the expression rates of CDX-2, CK20, and CK7 in totally 32 cases (excluding 2 cases of no cell type) were 30/32(93.8%), 31/32 (96.9%), and 3/16, respectively; the expression rates of CDX-2, CK20, and CK7 in 16 cases with distinct primary site were 15, 16, and 1, respectively; fifteen of 16 cases of tumors of unknown primary site were positive for CDX-2 and CK20, two of the them were positive for CK7. There was no difference in the expression of CDX-2, CK20 and CK7 between tumors with distinct primary site and tumors with unknown primary site (P>0.05). The expression rate of SATB2 in the cases was 56.3% (18/32), excluding 2 cases of no cell type. There was no significant difference between low grade and high grade tumors in the expression of SATB2 [15/23(65.2%) vs 3/9, P=0.102], also SATB2 was not related to the prognosis of the tumor (P=0.786).

CONCLUSIONThe prognosis of the mucinous tumor of the peritoneum was significantly different between low grade and high grade according to WHO 2010 classification, and most mucinous tumor of the peritoneum originated from the appendix.

Adenocarcinoma, Mucinous ; metabolism ; pathology ; secondary ; surgery ; Adult ; Aged ; Aged, 80 and over ; Appendiceal Neoplasms ; pathology ; surgery ; CDX2 Transcription Factor ; Female ; Follow-Up Studies ; Homeodomain Proteins ; metabolism ; Humans ; Keratin-20 ; metabolism ; Keratin-7 ; metabolism ; Lymphatic Metastasis ; Male ; Matrix Attachment Region Binding Proteins ; metabolism ; Middle Aged ; Neoplasm Invasiveness ; Neoplasm Recurrence, Local ; Peritoneal Neoplasms ; metabolism ; pathology ; secondary ; surgery ; Pseudomyxoma Peritonei ; metabolism ; pathology ; surgery ; Survival Rate ; Transcription Factors ; metabolism

OBJECTIVETo explore the pathological features and prognosis of appendiceal mucinous neoplasms (AMN) based on WHO classification 2010.

METHODSSeventy consecutive cases of AMN were classified into 5 groups according to WHO classification of digestive system tumors in 2010 including mucinous adenomas/cystadenoma (MA), low grade appendiceal mucinous neoplasms (LAMN), low grade pseudomyxoma peritoneum originated from appendix (PMP-L), invasive mucinous adenocarcinomas (MAC) and high grade pseudomyxoma peritoneum originated from appendix (PMP-H). Clinicopathological features, classification, treatment and prognosis of AMN were investigated retrospectively.

RESULTSThere were 11 cases of MA with neoplastic epithelium and mucin being defined in lumen and mucosa but without invasive lesions, and no relapse or death was found. In 41 LAMN cases, mucin was found in submucosa, muscularis proparis, or serosa of appendix, no or only scant mucinous epithelium with low grade dysplasia presented in mucinous pools in most cases (39/41). Among 41 LAMN cases, 3 developed relapse or PMP-L, and no death was observed. In 7 PMP-L cases, low grade dysplastic mucinous epithelium in mucinous pools could be found easily in 3 cases and was very scanty in 4 cases, with 1 relapse and 1 death. Eleven invasive carcinomas were found, including 7 MAC cases and 4 PMP-H cases, with local high grade dysplastic epithelium at least. In these invasive lesions, 4 cases recurred and 3 case died (including 2 recurred cases above). MA and LAMN were both non-invasive neoplasms histologically, however, PMP-L, MAC and PMP-H were regarded as adenocarcinomas according to their biological behavior.

CONCLUSIONAMN displays a relatively homogeneous group of neoplasms that pursues a predictable clinical course based on their nature, so it is necessary to diagnose and administrative accurately with consistently standards for these neoplastic entities.

Adenocarcinoma, Mucinous ; pathology ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Appendiceal Neoplasms ; classification ; pathology ; Child ; Female ; Humans ; Male ; Middle Aged ; Myxoma ; pathology ; Prognosis ; Young Adult

Local recurrence after endoscopic piecemeal mucosal resection (EPMR) for colorectal tumors is a crucial issue. However, such recurrence is usually detected within one year and cured with additional endoscopic treatment, which makes EPMR acceptable. Herein, we report a rare case of repeatedly recurrent colon cancer involving the appendiceal orifice after EPMR, which was not cured with additional endoscopic treatments. A 67-year-old man was referred to us for endoscopic treatment of a 25 mm cecal tumor spreading to the appendiceal orifice in May 2002. The tumor was resected with EPMR, showing well differentiated intramucosal adenocarcinoma with a positive lateral cut margin of tubular adenoma. Endoscopic surveillance was conducted and the first local recurrence was detected in August 2006. Although we resected it endoscopically, the second local recurrence was found in September 2007 and we removed it with endoscopic resection again. However, the third local recurrence was detected in March 2008. Although endoscopic resection was performed also for the third recurrence, curative resection was not achieved. In February 2009, laparoscopic assisted colectomy was performed and histopathological examination showed well differentiated adenocarcinoma with deep submucosal invasion. This case is important in considering indication for endoscopic resection in colorectal tumors involving the appendiceal orifice.
Adenocarcinoma/*diagnosis/pathology/surgery ; Aged ; Appendiceal Neoplasms/complications ; Colectomy ; Colonic Neoplasms/*diagnosis/pathology/surgery ; Colonoscopy ; Humans ; Intestinal Mucosa/pathology ; Male ; Neoplasm Recurrence, Local ; Recurrence

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

Adult ; Appendiceal Neoplasms ; immunology ; pathology ; Dendritic Cell Sarcoma, Follicular ; immunology ; pathology ; Humans ; Immunohistochemistry ; Male ; Proto-Oncogene Proteins c-kit ; analysis ; Receptors, Complement 3b ; analysis