Adenocarcinoma of the vermiform appendix presenting as a uterine tumor.
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The clinicopathologic features of an appendiceal adenocarcinoid that metastasized to the ovary and peritoneum after a 5-year disease-free interval are presented. A review of the literature reveals that appendiceal adenocarcinoid in women has a high frequency (32%) of ovarian metastasis. Among 18 cases with ovarian metastasis, the ovarian metastasis was diagnosed at initial presentation in 13 cases. In the remaining 5 cases the ovarian metastasis developed after disease-free intervals ranging from 6 months to 8 years. In most cases, the ovarian metastasis was the main clinical presentation of the patients. In 5 cases the ovary was the only site of metastasis clinically when metastasis was first diagnosed. There is no known effective treatment for disseminated adenocarcinoid. It is advisable that oophorectomy be included routinely in the initial surgical treatment of appendiceal adenocarcinoid, with the possible exception of the rare pure tubular variant.
To assess the role of appendectomy in the surgical procedures for ovarian cancer, we evaluated retrospectively the clinical charts of 435 patients who underwent surgery after diagnosis of ovarian cancer. The appendix was removed in 160 cases and pathological examination revealed 37 with metastatic implants (23%). All the patients with appendiceal metastases showed advanced disease (stages III-IV) with an incidence of 43%. Ninety-one percent (31/34) of the tumors with appendiceal involvement at the staging operation were of the serous cell type and grade II or III. No case with early stage, right ovary carcinoma showed appendiceal metastatic foci, denying the existence of a preferential lymphatic pathway. Microscopic involvement was found only in 4 patients with advanced disease (11.7%). No intra- or postoperative complication directly related to the appendectomy was recorded. We conclude, with these results, that appendectomy should be part of the cytoreductive operation for ovarian cancer.
A case of an isolated mesenteric desmoid (fibroma) is presented with two unusual characteristics. First, it arises from the mesentery of the appendix. Second, it presents as a twisted desmoid. The CT and US characteristics are presented. This patient was without risk factors for mesenteric fibrosis such as Gardner's syndrome or previous surgery.
A unique case of an infarcted carcinoid of the appendix is presented in which the computed tomography (CT) and clinical findings mimicked an appendiceal abscess. Though unusual, this possibility and appearance should be familiar to the radiologist particularly if percutaneous drainage is considered.
Pseudomyxoma peritonei is a rare disease caused by a perforated adenoma of the appendix. It results in extensive accumulation of mucinous tumour at specific locations within the abdomen and pelvis. The study was undertaken to examine patterns of recurrence in patients with grade I disease treated by cytoreductive surgery and early postoperative intraperitoneal chemotherapy. After a median follow-up of 1.9 years (range 0.5-7.4 years) 42 out of 118 patients had recurred. In 32 patients, detailed information regarding the anatomical location of recurrent tumour from CT-scan and second-look laparotomy were available and these form the basis of this study. The volume of recurrent tumour was recorded at eight abdominal sites, the laparotomy scar and at suture lines. Patient, tumour and treatment factors were analysed for possible relationship with the pattern of recurrence. With recurrence, true metastatic disease was observed in 3 patients and a distinctly higher grade of intraperitoneal tumour in another patient. Pleural spread of pseudomyxoma was found in 6 patients, always related to entering the pleural cavity during cytoreduction (P = 0.000031). Two abdominal sites consistently had an increase in tumour deposits at re-operation as compared to the initial cytoreduction. Small bowel had large deposits at re-operation in 17% versus 3% at initial cytoreduction and retroperitoneal surfaces 10% versus 0%. Recurrences were most frequent in the left subhepatic/lesser omentum area (28%), while the right subdiaphragmatic area (3%) was least involved. Pseudomyxoma peritonei recurrence in the laparotomy scar was found in 15/29 patients (52%), significantly more frequent if tumour had been present at former laparotomy scars during cytoreduction (P = 0.042). In 15/25 (60%) of patients, recurrences were found at suture lines. Differences in the completeness of cytoreduction, inadequate distribution of intraperitoneal chemotherapy to upper abdominal and small bowel surfaces, and entrapment of tumour within suture lines were thought to be causal factors consistent with this pattern of recurrence. Consequences for future treatment strategies are discussed.
A 37-year-old woman presented with acute right lower abdominal pain. Intraoperatively, the appendix was enlarged and distended. The lumen of the appendix was tightly filled with pearl-like globules, diagnostic of myxoglobulosis, a rare variant of mucocele of the appendix. A carcinoid of 2.0 cm diameter was found in the proximal region of the appendix. The appendiceal mucosa showed hyperplastic-adenomatous changes. A pseudodiverticulum interpreted as evidence of increased intraluminal pressure was detected. The latter may be an adjunct to the proximal partial obstruction of the appendiceal lumen by the carcinoid in the development of the spheroids of myxoglobulosis.
Neuroendocrine tumours (NETs) of the appendix (formerly 'carcinoids') are rare and are usually detected incidentally after appendectomy. Histopathologically they derive from a subepithelial cell population, which is different from NETs in other sites. They are preferentially located at the tip of the appendix. Tumours <1 cm hardly ever metastasize and are treated by appendectomy. Tumours >2 cm require right hemicolectomy because of a significant risk of metastatic spread. Treatment for lesions 1-2 cm is controversial and needs further characterization of the tumour (i.e. mesoappendiceal invasion, vascular invasion, mitotic activity, proliferation markers) and careful patient risk evaluation. Goblet-cell carcinoids have features resembling both carcinoid and adenocarcinoma and should be treated by hemicolectomy. Overall prognosis of small appendiceal NET is excellent in all ages.
Diagnosis of acute appendicitis is considered more difficult in pregnant than in non-pregnant women. The appendicial carcinoid tumor is a lesion that most frequently is discovered incidentally in the removed organ. We report a rare case of an unruptured acute appendicitis during pregnancy, which proved to be a large carcinoid tumor.
AIM: The aim of this study was to determine the value of medical history and physical examination, tumour marker testing, and CT-scanning in the follow-up of patients treated for peritoneal carcinomatosis of colorectal origin. METHODS: Between November 1995 and June 2003, 107 patients were treated by cytoreduction and hyperthermic intra-peritoneal chemotherapy. The treatment was considered effective if residual tumour after cytoreduction was no thicker than 2.5 mm. The follow-up consisted of history, physical examination, serum CEA and CA 19.9 testing three-monthly, and CT-scanning of the abdomen six-monthly. Location of the recurrence was categorized into intra-abdominal, hepatic, thoracic, and both intra-abdominal and systemic. The investigation that led to the detection of a recurrence was ranked according to its invasiveness and costs. The simplest investigation that could have led to the detection was marked. RESULTS: A recurrence developed in 63 patients of the 74 patients effective initial treatment during the study period. Physical examination revealed the recurrence in 38 patients, at least one of the markers was raised above normal value in 39 patients and in 37 patients the CT-scan showed the recurrence. History and physical examination could have triggered the finding of a recurrence in 38 patients, tumour markers in 21 patients and CT-scanning in only three of the 74 recurrences. CONCLUSION: Physical examination and tumour marker testing detect most recurrences. CT-scanning is not an effective tool in the follow-up, and should be reserved for on-demand use.
BACKGROUND: The aim of this study was to report the role of combined treatments, including cytoreductive surgery and perioperative regional chemotherapy, in patients with synchronous systemic and intraperitoneal dissemination of appendix cancer. METHODS: Patients with synchronous systemic and intraperitoneal dissemination of appendix cancer were treated with cytoreductive surgery and perioperative regional chemotherapy. Variables statistically analyzed with survival as an end point included demographic characteristics, prior surgical score (PSS), tumour marker levels, peritoneal cancer index (PCI), and completeness of cytoreduction (CC). RESULTS: Mean follow up was 42.6 months, median survival time (MST) for 15 patients was 28 months and 5-year survival rate was 29.4%. Female patients had a longer MST than male patients (p=0.0199). Survival was better in patients with PSS 0 and 1 (p=0.0277). Patients with elevated CEA and CA 19-9 levels had a shorter MST (p=0.0083 and p=0.0193, respectively). PCI and CC comparisons did not show significant differences. Morbidity (n=2) and mortality (n=2) rates were 13.3% respectively. CONCLUSION: Acceptable morbidity and mortality and a 29.4% 5-year survival rate allows cytoreductive surgery and regional chemotherapy to be considered as a treatment option for selected patients with synchronous systemic and intraperitoneal dissemination of appendix cancer.
Mucocele of the appendix secondary to appendicular carcinoid tumour is a rare entity. It occurs exceptionally during pregnancy. The interaction between carcinoid tumour or mucocele of the appendix and pregnancy is not yet elucidated. Prognosis depends on the size of the carcinoid tumour and its regional invasion. We report a case of appendicular mucocele caused by a carcinoid tumour in a 36-year-old pregnant woman who had been admitted because of acute appendicitis. Pathological examination of the appendicectomy specimen revealed a carcinoid tumour measuring 2 cm. Right hemicolectomy was performed two weeks after delivery with good evolution. According to this case and depending on the literature, the authors discuss the diagnosis and the therapeutic modalities proposed for this rare entity.
Melanomas of the gastrointestinal (GI) tract are very rare, and their primary or secondary nature is often difficult to establish. We report the case of a 55-year-old Caucasian female who presented with an appendiceal melanoma. To our knowledge, appendiceal localization of melanoma has never been reported. We review the literature and discuss the issues relating to the origin of gastrointestinal melanomas. In conclusion, even if a primary site of melanoma was not evident in this case, the metastatic nature of this appendiceal melanoma cannot be completely ruled out.
Gastrointestinal carcinoid tumors are often associated with other tumors, particularly colon adenocarcinomas; but the association between carcinoid tumors and hereditary nonpolyposis colorectal cancer (HNPCC) syndrome has not yet been explored. We report an unusual case of a 28-year-old woman with HNPCC who underwent surgery for a transverse colon adenocarcinoma in whom an appendix carcinoid tumor was incidentally found. To assess whether the carcinoid tumor displayed the characteristic molecular features of HNPCC tumors, we investigated the expression of mismatch-repair (MMR) proteins and microsatellite instability (MSI) status in both tumors. Both tumors demonstrated normal expression of the MMR proteins hMLH1, hMSH2, hMSH6, and hPMS2. Interestingly, the adenocarcinoma exhibited an MSI phenotype but the carcinoid tumor did not, indicating that these 2 tumors arose through different molecular pathways.
We report a female patient with cytomegalovirus (CMV) terminal ileitis and CMV viraemia, associated with a metastatic goblet cell carcinoid (GCC) tumour of the appendix. She was treated with ileocaecal resection followed by ganciclovir. We highlight the importance of vigilant histopathological assessment and discuss the existing literature on gastrointestinal CMV infection in immunocompetent patients.
Abstract Appendiceal intussusception and mucosa-associated lymphoid tissue lymphoma are both uncommon entities, and this is the first report of the two occurring concomitantly in an 8-year-old boy. This report of a mucosa-associated lymphoid tissue lymphoma localized in the appendix has not been reported previously.
Mucocele of the appendix is a thin-walled dilated appendix filled with mucus. It occurs secondary to chronic obstruction of the appendiceal lumen because of a range of pathologies. Cystadenomas in children are exceedingly rare and most frequently of ovarian origin. A mucous cystadenoma of the appendix in a 10-year-old boy with chronic abdominal pain is presented.