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Villous adenoma of the appendix is a rare pathologic entity, the treatment is controversial. We describe the case of a patient complaining of abdominal pain that was diagnosed as acute appendicitis. Pathological examination of the surgical specimen revealed a villous adenoma of the appendix. We comment the therapeutic options and we review the literature about this pathological entity.
In a glycohistochemical analysis of human appendix vermiformis we report the assessment of lectin binding in cells of the Gut Associated Lymphoid Tissue of normal samples and in acute appendicitis using a panel of plant, invertebrate and mammalian lectins with specificity for alpha-L-Fuc (UEA-I), alpha-D-Gluc and alpha-D-Man (Con A), alpha-D-GalNAc (DBA), GalNAc (SBA, HPA), beta-Gal (RCA-I, 14 kDa = galectin-1) and alpha-, beta-Gal (VAA). Moreover, we initiate the study of expression of carbohydrate-binding sites in this tissue and in colonic mucosa, employing several types of carrier-immobilized carbohydrate ligands as suitable probes for this purpose. Within the three populations of macrophages intra-/subepithelial macrophages of the dome region, the lamina propria of the intercryptal region and the follicle-associated epithelium were apparently reactive with most of the lectins and also with mannose and fucose residues of the tested neoglycoproteins. Distinguishing features of germinal center macrophages in relation to intra-/subepithelial phagocytes were the lack of binding of UEA-I and DBA. In comparison to all other types of phagocytes, macrophages of the T-region displayed a rather restricted binding capacity only to Con A and RCA-I. Labeling of macrophages with SBA, HPA and VAA in this location was only rarely found. With respect to dendritic cells no consistently positive reaction was seen for follicular cells, whereas interdigitating cells of the T-region bound Con A, HPA and RCA-I, and, less frequently, SBA. Lymphocytes in all anatomical subsites of the Gut Associated Lymphoid Tissue, centrocytes, centroblasts and plasma cells had binding sites for Con A and RCA-I in common. Notably, a small number of lymphocytes mostly in the T-region but also in B-cell-rich areas expressed intranuclear binding sites for fucose and mannose residues. Intraepithelial lymphocytes and lymphatic cells of the T-region differed from lymphocytes in other regions by a more frequent expression of VAA-binding sites. The epithelium of appendix vermiformis and colonic mucosa not only presents lectin binding sites, but also has the capacity to bind carbohydrate structures, as shown by labeled glycoligand-exposing neoglycoproteins. In normal mucosa the extent of binding appeared to be associated with maturation of cells, the surface epithelium showing the most intense staining reaction. This pattern is not detectable in colonic adenoma which reveal increased intensity, when compared to normal mucosa. In contrast to development of hyperplasia, acute inflammation in appendicitis caused no detectable changes of neoglycoprotein binding. Taking our previous assessment on lectin binding in appendicitis into account, we conclude that glycosylation of goblet cell mucus, but not the capacity to bind certain sugar epitopes responds to inflammatory processes, whereas tumorigenesis of colonic adenoma can also affect the binding of neoglycoproteins.
A case of primary acute appendiceal torsion in a 6-year-old boy with symptoms suggestive of acute appendicitis is presented. The appendix was abnormally long, measuring 13.5 cm in length. Although appendicitis is the most common intra-abdominal surgical emergency, there are few descriptions of primary acute appendiceal torsion, a rare cause of an acute abdomen. A review of the English language literature disclosed 19 reports, including the present, with 11 pediatric cases. The site of torsion occurs most frequently 1 cm or more from the appendiceal base. Rotation varies from 270 degrees to 1080 degrees with a mean of 580 degrees. The direction of the rotation is more frequently anticlockwise. Appendix is most commonly described as lying free or pelvic. In children the mean age is 9.1 years, the range 3-16 years, and the male-to-female ratio 4.5:1.
BACKGROUND: Mucocele is a cystic dilatation of the vermiform appendix that contains mucous material. It may be caused by benign or malignant diseases. AIM: To report and discuss four cases with mucocele. REPORT OF CASES: The main clinical manifestations were abdominal pain and changes in the bowel habits. In two cases, appendiceal mucocele was an incidental finding in the diagnostic work-up or operation for acute diverticulitis and acute cholecystitis, respectively. The diagnostic approach included barium enema and CT scan of the abdomen. In three cases, the mucocele was secondary to mucinous cystadenoma; two of them had a preoperative diagnosis of mucocele and underwent colonic preparation and right hemicolectomy, one patient underwent appendectomy alone. The remaining case underwent appendectomy alone, was found to have mucinous adenocarcinoma, and underwent a right hemicolectomy in a second operation. Postoperative outcome was adequate in all cases. CONCLUSION: Mucocele of the vermiform appendix is a rare disease. An appendectomy is an adequate treatment for benign disease. If malignant disease is demonstrated, a right hemicolectomy should be performed.
One hundred and five appendices removed at the time of surgical reduction of intussusception in children were studied by light microscopy after routine procedures to search for aetiological factors involved in intussusception. Normal pediatric appendix specimens served as controls (n = 30). Light microscopic examination showed viral inclusions in epithelial cells in 48 of 105 appendices (45%) from the cases of intussusception. No viral inclusion was observed in controls. Immunohistochemistry performed on 21 appendices from intussusception with a monoclonal antibody against adenovirus showed intranuclear positivity in all appendices with viral inclusions. Viral inclusions seen in epithelial cells of appendices from cases of intussusception are caused by virus and in particularly by adenovirus. The etiological factors involved in intussusception without viral inclusion in appendix remain unknown.
A case of a malakoplakia of the appendix in a sixty-year-old woman is reported. No involvement of the organs or important general diseases were present and the patient, after the appendicectomy, promptly recovered. Only three cases of malakoplakia of the appendix have previously been described in the literature and they are briefly summarized.
We investigated the molecular basis for the appearance of V(H)a2 allotype-bearing B cells in mutant Alicia rabbits. The mutation arose in an a2 rabbit; mutants exhibit altered expression of V(H) genes because of a small deletion encompassing V(H)1a2, the 3'-most gene in the V(H) locus. The V(H)1 gene is the major source of V(H)a allotype because this gene is preferentially rearranged in normal rabbits. In young homozygous ali/ali animals, the levels of a2 molecules found in the serum increase with age. In adult ali/ali rabbits, 20 to 50% of serum Igs and B cells bear a2 allotypic determinants. Previous studies suggested that positive selection results in expansion of a2 allotype-bearing B cells in the appendix of young mutant ali/ali rabbits. We separated appendix cells from a 6-wk-old Alicia rabbit by FACS based on the expression of surface IgM and a2 allotype. The VDJ portion of the expressed Ig mRNA was amplified from the IgM+ a2+ and IgM+ a2- populations by reverse transcriptase-PCR. The cDNAs from both populations were cloned and sequenced. Analysis of these sequences suggested that, in a2+ B cells, the first D proximal functional gene in Alicia rabbits, V(H)4a2, rearranged and was altered further by a gene conversion-like mechanism. Upstream V(H) genes were identified as potential gene sequence donors; V(H)9 was found to be the most frequently used gene donor. Among the a2- B cells, y33 was the most frequently rearranged gene.
The authors present a case of a 36 year old male, admitted with a 3 year history of cramping abdominal pain, fever, and transient finding of a right lower quadrant mass. The abdominal roentgenogram revealed a large opacity located on the right side of the fifth lumbar segment. The patient underwent laparotomy: an enlarged appendix was removed. The specimen had 4 calculi completely obstructing the lumen of the appendix. The diameter of calculi were 0.5 (in 3 instances) and 3 cm. The case herein described is rather uncommon, due to: 1) the rarity of multiple appendiceal calculi; 2) the size of the larger stone; 3) the inconstant finding of right lower quadrant mass; 4) the non development of acute appendicitis. Differential diagnosis involved several diseases presenting with palpable mass of the lower quadrant of the abdomen and/or laminated densities at the plain abdominal roentgenogram.
Two cases of invagination of the Vermiform appendix associated with endometriosis are presented. Classification, incidence, etiology, symptomatology, diagnosis and treatment are discussed. The literature is reviewed in brief, and it is concluded that the possibility of invagination of the Vermiform appendix should be considered in obsure abdominal cases with recurrent right lower quadrant pain.
Adenocarcinomas of the ileum and appendix are uncommon. Multiple synchronous adenocarcinomas of the small bowel without carcinoid features are even more unusual, with only seven reported cases in the literature. The radiologic and pathologic findings are reported in a case with 13 separate, well-documented synchronous primary adenocarcinomas of the ileum and appendix. The value of the double-contrast small bowel enema is emphasized.
A specimen from the small intestine with multiple (three) classic carcinoid tumors and one appendiceal carcinoid tumor displaying argentaffinity, argyrophilia (Grimelius stain), and serotonin and neuron specific enolase immunoreactivity was examined by light microscopy with regard to the tumor cell histopathogenesis. The smallest tumor (diameter 0.5 cm) from the small intestine was cut into 512 and the appendiceal tumor into 511 serial sections, which were stained with the argyrophil technique. In the small intestine an increased number of endocrine cells and small proliferating aggregates of endocrine cells were observed among nonendocrine enterocytes in the crypts of Lieberkühn. They seemed to grow initially inside the crypts and to later infiltrate through the basement membrane into the lamina propria of the mucosa. This finding suggests that classic carcinoid tumors of the small intestine develop from mucosal endocrine (enterochromaffin) cells. Since proliferating argentaffin cells were also seen in the mucosal crypts in one of the other two carcinoid tumors (2 cm in diameter) in the same intestine specimen, it is suggested that when multiple carcinoid tumors occur in the small intestine they arise from multiple sites. There was no apparent connection between the mucosal crypts and the carcinoid tumor of the appendix. Thus in this particular case, the appendiceal carcinoid tumor did not appear to derive from the mucosal endocrine cells but from the subepithelial endocrine cells that are present in the lamina propria and submucosa of the appendix wall. Supporting this view is the fact that S-100 protein immunoreactive cells are found both in close relation to subepithelial endocrine cells and as an integral component of appendiceal carcinoid tumors.
Ten cases of primary adenocarcinoma of the vermiform appendix are presented. The condition is rare and usually presents after middle age as acute appendicitis. It is seldom recognized during appendicectomy. The tumours resemble colonic adenocarcinomas but, because of peculiarities in the anatomy of the appendix, there is a tendency to early local spread. Right hemicolectomy performed as soon as the true nature of the lesion is recognized is the treatment of choice and has been shown to improve survival. Even after hemicolectomy the prognosis must be guarded, as local spread of disease may not be controlled in many cases.
Forty carcinoid tumours of the appendix were identified in children under 15 years of age between 1957 and 1986 from the records of the West Midlands Regional Children's Tumour Research Group. A significant excess of girls was observed (P = 0.02). All tumours were discovered incidentally and two patients underwent further surgery. Twenty-two of the 29 confirmed locations (76 per cent) were at the tip of the appendix and 34 of the 40 tumours (85 per cent) had invaded beyond the appendiceal submucosa. The reported incidence rate of 1.14 per million children per year remained constant throughout the study period. No recurrences, metastases or deaths from tumour were detected. Appendicectomy alone is confirmed as usually curative, although right hemicolectomy is occasionally necessary. Caution during routine appendicectomy is recommended to avoid possible tumour damage, as is consistent histological examination of all appendices in the paediatric age group. The importance of long-term follow-up is emphasized.
BACKGROUND: Traditionally epithelial malignancies of the appendix with or without carcinomatosis have been treated by right hemicolectomy. Recent accumulation of a large number of patients with this disease has enabled a re-evaluation of this surgical judgement. METHODS: Clinical data on 501 patients with epithelial malignancy of the appendix were collected prospectively. All patients had peritoneal seeding at the time of referral and were treated by cytoreductive surgery and perioperative intraperitoneal chemotherapy. The main independent variable for statistical analysis was the surgical procedure used to resect the primary cancer (appendicectomy alone versus right hemicolectomy). Nineteen other clinical and pathological variables were considered as control variables. The endpoint for all analyses was survival. RESULTS: Median follow-up after the initial diagnosis was 4 years. The rate of regional lymph node positivity was 5.0 per cent. When the incidence of lymph node metastasis was determined by histological type, it was statistically significantly higher in intestinal (66.7 per cent) than in mucinous (4.2 per cent) tumours (P < 0.001). The presence of lymph node metastases had no influence on prognosis (P = 0.155). The surgical procedure (appendicectomy alone versus right hemicolectomy) had an influence on patient survival by univariate analysis (P < 0.001), but not by multivariate analysis (P = 0.258). CONCLUSION: Right hemicolectomy does not confer a survival advantage in patients with mucinous appendiceal tumours with peritoneal seeding. These data suggest that right hemicolectomy should be avoided unless metastatic involvement of the appendiceal or distal ileocolic lymph nodes is documented by biopsy, or the resection margin is inadequate.
BACKGROUND AND OBJECTIVES: Tumor markers are a clinical tool frequently used in oncology in association with other clinical and radiologic information. For gastrointestinal cancer, carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA 19-9) tumor markers have found selected clinical application. The use of these tumor markers in mucinous epithelial tumors of the appendix has not been previously determined. METHODS: In patients with peritoneal dissemination of a mucinous epithelial malignancy of the appendix, tumor markers CEA and CA 19-9 were prospectively recorded preoperatively within 1 week prior to definitive treatment. Also, if the appendiceal tumor recurred, the tumor marker was determined. The accuracy of these two tumor markers in the management of this disease was determined for these two specific clinical situations. RESULTS: CEA was elevated in 56% of 532 patients and CA 19-9 was elevated in 67.1% of these patients. Although the absolute level of tumor marker did not correlate with prognosis, a normal value indicated an improved survival. CEA was elevated in 35.2% of 110 patients determined to have recurrent disease; CA 19-9 was elevated in 62.9% and at least one of the tumor markers was elevated in 68.2% of patients. An elevated CEA tumor marker at the time of recurrence indicated a reduced prognosis. CONCLUSIONS: Both CEA and CA 19-9 tumor markers were elevated in a majority of these patients and should be a valuable diagnostic tool previously underutilized in this group of patients. These tumor markers were also of benefit in the assessment of prognosis in that a normal level indicated an improved prognosis. At the time of a reoperative procedure, CEA and CA 19-9 tumor markers gave information regarding the progression of disease. These tumor markers have practical value in the management of epithelial appendiceal malignancy with peritoneal dissemination.
Primary adenocarcinoma of the appendix is rare; approximately 200 cases have been reported. Preoperative diagnosis is extremely difficult. We report two cases with two distinctive histologic patterns of adenocarcinoma resulting in different outcomes. The classification and treatment of primary epithelial malignant tumors of the appendix are also discussed.
An unusual case of a 22-year-old white female with known chronic ulcerative colitis presented with a several-day history of lower abdominal pain and a pelvic mass. Laparotomy revealed a primary carcinoma of the appendix with Krukenberg metastasis to both ovaries. Pathologically this tumor appeared to arise from an appendix which showed no evidence of chronic ulcerative colitis and therefore could not be associated with the above-mentioned entity.
Five patients with primary adenocarcinoma of the appendix are reported. All patients presented with symptoms resembling those of acute appendicitis or periappendicular abscess. In none of them was malignancy suspected prior to operation. It is advisable that every patient above 50 years of age presenting with symptoms of appendicitis undergo laparotomy, thus enabling a better exploration of the cecal region. If an appendiceal mass is present and frozen section shows malignancy, a right hemicolectomy should be performed. The prognosis of adenocarcinoma of the appendix is dismal as most patients present with an advanced stage of the disease.