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Adenocarcinoma of the appendix: an unusual disease.

OBJECTIVE: To describe our experience with primary appendiceal tumours. DESIGN: Retrospective study. SETTING: University hospital, Israel. SUBJECTS: 2520 patients who had appendectomies during the 14 years, January 1982-December 1996. RESULTS: 22 patients 5 male and 17 female, mean age 56.2 years, had primary neoplasms; 14 were carcinoid tumours and villous adenomas and were treated by appendicectomy only. Adenocarcinoma was diagnosed in 8 patients (0.3%), 5 after appendicectomy (0.2%) which is twice the reported incidence. They were all treated by right hemicolectomy. Seven of the patients were classified as Dukes' B and one as Dukes' C. All patients were alive and disease-free after a mean follow-up period of 57.4 months. CONCLUSION: Right hemicolectomy is the treatment of choice for adenocarcinoma of the appendix.

Adenocarcinoma↗

Laparoscopic management of pseudomyxoma peritonei secondary to adenocarcinoma of the appendix.

Pseudomyxoma peritonei is a rare disease in which the abdominal cavity fills with thick mucoid material secondary to either benign or malignant conditions. We discuss a case where pseudomyxoma peritonei secondary to adenocarcinoma of the appendix was diagnosed and managed laparoscopically. The laparoscopic approach allows thorough exploration of the abdomen, as well as irrigation and aspiration of the thick mucinous material using a 10-mm suction cannula and the instillation of mucolytic agents such as 5% dextrose solution. Appendectomy or right hemicolectomy can be performed with minimal disturbance of the anterior abdominal wall, thus minimizing future adhesions as well as possible tumor-cell implantation. Intraperitoneal catheters for chemotherapy can be placed easily through the port sites. These measures offer an alternative to radical peritoneal dissection and can be accomplished during the initial laparoscopic exploration.

Adenocarcinoma↗

Crypt cell carcinoma of the appendix (so-called adenocarcinoid tumor).

A series of 16 carcinoid tumors of the appendix, including four examples of so-called adenocarcinoid tumors, has been studied immunohistochemically for the presence of lysozyme, secretory component (SC) and IgA, within tumor cells. Stains for mucin, Paneth cells, and APUD cells were also performed. Of the conventional carcinoid tumors, eight showed focal tubule formation with production of PAS-positive material. In contrast to conventional carcinoids, including those showing tubule formation, adenocarcinoids contained few APUD cells and showed positive staining of tumor cells for lysozyme, SC, and IgA. Paneth cells, staining positively for lysozyme, were present in two cases and mucin-containing Paneth cells were observed. A parallel study of normal small intestinal mucosa demonstrated a population of lysozyme containing goblet cells within the crypts. It is suggested that so-called adenocarcinoid tumors are not derived from APUD cells but from lysozyme-producing cells of the type normally present in small intestinal crypts.

Adenocarcinoma↗

Association of mucinous tumors of the ovary and appendix. A clinicopathologic study of 25 cases.

Twenty-five patients with mucinous tumors of the ovary and appendix were studied. The average age of the patients was 52 years, and the ovarian and appendiceal tumors were discovered synchronously in all but two cases. The majority had either a pelvic mass or abdominal or pelvic pain. A high frequency of bilateral ovarian tumors (11/25), and right-sided predominance for the unilateral ovarian tumors (nine right, five left) were found. Four patients had ovarian mucinous carcinomas, 10 had mucinous tumors of low malignant potential, 10 had mucinous cystadenomas, and one had a mucinous cyst. Pseudomyxoma ovarii was present in 22 cases. Twenty-two of 24 appendices were grossly abnormal. There were six appendiceal mucinous adenocarcinomas, 10 mucinous tumors of uncertain malignant potential, seven mucinous cystadenomas, one hyperplastic polyp, and one mucocele. Twelve patients had ovarian and appendiceal tumors of similar malignant potential, nine had appendiceal tumors with more aggressive morphologic features than the corresponding ovarian tumor, and four had ovarian tumors with more aggressive morphologic features than the appendiceal tumor. Eighteen patients had peritoneal involvement by mucinous epithelium admixed with mucus (nine localized, nine diffuse). Immunoperoxidase reactions for four epithelial antigens in 15 cases showed complete concordance between ovarian and appendiceal lesions in only five cases and were not helpful in determining the site of origin of the peritoneal tumor. Our findings suggest an independent origin of the ovarian and appendiceal tumors in most cases and do not favor an origin in a single site. Furthermore, it is proposed that the peritoneal lesions may arise de novo as part of a multifocal neoplastic process.

Adenocarcinoma↗

Synchronous mucinous tumors of the appendix and the ovary associated with pseudomyxoma peritonei. A clinicopathologic study of six cases with comparative analysis of c-Ki-ras mutations.

Mucinous tumors of the ovary are often associated with mucinous tumors of the appendix. It has not been clearly determined whether they are independent or metastatic neoplasms. A clinicopathologic study and a comparative analysis of c-Ki-ras mutations were done in six cases of synchronous ovarian and appendiceal tumors. The clinicopathologic features (simultaneous presentation, bilaterality or right-sided predominance, similar histopathologic findings, presence of pseudomyxoma peritonei) suggested that they were primary appendiceal tumors metastatic to the ovaries. DNA was extracted from formalin-fixed, paraffin-embedded tissue, and target sequences were amplified in vitro by the polymerase chain reaction. Mutations were detected by the presence of restriction fragment length polymorphism, artificially introduced by the use of mutant amplimers. The pattern of c-Ki-ras mutations was identical in the ovarian and appendiceal tumors of all patients. Four patients had a GGT --> GAT (Gly --> Asp) transition and one a GGT --> GTT (Gly --> Val) transversion, all detected in codon 12. No mutation was found in the sixth patient in either the ovarian or the appendiceal tumor. Because c-Ki-ras mutations are considered to represent an early event in tumorigenesis, our results support a clonal nature for both tumors and suggest that they are not independent tumors but rather originate one from another.

Adenocarcinoma, Mucinous↗

Carcinoid tumors of the appendix. Mesoappendiceal extension and nodal metastases.

Carcinoid of the appendix occurred in 92 of 400,000 surgical pathology specimens covering 70 years, and followed previously reported incidences by age, sex, symptoms, size, localization and histology. Invasion of the mesoappendix occurred in 13 cases, and in two of these, a reoperative ileocolectomy demonstrated regional node metastases. No distant metastases or fatalities appeared. Tumor size correlated with mesoappendiceal invasion. Carcinoid invasion of the mesoappendix may provide a valuable marker for more distant tumor spread, and justify an aggressive surgical approach in selected patients.

Adolescent↗

Incidental appendix carcinoid. Value of somatostatin receptor imaging.

PURPOSE: Somatostatin receptor scintigraphy is used to diagnose carcinoid of the gastrointestinal tract. Its sensitivity ranges from approximately 75%-100%. Therefore, it was hypothesized that it can be used in the postsurgical follow-up to detect residual carcinoid, recurrence, and metastatic disease. RESULTS: This article is concerned with the findings of somatostatin receptor imaging performed on a 12-year-old girl 8 weeks after appendectomy. Histologic examination showed an incidental appendix carcinoid. Somatostatin receptor scintigraphy performed for detection of lymph node metastatic spread of the carcinoid showed focal tracer accumulation at the former operative site; subsequently, a right hemicolectomy was performed. However, histologic examination of the surgical tissue showed no evidence for carcinoid. CONCLUSION: It is concluded that there are some potential pitfalls for somatostatin receptor imaging at least soon after surgery. Therefore, it should not be used to aid in reoperation.

Appendiceal Neoplasms↗

Torsion of lipoma of appendix epiploica: CT evaluation.

We present a case of a large abdominal mass of fat density on CT, which proved at surgery to be a lipoma arising in an appendix epiploica. The clinical, pathological, and radiological aspects of appendices epiploicae are discussed.

Aged↗

Peridiverticulitis of the appendix: an unusual ultrasonic "target lesion".

In a 26-year-old man with perforating diverticulitis of the appendix ultrasonography demonstrated a "target lesion" with a hyperechoic center surrounded by a hypoechoic rim similar to the findings commonly seen in benign and malignant conditions of the gastrointestinal tract. This ultrasonographic appearance is quite different from the ultrasonic findings usual in acute appendicitis. We take this to be the first ultrasonic demonstration of appendiceal diverticulitis, and discuss the clinical features, ultrasonic differential diagnosis, and pathology of this condition.

Adult↗

Testicular microlithiasis in a child with torsion of the appendix testis.

We report on an 8-year-old boy with a unilateral testicular mass and bilateral diffusely echogenic testes on sonography. At inguinal exploration torsion of an appendix testis with a reactive phlegmon was found and testis biopsy revealed significant testicular microlithiasis. To our knowledge our case demonstrates a previously undescribed presentation of testicular microlithiasis. The literature is reviewed and this disease entity is discussed.

Calculi↗

Infarction of an epiploic appendix.

We have presented four cases of infarcted appendices epiploicae. Because the clinical picture can be confusing, the diagnosis is frequently delayed or missed. Removal of the infarcted appendix results in cure, with no reported cases of recurrence. The disease can be fatal, four deaths having been reported in the literature.

Adult↗

Torsion, necrosis, and inflammation of an epiploic appendix of the large bowel: a diagnostic and therapeutic dilemma.

We present the case of a young patient with abdominal pain of 3 weeks' duration and a preoperative presumptive diagnosis of acute appendicitis, diverticulitis, colon perforation, or possibly neoplasm. Even after resection of a large mass involving the right colon, the diagnosis was not confirmed until surgical pathology identified torsion, partial necrosis, and inflammation of an epiploic appendix of the right colon.

Adult↗

Cancer of appendix as a presenting feature of Crohn's disease.

Appendiceal neoplasms are rare. Colonic malignancies, including appendiceal carcinomas, have been rarely reported in Crohn's disease patients. The involvement of the appendix in Crohn's disease is not often seen. We report an interesting case in which a caecal Crohn's disease patient presented for the first time with an appendiceal adenocarcinoma.

Adenocarcinoma, Mucinous↗

Mixed carcinoid and adenocarcinoma of the appendix: report of 4 cases with immunohistochemical studies and a review of the literature.

Four cases of mixed carcinoid and adenocarcinoma of the appendix were reported. All cases presented with a dominant cecal-appendiceal tumor mass and local metastasis. Two patients had multiple peritoneal implants mimicking primary peritoneal serous adenocarcinoma or carcinomatosis. Histopathologic features of the tumors are similar, with infiltrating microglandular and cribriform patterns of tumor nests, and variable numbers of goblet cells. A literature review of "goblet cell carcinoid" that included nonlocalized cases revealed a significant percentage (>14%) of tumor-associated death, in contrast to the classic carcinoid tumor. Immunohistochemical stains were helpful to separate these tumors from carcinoid tumors and primary peritoneal serous adenocarcinoma. Mixed carcinoid and adenocarcinomas were cytokeratin (CK)-20 positive, and negative or weakly positive for chromogranin A and synaptophysin. Carcinoid tumors were CK20 negative and diffusely positive for chromogranin A and synaptophysin. Peritoneal serous adenocarcinomas were CK20 negative. These cases were clinically aggressive, and 1 patient had multiple recurrences and responded partially to chemotherapy.

Adenocarcinoma↗

Laparoscopic two-stage surgery for goblet cell carcinoid of the appendix: report of a case and review of the Japanese literature.

Goblet cell carcinoid of the appendix is a rare clinical entity exhibiting features of both carcinoid and adenocarcinoma. Here, we present the first report of laparoscopic 2-stage surgery for goblet cell carcinoid with a review of the Japanese literature. A 49-year-old man underwent laparoscopic appendectomy under the diagnosis of acute appendicitis. A pathologic diagnosis of goblet cell carcinoid, accompanied by the aggressive proliferation, with acute appendicitis was made. Subsequent laparoscopic ileocecal resection was performed, and it was verified that there were neither residual tumor nor lymph node metastases. The postoperative course was uneventful. Because goblet cell carcinoid may be difficult to clinically distinguish from acute appendicitis, pathologic examination is essential. Depending upon the grade of tumor proliferation, additional resection should be considered, and our experience with this case suggests that laparoscopic 2-stage surgery is feasible for the adequate treatment of goblet cell carcinoid without complications.

Anastomosis, Surgical↗