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[Carcinoma of the appendix. Presentation of one clinical case and review of the literature (author's transl)].

After presenting one case of carcinoma of the appendix of their own observation, the authors discuss several aspects of the anatomopathology, clinical course, and diagnosis of tumors of the appendix. From a review of existing literature it emerges that no case of carcinoma of the appendix was ever diagnosed preoperatively. The authors evaluate the difficulties inherent in such diagnosis and recommend some means of investigation that are currently applied to other organs.

Adenocarcinoma↗

Villous adenoma of the vermiform appendix with cecal intussusception: a case report and review of literature.

Villous adenoma of the appendix is an uncommon neoplasm. Intussusception of the appendix is a rare pathological entity. We report here an intussusception of the appendix, the lead point being a villous adenoma, presenting in a 62-year-old male whose initial complaint was a progressive enlargement of a right inguinal hernia. The endoscopic, surgical, and pathologic data are presented. A review of the literature of this uncommon entity is also presented.

Adenoma, Villous↗

Pseudomyxoma extraperitonei: a lethal complication of mucinous adenocarcinoma of the appendix.

We report the unusual case of a 56-yr-old man who presented with an appendix abscess. This abscess failed to respond to standard treatment, and recurred despite operative intervention. A colocutaneous fistula developed, which initially discharged frank pus, then began discharging mucus via two cutaneous sinuses. No evidence of malignancy was detected from either repeat curettings or a diagnostic laparotomy. One and a half years after the initial presentation, histologic examination finally revealed mucinous adenocarcinoma of the appendix. We postulate that this malignancy in a retrocecal appendix resulted in a mucocele, which ruptured into the retroperitoneal space and drained via cutaneous sinuses, so-called pseudomyxoma extraperitonei. The patient declined further surgical intervention, and despite initial control by radiotherapy, the tumor spread locally to involve the entire right lower quadrant of the abdomen. The patient eventually died 39 months after the initial presentation.

Adenocarcinoma, Mucinous↗

[Mucinous cystadenocarcinoma (malignant mucocele) of the cecal appendix].

Mucinous cystadenocarcinoma of the cecal appendix is a very rare malignant neoplasia. The majority of these tumours are discovered during surgery performed for other pathologies or as a chance finding in the histological analysis of surgically removed inflamed appendix. There is no characteristic symptomatology of this neoplasia which requires oncologically correct surgery, such as right hemicolectomy. The authors report a case study o peritoneal recidivation of a mucinous cystadenocarcinoma of the appendix.

Aged↗

Duplication of the appendix mimicking adenocarcinoma of the colon.

Duplication of the appendix is a rare anomaly that has been reported fewer than 60 times. Three anatomic types of appendiceal duplications have been described--each the result of a different embryologic occurrence. A unique case of appendiceal duplication is presented, in which the duplication caused a constricting lesion of the colon and mimicked carcinoma. The anomalous appendix in this patient was not compatible with any of the previously described types of appendiceal duplications.

Adenocarcinoma↗

Does the retrocecal position of the vermiform appendix alter the clinical course of acute appendicitis? A prospective analysis.

Ninety-four adult patients undergoing appendectomy for acute appendicitis were prospectively studied during a 2-year period. Patients were divided into retrocecal (group 1; n = 27 [29%]) and anterior (group 2; n = 67 [71%]) groups according to the position of the appendix. There was no statistical difference between the two groups in duration of symptoms, presenting signs and symptoms, and initial white blood cell count. Furthermore, retrocecal appendicitis was not associated with a higher rate of perforation or increased morbidity. We conclude that the retrocecal position of the appendix does not alter the presentation of appendicitis.

Acute Disease↗

Agenesis of the vermiform appendix in a thalidomide child.

A case of congenital absence of the appendix occurring in a child with severe thalidomide deformities is described. The literature on agenesis of the appendix is reviewed. The frequency and significance of internal abnormalities in thalidomide children are discussed.

Abnormalities, Drug-Induced↗

Use of the appendix in reconstructive surgery: a case against incidental appendicectomy.

Ignored for years, the appendix is once again being promoted as having useful surgical potential, notably in the management of incontinence. Incidental and prophylactic appendicectomy with their established complications become less easy to justify as the incidence of appendicitis declines. The role of the appendix in reconstructive surgery is reviewed, with particular reference to urinary reconstruction and to the treatment of chronically constipated or faecally incontinent children by use of the antegrade continence enema.

Appendectomy↗

Sonographic appearance of mucocele of the appendix.

Mucocele of the appendix is an uncommon entity infrequently diagnosed prior to surgery or autopsy. Patients may be asymptomatic or present with a palpable right lower quadrant mass and/or pain. Barium enema findings are nonspecific and include nonfilling of the appendix and occasionally mass impression on the cecum. When visualized, calcification of the rim of a right lower quadrant mass may aid in diagnosis. The sonographic appearance in two cases is presented and compared with that in three other case reports in the literature, illustrating a spectrum of sonographic findings. Differential diagnosis is discussed.

Aged↗

MR imaging of the normal appendix and acute appendicitis.

PURPOSE: To describe the MR appearance of the normal appendix and the MR imaging characteristics of acute appendicitis with correlation to pathological severity. MATERIALS AND METHODS: A total of 20 volunteers participated in this study to demonstrate normal appendices by MR imaging. A total of 37 consecutive patients with clinically diagnosed acute appendicitis were also scanned. T1-weighted (T1WI) spin-echo images, T2-weighted (T2WI) fast spin-echo, and fat-suppressed spectral presaturation inversion recovery T2-weighted (T2SPIR) fast spin-echo images were obtained. The MR criteria for considering acute appendicitis were as follows: 1) thickening of the appendiceal wall with high intensity on T2WI or T2SPIR; 2) dilated lumen filled with high intensity material on T2WI or T2SPIR; and 3) increased intensity of periappendiceal tissue on T2WI or T2SPIR. RESULTS: The visibility of a normal appendix on MR imaging was 90% (18/20). It appeared as a cord-like structure of medium intensity without fluid collection in the lumen. A total of 30 cases with clinically diagnosed acute appendicitis had positive MR findings and all except one were pathologically proven. The one had cecal diverticulitis. These cases demonstrated filled lumen, with a hypointense wall on T1WI and slightly hyperintense on T2WI or T2SPIR. MR findings correlated well with pathological severity, especially a thicker wall, periappendiceal high intensity, and ascites were useful for suspecting severe appendicitis. CONCLUSION: Correct diagnosis of acute appendicitis was obtained with MRI, and correlated well with its pathological severity. MRI is a powerful alternative for diagnosing acute appendicitis especially for the patients in whom the radiation is major concern.

Acute Disease↗

Mast cells, nerves and fibrosis in the appendix: a morphological assessment.

Mast cells are closely associated with nerves in the mucosa of the appendix vermiformis, and obliteration of the appendiceal lumen by fibrous tissue is accompanied by neurogenous hyperplasia. However, changes in the density of mast cells in this process have not been reported. Accordingly, fibrosis was graded in haematoxylin and eosin sections from 46 samples of human appendix. This was compared with mast cell number in toluidine blue-stained slides and nerve density in PGP9.5-immunoreactive sections. In the mucosa, the mast cell number in the samples with minimal fibrosis was three times greater than in those classified as normal (P less than 0.0001), and this declined in the more fibrotic samples. The mucosal nerve scores paralleled the mucosal mast cell changes, and stereological analysis revealed a correlation of mast cell number and nerve density within the lamina propria of the same specimens (r = 0.49-0.90). In the submucosa, mast cell numbers and nerve scores were not significantly different in the different histological grades and obliterated samples resembled normal submucosa, except that a dense axial block of nerve staining was often present. The progressive fibrotic changes in appendices provide a human model for studying the relationships of nerves, mast cells, and fibrosis in the gastrointestinal tract.

Appendix↗

Role of appendix and spleen in experimental colitis.

There is growing clinical evidence suggesting that certain secondary lymphoid tissues (e.g., appendix and spleen) contribute to the initiation and/or perpetuation of ulcerative colitis. In this study, the importance of secondary lymphoid tissues in inducing colitis was assessed experimentally by removing the spleen and/or appendix (or sham operation) prior to inducing colitis in mice. Feeding 2.5% dextran sulphate sodium (DSS) in drinking water over 7 days induced colitis. Clinical disease activity was assessed based on weight loss, stool consistency, and presence of blood in stools. Additional measurements included white blood cell count and hematocrit, and myeloperoxidase activity (MPO) in colon samples. Colonic injury was assessed by histology and computerized image analysis. DSS treatment in sham-operated mice produced colitis associated with weight loss, bloody diarrhea, and mucosal ulceration. Clinical assessment of DSS-treated mice subjected to appendectomy or combined appendectomy/splenectomy exhibited a delayed onset and course of disease activity. Histomorphologic examination revealed significantly lower damage scores and a reduction in ulcerated mucosal surface area. Colonic MPO activity, which correlated with tissue injury and disease activity, was lowest in appendectomized mice. No beneficial effects of splenectomy were observed after 7 days of colitis. These findings support the hypothesis that appendicular lymphoid tissue, but not the spleen, contributes to the development of colitis.

Animals↗

Demonstration of fibronectin in normal and acutely inflamed appendix.

The presence and localization of fibronectin in normal and acutely inflamed appendices in man was studied using indirect immunoperoxidase technique on sections of formaldehyde fixed and paraffin embedded tissue, following pretreatment with pepsin and testicular hyaluronidase. In the normal appendix fibronectin was demonstrated in the region of the basement membrane of the surface epithelium, in the loose connective tissue, in the perimysium around the individual smooth muscle cells and in the vessel walls. In the acutely inflamed appendices, fibronectin was found in the luminal necrotic area, both intercellularly and in the cytoplasma of some inflammatory cells. In relation to the surface inflammation and in the tissue matrix corresponding to the acute inflammatory reaction fibronection was, compared to the normal appendix, found in increased amount. Furthermore, a comparison between the use of a primary antibody to fibronectin, produced in our collaborating laboratory and two different commercial primary antibodies showed that the staining results concerning the localization of fibronectin were equal for all three antibodies, whereas the commercial antibodies showed a weaker staining intensity and some unspecific staining compared to the antibody produced in our collaborating laboratory.

Acute Disease↗

The neuroendocrine system of normal human appendix, ileum and colon, and in neurogenic appendicopathy.

Epithelial endocrine cells containing 5-hydroxytryptamine, substance P, somatostatin, enteroglucagon and vasoactive intestinal polypeptide-immunoreactivity were localized by immunocytochemistry in the mucosa of normal appendices, ileum and proximal colon, and in neurogenic appendicopathy. In neurogenic appendicopathy a large number of proliferating nerves were visualized independently of neurotransmitters by immunostaining for neuron-specific enolase. A large number of nerve fibers were shown to contain substance P-immunoreactivity and to be of intrinsic origin. Stromal endocrine cells containing 5-hydroxytryptamine, somatostatin- and possibly substance P-immunoreactivity, were observed in substantial numbers in neurogenic appendicopathy. Substance P may be involved as a neurotransmitter and/or as a paracrine/endocrine peptide in the pathogenesis of spastic contractions and abnormal peristalsis of the appendix, which are characteristic of neurogenic appendicopathy. Stromal endocrine cells may be considered to be the origin of certain carcinoids in the appendix.

Appendix↗

S-100 protein in carcinoid tumours of appendix.

A series of 12 carcinoid tumours of the appendix were examined with regard to S-100 protein immunoreactivity. All tumours were both argentaffin and argyrophil, and displayed immunoreactivity after application of a monoclonal antibody against serotonin. The S-100 protein immunoreactivity appeared in 11 of the 12 tumours, preferably in cells presumably of Schwann cell origin with long slender processes localized at the periphery of the carcinoid tumour buds. Immunoreactive cells with cytoplasmic processes were also seen extending between individual tumour cell in the tumour aggregates. In a few tumours S-100 immunoreactivity occurred in the cytoplasm of tumour cells with or without cytoplasmic extensions. The presence of S-100 protein immunoreactive cells, apparently as an integral component, and its shape and distribution indicate that the peripheral nervous system (PNS) is histogenetically involved in the development of carcinoid tumours of the appendix.

Appendix↗

Neuropeptides in the human appendix. Distribution and motor effects.

At present our knowledge of enteric peptide-containing neurons in man is limited. In this study we have used human appendices removed at surgery to examine the peptidergic innervation by immunocytochemistry, immunochemistry, and pharmacological in vitro experiments. Immunocytochemistry revealed a variety of peptide-containing nerve fiber populations in the human appendix. VIP/PHI-, VIP/PHI/NPY-, SP/NKA-, galanin-, and enkephalin-containing nerve fibers were numerous; CGRP- and GRP-containing nerve fibers were moderate in number, while only scattered NPY-, enkephalin/BAM-, and somatostatin-containing nerve fibers could be found. No CCK-, dynorphin A-, or dynorphin B-immunoreactive nerve fibers could be detected. The coexistence of VIP/PHI, SP/NKA, and enkaphalin/BAM can be anticipated from the known sequence of their respective precursors. However, the coexistence of VIP/PHI and NPY was unexpected but corroborates previous observations in other species. Interestingly, SP and CGRP did not seem to coexist in nerve fibers of the human appendix. Immunochemistry (RIA and HPLC) confirmed the presence of VIP, NPY, SP, galanin, CGRP, GRP, enkephalin, and somatostatin. Motor activity studies suggest that acetylcholine plays a major role in the electrically evoked contractions, since atropine suppressed these contractions. Galanin (10(-8)-10(-6) M) and GRP (10(-9)-10(-7) M) caused concentration-dependent contractions that were unaffected by tetrodotoxin and thus probably reflect a direct action on smooth muscle receptors. GRP (10(-9) M) enhanced the electrically induced cholinergic contraction (to 193 +/- 24%), while met-enkephalin (10(-6) M) reduced it (to 54 +/- 6%). Both peptides failed to affect the contractile response to exogenous acetylcholine and probably act to modulate the release of acetylcholine. NPY, VIP, CGRP, SP, and somatostatin failed to induce contraction or to affect the electrically evoked contractions.

Appendix↗

Isolated adenomatous polyposis of the appendix: report of a case.

This case study describes a 61-year-old patient who had previously undergone sigmoid polypectomy. A follow-up colonoscopy revealed a polyp prolapsing into the colonic lumen through the base of the appendix. A laparoscopic-assisted appendectomy was performed, in which the cecum was exteriorized through the trocar hole so that a wide excision of the base could be performed. Fifty-eight polyps were observed in the appendix, but the base was free of polyps. Histologic studies reveal well-differentiated villous adenomatous polyposes. The patient was symptom free four years after surgery.

Adenomatous Polyps↗

The appearance of a normal appendix on barium enema examination does not rule out a diagnosis of chronic appendicitis: report of a case and review of the literature.

We report herein the rare case of a 15-year-old girl with chronic appendicitis in whom the appendix was clearly depicted by a barium enema (BE) examination. The patient presented with a 4-month history of diffuse abdominal pain and tenderness in the lower abdomen. Neither computed tomography nor ultrasonography showed any abnormal findings in the lower abdomen or pelvic cavity. Furthermore, BE examination depicted an appendix filled with the contrast medium. However, at elective surgery, she was found to have chronic appendicitis which was later confirmed histologically. Following this case report, a discussion on the value and limitations of BE examination for the diagnosis of acute and chronic appendicitis is presented.

Abdominal Pain↗