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Appendiceal mucinous cystadenoma presenting as "porcelain" appendix with myxoglobulosis--a rare cause of a right lower quadrant mass.

Mucinous cystadenoma is a rare tumour of the vermiform appendix and is associated with cystic dilatation of the appendix, to which the more general term of mucocoele has been applied. Mucocoele of the appendix is only a descriptive term for abnormal mucus accumulation causing distension of the appendiceal lumen, irrespective of the underlying cause. Pre-operative diagnosis of appendix mucocoele, though infrequently made, is important as some of these lesions may be malignant, and also is essential in order to avoid the risk of rupture at surgery with subsequent development of pseudomyxoma peritonei. The appearances of mucocoele of the appendix and its uncommon variant of myxoglobulosis on plain radiograph, ultrasound and barium study are presented, together with review of the literature.

Appendectomy↗

Epithelial noncarcinoid tumors and tumor-like lesions of the appendix. A clinicopathologic study of 184 patients with a multivariate analysis of prognostic factors.

BACKGROUND: The current understanding of appendiceal epithelial tumors is based on series composed of relatively small numbers of patients and on case reports. The aim of this study was to perform clinicopathologic correlation, particularly concerning prognosis, on a relatively large series of patients with epithelial tumors and tumor-like lesions of the human vermiform appendix. METHODS: One hundred eighty-four cases of simple mucoceles, hyperplastic polyps, adenomas, carcinomas, and mucinous tumors of uncertain malignant potential (UMP) were reviewed. Retrospective follow-up data were obtained in carcinoma and UMP tumor cases. RESULTS: Most neoplasms were of the mucinous type characteristic of the appendix, but a small fraction were nonmucinous and resembled neoplasms typical of the spectrum encountered in the colorectum. Both benign and malignant conditions in some cases showed acellular mucin dissecting the appendiceal wall and mucin outside the appendix. Immunostains were sometimes helpful in identifying epithelial cells within mucus, but hematoxylin and eosin and mucin stains were adequate in most cases. Two factors were significantly associated with survival by multivariate analysis: the presence of mucin outside the right lower quadrant of the abdomen and the presence of epithelial cells in the peritoneal cavity outside the appendix. CONCLUSIONS: The distribution of mucin within the abdomen and the presence of cells outside the visceral peritoneum of the appendix are important prognostic factors that should be recorded in these neoplasms. Tumors designated as UMP lesions behave in a benign or low grade fashion.

Adenocarcinoma↗

Primary adenocarcinoma of appendix, colonic type associated with perforating peritonitis in an elderly patient.

We report a case of colonic type adenocarcinoma of the appendix with perforating peritonitis in a 92-year-old man. The preoperative diagnosis was localized peritonitis due to acute appendicitis and emergency laparotomy was performed. A gray, hard tumor was palpated at the base of the appendix. Appendiceal cancer was suspected, and right hemicolectomy was performed. The histopathological diagnosis was moderately differentiated adenocarcinoma of the appendix. The tumor obstructed the orifice of the appendix, and this may have caused the perforation of the appendix. The patient had an uneventful postoperative course and there have been no signs of recurrence in the 2 years since the operation.

Adenocarcinoma↗

Androgen and oestrogen receptor status of the human appendix testis.

Since the human appendix testis is a Mullerian duct derivative it has been theorised that rising oestrogen levels in puberty result in enlargement of this organ and consequent predisposition to torsion. This study sought to establish the presence or absence of both androgen and oestrogen receptors in the human appendix testis. Bilateral appendix testis specimens surgically excised from ten patients undergoing scrotal exploration for acute scrotum were stained immunohistochemically for androgen and oestrogen receptors. These were examined by light microscopy. The human appendix testis was found to express both oestrogen and androgen receptors. Marked regional variation of androgen and oestrogen receptor positivity was demonstrated. The surface epithelium and some stromal cells were androgen receptor positive, whereas oestrogen receptors were confined to ductular invaginations, gland-like structures and some stromal cells. Rising levels of androgens and oestrogens in pubertal boys may account for enlargement and the predisposition of the human appendix testis for torsion since this structure contains receptors for both.

Adolescent↗

Leukaemia and lymphoma of the appendix presenting as acute appendicitis or acute abdomen. Four case reports with a review of the literature.

Leukaemic and lymphomatous infiltration of the appendix is rare and even rarer is acute appendicitis as the initial manifestation. From our routine biopsy material we collected four cases of haematological malignancies presenting as acute appendicitis or acute abdomen, caused or accompanied by tumoral infiltration of the appendix. Appendicitis was the initial manifestation that allowed diagnosis of the underlying disease. The clinical histories and histological examinations of the appendices and of one autopsy are described. We report the first detailed description of acute myeloid leukaemia involving the appendix, and three cases of lymphomatous infiltration of the appendix presenting with appendicitis, and give an overview of the literature. In these days of budgetary cuts in national health services, where one may be tempted not to have seemingly commonplace cases of appendicitis histologically verified, our cases emphasize that careful histopathological examination of all appendectomy specimens should be mandatory. Despite the fact that leukaemia and lymphoma of the appendix are rare, our cases illustrate that these must be included in the differential diagnosis of acute appendicitis and that physicians and surgeons have to be aware of these conditions.

Abdomen, Acute↗

Goblet cell carcinoid of the appendix endoscopically diagnosed and examined with p53 immunostaining.

We report a case of a 62-year-old woman with goblet cell carcinoid of the appendix. She was admitted to our hospital in September 1994 after the discovery of liver tumors. After admission, a tumor in the right kidney and multiple tumors in the liver were found. She was diagnosed with renal cell cancer and metastasis to the liver and underwent excision of the kidney and enucleation of the largest liver tumor. Histological examination revealed that the liver tumor was a metastatic carcinoid tumor. As carcinoid tumors have frequently been found in the appendix, endoscopic examination was performed and a lesion was found in the appendix by colonoscopy. As predicted, the biopsy specimen was a carcinoid tumor, and she underwent an appendectomy. Histologically, the tumor was a goblet cell carcinoid. Goblet cell carcinoid is a rather rare neoplasm that has the histologic features of both carcinoids and adenocarcinoma. Forty-two cases of goblet cell carcinoid of the appendix have been reported thus far in Japan. However, few were diagnosed via endoscopic examination before surgical operation. We also carried out an immunohistochemical study with anti p53 antibody on the goblet cell carcinoid tumor of the appendix. Most tumor cells were strongly positive, while in three benign carcinoid tumors investigated simultaneously they were negative. These findings suggest that goblet cell carcinoid has an aggressive phenotype compared with benign carcinoid tumors.

Appendiceal Neoplasms↗

Laparoscopic appendectomy for Crohn's disease of the appendix presenting as acute appendicitis.

BACKGROUND: Crohn's disease confined to the appendix is rare but has been well described in the literature. It can mimic acute appendicitis clinically. After surgical treatment, recurrences of Crohn's disease are rare. We report the first case of treatment by laparoscopic appendectomy of Crohn's disease confined to the appendix. METHODS: A healthy 32-year old man presented with a week-long history of vague lower abdominal pain. Diagnostic work-up, which included CT, enteroclysis, and routine blood work, revealed a patent appendiceal lumen with an inflammatory mass in the right lower quadrant. RESULTS: Diagnostic laparoscopy revealed an inflamed appendix, and a laparoscopic appendectomy was performed, with frozen-section examination revealing Crohn's disease of the appendix. Two years after surgery, the patient has not had a recurrence of symptoms. CONCLUSIONS: Crohn's disease of the appendix can mimic acute appendicitis, although often with a more indolent course. The disease may be treated successfully by laparoscopic appendectomy, with good long-term results.

Acute Disease↗

Appendiceal colic in childhood: the role of inspissated casts of stool within the appendix.

Seventy pediatric patients with histories of recurrent crampy abdominal pain, right lower quadrant tenderness, and proven filling defects or distention of the appendix on radiographs, have been subjected to appendectomy. Inspissated fecal material was found within the appendix in 72.8% of such children. Fecal material was found in only 12.9% of 155 normal appendices and in 33.8% of 530 acutely inflamed appendices. It is postulated that inspissated fecal material acts as a foreign body of the appendix, leading to intermittent obstruction and distention of the appendix--appendiceal colic. An 86% correlation between the appendiceal filling defects seen on radiographs and the inspissated fecal material found within the appendix at operation has been noted. Ninety-six per cent of these 70 children had been relieved of their recurrent abdominal pain by appendectomy. Appendiceal colic characterized by crampy abdominal pain, right lower quadrant tenderness, and appendiceal filling defects appears to be a veritable and verifiable diagnosis.

Abdomen, Acute↗

[Torsion of the appendix testis].

The differential diagnosis of an acute scrotum includes spermatic cord torsion, torsion of a testicular appendage, torsion of spermatocele, epididymitis, orchitis, trauma, hernia, testicular segmental infraction and tumor. Among these, torsion of appendix testis could got dramatic improvement if accurately diagnosed and treated. 5 patients of torsion of appendix testis have been identified in our hospital in the recent 5 years. Ages ranged from 9 to 13 years old (mean age 11). The lesions were on the right side in 2 cases and left side in 3 cases. Duration of scrotal pain ranged from 2 to 7 days (mean of 4 days). The urine analyses were normal in all cases. The white blood counts were all within normal limits with mean of 5.72 x 10(3)/ul. No abnormality in passing urine or other infectious sign could be detected. Doppler ultrasonography or nuclear medicine testicular scan were performed selectively due to clinical availability. The result was equivocal and did not support a definite diagnosis. All 5 cases received surgical treatment. The necrotic testicular appendix was excised and reactive hydrocele treated. All the patients were discharged from the hospital the day after operation and recovered quickly. The pathological report revealed congestion and extensive hemorrhagic necrosis of the testicular appendix. Advances in technology have been helpful in improving the accuracy of diagnosis but technology is not infallible and an over reliance on it can also result in misdiagnosis. Various reports supporting the use of scrotal ultrasound in evaluating cases of acute scrotum pain. Ultrasonography has definitely helped in detecting scrotal pathology but its limitations need to be appreciated. Because of the difficulty of making an accurate diagnosis in acute scrotum, misdiagnosis and delayed operation offer result in disappointment of testis salvage rates. We have supported a policy of early scrotal exploration in any case suspicious of torsion of appendix testis.

Child↗

[Association of carcinoid tumor of the appendix and Crohn disease (case report and review of the literature)].

The authors describe the coexistence of the carcinoid of the appendix and Crohn's disease. In the case of their woman patient the carcinoid was identified with the examination of the resected ileoascendent part of the bowel resulting of the complication of the Crohn's disease. 10 similar association is known in the literature but none of the patients had the signs of the carcinoid syndrome. Subsequent adrenerg syndrome after an alimentary hypoglycemia (increased evacuation of the cathecolamines and their metabolites in the urine), food allergy (increased IgE type antibody to milk protein) or gastroenteropancreatic (GEP) neuroendocrine tumor (based on the result of the 111In-octreoscan and the increased 5-hydroxyindolaceticaciduria) equally suspected of the symptoms (palpitation, flush) manifested after the operation. They look for the connection between the genesis of the intestinal carcinoid and the Crohn's disease with working up the bibliography. Summing up the references with a view of the latest it can be stated that the carcinoid of the appendix occurs more than orders of magnitude in the samples from inflamed surrounding than the samples from autopsy (0.24%--820/338,000 inflamed appendix and 0.03%--19/53,430 appendix from autopsy). Consequently the inflammation create favourable condition for the development of the carcinoid of the appendix.

Adult↗

Mucocele of the appendix with hematuria.

Mucocele of the appendix, a nonspecific and descriptive term for an abnormal mucous accumulation within the appendiceal lumen, regardless of the underlying cause, is a rare clinical disease that is not usual as a consideration in the differential diagnosis of right lower quadrant lesions. The reported prevalence of mucocele of the appendix in appendectomy specimens is 0.2-0.3 percent. Hematuria due to mucocele of the appendix is extremely rare. Up to now, only few cases of such condition have been recorded in the literature. We describe a woman who experienced intermittent episodes of right lower quadrant pain and hematuria. Abdominal exploration incidentally displayed mucocele of the appendix. No evidence of other lesions was found. The patient was still in good health after operation. This case highlights mucocele of the appendix as a consideration in the differential diagnosis of right lower quadrant pain with hematuria.

Aged↗

Carcinoid tumors of the appendix. Our experience in a university hospital.

OBJECTIVE: To present our experience of carcinoid tumors of the appendix managed at a university teaching hospital. Complex symptomatology, varied biochemical affections and different surgical therapeutic modalities are discussed. METHODS: The medical records of all the patients who underwent consecutive appendectomies at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia from 1994 to 1999 were retrospectively analyzed. The data of patients identified to have histological evidence of carcinoid tumors of the appendix were further reviewed for the demographic details, indications for surgery, surgical procedure, tumor localization in the appendix and size; concomitant appendicitis and further surgical procedures were considered. RESULTS: During the study period, 1547 appendectomies were performed and, out of these, 9 (0.6%) cases were reported to have carcinoid tumors of the appendix. There were 4 male and 5 female patients, age range 17-51 years (median 29.8 years). Seven subjects had a clinical evidence of appendicitis while 2 presented with chronic abdominal pain. There were 6 open and 3 laparoscopic appendectomies. Six carcinoid tumors were encountered at the appendiceal apex, 2 at the midportion, and one at the base with a mean diameter of 9.5 mm (range, 4-19 mm). One patient had histologically confirmed residual tumor, which necessitated a right hemicolectomy 3 weeks later. All patients remained disease-free during a mean follow up of 7 years (range, 4-10 years). CONCLUSION: Carcinoid tumors of the appendix are extremely rare and invariably remain asymptomatic. Simple appendectomy offers adequate relief while the need for further extensive surgery depends on tumor characteristics and dissemination. Despite an excellent prognosis, all reported patients should be followed up with urinary 5-hydroxyindoleacetic acid and abdominal ultrasonography.

Abdominal Pain↗

Effect of cecum and appendix on 7 alpha-dehydroxylation and 7 beta-epimerization of chenodeoxycholic acid in the rabbit.

The effect of surgical removal of cecum and appendix on bile acid metabolism was studied in the rabbit. Bile acid composition of bile and intestinal contents were analyzed by gas-liquid chromatography and individual bile acids were further identified by mass spectrometry. In normal, intact rabbits (group I), the biliary bile acids consisted largely of deoxycholic acid (DA) 95.3 +/- 1.0 (mean +/- SE) %; cholic acid (CA) 2.3 +/- 1.1% and lithocholic acid (LA) 1.0 +/- 0.3% were also detected. Removal of cecum and appendix (group II) produced significant changes in biliary bile acid composition: DA fell to 58.3 +/- 31.8%, CA rose to 37.7 +/- 10.4%, and LA was barely detectable (0.3 +/- 0.1%). Administration of chenodeoxycholic acid (CDA), 125 mg/day, produced severe hepatotoxicity, reduced food intake, and produced weight loss (group III). Biliary LA rose to 15.0 +/- 1.3%, while DA was 61.3 +/- 14.0% and CDA 21.8 +/- 14.6% of total biliary bile acids. Feeding CDA to animals without cecum and appendix (group IV) resulted in a slight increase of LA (3.2 +/- 2.2%) compared to group III, and appreciable amounts of ursodeoxycholic acid (UDA) 32.0 +/- 9.8% and of 7-ketolithocholic acid (7-KLA) 3.0 +/- 0.6% appeared in bile. The animals of group IV exhibited no hepatotoxicity and ate and gained weight normally. These results indicate that the microbial population of cecum and appendix is active in 7alpha-dehydroxylation of primary bile acids and that removal of these organs results in an increased formation of UDA by an unknown mechanism.-Yahiro, K., T. Setoguchi, and T. Katsuki. Effect of cecum and appendix on 7alpha-dehydroxylation and 7beta-epimerization of chenodeoxycholic acid in the rabbit.

Animals↗

The vermiform appendix: not a useless organ.

The appendix has often been seen more as a nuisance rather than an important part of the human anatomy. Early misconceptions have led to the indiscriminate removal of the appendix from the body. Long thought to be an evolutionary remnant of little significance to normal physiology, the appendix has more recently been identified as an important component of mammalian mucosal immune function, particularly B-lymphocyte-mediated immune responses and extrathymically derived T-lymphocytes. This structure helps in the proper movement and removal of waste matter in the digestive system, contains lymphatic vessels that regulate pathogens, and lastly, might even produce early defences that prevent deadly diseases. The appendix is one of the guardians of the internal environment of the body from the hostile external environment.

Appendix↗

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↗

Experimental model for the study of the human appendix.

A simple and reproducible model for the experimental study of the normally functioning human appendix is described. The appendix removed from a human is transplanted into a nude mouse and is kept vital for at least 4 hours. This model is an improvement on previous models. It corresponds well with the working of the normal human appendix as regards tissue type and characteristics. Results are easily quantified and a paired control is possible in the experimental animal. This simplifies the experimental design and statistical calculations. The model can be used to gain additional information about the pathophysiology of the human appendix and the possible influence of drugs on this organ.

Animals↗

Germinal centers and the B-cell system. VI. Migration pattern of germinal-center cells of the rabbit appendix.

The migration pattern of germinal center cells of the rabbit appendix was studied and compared with that of appendix dome cells, spleen cells, thymus cells and thoracic duct lymphocytes. To discriminate T- and B-cell migration pathways, normal or T-cell-depleted rabbits were used as donors. Cell suspensions were labeled in vitro with 3H-leucine followed by intravenous transfer. The migration of labeled cells in lymphoid organs was studied using autoradiography, particular attention being paid to the spleen of the recipient. B-cells from the appendix dome, spleen and thoracic-duct lymph migrate to primary follicles or the corona of secondary follicles via thymus-dependent areas of peripheral lymphoid organs. In contrast, a B-cell subpopulation from the germinal centers of the appendix migrates to the center of splenic primary follicles and into germinal centers. The migration of germinal center cells to splenic follicle centers is not enhanced by specific antigens. The migration properties of B-cells, possibly changing during differentiation, may be instrumental in the two types of immune reactions, i.e., plasma-cell reaction and germinal-center reaction.

Animals↗

A case of cecocolic intussusception with complete invagination and intussusception of the appendix with villous adenoma.

Villous adenoma of the appendix is a rare neoplasm and intussusception of the appendix is a rare pathologic condition. A very rare case seen in a 35-year-old male with pain in the right lateral abdomen is reported. In this patient, the appendix along with the villous adenoma intussuscepted and invaginated into the cecal lumen, and presented as cecocolic intussusception. A polypoid lesion was diagnosed in the cecum by fiberoptic colonoscopy. Unlike polypoid lesions at other sites in the large intestine, polypoid lesions of the cecum may accompany intussusception and invagination of the appendix. Consequently, caution is required in performing endoscopic polypectomy in cases of polypoid lesions of the cecum.

Adenoma↗