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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

Mucocele of the appendix: imaging findings.

Mucocele of the appendix, a cystic mass resulting from a dilated appendiceal lumen caused by abnormal accumulation of mucus, is a rare entity that often is not considered when problems of the right lower quadrant are assessed. Preoperative recognition of mucocele of the appendix is important because of the possibility of rupture at surgery with development of pseudomyxoma peritonei and to predict malignant transformation. The appearances of mucoceles of the appendix on sonography, CT, and barium studies are illustrated.

Appendix

Right ureteral occlusion due to isolated Crohn's disease of the appendix.

A case of Crohn's disease limited to the vermiform appendix is described. The patient was initially thought to have an appendiceal abscess. The condition was complicated with right ureteral occlusion. Urinary stasis completely ceased after removal of the appendix. Our patient is apparently the first reported case of obstructive uropathy due to isolated Crohn's disease of the appendix.

Adult

Arterial vascularization of the vermiform appendix in human fetus.

In 50 human fetuses of the crown-rump length ranging from 88 mm to 185 mm (12 to 20 weeks), the variability of the arterial vascularization of the vermiform appendix was investigated using the injection method. 3 vascularization types were distinguished on the basis of the size and number of the supplying arteries. The obtained results were compared with those reported by other authors who studied vermiform appendix vascularization in adults. In most cases the vermiform appendix is supplied by single ramus arising from ileocolic artery.

Appendix

Motor response to electric spatial stimulation of isolated intact and inflamed human appendix.

The smooth muscle responses to electric stimulation of intact, slightly or severely inflamed, isolated human appendix strips were studied. No changes were noted in the motility of the not inflamed and slightly inflamed appendices, (simple appendicitis), the preparations responded to nervous stimulation with contractions of the same size. The smooth muscle contractions were blocked by atropine, and enhanced by physostigmine, which proves the cholinergic nature of the innervation. The severe inflammation (acute phlegmonous appendicitis) reduced also the contraction and relaxation of the appendix strips. With the advancement of the inflammatory process, the decreased motility of the appendix can play an in vivo role in the rapid progress of the process.

Appendicitis

[Goblet cell carcinoid of the vermiform appendix].

The goblet-cell-carcinoid of the appendix vermiformis lies somewhat between the carcinoid and the adenocarcinoma of the appendix concerning the biological behaviour and the histological characteristics. Referring an own well documented case (76 are well known in the available literature) the importance of the very conscientiously managed histological examination of each appendix specimen is emphasized with regard to the 5-year-survival of 73%.

Appendectomy

Crohn's disease of the appendix, manifested as acute appendicitis with postoperative fistula.

Two cases are reported of Crohn's disease localized to the appendix and manifested as acute appendicitis; after appendectomy a fistula developed. In none of 18 patients with Crohn's disease reported by other authors, where the appendix was the primary site, did a fistula develop postoperatively. When the appendix is the primary site of Crohn's disease the presence of mild inflammation of adjacent organs such as the terminal ileum may be mistakenly attributed to ordinary appendicitis. If, at exploratory laparotomy performed on a tentative diagnosis of appendicitis, Crohn's disease is suspected in the adjacent intestine, it is proposed that appendectomy should be followed by at least 10 days of total parenteral nutrition to minimize the risk of a fistula developing.

Acute Disease

[Histochemical characteristics of the secretory elements of the vermiform appendix following surgical trauma, circulatory hypoxia and immunization].

Histochemical methods were applied to determination of salomycin, dermatansulfate, keratosulfate and neutral mucopolysaccharides in the goblet cells of rabbit appendix. Sialomycin and neutral mucopolysaccharides were found in the glands. Immunization with human gamma globulin was followed in the productive phase of immunogenesis by a rise of the secretory activity of the mucosa of the appendix. An increase of the mucus formation in the appendix occurred after laparatomy and during the initial period of experimental appendicitis. However, surgical trauma caused an opposite effect against the background of increased functional activity of the secretory elements of the immunized animals, and during experimental appendicitis no activation of mucus formation was seen. The influence of the abovementioned factors on the secretory activity of the mucous membrane was accompanied by qualitative changes in the synthesized mucus.

Animals

Complement receptor lymphocytes in the rabbit I. an SIg-negative subpopulation in the appendix.

Complement receptor lymphocytes (CRL) were detected in various rabbit lymphoid tissues by the ability of these CRL to form rosettes with sheep red blood cells coated sequentially with rabbit antiserum directed against sheep red blood cell stroma and horse serum as a nonhemolytic source of complement (EAC). The rosette assay was shown to be specific for complement receptor (CR) activity and the EAC capable of detecting both C3b and C3d specific receptors. With lymphocyte preparations containing less than 5% phagocytic cells, the average per cent CRL in the various tissues studied was as follows: thymus 1%, popliteal lymph node 18%, spleen 30%, appendix 35%, and peripheral blood 45%. Double assays in which the lymphocytes were prestained with an FITC-labeled Fab fragment of a goat anti-rabbit Fab antibody before rosetting indicated that CRL were a subpopulation of surface immunoglobulin (SIg)-bearing lymphocytes in popliteal lymph node, spleen, and peripheral blood. In the appendix, however, in addition to finding SIg+ CR+ and SIg+ CR- populations, an SIg- CR+ population was consistently found. Double assays employing FITC-labeled goat antibodies specific for mu, alpha, and gamma determinants were also performed to determine if there was any relationship between the class of Ig displayed and presence of CR. It appeared that an approximately equivalent percentage of both IgM- and IgG-bearing cells also displayed CR. Experiments in which appendix cells were treated with Pronase to remove SIg and CR and the cells cultured in vitro to allow regeneration of surface markers confirmed the existence of SIg+CR+, SIg+CR-, and SIg-CR+ lymphocyte subpopulations. Whether the SIg-CR+, population represents a developing B cell population which will eventually also express SIg or whether it belongs to the T or "null" cell populations is unclear at present.

Animals

Granular cell tumour of the appendix in a patient irradiated for a rectal carcinoma.

We report on a 47-year-old man with a granular cell tumour of the appendix, discovered incidentally during surgery for a rectal adenocarcinoma that had been irradiated preoperatively. A detailed immunocytochemical analysis revealed positivity for S-100 and neuron-specific enolase (NSE). Electron microscopically, the cytoplasm of the tumour cells contained numerous pleomorphic lysosomes. In the appendix tissue adjacent to the tumour a neuroma and the histological features of radiation injury were present. Our findings suggest that this granular cell tumour may have originated from a pre-existing appendix neuroma which underwent granular degeneration, possibly as a result of radiation.

Appendiceal Neoplasms

Calcified mucocele of the appendix presenting as ureteral obstruction.

Mucocele of the appendix, a rare lesion, occurs in 0.3% of patients undergoing appendectomy. Only 46 cases of calcified mucocele have been reported. Complications reported include appendiceal intussusception, rupture resulting in acute abdomen, and infection. We report the case of a 74-year-old man with a calcified mucocele of the appendix that was discovered in the evaluation of a ureteral obstruction. During exploratory surgery, the patient was found to have a 6 x 5 cm appendiceal tumor and underwent a right ileocolectomy. Pathologic examination showed calcified mucous cystadenoma of the appendix. Calcification of a mucocele is believed to denote chronicity. Our case is the first report of ureteral obstruction secondary to calcified mucocele and the second calcified mucocele to be seen on computerized tomography. Calcified mucocele should be included in the differential diagnosis of any calcified tumor in the right lower quadrant.

Aged

[Carcinoid tumor of the appendix in children].

Carcinoid tumor of the appendix has been known since 1808, and currently we know that it may appear all along the intestinal tract, biliary tree, ovaries, bronchi, lungs and pancreas. It is usually uncommon during infancy, representing 1.8% of all the tumors of the intestinal tract, approximately 70% of the appendiceal tumors and 0.16 to 0.7% of all operated appendixes. It is more frequent in females. With no typical clinical picture, it is usually an incidental finding, as well as a cause of inflammatory processes of the appendix. When the diameter is less than 1.0 cm, the treatment of choice is appendectomy; and in tumors of larger size, surgery is more extensive. Two cases are reported.

Appendectomy

[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

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