1/5. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/5. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/5. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/5. pseudomyxoma peritonei, origin from appendix: report of cases with images.This report demonstrates the cases of a 47-year-old housewife, a 54-year-old female, and an 80-year-old woman in whom pseudomyxoma peritonei was found accidentally during surgery. pseudomyxoma peritonei is a rare disease. No effective treatment is known. Modern treatments include radical surgical excision with appendicectomy and oophorectomy in women and adjuvant hyperthermic intraperitoneal and systemic chemotherapy. Major causes of morbidity and mortality are bowel obstruction and biliary obstruction. Five-year survival rate, depending on whether the disease is benign or malignant, is about 53-75%. Median survival is about 2 years under surgical management.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/5. Pulmonary metastases in pseudomyxoma peritonei syndrome.BACKGROUND: pseudomyxoma peritonei (PMP) is a rare disease arising from a mucinous cystadenoma of appendiceal origin. The syndrome has been characterized by progressive growth of mucinous tumors, tense mucinous ascites, and ultimately death. Abdominal and pelvic recurrence after resection of intraperitoneal disease occurs in all patients unless adjunctive measures are taken. Local spread of PMP by direct extension to the pleural or pericardial space is uncommon but has been reported in the literature. Here we report development of pulmonary parenchymal metastases after treatment for PMP. methods: The charts of 3 patients were retrospectively reviewed for the presentation and management of metastatic PMP. RESULTS: Three patients underwent resection for pulmonary parenchymal metastases of PMP. All patients recovered uneventfully. The continue to do well after 2 to 8 years of follow-up. CONCLUSIONS: Pulmonary metastasectomy for PMP is safe and effective after treatment of intraperitoneal disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |