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PubMed2000

The coexistence of low-grade mucinous neoplasms of the appendix and appendiceal diverticula: a possible role in the pathogenesis of pseudomyxoma peritonei.

We examined 38 appendectomies with diagnoses of mucocele, diverticulum, or adenoma to study the coincidence of appendiceal diverticula and appendiceal low-grade mucinous neoplasms and to examine the possible role of diverticula in the pathogenesis of pseudomyxoma peritonei. Invasive adenocarcinomas and retention cysts were excluded (six cases). Cases were classified as adenomas or mucinous tumors of unknown malignant potential, with or without diverticula. Medical records were reviewed for multiple parameters, including presenting symptoms, presence of pseudomyxoma peritonei, and presence of associated malignancies. Binomial statistics were used to calculate the probability that the observed prevalence of low-grade mucinous neoplasms and diverticula together was significantly different from the expected prevalence of diverticula or low-grade mucinous neoplasms alone, using historical controls from the literature. Twenty-five percent of the total cases (8 of 32) contained both a low-grade mucinous neoplasm (7 cystadenomas and 1 mucinous tumor of unknown malignant potential) and a diverticulum. Thus, 8 of 19 low-grade mucinous neoplasms (42%) were associated with diverticula. Of the appendices with both low-grade mucinous neoplasms and diverticula, three contained dissecting acellular mucin within the appendiceal wall, four showed diverticular perforation, and one had pseudomyxoma peritonei associated with the ruptured diverticulum. A significant percentage (P < .001) of cases contained low-grade mucinous neoplasms and diverticula together. The case of coexistent low-grade mucinous neoplasm, diverticulum, and pseudomyxoma peritonei suggests that diverticula could play a role in the pathogenesis of pseudomyxoma peritonei. This could occur either by involvement of preexisting diverticula by the neoplasm or by distention of the appendiceal lumen by mucin, leading to increased intraluminal pressure and subsequent diverticulum formation at a weak area in the wall. Either mechanism might allow low-grade mucinous neoplasms to penetrate the appendiceal wall more easily.

Adult↗
PubMed2004

Primary epithelial neoplasms and other epithelial lesions of the appendix (excluding carcinoid tumors).

Epithelial tumors of the appendix range from low-grade mucosal-based tumors which, when confined to the appendix, have an excellent prognosis but, once outside the appendix, have a fair prognosis and often a prolonged disease course, to high grade invasive carcinomas that are rapidly fatal. Low grade mucinous neoplasms may rupture and spread to the peritoneum as pseudomyxoma peritonei, and the nomenclature of these tumors has been the subject of considerable disagreement among pathologists; the designation "low grade appendiceal mucinous neoplasm" has recently been proposed for reasons discussed herein. Demonstrating rupture of these neoplasms may require particularly diligent gross and microscopic evaluation as the rupture site often heals over leaving only subtle evidence of its presence. Invasive adenocarcinomas are often mucinous and may also spread to the peritoneum. Against this backdrop, the clinical and pathologic features of low grade appendiceal mucinous neoplasms and mucinous adenocarcinomas, as well as other types such as typical colorectal type and signet-ring cell type, are reviewed. In addition, emerging entities, serrated polyps and serrated adenomas, whose significance is only beginning to be understood, are considered. Retention cysts, hyperplastic polyps, and diffuse mucosal hyperplasia, although not necessarily neoplastic, are reviewed here as they may enter into the differential diagnosis of appendiceal mucinous neoplasms.

Appendiceal Neoplasms↗
PubMed1963

CARCINOID TUMORS.

Carcinoid tumors are diminutive growths found along the gastrointestinal tract, in the lungs and, occasionally, within the confines of teratomas. Carcinoid tumors of the intestinal tract are found in decreasing order of frequency in the rectum, the appendix, the ileum, the jejunum, the lung, the stomach and the duodenum. Arising from argentaffin elements in the crypts of Lieberkuehn, and secreting serotonin, carcinoid tumors belong to the order of functioning neoplasms. Their indolent growth seems to place them between benign neoplasms and carcinomas. However, they possess propensities for metastasis that correlate with increase in size. The symptoms that are produced are almost always due to the presence of metastatic lesions. In ileal and gastric carcinoid tumors, the tendency to metastasis is augmented and the metastatic masses are sometimes of sufficient bulk to cause the carcinoid syndrome or to interfere with the supply of blood to the affected segment. More often they produce the clinical picture of intestinal obstruction. The unusually long interval from onset to death associated with carcinoid tumors makes palliative subtotal resections and short-circuiting operations in symptomatic patients with advanced disease worthwhile, for by such unorthodox procedures the patients may be afforded many additional years of useful life.

Appendiceal Neoplasms↗
PubMed1999

[Abdominal metastases from signet cell carcinoma of the appendix vermiformis].

Signet cell carcinomas are often aggressive tumours. A patient with this tumour, originally located in the appendix, was diagnosed with peritoneal metastases. When she later got abdominal symptoms, computer axial tomography indicated large infiltrating tumour masses in the pelvis. However, when a laparotomy was performed, the only macroscopic tumour masses was localised to the ovaries (Krukenberg tumour). The surgical intervention gave the patient an improved quality of life, and most likely prolonged survival. We stress the importance of thinking of this possibility.

Abdominal Neoplasms↗