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PubMed1998

[Endometriosis of the appendix. A case report].

The authors report a case of endometriosis of the appendix found by chance during the course of left adnexectomy owing to endometriotic cysts. Endometriosis of the appendix is usually associated with right ovarian endometriosis, which is sometimes only present at a microscopic level. A situation of acute endometriotic appendix is the result not only of the disintegration of the appendix muscles, as was thought in the past, but also recent catamenial hemorrhage within the endometriotic tissue.

Acute Disease↗
PubMed1999

K-ras and p53 mutations in the pathogenesis of classical and goblet cell carcinoids of the appendix.

BACKGROUND: Mutations in the K-ras oncogene and the p53 tumor suppressor gene are present in approximately 50% of colonic adenocarcinomas. Goblet cell carcinoids (GCCs) are uncommon neoplasms of the appendix that appear to be intermediate between carcinoid tumors and adenocarcinomas, both histologically and biologically. The current study was undertaken to examine the role of p53 and K-ras mutations in the pathogenesis of GCCs and typical carcinoids (TCs) of the appendix. METHODS: Archival materials from 22 GCCs and 18 TCs were analyzed. K-ras mutations in codons 12, 13, and 61 were studied by a polymerase chain reaction (PCR) based designed restriction fragment length polymorphism method using mismatched nested primers. Mutations in exons 5-8 of the p53 tumor suppressor gene were analyzed in 16 GCCs and 18 TCs by PCR and single-strand conformational polymorphism followed by direct sequencing. Immunostains for p53 and chromogranin were performed in all cases. RESULTS: K-ras mutations and nuclear accumulation of p53 by immunohistochemistry were not detected in any of the GCCs or TCs. p53 mutations were found in 4 of 16 GCCs (25%) and 8 of 18 TCs (44%). Immunoreactivity for chromogranin was seen in the vast majority of GCCs and TCs. CONCLUSIONS: p53 mutations appear to play a role in the pathogenesis of some GCCs and in approximately 50% of TCs of the appendix, whereas mutations in the K-ras oncogene do not appear to be important in the development of these tumors. The minimal cytologic atypia, low incidence of metastases, and lack of K-ras mutations in goblet cell appendiceal neoplasms suggest that they are variants of carcinoid tumors. Our findings lend support to the recommendation that the therapeutic guidelines applied to TCs of the appendix should be the same for GCCs.

Appendiceal Neoplasms↗
PubMed2002

Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973-1998.

BACKGROUND: Cancer of the appendix is an uncommon disease that is rarely suspected rarely before surgery. Although several case series of these tumors have been published, little research has been anchored in population-based data on cancer of the appendix. METHODS: This analysis included all actively followed cases of appendiceal neoplasms reported to the National Cancer Institute's Surveillance, Epidemiology and End-Results (SEER) program between 1973 and 1998. Tumors were classified as "colonic type" adenocarcinoma, mucinous adenocarcinoma, signet ring cell carcinoma, goblet cell carcinoid, and "malignant carcinoid" (SEER only collects data on carcinoids specifically classified as malignant). We compared incidence, overall survival and survival rates by extent of disease at diagnosis. RESULTS: Between 1973 and 1998, 2117 appendiceal malignancies were reported to the SEER program, of which 1645 cases were included in the analysis. Age-adjusted incidence of cancer of the appendix was 0.12 cases per 1,000,000 people per year. Demographic characteristics of patients with goblet cell carcinoid tumors were midway between those of patients with malignant carcinoid and all types of adenocarcinomas. After controlling for age and extent of disease at diagnosis, the overall survival rate for patients diagnosed between 1983 and 1997 (n = 1061) was significantly worse for those with signet ring cell carcinoma than for those with any other tumor type (P < 0.01). In addition, overall survival rates were better for patients with malignant carcinoid (P = 0.01). CONCLUSIONS: Demographic characteristics of patients with cancer of the appendix vary by histology. Except for signet ring cell carcinoma and malignant carcinoid, the extent of disease at time of diagnosis is a more important predictor of survival than histology.

Adenocarcinoma↗
PubMed1983

Mucinous cystadenoma of the appendix: a case report of the diagnoses and management.

Cystadenoma of the appendix as a factor in the formation of mucous cysts (mucocele) of the appendix is well recognized. The diagnosis can be made in patients prior to surgery if appropriate investigations are utilized. A 66-year-old white woman with a complaint of increased frequency of bowel movements was investigated and a primary diagnosis of mucocele of the appendix was made preoperatively. As cecal involvement was suggested by these studies, a limited cecal resection was planned. These findings were confirmed at surgery. The appendectomy and limited cecal resection was curative for mucinous cystadenoma of the appendix.

Aged↗
PubMed1998

Peutz-Jeghers syndrome manifesting complete intussusception of the appendix and associated with a focal cancer of the duodenum and a cystadenocarcinoma of the pancreas: report of a case.

The unusual occurrence of an "inside-out" appendix reported here is a case of complete intussusception of the appendix of a 45-year-old woman with Peutz-Jeghers syndrome in whom the diagnosis of intussusception was made preoperatively. At laparotomy, the lead point of intussusceptum was revealed to be a Peutz-Jeghers syndrome polyp of the appendix. There was also a cystic lesion in the pancreas, and subsequent distal pancreatectomy revealed a cystadenocarcinoma of the pancreas. Two jejunal Peutz-Jeghers syndrome polyps and two duodenal Peutz-Jeghers syndrome polyps were found via intraoperative endoscopies. The duodenal polyps were endoscopically removed, whereas a jejunal wedge resection was performed for the adjoining jejunal polyps. One of the two duodenal polyps possessed an adenocarcinoma focus. To our knowledge, this is the first report of complete intussusception of the appendix caused by a Peutz-Jeghers syndrome polyp.

Appendiceal Neoplasms↗
PubMed1988

A 25-year review of adenocarcinoma of the appendix. A frequently perforating carcinoma.

A study was undertaken to compare the age, sex, preoperative diagnosis, and operative findings of patients who had adenocarcinoma of the appendix with patients who had adenocarcinoma of the colon. The study consisted of an analysis of 316 case reports and collective reviews of adenocarcinoma of the appendix that were reported in the literature between 1960 and 1985. The mean age of patients with this disease was 57.1 years and the male-to-female ratio for adenocarcinoma of the appendix was 1.4:1. Only rarely was a malignancy suspected, as 68 percent of the patients presented with signs and symptoms of inflammatory disease of the appendix. The tumor was perforated in 55 percent of patients, making it the most frequently perforating carcinoma of the entire gastrointestinal tract. One half of these perforations were localized as abscesses. The presence of perforation did not necessarily predispose a poor prognosis. Synchronous appendiceal and other colonic neoplasms occurred in 2.7 percent of patients. Pseudomyxoma peritonei occurred as a presenting feature in 5.6 percent of patients and was generally a poor prognostic indicator. Carcinomatosis peritonei was found at initial exploration in 10.3 percent of patients and these patients rarely survived one year.

Abscess↗
PubMedDate not supplied

Synchronous mucinous tumors of the ovary and the appendix associated with pseudomyxoma peritonei: CT findings.

BACKGROUND: To present the computed tomographic (CT) findings of synchronous mucinous tumors of the ovary and the appendix associated with pseudomyxoma peritonei (PMP). METHODS: Imaging studies, mainly abdominal CT scans, of three women aged 49-75 years were reviewed. Attention was directed to the ovarian masses, peritoneal seeding, and the presence of an appendiceal mucocele. RESULTS: The ovarian tumors and the appendiceal mucocele were clearly demonstrated in two cases, and they were part of the extensive PMP in the third patient. Ascites was found in all cases, with internal septation in one. Associated scalloping of the liver margins and hypodense peritoneal implants, with extensive bowel involvement, were seen in another one. Pathologically, there was one case of right ovarian mucinous cystadenoma and villous adenoma of the appendix, one case of right ovarian and appendiceal mucinous cystadenocarcinoma, and one case of bilateral metastatic ovarian implants of appendiceal mucinous cystadenocarcinoma. PMP was found in all. In the case with benign tumors of the ovary and the appendix, the PMP was classified as a benign mucinous spillage. This patient returned 33 months after surgery with PMP, in which epithelial cells were found. CONCLUSIONS: Radiologists should be familiar with the clinical occurrence of synchronous mucinous tumors of the ovary and the appendix associated with PMP and with the typical CT findings of the latter two entities. Alternatively, when the imaging findings suggest ovarian cystic tumor with PMP, the radiologist should be alerted to the probability of a clinically unsuspected appendiceal mucocele and should search for it.

Aged↗
PubMed2001

Carcinoid tumors of the appendix in children.

We report ten cases of carcinoid tumor of the appendix observed in children from 1988 to 1996. The patients included six females and four males with an average age of 13 years at presentation. They were admitted after complaining of pain in the lower abdominal quadrant. In eight children who presented with symptoms of acute appendicitis, the tumor was located at the tip of the appendix. Diagnosis was performed after appendicectomy (AE) and pathologic examination, which revealed a tumor slightly under 1 cm in size. Two other children were admitted with clinical signs of peritonitis due to larger tumors measuring more than 2 cm on the base of the appendix. One patient underwent a cecectomy, the other a right hemicolectomy. For all patients follow-up was 3 years, and all recovered fully. According to these findings and a review of the literature, we suggest conservative surgical procedures in children. More than 70% of these tumors are localized at the tip of the appendix and represent an incidental pathologic finding during AE; AE alone is curative. Patients with a bulky tumor of the appendicular base measuring 2 cm and invading the serosa and mesoappendix without metastases may be treated with a cecectomy; ileocecal resection may be indicated in cases where the tumor has infiltrated tissue beyond the cecum with localized metastases and in patients with incomplete gross resection. Right hemicolectomy is questionable in this age group and restricted to rare conditions.

Adolescent↗
PubMed2001

Mucinous cystadenocarcinoma in the appendix in a patient with nonrotation: report of a case.

Mucinous cystadenocarcinoma in the appendix is uncommon. An anomaly in the rotation of the intestine is also uncommon in adults. We herein report a case of mucinous cystadenocarcinoma in the appendix in a patient with nonrotation. To the best of our knowledge, this is the first report of appendiceal carcinoma in a patient with an anomaly of intestinal rotation. A 76-year-old woman was admitted to our hospital with left low abdominal pain. Physical examination revealed tenderness with muscle rigidity in the left lower quadrant. The patient was diagnosed to have intussusception by computed tomography and ultrasonography. An emergency operation showed nonrotation and the top of the appendix situated in the left iliac fossa. An appendectomy was performed because of gangrenous acute appendicitis. However, the cut surface of the appendix showed a mucocele measuring 4 x 4 cm in size. It was diagnosed to be mucinous cystadenocarcinoma histopathologically. A right hemicolectomy with lymph node dissection was performed, and no remaining cancer cells or lymph node metastases were found in the resected specimen pathologically. The patient had an uneventful postoperative course. No signs of recurrence have been observed for 23 months since her last operation.

Aged↗
PubMed2003

Adenocarcinoid of the appendix: report of two cases.

Adenocarcinoid of the appendix is a rare tumor with the histological features of both adenocarcinoma and carcinoid tumor. However, its biological behavior and malignant potential are still unclear. We treated two patients with this unusual tumor; a 60-year-old man and a 79-year-old woman. Both patients were initially diagnosed with acute appendicitis followed by an appendectomy. At surgery, the appendix was seen to be acutely inflamed without any macroscopic signs of tumor. Postoperative histological analysis revealed an adenocarcinoid tumor in the appendix, which had spread diffusely into its wall without forming a mass. Immunohistochemical staining with p53, MIB-1, bcl-2, and carcinoembryonic antigen suggested that neither of these tumors were particularly aggressive. Adenocarcinoid of the appendix is a rare tumor, which is very difficult to diagnose preoperatively and even macroscopically, making histological examination essential.

Adenocarcinoma↗
PubMed2000

An albumin-like protein in the serum of non-parasitic brook lamprey (Lampetra appendix) is restricted to preadult phases of the life cycle in contrast to the parasitic species Petromyzon marinus.

Previous work showed that the parasitic sea lamprey, Petromyzon marinus, has two different albumin-like serum proteins during the course of its life cycle. One of these, AS, is the predominant protein in the serum of larval and metamorphosing intervals but is absent in the upstream migration phase of the life cycle; the other, SDS-1, is found at its highest level in the upstream migration phase and is the predominant serum protein in this phase. The present investigation examines the nonparasitic brook lamprey Lampetra appendix for the presence of albumins. Using electrophoresis and antisera monospecific for serum proteins, it was found that L. appendix has a serum protein that is antigenically similar to the AS protein of P. marinus. This protein (LAS) makes up about 70% of the total serum protein of the Lampetra larva, is present in metamorphic stages 1,2,3,4 and 5 but is virtually absent from the remaining metamorphic stages and the adult. Furthermore, the adult of L. appendix has no serum protein that is antigenically similar to SDS-1 of the adult P. marinus; our study shows that, unlike P. marinus, there is no single protein that represents a major portion of the total serum protein content in the L. appendix adult.

Albumins↗
PubMed1984

Combined production of mucus, amines and peptides by goblet-cell carcinoids of the appendix and ileum.

Goblet-cell carcinoids are particular mucus-producing tumors combining features of typical carcinoids and adenocarcinomas. The immunoreactivity of five goblet-cell carcinoids of the appendix and one tumor of the ileum for 5-hydroxytryptamine (5-HT, serotonin), glucagon, somatostatin, substance P (SP), neuron-specific enolase (NSE), lysozyme, secretory component (SC) and carcino-embryonic antigen (CEA) was compared with that of the mucosa of the appendix (n = 24) and ileum (n = 12), and of typical carcinoids (appendix: n = 10; ileum: n = 3). The goblet-cell carcinoids were consistently lysozyme-, SC- and CEA-reactive and contained weakly NSE reactive endocrine cells, while typical carcinoids were lysozyme-, SC- and CEA-negative, but strongly NSE- reactive. Two goblet-cell carcinoids were glucagon-reactive, one displayed SP-reactivity, one malignant tumor was reactive to the alpha-chain of glycoprotein hormones; six of ten typical appendix carcinoids were SP reactive, as were the three typical ileum carcinoids. Using the immunogold technique combined with the alcian-blue reaction, the presence of 5-hydroxytryptamine (5-HT) and mucus was demonstrated within the same cell. These findings suggest histogenetic differences between goblet-cell carcinoids and typical carcinoids; the former are possibly derived from undifferentiated stem cells, whereas the latter probably arise from endocrine cells in the mucosal stroma.

Aged↗
PubMed2004

Neuroendocrine tumors of the appendix.

The vast majority of neuroendocrine neoplasms of the appendix are carcinoid tumors. Most are of enterochromaffin (EC) cell type, although rare examples are of L cell type. EC cell carcinoids of the appendix differ from those encountered elsewhere in the gastrointestinal system. For example, they are remarkably common given the small size of the appendix, are usually benign, occur in younger patients, and typically contain sustentacular cells that express S-100. Origin from subepithelial neuroendocrine cells could explain these characteristics. It has also been suggested that most appendiceal carcinoids are hyperplastic rather than neoplastic, although this hypothesis requires further study. Nevertheless, truly neoplastic EC cell carcinoids of the appendix undoubtedly occur, and those greater than 2 cm in diameter have a significant risk of producing distant metastases. Carcinoid syndrome is a very rare presentation. Tubular carcinoids are unusual benign neoplasms; it has been proposed that they represent L cell carcinoids with a predominant tubular pattern of growth. Goblet cell carcinoids tend not to produce a grossly visible tumor mass but diffusely infiltrate the wall. They typically exhibit tight clusters of goblet cells, usually with scattered neuroendocrine cells and sometimes with Paneth cells, sometimes surrounding a small lumen. They may behave as a low-grade malignancy. The distinction between goblet cell carcinoid and other types of tumor is of great importance because of the implications for treatment and prognosis. Frank adenocarcinoma can arise from goblet cell carcinoids, and tumors with both components are classified as mixed goblet cell carcinoid-adenocarcinoma. The carcinoma component of the latter determines their prognosis, which would be worse than for a goblet cell carcinoid alone.

Appendiceal Neoplasms↗
PubMed1994

Insurance-related differences in the risk of ruptured appendix.

BACKGROUND: We studied differences in the incidence of appendiceal perforation in patients with acute appendicitis according to their insurance coverage. METHODS: In a retrospective analysis of hospital-discharge data, we examined the likelihood of ruptured appendix among adults 18 to 64 years old who were hospitalized for acute appendicitis in California from 1984 to 1989. RESULTS: After controlling for age, sex, psychiatric diagnoses, substance abuse, diabetes, poverty, race or ethnic group, and hospital characteristics, we found that ruptured appendix was more likely among both Medicaid-covered and uninsured patients with appendicitis than among patients with private capitated coverage (odds ratios, 1.49 [95 percent confidence interval, 1.41 to 1.59] and 1.46 [95 percent confidence interval, 1.39 to 1.54], respectively). After adjustment for the above factors, the risk of appendiceal rupture associated with a lack of private insurance was elevated at both county and other hospitals, but admission to a county hospital was an independent risk factor. In all income groups, appendiceal rupture was more likely with fee-for-service than capitated private coverage (overall odds ratio, 1.20 [95 percent confidence interval, 1.15 to 1.25]). CONCLUSIONS: Among patients with appendicitis an increased risk of ruptured appendix may be due to insurance-related delays in obtaining medical care. Both organizational and financial features of Medicaid and various types or levels of private third-party coverage may be involved. The significant association between ruptured appendix and insurance coverage after adjustment for socio-economic differences suggests barriers to receiving medically necessary acute care that should be considered in current deliberations on health policy.

Adolescent↗
PubMed2000

Should an appendix that looks 'normal' be removed at diagnostic laparoscopy for acute right iliac fossa pain?

OBJECTIVE: To find out whether the removal of the appendix from patients in whom laparoscopy for acute right iliac fossa pain shows no abnormality is justified to avoid the risk of missing acute appendicitis. PATIENTS: The records of patients who, between 1990 and 1997 had emergency laparoscopy for acute right iliac fossa pain were reviewed. Only those in whom laparoscopy had shown no abnormality and had not had the appendix removed were included in the study. METHODS: Outcome was assessed by telephone questionnaire to the patient, the general practitioner, or both. RESULTS: Emergency laparoscopy had been done for 254 patients. No abnormality was detected in 41. Full follow up was available on 34 patients (83%). 21 patients have remained entirely free of symptoms. Of the 13 patients who had recurrent symptoms, 2 subsequently had a histologically normal appendix removed, yet still had symptoms; 2 had a second laparoscopy that showed no abnormality; 5 had ultrasound; and 4 had colonoscopy or a barium enema examination. CONCLUSION: Removal of an appendix that looks 'normal' at emergency laparoscopy for right iliac fossa pain is unjustified.

Abdominal Pain↗
PubMed1994

The natural history of surgically treated primary adenocarcinoma of the appendix.

OBJECTIVE: The aim of this investigation was to determine the prognostic variables and optimal surgical procedure for patients with adenocarcinoma of the appendix. SUMMARY BACKGROUND DATA: Primary adenocarcinoma of the appendix is a rare malignancy that constitutes less than 0.5% of all gastrointestinal neoplasms. However, the prognostic factors and the preferred surgical procedure and outcome are poorly understood. METHODS: The authors reviewed their institutional experience from 1976 to 1992 in treating 94 consecutive patients with primary adenocarcinoma of the appendix. Patients with carcinoid tumors or those in whom the diagnosis of primary cecal cancer could not be ruled out were excluded from the study. RESULTS: Fifty-two (55%) patients had the mucinous variety, of which 22 had pseudomyxoma peritonei; the other 45% had the colonic and adenocarcinoid types of tumor. The most common presentation was that of acute appendicitis. Interestingly, in no patients was the correct diagnosis made before surgery, and it was entertained intraoperatively in only 30 patients (32%). The cure 5-year survival rate was 55%, but it varied with stage (A, 100%; B, 67%; C, 50%; and D, 6%; p < 0.01) and with grade (I, 68%, and III, 7%; p < 0.01). Patients with the mucinous type had a better prognosis than those with the colonic type (p < 0.01). The survival rate was superior after right hemicolectomy versus appendectomy alone (68% vs. 20%, p < 0.001). Right hemicolectomy performed as a secondary procedure resulted in the upstaging of 38% of the patients' tumors. A second primary malignancy occurred in 33 patients (35%), of which 17 were located in the gastrointestinal tract. CONCLUSIONS: Primary adenocarcinoma of the appendix should be treated by right hemicolectomy, even if it is a secondary procedure. Surveillance for synchronous or metachronous tumors, especially in the gastrointestinal tract, is warranted.

Actuarial Analysis↗
PubMed1995

Plasminogen activator inhibitor-2 expression in inflamed appendix.

Plasminogen activator inhibitors are thought to be responsible for the abolition of fibrinolytic activity in inflamed peritoneum. This reduction in the fibrin clearing capacity of the peritoneum promotes the formation of intra-abdominal adhesions. High concentrations of plasminogen activator inhibitor-2 (PAI-2) have been previously found in inflamed peritoneal tissue using immunoassays, but it is undetectable in normal peritoneum. The aim of this study was to localize plasminogen activator inhibitor-2 production in tissue by in situ mRNA hybridisation. Sections of normal and inflamed human appendix were hybridised with a digoxigenin labelled cDNA probe. In normal appendix staining was confined to macrophages in the mucosa. Macrophage staining was also seen in inflamed tissue but with a wider distribution throughout the appendix wall. PAI-2 was also localized to mesothelial cells of inflamed but not normal appendix. Cell identities were confirmed using immunohistochemistry directed against cell specific markers. Staining was absent from control slides incubated with plasmid DNA or PAI-2 probe following ribonuclease digestion. The identification of the cells expressing the PAI-2 gene in peritoneum increases our understanding of the pathophysiological process leading to fibrin deposition within the abdomen during peritonitis.

Appendicitis↗
PubMed2006

Torsion of the testicular appendix: importance of associated acute inflammation.

BACKGROUND: Torsion of the testicular appendix is the commonest cause of acute scrotum in children. The histological picture of these cases is variable and many show a heavy acute inflammatory cell infiltrate, unlike the response to pure ischaemic necrosis in other organs. The clinical implications and consequences of this associated inflammation are not clear. METHODS: A retrospective review of all cases presenting with torsion of the testicular appendix between 1987 and 2005 was carried out. In all, 79 cases were reviewed and the degree of acute inflammatory cell infiltrate and other morphological parameters were assessed and compared with the patients' clinical, operative and postoperative findings. Stains for bacteria and fungi were examined. RESULTS: A positive correlation was found between the severity of acute inflammatory cell infiltrates and the longer duration of symptoms and presence of clinical evidence of torsion of the testicular appendages. No other association was detected between the pattern or degree of acute inflammatory cell infiltrate and any other clinicopathological variable that may indicate pyogenic infection. No bacteria or fungal elements were identified. Marked lymphatic dilation may be the only histological finding to indicate the presence of early torsion in cases of scrotal pain secondary to torsion of the appendix testis. CONCLUSION: Heavy acute inflammatory cell infiltrates in the torted testicular appendix can be regarded as marker of progression of this condition. Investigation for infection is therefore not necessary in such cases.

Acute Disease↗