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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

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

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

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

Primary Crohn's disease of the appendix: report of 14 cases and review of the literature.

Fourteen patients with primary Crohn's disease of the appendix have been seen in a 12 year period. These patients represent 12.8% of the total number undergoing surgical resection because of Crohn's disease. Twenty-three cases of appendiceal Crohn's disease have been previously reported in the literature. A correct preoperative diagnosis is rarely made; the usual diagnosis is that of acute appendicitis or appendiceal abscess. The removed appendix in twelve of our 14 cases had marked thickening of the wall with transmural fibrosis and often with granulomatous inflammation. The enlarged appendix had an external appearance similar to that of ileal Crohn's disease, and we consider a correct surgical diagnosis might be possible with better awareness of its existence. The diagnosis might be suspected earlier when the clinical course of apparent appendicitis is protracted or atypical. Contrary to the previous estimation of high recurrence rate, this series and the cumulative evidence in the literature show a relatively low rate at 14%. The feared fistula formation following the removal of the appendix has not been seen in either our series or the literature. These patients, however, merit long-term follow-up.

Adolescent

Mucoceles of the appendix. Their relationship to hyperplastic polyps, mucinous cystadenomas, and cystadenocarcinomas.

Sixty-four cases of hyperplastic polyp, mucinous cystadenoma, and cystadenocarcinoma of the appendix were studied in relation to the development of mucocele and "pseudomyxoma peritonei." Thirty-three cases were examples of hyperplastic polyps. In 11, the appendix was transformed into a mucocele; eight were associated with mucinous cystadenoma and one with mucinous cystadenocarcinoma. The hyperplastic polyp alone was the cause of mucocele formation in two. Thirty-five cases represented examples of mucinous cystadenoma; 32 resulted in mucocele formation. Rupture with localized pseudomyxoma peritonei was found in six; generalized pseudomyxoma peritonei was encountered only once. In four of the five cases of mucinous cystadenocarcinoma, the appendix was grossly transformed into mucoceles. The histological features of mucinous cystadenoma are identical to villous adenoma of the large bowel and probably represent its counterpart within the appendix.

Aged

[The first stages of the appendix development in the human embryo].

The cecoappendicular area of 14 human embryos with a length ranging from 6 to 17 mm from vertex to coccyx (28 to 48 days old; Carnegie stages 13 to 19) was studied. The appendix is not an atrophic remain of the caecum, it develops at the same time as the caecum. The first morphological anlage of the organ is observed in human embryos from 6 to 10 mm (28 to 37 days of age; Carnegie stages 13 to 16) in the form of an elevation covered with coelomic epithelium, constituted by the mesenchyme which surrounds the anlage of the caecum. In later stages, on human embryos of 11 to 16 mm (37 to 44 days of age; Carnegie stages 16 to 18), the mesoblastic anlage of the appendix is more evident, but it is not invaded by the entoblastic cells which come from the caecum on embryos of 12 to 13 mm. On embryos of about 17 mm (48 days old; Carnegie stage 19) the entoblastic anlage of the appendix has invaded the original mesoblastic anlage. The formation of the appendix by two anlage one earlier, mesoblastic, and another later, entoblastic, is similar to that of other lymphoid organs like the sack of Fabricius in birds.

Appendix

Intussusception of the appendix caused by an adenovillous papilloma.

A case of intussusception of the appendix caused by an adenovillous papilloma in the appendix is reported. The condition was combined with acute appendicitis. The different clinical pictures are described. Due to the varied symptoms the diagnosis of intussusception of the appendix is rarely made preoperatively. The aetiology is briefly discussed. The treatment is operative with removal of the appendix or an appropriate resection.

Acute Disease

[Effect of experimental inflammation of the appendix on immunogenesis].

Effects of an operation trauma and inflammation of the appendix caused by ligation of appendicular vessels on immunogenesis were studied in experiment on rabbits. A number of antibody-containing cells of plasmatic series in the blood serum and their percentage interrelation in the appendices were determined. A stimulating effect of laparotomy on the quantitative content of antibodies in the blood was established. Accelerated differentiation and restitution of the percentage interrelationship of antibody-containing cells in the appendix were noted at earlier periods as compared with controls in which similar phenomena were observed at later terms. The development of inflammation in the appendix in the majority of animals revealed itself in an increase of the antibody content in the blood at earlier periods and a decrease thereof at later periods. In the appendix the accelerated differentiation at earlier periods wes not completed subsequently by restitution of the percentage interrelationship of antibody-containing cellular forms.

Animals

Carcinoid tumors of the appendix in children. A report of 25 cases.

Carcinoid tumors of the appendix in 25 children (21 girls, 4 boys) below 15 years corroborated the previously reported preponderance of females. 17 children were operated upon because of acute appendicitis, one because of oxyuriasis in the appendix and the remaining 7 because of diffuse recurrent abdominal pain. All the patients were subjected to appendectomy and one of them later also to right-sided hemicolectomy because of carcinoid in the margin of the resection of the appendix but not in the cecum. Despite deep infiltration of the wall of the appendix to the serosa in 9 children and lymph node metastases in one, no signs of a recurrence have been seen during follow-up of 5 to 17 years (mean 12 years).

Adolescent

Trauma to the appendix. A report of two cases.

Only two cases of trauma to the vermiform appendix are recorded in the English literature. This report adds two more cases: one due to a penetrating bullet wound of the abdomen where tangential laceration of the appendix was the only intra-abdominal injury, the other an avulsion of the appendix from the mesoappendix resulting from blunt abdominal trauma. Preoperative diagnosis of this specific lesion could not be made. Both patients were successfully managed by appendectomy.

Abdominal Injuries

Crohn's disease of the appendix: report of a case and review of the literature.

A case of Crohn's disease of the appendix that simulated a cecal tumor is presented and 27 additional cases are reviewed. Crohn's disease of the appendix most commonly occurs in the younger patient, with 90 per cent in the second and third decades of life. Before operation, appendicitis or an appendiceal abscess is commonly diagnosed. Appendectomy should be performed, if possible; otherwise a limited ileocolectomy should be done. These patients should have a long-term follow-up program because inflammation can develop later in any part of the bowel. Crohn's disease of the appendix should be included in the differential diagnosis of pain or a palpable mass in the right lower quadrant, especially in the young adult patient.

Adult

The normal and abnormal development of the appendix. A radiographic assessment.

Radiographic examinations of the colon in the adult demonstrate a wide variety of contours of the interior cecal segment and appendix. These findings are a result of the normal developmental process of the appendix which can be arbitrarily separated into four stages. Examples of appendiceal development arrested at different stages, as well as variations of the normal adult appendix, are explained and illustrated. Forms of primitive development and lack of development (agenesis) are discussed.

Appendix

Development of the rabbit appendix. I. Electron-microscopic observations.

The progressive lymphoepithelial development of the rabbit appendix was examined by light and electron microscopy. At 5 days postnatally, the appendix wall primitively resembles the mature organ but lacks both the discrete lymphoid organization and most cell types. The dome appears to be the first recipient of lymphoid cells. By 18 days the lymphoid nodule has become compartmentalized and lymphoid. The epithelium, although not fully developed, is in contact with the lymphocytes of the dome. The 5-week-old appendix has all the lymphoepithelial components of the adult but lacks the size of the mature organ. At all ages reported, plasma cells and dendritic cells are absent. Bacteria, thought to be essential to appendical lymphoid development, are not apparent at 5 or 18 days. The features common to central or peripheral lymphoid tissues are discussed.

Animals