Cases reported "pseudomyxoma peritonei"

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1/102. Pseudomyxoma retroperitonei with spontaneous skin fistula.

    We describe the imaging features in a patient with pseudomyxoma retroperitonei arising from a mucinous carcinoma of the appendix with spontaneous development of a skin fistula. The thick wall and septa characteristic of pseudomyxoma were best demonstrated on gadolinium-enhanced magnetic resonance imaging. This patient illustrates (a) the potential of pseudomyxoma to cause skin fistula and (b) the possibility that this intraabdominal process can first present as a dermatologic complication. ( info)

2/102. Pseudomyxoma retroperitonei.

    We report a rare case of pseudomyxoma retroperitonei in a 58-year-old woman with a past history of severe appendicitis. The imaging showed a multicystic mass similar to pseudomyxoma peritonei, but the tumor was located in the retroperitoneal space. ( info)

3/102. pseudomyxoma peritonei in the pleural cavity: report of a case.

    PURPOSE: pseudomyxoma peritonei is a rare disease characterized by mucinous ascites and associated with ruptured mucocele, cystadenoma, and low-grade carcinoma arising from the appendix, ovaries, or colon. Metastases and extraperitoneal involvement are extremely rare events. METHOD: This is a case report of a patient with pseudomyxoma peritonei with pleural involvement. RESULTS: A 38-year-old male patient with a pseudomyxoma peritonei from appendiceal origin underwent an extensive cytoreduction procedure. During the operation pleural involvement was noted. This was later confirmed by thoracoscopy. An expectant policy was followed until the patient became symptomatic with progressive disease in the abdomen and both pleural cavities. With systemic chemotherapy (5-fluorouracil and leucovorin), a good clinical response was obtained, and the patient was alive with stable disease 2.5 years after the first diagnosis. CONCLUSIONS: Involvement of the pleural cavity by pseudomyxoma peritonei is rare and carries an unfavorable prognosis. Whenever possible, the same guidelines as for intra-abdominal disease should be followed: extensive cytoreductive procedures with local and/or systemic chemotherapy. In our patient we hope to achieve a prolonged palliation with systemic chemotherapy. ( info)

4/102. Laparoscopic management of pseudomyxoma peritonei secondary to adenocarcinoma of the appendix.

    pseudomyxoma peritonei is a rare disease in which the abdominal cavity fills with thick mucoid material secondary to either benign or malignant conditions. We discuss a case where pseudomyxoma peritonei secondary to adenocarcinoma of the appendix was diagnosed and managed laparoscopically. The laparoscopic approach allows thorough exploration of the abdomen, as well as irrigation and aspiration of the thick mucinous material using a 10-mm suction cannula and the instillation of mucolytic agents such as 5% dextrose solution. appendectomy or right hemicolectomy can be performed with minimal disturbance of the anterior abdominal wall, thus minimizing future adhesions as well as possible tumor-cell implantation. Intraperitoneal catheters for chemotherapy can be placed easily through the port sites. These measures offer an alternative to radical peritoneal dissection and can be accomplished during the initial laparoscopic exploration. ( info)

5/102. pseudomyxoma peritonei with high serum CA19-9: report of three cases.

    pseudomyxoma peritonei (PMP) is an unusual form of intraabdominal neoplasm that produces a large amount of extracellular mucin. It is often associated with mucinous tumors of gastrointestinal tract or ovary. Herein, we report 3 patients with pseudomyxoma peritonei with high serum carbohydrate antigen 19-9 (CA19-9) levels. The first patient, who had a CA19-9 level of 1132 U/ml, had well-differentiated rectal cancer and died of chemotherapy complications, pneumonia and septic shock; one month after admission. The other 2 cases with CA19-9 levels of 2520 U/ml and 679 U/ml had tumors of unknown origins and had survived more than 1 year and 3 months after treatment, respectively. Usually, elevated serum CA19-9 levels are found in patients with pancreatic, biliary, colorectal, gastric or liver cancers. However, many studies have shown high serum CA19-9 levels are associated with mucinous carcinoma. Immunochemical studies also showed positive staining of CA19-9 in mucinous tumors. PMP is composed of large amounts of mucin, therefore, we suggest that serum and ascites CA19-9 levels should be routinely checked in patients with PMP. ( info)

6/102. Acidic glycosaminoglycans of abdominal mucin in a case of pseudomyxoma peritonei caused by appendiceal cancer.

    Although the use of intraperitoneal mucolytic agents is useful in the management of pseudomyxoma peritonei, effective removal of mucin is difficult even by repeated mucolysis in cases with massive ascites. To establish mucolytic therapy as a further effective procedure, biochemical analysis of mucin is required in greater detail. We reported here a case of pseudomyxoma peritonei caused by primary appendiceal cancer with biochemical analysis of mucin. The abdominal cavity in this case was filled with massive mucinous ascites. Although the mucolytic therapy was performed repeatedly, it was not effective in preventing mechanical ileus which was followed by surgical exclusion. Biochemical study of mucin showed that the mucin contained a small amount of hyaluronic acid, as characterized by electrophoretic study. These data led us to the hypothesis that the hyaluronic acid component plays a role in the pathogenesis of the replacement of mucin in the abdominal cavity. ( info)

7/102. Mucinous epithelial cysts of the spleen associated with pseudomyxoma peritonei.

    AIMS: We report two rare cases of neoplastic pseudomyxoma peritonei associated with splenic mucinous epithelial cysts and review previously reported cases of splenic mucinous lesions in order to investigate the extent and implications of such an association. methods AND RESULTS: The majority of mucinous lesions of the spleen appear to be associated with pseudomyxoma peritonei. The clinicopathological profile of these cases conforms to that of neoplastic pseudomyxoma peritonei, showing a similar age of onset, outcome and histological features. Most of the cases were associated with a confirmed or suspected appendiceal primary. The immunophenotype (cytokeratin 7 negative; cytokeratin 20 and CEA positive) of the lesions of both our cases, including those in the ovary, was suggestive of a gastrointestinal origin. CONCLUSIONS: splenomegaly due to cystic intrasplenic mucinous epithelial lesions may occasionally be the presenting feature of pseudomyxoma peritonei or herald tumour recurrence. Mucinous epithelial cysts of the spleen may also precede the development of pseudomyxoma peritonei. All cases of pseudomyxoma peritonei should be investigated for splenic involvement and, conversely, a primary mucinous neoplasm sought elsewhere in the abdomen in all cases of splenic mucinous cysts. ( info)

8/102. Congenital pleuroperitoneal communication in a patient with pseudomyxoma peritonei.

    BACKGROUND AND OBJECTIVES: pseudomyxoma peritonei syndrome is a rare disease arising from a perforated appendiceal adenoma. The syndrome is characterized by progressive accumulation of mucinous ascites and tumor within the peritoneal cavity. Direct extension of pseudomyxoma peritonei to the pleural cavity is uncommon and has been associated with surgical penetration of the diaphragm at the time of cytoreduction. methods: We review the case of a patient who presented with mucoid peritoneal and pleural fluid consistent with spontaneous pleural spread of pseudomyxoma peritonei. RESULTS: Surgical exploration confirmed direct pleuroperitoneal communication by macroscopic diaphragmatic fenestration. CONCLUSIONS: This is a rare phenomenon. We outline a therapeutic approach to be applied when pleural involvement is suspected in patients with pseudomyxoma peritonei syndrome. ( info)

9/102. Pseudomyxoma peritoneii.

    A middle aged male patient presented with gradual distension of the abdomen. Imaging modalities showed classical features of pseudomyxoma peritoneii which was confirmed by aspiration cytology. Details of the case are described and relevant literature is reviewed. ( info)

10/102. Malignant peritoneal mesothelioma with mimicry of pseudomyxoma peritonei in a patient with a history of perforated sigmadiverticulitis.

    We describe a 57-year-old man who presented with diffuse abdominal pain, abdominal enlargement, vomitus, dyspnea and a weight loss of 30 kg within 6 months. These acute symptoms were preceded by an episode of ascites and an acute sigmadiverticulitis 7 months ago. ultrasonography and computed tomography were suggestive of pseudomyxoma peritonei. However, malignant mesothelioma peritonei was diagnosed by open surgery with biopsy for histological examination. Despite R-2-resection of the tumor and following open hyperthermic intraperitoneal chemotherapy with initial remarkable recovery the patient died 5 months after therapeutical intervention. Malignant peritoneal mesothelioma is an extremely rare tumor with great diagnostic and therapeutic difficulties. We report a case including diagnostical work up and the medical surgical therapy of this disease. ( info)
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