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Epithelial neoplasms of the vermiform appendix (exclusive of carcinoid). II. Cystadenomas, papillary adenomas, and adenomatous polyps of the appendix.

Forty-two cases of benign epithelial neoplasms of the appendix gleaned from the files of the Laboratory of Surgical Pathology, Columbia University, were studied. The majority were associated with dilatation of the appendix, or "mucocele." Conversely only 50% of all cystically dilated appendices examined had definite underlying adenomatous changes. Thus it is important to distinguish between cystic neoplasms (cystadenomas) and non-neoplastic retention cysts of the appendix. The most frequently found benign epithelial neoplasms were papillary adenomas. Even in the presence of atypia we labeled them benign. Appendectomy is usually adequate treatment for all adenomatous lesions occurring in the appendix. Almost a quarter of the cases reviewed were found to have synchronous or metachronous neoplasms elsewhere in the colon, while several patients harbored multiple colonic neoplasms in addition to the appendiceal adenomas. Thus a patient with an adenomatous lesion of the appendix should undergo a systematic search for other colonic lesions.

Adenoma

Epithelial neoplasms of the vermiform appendix (exclusive of carcinoid). I. Adenocarcinoma of the appendix.

A study of primary epithelial neoplasms of the appendix (with the exception of classical carcinoids), produced 24 adenocarcinomas and 42 benign lesions from the files of the Laboratory of Surgical Pathology of Columbia University. Invasive adenoarcinomas were often complicated by appendicitis and were then unexpectedly discovered during appendectomy. In one third of the cases the carcinomas were found in juxtaposition to adenomatous lesions of the appendix. Well-differentiated adenocarcinomas of the colonic type generally had a better prognosis than signet-ring cell (or microglandular) tumors. The most reliable criterion for correlating prognosis was the Duke's method of staging. Since invasive adenocarcinomas of the appendix can metastasize to regional lymph nodes, treatment should ideally consist of ileocolectomy.

Adenocarcinoma

Mucinous tumors of the appendix associated with mucinous tumors of the ovary and pseudomyxoma peritonei. A clinicopathological analysis of 22 cases supporting an origin in the appendix.

Twenty-two cases in which mucinous tumors of the appendix were associated with mucinous tumors of the ovary are reported. The patients ranged from 23 to 83 (average 49) years of age and usually presented with increasing abdominal girth. The appendiceal and ovarian tumors were synchronous in 21 cases. Laparotomy typically disclosed large cystic ovarian tumors that averaged 16 cm in diameter and were usually multiocular, an appendix that was usually dilated and covered with mucus, and abundant intra-abdominal mucus. The ovarian tumors were bilateral in seven cases. The ovarian and appendiceal tumors were typically similar histologically, with features similar to those of ovarian mucinous cystadenomas and cystadenomas of borderline malignancy. In most of the ovarian tumors, mucin dissected through the ovarian stroma (so-called pseudomyxoma ovarii). Eight of the 20 patients with follow-up information were well when last seen, but the duration of follow-up was 3 years or less in six of them. Two patients died of pseudomyxoma peritonei 4 and 5.5 years after presentation. One patient died of a myocardial infarct shortly after laparotomy for recurrent pseudomyxoma peritonei at 11 years. The remaining patients had definite or probable recurrent or residual disease but were alive at the time of the last follow-up information. The typical synchronous presentation of the ovarian and appendiceal tumors, their histologic similarity, the frequency of bilaterality of the ovarian tumors, the predominance of right-sided ovarian involvement, and the usual presence of mucin and atypical mucinous cells on the ovarian surfaces all point toward the probable secondary nature of the ovarian tumors.

Adult

The frequency of histopathological abnormalities in incidental appendectomy in urological patients: the implications for incorporation of the appendix in urinary tract reconstruction.

In 1980 Mitrofanoff described the use of the isolated appendix as an intermittent catheterization route to empty a continent urinary reservoir. The procedure was popularized and numerous variations on the same principle were reported. Presence of histopathological abnormalities in the appendix may limit its suitability for reconstructive purposes. We studied the frequency of incidental histopathological abnormalities in appendixes removed electively in 122 urological patients during a radical pelvic operation. The implications for incorporation of the appendix in urinary tract reconstruction are evaluated. A total of 38 patients (31.1%) had notable histological abnormalities of the appendix: 35 had fibrous obliteration of the lumen, 2 had carcinoid tumor and 1 had a mucocele of the appendix. The rate of abnormal appendixes was significantly higher in elderly patients (more than 70 years old). Incidental pathology of the appendix is a frequent finding that may affect the immediate results and the late outcome of urinary tract reconstruction using the appendix. When such strategy of urinary tract reconstruction is considered, potential histopathological abnormalities should be anticipated. The patients should be informed and aware of possible unexpected changes in the preplanned procedure, while the surgeon must be familiar with these alternative reconstructive methods.

Adult

Primary epithelial tumors of the appendix and a reappraisal of the appendiceal "mucocele".

A 28-year review of the records at Hines V.A. Medical Center revealed 17 primary epithelial tumors of the appendix. Five of these tumors were benign and 12 malignant. It is suggested that the term mucocele be abandoned, because it represents the end result rather than a definite pathologic entity. The majority of benign tumors and carcinoid tumors of the appendix are discovered incidentally to other procedures. The majority of adenocarcinomas cause symptoms and signs of appendicitis. Simple appendectomy is sufficient treatment of all benign tumors of the appendix, and for all carcinoids that show no gross local metastases and are less than 2 cm in diameter. Simple appendectomy followed by right hemicolectomy or initial right hemicolectomy is the treatment of choice for all carcinoids of the appendix that show gross local metastases and are 2 cm or more in diameter and for all adenocarcinomas of the appendix, whether mucinous or colonic, in the absence of distant metastasis. A new classification for primary epithelial tumors of the appendix is suggested.

Adenocarcinoma

The gas-containing appendix: potential sonographic pitfall in the diagnosis of acute appendicitis.

The presence of gas within the appendix on plain abdominal radiographs is nonspecific and may or may not be associated with acute appendicitis. This finding, however, has not previously been reported with graded compression sonography of the right lower quadrant. Gas within the appendix was identified in four of 154 patients with a visualized appendix. All four patients had surgically confirmed acute appendicitis. Diagnostic difficulties were encountered in three of these four patients. In two patients, the findings were misinterpreted as an extraluminal gas-forming periappendiceal abscess. In an additional patient, the gas-filled appendix was initially mistaken for a segment of normal terminal ileum. The gas-filled appendix is a potential pitfall in the sonographic diagnosis of acute appendicitis. However, if other diagnostic criteria are met, gas within the appendix should not preclude establishing a sonographic diagnosis of appendicitis.

Acute Disease

Evaluation of two different dosage regimens of clindamycin and the penetration into human appendix.

The study objective was to evaluate serum, peritoneal fluid, and appendix tissue concentrations of clindamycin using two differing clindamycin regimens. Patients age 16 years and older who were about to undergo appendectomies were randomly assigned to receive gentamicin 1.5 mg/kg every 8 h admixed with clindamycin 900 mg every 8 h (8-h group) or clindamycin 600 mg every 6 h given separately (6-h group). Doses of each regimen were given preoperatively. Serum, peritoneal fluid, and appendix tissue samples were obtained intraoperatively, and frozen at -70 degrees C for gas chromatographic drug analysis. Twenty-one patients were evaluated, 11 patients in the 8-h group and 10 patients in the 6-h group. Values are reported as means +/- standard deviations. The values in the 8-h group were 12.3 +/- 14.1 micrograms/ml, 8.7 +/- 3.9 micrograms/ml, and 9.8 +/- 10.3 micrograms/g for serum, peritoneal fluid, and appendix tissue, respectively. The values in the 6-h group were 9.7 +/- 5.1 micrograms/ml, 5.8 +/- 5.3 micrograms/ml, and 6.2 +/- 4.9 micrograms/g for serum, peritoneal fluid, and appendix tissue, respectively. The 6-h group received more doses preoperatively (1.8 +/- 0.6) than the 8-h group (1.2 +/- 0.4; p less than 0.05). No differences in penetration of clindamycin into the serum, peritoneal fluid, and appendix tissue for the 8-h group and the 6-h group were noted. The study revealed a similarity in penetration of clindamycin into the serum, peritoneal fluid, and appendix tissue using either clindamycin 900 mg given by intermittent intravenous infusion every 8 h admixed with gentamicin or clindamycin 600 mg given every 6-h separately.

Adolescent

[Cystadenocarcinoma of the appendix. Report of a case].

Primary adenocarcinoma of the appendix is an uncommon disease frequently diagnosed surgery. It has an occurrence between 0.01 and 0.11% of all appendectomized patients, and it is more often found in patients older than fifty years. Symptoms are usually similar to those seen in acute appendicitis. Some authors consider the mucocele of the appendix a primary carcinoma as it is related with obstruction of the appendix lumen followed by a cystic dilatation with mucosal changes, mucus hypersecretion and extension outside of the appendix. We present one case with abdominal pain, constipation, weight loss of 26 kg, and a mass localized in the right lower quadrant. An exploratory surgery showed a partially retroperitoneal mass of 15 by 20 cm, irregular, cystic, with mucus content and the appendix included in it. The mass was removed and appendectomy was performed. Postoperatively the patient did well. The final pathologic report was a cystadenocarcinoma of the appendix.

Aged

Responsiveness of rabbit spleen and appendix cells to bacterial mitogens.

Rabbit spleen and appendix cells were used to test the mitogenic activity of a commercial lipopolysaccharide (LPS) preparation from Salmonella typhimurium (Difco), a preparation extracted from it, and cell wall preparations from smooth (45/0) and rough (45/20) strains of Brucella abortus. On the basis of [3H]thymidine incorporation ratios (E/C), that is, the incorporation rate among cells treated with the mitogen relative to that of untreated cells, the extracted LPS and both Brucella cell wall preparations, but not the commercial LPS were potent mitogens for rabbit spleen cells. By the same criterion, only the Brucella cell wall preparation produced a significant, but minimally so, response among appendix cells. The weak responsiveness of appendix cells may be more apparent than real, however, and may not imply a difference in intrinsic susceptibility to mitogens by these two populations, because unstimulated appendix cells incorporated thymidine at 10 times the rate of unstimulated spleen cells. Appendix cells, then, may not be susceptible to further stimulation, even by active mitogens. Therefore, the significance of E/C ratios may be equivocal when materials are assayed for mitogenic activity on lymphoid populations whose basal activity is relatively high.

Animals

Sonographic detection of the normal and abnormal appendix.

A prospective study of 170 patients with suspected appendicitis was performed to assess the value of sonography in detecting the normal and abnormal appendix. The wall thickness (normal, less than or equal to 3 mm), compressibility of the appendix, and echogenicity of surrounding fat were the primary criteria used to determine the status of the appendix. Of 60 patients who underwent surgery, appendicitis was proved in 45. The remaining 110 patients who did not have surgery, contacted by telephone at the end of the study, had no clinical follow-up evidence of acute appendicitis. A normal appendix was clearly identified in 102 (82%) of 125 patients without acute appendicitis. The sensitivity of sonographic examination in detecting appendicitis was 93%, the specificity was 94%, and the accuracy was 94%. The predictive value of a positive test was 86%; that of a negative test was 98%. Ruptured appendicitis was predicted in all cases (11/11). Sonography is useful in detecting acute appendicitis and can clearly show the normal appendix more frequently than previously reported.

Acute Disease

Granulomas of the appendix: is it Crohn's disease?

An epidemiologic study was performed 1) to estimate the occurrence of epithelioid granulomas in the appendix wall among patients in whom an appendectomy had been performed, 2) to identify patients with granulomas in the appendix who had symptoms and history compatible with Crohn's disease, and 3) to estimate the long-term prognosis. Within a 5-year period 6051 patients in Copenhagen County underwent appendectomy. Six patients (0.1%) had epithelioid granulomas of the appendix (0.2 per 10(5) inhabitants per year). Follow-up of the six patients for 9-11 years (median, 9.5 years) showed that all had been free of gastrointestinal symptoms since the operation. Among the 373 patients diagnosed as having Crohn's disease in Copenhagen County between 1962 and 1987, 3 patients had their disease initially confined to the appendix. Follow-up in these patients showed no recurrence within a median of 6 years (range, 4-7 years). Patients with epithelioid granulomas of the appendix have an excellent long-term prognosis, which could be explained by the fact that the condition seems to be unrelated to Crohn's disease.

Adolescent

[Mainz pouch using the appendix as the efferent limb].

Mainz pouch procedure requires intussuscepted ileum only at the efferent limb because ureters are tunneled into the colon wall in stead of the intussuscepted ileum as done in the Kock pouch procedure. We modified the Mainz pouch procedure using the tunneled appendix as the efferent limb in which intussuscepted ileum was no longer required. Four patients, three with bladder cancer and one with prostatic cancer, underwent this procedure. After total cystectomy and pelvic lymph-adenectomy the pedicled ileocecal segment was isolated. The appendix with the intact vascular supply was separated from the ileocecal segment. After the pouch was constructed, both the ureters and the appendix were tunneled into it. The pouch was then drained by a 24 Fr nephrostomy catheter. The ureters were splinted. The pouch was closed in one layer with locked running sutures. Three patients had ureteral strictures, 2 of which were corrected by percutaneous dilatation. The third one required persistent nephrostomy drainage. The appendix of all 4 cases functioned well as the efferent limb, preventing urinary leakage and allowing easy catheterization. Three of the 4 patients satisfied with this type of urinary diversion. The modified Mainz pouch procedure may be very useful because it does not require any staples to fix the intussuscepted ileum nor a long intestinal segment. Patients whose appendix can not be utilized may be candidates for the original Mainz pouch procedure.

Aged

Intussusception of the appendix. A report of four cases and review of the literature.

The clinical and pathologic features of four cases of intussusception of the appendix are reported and the literature is reviewed. All patients had vague abdominal symptoms. The diagnosis of intussusception of the appendix was not made preoperatively in any of these cases. All four patients were females who ranged from 37 to 70 years of age (mean age, 46 years). Examination of the surgical specimens showed tow appendixes that had completely inverted, one with a polyp attached at the base of the appendix forming the intussusceptum and the other with inversion of the appendiceal tip. Three cases were associated with endometriosis and one with a tubulovillous adenoma. Radiologically and endoscopically, the intussuscepted appendix may mimic a neoplastic lesion. Since intussusception may be caused by both benign and malignant conditions, appropriate management will depend on the associated cause.

Adult

Diverticular disease of the appendix.

A retrospective study of diverticular disease of the appendix was made in 3,343 consecutive instances of appendectomies. A 2 per cent incidence of diverticular disease was found. These instances were classified into four morphologic types: 1, acute diverticulitis; 2, acute appendicitis with acute diverticulitis; 3, acute appendicitis with diverticulum, and 4, appendix with diverticulum. Types 1, 2 and 3 were divided into subgroups with or without perforation. The elements of clinical behavior in each group were examined in detail. Diverticulitis of the appendix is presented as a clinically variant form of the inflamed appendix. Some followed the pattern of typical acute appendicitis. However, most were distinctive at a later age of onset, longer interval of disease, fewer or absent symptoms of the gastrointestinal tract, failure of typical abdominal pain progression, delay in surgical treatment and a remarkably high incidence of perforation. In a chi-square analysis of 56 patients with acute diverticulitis of the appendix compared with 2,503 patients with acute appendicitis, more than a fourfold incidence of perforation in acute diverticulitis was significant beyond the 0.001 level. These findings of variant behavior and high incidence of perforation are cautionary features of this frequently overlooked disease.

Acute Disease

Gas in the appendix: a sometimes significant but nonspecific diagnostic sign.

The gas-filled appendix is an uncommon roentgenographic sign that has been considered to be virtually diagnostic of acute appendicitis when the appendix is positioned caudad to the cecum in the right lower quadrant of the abdomen. Three patients had gas in the appendix in the absence of acute appendicitis. This diagnostic sign is therefore not specific for appendicitis. Regardless of its location, the appendix may contain gas despite the absence of intrinsic disease.

Acute Disease