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

OBJECTIVETo investigate the clinicopathological and immunohistochemical features of primary malignant tumor of the appendix.

METHODSThe clinical data were reviewed; and histopathological and immunohistochemical features were analyzed in 22 cases with primary malignant tumor of the appendix.

RESULTIn 22 cases of primary malignant tumor of the appendix, 19 cases were carcinoid and 3 were adenocarcinoma. Immunohistochemistry showed that the carcinoid was positively reacted to the neuroendocrine markers, and the adenocarcinoma was negatively reacted to the neuroendocrine markers.

CONCLUSIONImmunohistochemistry is useful in diagnosis of primary malignant tumor of the appendix, a rare type of cancer.

Adenocarcinoma ; diagnosis ; pathology ; surgery ; Adolescent ; Adult ; Aged ; Appendiceal Neoplasms ; diagnosis ; pathology ; surgery ; Carcinoid Tumor ; diagnosis ; pathology ; surgery ; Female ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; 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 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

BACKGROUND/AIMS: Primary appendiceal adenocarcinoma is a rare neoplasm that constitutes less than 0.5% of all gastrointestinal neoplasm. The aim of this study was to figure out its clinicopathologic characteristics that are not well understood. METHODS: We reviewed the medical records of nineteen patients (9 males and 10 females) with histologically proven appendiceal adenocarcinoma. They had been treated at Asan Medical Center between June 1989 and December 2002. Their median follow-up duration was 72.5 months. RESULTS: Their median age was 56.5 (range, 33~80) years. Thirteen patients had mucinous variants and the other five had adenocarcinoma. Seven patients (36.8%) were diagnosed as acute appendicitis. In fact, none of the patients was diagnosed correctly before surgery. The operative procedure, included right hemicolectomy in 9 patients, appendectomy alone in 2 patients, and debulking of their tumors or a biopsy in 8 patients. The 5-year survival rate was 20.5%. The patients with mucinous type had better prognosis than those with the non-mucinous type (p<0.01). In the patients with mucinous type, the survival rate after debulking operation was similar to that after right hemicolectomy. CONCLUSIONS: The most important prognostic factor of primary appendiceal adenocarcinoma was histology. The outcome of debulking operation is being watched compared with that of right hemicolectomy in mucinous variant.
Adenocarcinoma/*diagnosis/mortality/surgery ; Adult ; Aged ; Appendiceal Neoplasms/*diagnosis/mortality/surgery ; English Abstract ; Female ; Humans ; Male ; Middle Aged ; Prognosis ; Survival Rate

Adenocarcinoma, Mucinous ; pathology ; Aged ; Appendectomy ; methods ; Appendiceal Neoplasms ; pathology ; surgery ; Appendicitis ; pathology ; Appendix ; pathology ; Carcinoid Tumor ; pathology ; surgery ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Male

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

OBJECTIVETo clarify the various diagnostic connotations of pseudomyxoma peritonei (PMP) and to study their prognostic implications.

METHODSClinicopathologic features and follow-up data of 40 patients with PMP diagnosed in The General Hospital of PLA were retrospectively reviewed. The cases were histologically classified into 3 subcategories: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and PMCA with intermediate or discordant features (PMCA-I/D). The survival rate was calculated using Kaplan-Meier method and the difference was statistically analyzed.

RESULTSTwelve of the 40 patients died on follow up. The duration of survival ranged from 2 to 348 months (medium = 37.5 months). In general, the 3-year, 5-year and 10-year survival rates were 79.0%, 69.4% and 53.0%, respectively. The mean age of the patients at the time of diagnosis was 50.3 years (age range = 22 to 76 years). The male-to-female ratio was 1:1. The age and sex of patients, frequency of operation and presence of ovarian involvement did not correlate with duration of survival. On the other hand, the presence of appendiceal tumor, parenchymal invasion of abdominal viscera, cellularity, architecture, nuclear atypia and mitotic activity of the peritoneal lesion significantly correlated with survival. There was also significant difference in survival between DPAM, PMCA-I/D and PMCA subcategories (P = 0.018). The difference in survival rate between PMCA-I/D and PMCA subgroups however was not statistically significant (P = 0.096). The outcome of DPAM was significantly better when compared with the combined group of PMCA-I/D and PMCA (P = 0.006).

CONCLUSIONSIn general, the 10-year survival rate of PMP was low, despite the relatively benign-looking or low-grade pathologic appearance. Peritoneal lesions with higher cellularity, conspicuous nuclear atypia and higher mitotic activity are associated with a lower survival rate. The prognosis was even worse in the presence of appendiceal carcinoma or parenchymal invasion of abdominal viscera. It is thus advisable to subclassify PMP into DPAM, PMCA and PMCA-I/D, due to the difference in prognostic implication.

Adenocarcinoma, Mucinous ; pathology ; surgery ; Adenoma ; pathology ; surgery ; Adult ; Aged ; Appendectomy ; Appendiceal Neoplasms ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Neoplasm Invasiveness ; Peritoneal Neoplasms ; classification ; pathology ; surgery ; Pseudomyxoma Peritonei ; classification ; pathology ; surgery ; Retrospective Studies ; Survival Rate ; Young Adult

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