[CONSIDERATIONS ON SURGERY OF THE CECUM].
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Appendiceal mucocele is a rare entity frequently associated with colorectal cancer. We report two cases of mucocele associated with colorectal tumours. The first case (male, 64 yrs) is an appendiceal mucinous cystadenoma found incidentally during surgery for colon cancer. There is no evidence of disease after a 4-year follow-up. The second case (male, 66 yrs) is a mucocele associated with mucosal hyperplasia that was found during surgery for acute appendicitis with a periappendicular abscess. Endoscopic follow-up showed a rectal adenocarcinoma that was initially treated with local excision with T.E.M.. Examination of the pathology specimen documented vascular invasion and the patient underwent curative colorectal resection. The preoperative radiological and endoscopic diagnostic procedures and the current therapeutic approaches described in the literature are reviewed. The relevance of the association between appendiceal mucocele and colorectal cancer is emphasized. Thorough investigation of the colorectal tract is recommended after diagnosing an appendiceal mucocele.
Nineteen patients (10 men, 9 women) with pseudomyxoma peritonei were studied to determine the site of origin of the disease and the nature of associated ovarian tumors. A primary appendiceal mucinous neoplasm was found in 16 of the 17 patients with an evaluable appendix. One woman's appendix had intramural and serosal mucinous deposits without neoplastic epithelial cells. The appendiceal tumors were classified as mucin-producing adenoma ("mucinous cystadenoma") in 13 patients (seven women, six men), intramucosal adenocarcinoma associated with mucin-producing adenoma in one man, and invasive adenocarcinoma associated with mucin-producing adenoma in one man and one woman. Five of the nine women had cystic mucinous ovarian neoplasms; each also had an appendiceal mucinous neoplasm (adenoma in four, invasive adenocarcinoma with adenoma in one). The ovarian neoplasms had histologic features resembling a borderline (low malignant potential) mucinous tumor in four and a mucinous cystadenoma in one; all five ovarian tumors also had features of pseudomyxoma ovarii. Mucinous implants were also on the ovarian surface of the contralateral ovary in four of the five women with ovarian tumors and in the other four women without ovarian tumors. The intraperitoneal mucus deposits contained neoplastic mucinous epithelial cells in 16 patients (eight men, eight women) and were acellular in three (two women, one man). Of 17 patients with more than 6 months of follow-up, 12 (seven women, five men) were alive after postoperative intervals of 7 to 147 months, including three with known residual disease. Five (three men, two women) died of disease 16 to 60 months after initial operation, including two patients with appendiceal carcinoma. Acellular intraperitoneal mucus appeared to be a favorable prognostic feature. We conclude that (a) the appendix is the primary site of origin of pseudomyxoma in the vast majority of cases in both men and women, and (b) the associated mucinous ovarian tumors are most likely secondary neoplasms resulting from incorporation of implanted mucus and neoplastic mucinous epithelial cells of the pseudomyxoma peritonei.
We report the first case of appendiceal mucinous cystadenoma associated with the kidney. A 57-year-old man with chronic renal failure presented with a right renal mass. During laparoscopic nephrectomy, frozen analysis of a tubular structure extending from the mass showed appendiceal tissue. The mass, appendix, and what was believed to be the right kidney were removed. Pathologic examination demonstrated mucinous cystadenoma of the appendix. No renal tissue was identified, but the patient refused further treatment. This case demonstrates that mucinous cystadenomas arising from retroperitoneal structures might mimic renal neoplasms and should be considered in the differential diagnosis of cystic renal masses.
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Neoplasms occur in 0.5 per cent of appendices. Ultrasonography or computed tomography is beneficial, but preoperative detection is rare. At operation, the diagnosis is considered in under half of cases. Mucocele, localized pseudomyxoma peritonei, benign tumours and most appendiceal carcinoids are cured by appendicectomy alone. Right hemicolectomy is indicated for: (1) invasive adenocarcinoma; (2) tumours close to the caecum; (3) lesions larger than 2 cm; (4) mucin production; (5) invasion of the lymphatics, serosa or mesoappendix; and (6) cellular pleomorphism with a high mitotic rate. Tumours of 1-2 cm, small mucinous carcinoids, adenocarcinoma confined to the mucosa, and tumours in children may be treated by appendicectomy alone at the surgeon's discretion. The 5-year survival rate associated with classical carcinoid is more than 90 per cent. The prognosis of mucinous carcinoid is intermediate between that of classical carcinoid and well differentiated adenocarcinoma. The prognosis of adenocarcinoma is determined by Dukes' stage and is similar, stage for stage, to that of colorectal carcinoma.
COX-2 expression was studied using an immunohistochemical method in 75 patients with pseudomyxoma peritonei (PMP). Twenty-five patients presented with disseminated peritoneal adenomucinosis (DPAM) and 50 with peritoneal mucinous carcinomatosis (PMCA). COX-2 was expressed in neoplastic mucinous epithelium of 30 cases (40%): 20 in PMCA (40%), 10 in DPAM (40%). Weak COX-2 expression was also noted in four of five patients with appendiceal mucinous neoplasms without peritoneal dissemination. In addition, COX-2 was detected in stromal, endothelial, inflammatory cells and reactive mesothelium. This preliminary information indicates a potential for the use of COX-2 inhibitors in patients with PMP.
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AIMS: CDX-2 is a highly sensitive and specific marker of intestinal epithelial cells and their neoplastic counterparts. CDX-2 status in pseudomyxoma peritonei (PMP) has been barely reported. The aim of this study was to investigate the clinicopathological features of 42 cases of PMP with a special emphasis on CDX-2. METHODS AND RESULTS: All patients were treated by cytoreduction. Immunohistochemistry was performed for CDX-2, MUC-2, MUC-5AC, cytokeratin (CK) 7 and CK20. Statistical correlation was evaluated for age, sex, completeness of cytoreduction and histological subtype with overall and progression-free survival (OS and PFS). PMP consisted of 32 cases of disseminated peritoneal adenomucinosis and 10 cases of peritoneal mucinous carcinomatosis. The appendix evaluated in 25 cases showed two mucinous adenocarcinomas and 21 low-grade appendiceal mucinous neoplasms. CDX-2 was diffusely positive in 40 cases, with the remaining two cases being focally positive. All cases demonstrated diffuse reactions to CK20 and MUC-2, and variable reactions to MUC-5AC, while CK7 was variably positive in 38 cases. Five-year OS was 97%. Histological type was significantly correlated with PFS (P=0.02). CONCLUSIONS: CDX-2 is diffusely and strongly positive in PMP. This is a useful marker to confirm an appendiceal origin of PMP, particularly when used in conjunction with CK7, CK20, MUC-2 and MUC-5AC.
BACKGROUND: Appendiceal mucinous neoplasms present, in most patients, with peritoneal dissemination at the time of initial diagnosis. Patients may have a borderline tumor showing disseminated peritoneal adenomucinosis or an aggressive malignancy identified as peritoneal mucinous adenocarcinoma. Patients with these diagnoses were treated by cytoreductive surgery and perioperative intraperitoneal chemotherapy. METHODS: A database was established in 1998 that prospectively evaluated the morbidity and mortality of this group of patients. By using common toxicity grading criteria, 8 categories were scored on a grade of I to V. Grade IV indicated that the adverse event required urgent and definitive intervention: often a return to the operating room or to the surgical intensive care unit. Grade V indicated that the adverse events resulted in the patient's death. Adverse events were tabulated for each cytoreduction performed in these appendiceal malignancy patients. RESULTS: There were 356 procedures in patients taken to the operating room who received cytoreductive surgery with peritonectomy procedures plus heated intraoperative intraperitoneal chemotherapy. Only patients who had this combined treatment at our institution were included in the analysis. The total 30-day or in-hospital mortality was 2.0%. Nineteen percent of procedures were accompanied by at least one grade IV adverse event, and 11.1% of patients returned to the operating room. The most common category of grade IV complications was hematological (28%), followed by gastrointestinal (26%). CONCLUSIONS: The mortality of 2.0% and the overall grade IV morbidity of 19% in these patients may be acceptable in light of modern standards for the management of gastrointestinal cancer.
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Primary appendiceal cancer is a rare neoplasm. We analyzed the clinicopathologic characteristics and clinical outcomes of patients with primary appendiceal cancer treated at Buddhist Tzu Chi General Hospital in Eastern Taiwan. We reviewed the medical records of patients who had appendectomy at our hospital over a 10-year period and studied those who had histologically proven malignant appendiceal neoplasms. We treated eight such patients: seven males and one female. Their median age was 66 years (range, 59-78 years). There were three mucinous adenocarcinomas, two colonic type adenocarcinomas, and three adenocarcinoids. No patient was diagnosed correctly before surgery, and five (62.5%) had a preoperative diagnosis of acute appendicitis. Operative procedures included right hemicolectomy or partial colectomy in four, debulking and right hemicolectomy in three, and appendectomy only in one. With a mean follow-up of 64 months (range, 3-132), patients with adenocarcinoid lesions had better prognosis than those with adenocarcinomas. The important prognostic factors of primary appendiceal cancer included histologic subtypes and the extent of dissemination. In our series, palliative resection for disseminated lesions with or without additional chemotherapy resulted in long-term survival.
An unusual case of adenocarcinoma of the appendix is presented. Abnormal growth of the tumor toward the abdominal wall resulted in a cutaneous mass, which was the first manifestation of the neoplasm.
The production of monoclonal antibodies by the hybrid technique introduced by Köhler and Milstein in 1975, led to new dimensions in the diagnosis and therapy of malignant diseases. Labelling such monoclonal antibodies, directed against tumour cells, by a radioactive isotope, makes them usable for visualising malignant tumours and their metastases. Early detection of cancer may represent a major step forward in cases where early detection is not possible with common noninvasive methods, such as ovarian cancer in gynaecological oncology. Radioimmunoscintigrams (RIS) were performed in seven patients, all of whom were suspect of having ovarian cancer. The RIS-results showed very good correlations with the operation reports. According to these first results we assume that the RIS method using I123-labelled monoclonal antibodies directed against tumour cells may be successful in the early detection of ovarian cancer. Other important information supplied by radioimmunoscanning is determination of tumour status, localisation, extent or metastatic spread.