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Mucinous cystadenoma and cystadenocarcinoma of the vermiform appendix with particular reference to mucocele and pseudomyxoma peritonei.

Ten cases of mucocele of the vermiform appendix are described. Eight cases were of mucinous cystadenoma of the appendix and six cases showed acute inflammation. Two of the six cases showed pseudoinvasion of the appendix and in a further case the appendix had perforated with extrusion of a misplaced neoplasm. Two cases were of mucinous cystadenocarcinoma and one of these was diagnosed as ;pseudomyxoma peritonei'. ;Pseudomyxoma peritonei' is a misnomer and is caused by dissemination of a mucinous cystadenocarcinoma within the peritoneal cavity. The special problems of histological diagnosis are discussed.

Aged↗

Primary adenocarcinoma of the appendix.

Two cases of carcinoma of the appendix presenting as appendicular masses are discussed. Carcinoma of the appendix is rare and hence a pre-operative diagnosis is seldom made. Awareness of the condition would naturally arouse suspicion of its presence, especially in elderly patients who present with cute appendicitis or an appendix mass. It is known to have occurred in an appendix stump several years after the initial appendicectomy, and also in a 17-year-old patient. The need for histological examination of all appendicetomy specimens is stressed. The operation of choice is right hemicolectomy, either as a primary or as a secondary procedure. This is associated with better survival rates than when the condition is treated by appendicectomy alone.

Adenocarcinoma↗

Implications of removing a normal appendix.

BACKGROUND: The diagnosis of acute appendicitis remains difficult, and therefore 15-30% of the removed appendices appear to be normal. The aim of this study is to investigate the morbidity, mortality and costs of removing a normal appendix in patients with suspected appendicitis. PATIENTS AND METHODS: A retrospective study was performed on patients who underwent a negative appendectomy for suspected appendicitis in the period 1991-1999 with a median follow-up of 4.4 years. Patients who underwent an elective appendectomy or appendectomy for other reasons were excluded. RESULTS: In 285 patients (70% women, 30% men) a normal appendix was removed. In 192 (67%) patients a muscle-splitting incision was performed, in 6 (2%) a median laparotomy, and in 51 (18%) the normal appendix was removed by laparoscopy. In 36 patients (13%) a diagnostic laparoscopy was converted to a muscle-splitting incision. Complications occurred in 16 (6%) patients, in 5 (2%) a reoperation was needed. The mean hospital stay was 4.4 (SE 2.8) days, in case of complication 7.4 (SE 4.2) days. The mean extra hospital costs of a negative appendectomy were EUR 2,712. CONCLUSION: The removal of a normal appendix has considerable complications and costs. In an attempt to prevent these costs, extra diagnostic tools should be considered. Expensive diagnostic tools as diagnostic laparoscopy should be used selectively in order to not further exceed costs.

Acute Disease↗

Appendix carcinoma invading the urinary bladder.

We report a case of an appendix carcinoma invading the urinary bladder. In contrast to other bowel tumors invading the bladder, history and symptoms were consistent with a primary bladder tumor. This is due to the unique anatomical position of the appendix where the tumor did not hinder passage of bowel contents or cause melena. Findings on physical examination as well as diagnostic imaging and transurethral resection were inconclusive. Consideration of local progression of an appendix carcinoma is an important differential diagnosis. In contrast to other vesical or extravesical T4 tumors, the appendix carcinoma offers a good chance for resection en bloc by right-sided hemicolectomy and partial cystectomy.

Adenocarcinoma, Mucinous↗

Goblet cell carcinoids of the appendix: immunophenotype and ultrastructural study.

BACKGROUND: Goblet cell carcinoids of the appendix are rare neoplasms with uncertain biological behavior. OBJECTIVE: The aims of our study were to evaluate the immunophenotype of this neoplasm with cell cycle/cell proliferation markers and to understand their histogenesis with ultrastructural analysis using conventional carcinoids as a frame of reference. METHODS: Clinical data and archival pathologic material of all goblet cell carcinoids of the appendix recorded by the Saskatchewan Cancer Registry between 1970 and 1998 were reviewed and evaluated by light microscopy, histochemistry, immunohistochemistry, and electron microscopy. RESULTS: Seven cases of goblet cell carcinoids were identified among 110 cases of conventional carcinoids of the appendix. Histopathology revealed widespread infiltration of the periappendiceal fat in all cases, with extensive perineural invasion. The cells stained strongly positive for mucicarmine, periodic acid-Schiff, periodic acid-Schiff diastase, Alcian blue, cytokeratin, and carcinoembryonic antigen. Most cases were positive for synaptophysin. Increased expression of cell proliferation markers and cell cycle markers was observed. Expression of p53 was strong in one case. Electron microscopy demonstrated the presence of mucinous vacuoles of varying sizes and occasional membrane-bound neuroendocrine granules. CONCLUSIONS: Goblet cell carcinoids of the appendix arise from a pluripotent cell with divergent neuroendocrine and mucinous differentiation. These neoplasms are widely invasive; they demonstrate a high cellular proliferation rate and dysregulation of the cell cycle with up-regulation of cyclin D1 and p21, and down-regulation of p16. Complete removal of the tumor is recommended because of the unpredictable biological behavior of this tumor, which includes delayed local recurrences and lung metastases.

Adult↗

[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↗

Synchronous serrated adenoma of the appendix and high-grade ovarian carcinoma: a case demonstrating different origin of the two neoplasms.

Association of mucinous adenomas of the appendix and mucinous ovarian tumors is well known. The origin of the ovarian tumor (metastasis from the appendix vs independent primary) is still debated. Serrated adenoma is a rare neoplasm of the distal gastrointestinal tract, and its precancerous role in the colorectum was recently postulated. A 74-year-old patient was subjected to hysterectomy with routine appendectomy due to a 17-cm tumor of her right ovary. Histological examination revealed a high-grade ovarian adenocarcinoma with peritoneal involvement. The appendix, grossly unremarkable, harbored a serrated adenoma with no evidence of invasion or malignant transformation. Immunohistochemical examination revealed CD7+, CK20-phenotype of the ovarian and reverse (CK7-, CK20+) phenotype of the appendiceal tumor. Microsatellite analysis demonstrated microsatellite instability (MSI-high) within the serrated adenoma (4/5 markers with positive amplification) and no MSI (0/6 amplified markers) in the samples from the ovarian carcinoma, its metastases and the uninvolved uterine cervix. There were also differences in LOH pattern between the ovarian adenocarcinoma and the serrated adenoma. The findings suggest two independent primaries with profound differences in tumorigenetic pathways of both lesions. To the best of our knowledge this is the first report of synchronous serrated adenoma of the appendix and ovarian carcinoma.

Adenocarcinoma↗

[Primary adenocarcinoma of the appendix. Case report and review of the literature].

A rare case of primary adenocarcinoma of the vermiform appendix (less than 250 cases described in the literature) in a 36 year-old female patient presenting signs and symptoms of an acute appendicitis is reported. Adeno-carcinoma of the vermiform appendix is a rare neoplasm of the gastrointestinal tract with an incidence of about 0,01-0,2%. Usually the diagnosis of adenocarcinoma of the vermiform appendix is difficult because symptoms and signs are not pathognomonic. In the case described, the diagnosis was intra and postoperative and confirmed by the pathological examination of the surgical specimens. At laparotomy, performed under suspicion of an acute appendicitis, disseminated disease was discovered, characterized by the involvement of the two ovaries, the left colon, the cecum, the vermiform appendix, with a peritoneal carcinosis and a hydroureteronephrosis. According to the dissemination of the disease, the surgical treatment was right hemicolectomy, anterior resection of left colon, bilateral oophorectomy and omentectomy. The post-operative course was regular. Adjuvant therapy was performed for 6 cycles, with 5FU and oxaliplatinum. The patient is still alive after 6 months and there is no sign of progression of the disease. A mild left hydroureteronephrosis is persistent.

Adenocarcinoma↗

Extensive stage small cell lung cancer presenting as an acute perforated appendix: case report and review of the literature.

Malignancies of the appendix are uncommon; a small subset of these lesions are actually metastatic cancers. In some rare cases, these lesions can cause obstruction, appendicitis, and perforation. M.K. is a 54-year-old man who presented to our institution with a 1-day history of right lower quadrant pain and a past medical history significant only for a 75-pack-year smoking history. CT scan revealed a perforated appendix, and the patient was taken to the operating room where a gangrenous appendix was removed uneventfully. Two days post-procedure, the patient was found to have acute mental status changes, requiring intubation and transfer to the surgical intensive care unit. As part of a workup, a CT scan of the head revealed multiple lesions compatible with metastatic disease. At that point, the pathology from the appendix came back as small cell lung cancer. Chest CT revealed hilar adenopathy and a hilar mass. The patient received emergent whole-brain irradiation therapy with improvement in his mental status, allowing him to be extubated and discharged from the hospital within 10 days of admission. Surgeons should remember that an underlying oncologic process may be the etiology of appendicitis in a small but important subgroup of patients.

Acute Disease↗

Adenocarcinoma of the appendix in pregnancy: a case report.

BACKGROUND: Perforation of an adenocarcinoma of the appendix in a pregnant woman is rare and may present as a pelvic mass and acute appendicitis. CASE: A 30-year-old woman, gravida 3, para 2-0-0-2, presented at 26 weeks' gestation with an acute abdomen, right-sided pelvic mass and uterine contractions. Tocolysis and prompt surgical exploration revealed a perforated appendix. The patient underwent a right hemicolectomy with primary anastomosis, her postoperative course was benign, and she delivered a viable infant at term vaginally. Pathology revealed a perforated adenocarcinoma of the appendix. The patient was free of disease at 36 months. CONCLUSION: Adenocarcinoma of the appendix in pregnancy is rare, may present as an acute abdomen with a mass and is managed with right hemicolectomy.

Abdomen, Acute↗

[Actinomycosis of the appendix. Case report].

Abdominal actinomycosis is a chronic granulomatous suppurative disease caused by anaerobic Gram positive germs from Actinomyces species. The vermiform appendix and ileocaecal region have been recognized as the most commonly involved sites. Actinomycosis of the appendix is generally acute in presentation, mimicking a common acute appendicitis. We present a case of actinomycosis of the appendix in a 62-year-old male patient. The clinical presentation was as for a common acute appendicitis. The ultrasound examination sustained the diagnosis of acute appendicitis and an appendectomy was performed. The diagnosis was established by histo-pathologic examination. The cultures from intraperitoneal fluid were negative. The association of a long-standing antibiotic therapy to the surgical treatment resulted in definitive cure for this patient. Actinomycosis of the appendix is a rare disease that must be known, the diagnostic being the key for a successful treatment.

Actinomycosis↗

[Metastasis of breast cancer presenting in the appendix].

A 45 year-old woman was referred to the Emergency Department of Jósa András County Hospital with typical history and signs of appendicitis. Other than abdominal findings during physical examination we discovered a palpable mass in the right breast. The patient was transferred to the Department of Surgery and she was operated on, appendicectomy was performed. Following the uncomplicated postoperative course investigations were carried out to diagnose the mass in the right breast (mammography, ultrasound, chest X-ray, bone scan). The pathology report of the appendix suggested the possibility of a metastasis of ductal breast carcinoma in the perforated appendix. Probably the inflammation was caused by the infiltration of the appendix wall leading to perforation. Following the investigations a right-sided mastectomy was performed with dissection of axillary lymph nodes. Immunohistochemical examination definitely proved that the metastasis in the appendix originated from the breast tumour.

Appendiceal Neoplasms↗

[Carcinoid tumors of the appendix: when right colectomy?].

Three cases of carcinoid tumour of the appendix (about 0,3 % of all performed appendectomies) has induced the Authors to a review of the literature with the aim to underline the most important biological and pathological findings and the current clinic and therapeutic knowledges. The diagnosis before surgery is rarely made; it is formulated incidentally in most patients by the histological exam during the operation for an appendicitis or during other surgical procedures. The kind of surgical intervention, that is the entity of the surgical demolition, for the treatment of the carcinoid tumours of the appendix is still controversial: appendectomy or right colectomy? It is possible identify, also during the operation for an appendicitis or for other abdominal lesions, criteria that can orient toward a major surgery (size of the neoplasia, subserosal lymphatic invasion, infiltration of the serosa, diffusion in the meso-appendix, location in closeness of the base of the appendix, invasion of the the locoregional lymph nodes, presence of metastases, section ?margins, number of mitoses, cellular pleiomorfism).

Adult↗

[Mucinous cystoadenocarcinoma of the appendix. A case report].

CASE REPORT: The Authors report a case of a 66-years-old male patient with mucinous cystoadenocarcinoma of the appendix. Preoperative diagnosis was appendiceal mucocele. At laparatomy was revealed the presence of a large mass of the appendix involving the caecum. No mucinous ascites was present and no mucinous implants on the peritoneum surface. Operation consisted in appendectomy and resection of the caecum bottom. Hystology revealed diagnosis of mucinous cystoadenocarcinoma of the appendix. After two weeks patient underwent right hemicolectomy, he is alive without disease at 36 months follow up. Mucinous cystic neoplasms of the appendix is an uncommon disease that is rarely suspected before surgery. CONCLUSIONS: The Authors stress the importance of an appropriate surgical treatment. Right hemicolectomy appears to be the appropriate treatment with aggressive debulking when mucinous cystoadenocarcinoma is associated with pseudomyxoma peritonei. The role of chemotherapy in the treatment of patients with appendiceal cancers remains controversial. No controlled data on the efficacy of adjuvant chemotherapy are available. All patients with any form of appendiceal tumor appear to have an increased incidence of synchronous and metachronous neoplasms, especially in the gastrointestinal tract, and should be investigated and followed up appropriately.

Aged↗

[Carcinoid tumor of the cecal appendix].

In connection with carcinoid tumor of the appendix as an incidental finding in appendectomies, we carried out revision in a period of 5 years, from 1983 through 1987, in the General Hospital of Venezuelan Institute of Social Security in San Felix, Bolivar State. We found two cases of carcinoid tumor of the appendix in 1433 appendixes that were examined by the Service of Pathology. Both cases were males patients who were admitted due to acute appendicitis. They represented 0.14% of the total appendectomies. We review on the subject and we find that the frequency is like to that published up to now. We agree with others authors in that simple appendectomy in the carcinoid tumor of the appendix is curative in tumors < 2 cm.

Appendectomy↗

[Carcinoid of the appendix].

Within the framework of reflections on the extent of surgical operations in case of carcinoid of the appendix the author evaluates the size of the tumour, site of the tumour on the appendix and the presence of metastases in the appropriate lymph nodes or in remote organs. The author presents two case-histories. In the first one he demonstrates difficulties associated with evaluation of metastases of the mesenterial lymph nodes in case of mesenterial lymphadenitis concurrent with unrecognised carcinoid of the appendix. From the second case-history it is apparent that in advanced appendicitis with marked inflammatory changes of the appendix also colliquation of part of the tumour may occur and even microscopis examination does not provide information on the size of the tumour.

Adolescent↗

[Carcinoma metastasis into the vermiform appendix].

Metastases into the appendix vermiformis are very rare. The authors present a case of a secondary of gastric carcinoma into the appendix vermiformis. In the patient the malignant disease was manifested with the clinical picture of diffuse peritonitis caused by perforation of the aboral portion of the appendix vermiformis at the site of the exulcerated secondary of the carcinoma. The authors give also an account of secondaries into the appendix vermiformis described in the literature.

Adenocarcinoma↗

Carcinoid tumors of the appendix.

Forty-six carcinoid tumors of the appendix, diagnosed in surgically resected specimens during an 11-year period (1965 to 1975) were reviewed to determine the features of metastasizing tumors. Of the carcinoid tumors of the appendix, 8.8% were found to metastasize to regional lymph nodes. The most reliable criterion of metastasizing tumors was the size of the primary tumor. Such tumors measured 2.0 cm or larger in greatest dimensions. Depth of invasion, extension to serosa, perineural involvement, histologic features, and location of the tumor relative to the length of the appendix were common to both the metastasizing and localized tumors. The surgical management of patients with carcinoid tumors of the appendix is controversial, ranging from a simple appendectomy to a more radical operative procedure. In the absence of distant metastases, it would appear that this group of patients with tumors 2.0 cm or more would require a right hemicolectomy rather than a simple appendectomy to accomplish removal of all disease-bearing tissue.

Adolescent↗