1/27. 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) |
2/27. 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) |
3/27. Cystic peritoneal mesothelioma: report of a case.Peritoneal mesothelioma is a rare disease, especially when it arises in a cystic form with tardive and often nonspecific symptoms. While diffuse neoplasms have an unfavorable prognosis, cystic forms are usually benign. An accurate diagnosis can only be made only with electron microscopy and immunohistochemical studies. A 92-year-old woman with an ultrastructurally ascertained cystic peritoneal mesothelioma was admitted to the hospital's emergency ward, and was considered to be unusual because of the size of the mass and the patient's age. A review of the literature is made, and the clinical and diagnostic aspects of this disease are also discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/27. 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) |
5/27. Recurrent leiomyomatosis peritonealis disseminata exacerbated by in vitro fertilization.leiomyomatosis peritonealis disseminata is a rare disease that is most often discovered during pregnancy or in patients with a history of oral contraceptive use. We report the first case of a patient with the disease who conceived by in vitro fertilization and the complications that occurred.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/27. Malignant peritoneal mesothelioma presenting with respiratory symptoms.Malignant peritoneal mesothelioma is a rare disease associated with mild, nonspecific abdominal symptoms and a wide spectrum of imaging findings, with thickened mesentery and peritoneum being the most common ones. A case of a malignant peritoneal mesothelioma presenting with manifestations of pulmonary disease is reported. Imaging evaluation revealed pleural, lung and pericardial involvement together with retroperitoneal lymphadenopathy, little ascites and extensive omental, but only subtle, mesenteric thickening.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/27. Primary diffuse malignant peritoneal mesothelioma: case report and update of therapy.BACKGROUND AND OBJECTIVES: Primary Diffuse Malignant Peritoneal mesothelioma is a rare disease, with an incidence of 2.2 cases in 1. 000.000 in the USA. It occupies 10% of all mesotheliomas referred in literature. methods: We describe a case of diffuse malignant peritoneal mesothelioma arising in a 54-year-old woman who presented a small bowel occlusion. A middle line laparotomy was done; multiple biopsies and an ileostomy were performed. There was not a history of exposure to asbestos. Histologic diagnosis was based on light microscopy, histochemistry, and immunohistochemistry. RESULTS: Patient had no further treatment because of her poor general conditions. She died 4 months later. CONCLUSIONS: Update of treatment is briefly described with particular attention to multimodality approach (surgery, chemotherapy, radiotherapy) and other new therapeutic options (iperthermochemotherapy, immunotherapy, gene therapy), currently in clinical trials.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/27. Mesenteric lipoma: report of a case with emphasis on US findings.Mesenteric lipoma (ML) is a relatively rare disease that has been very infrequently reported. We present the clinical data and medical imaging results of an asymptomatic case with ML incidentally detected by sonography (US). On US the lesion was imaged as a well-demarcated hypoechoic mass with multiple linear echoes. The mass also changed location under probe compression. The lesion was imaged as a clearly demarcated non-enhanced homogeneous fat-density mass on CT. color Doppler US and angiography confirmed the avascular nature of the mass. knowledge of the US findings in this case is useful for the diagnosis of ML.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/27. pseudomyxoma peritonei: report of an unusual case.pseudomyxoma peritonei, a rare disease, is characterized by accumulation of mucinous material in the peritoneal cavity and is commonly caused by ovarian tumors or appendicular lesions. This report presents necropsy findings of an unusual case of pseudomyxoma peritonei caused by dissemination of mucinous adenocarcinoma of the stomach, hitherto unreported in literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/27. Peritoneal cystic mesothelioma: a case series.BACKGROUND: Cystic peritoneal mesothelioma is a rare disease associated with a favorable short-term prognosis. Longer follow-up documenting a persistence of symptoms and a high rate of recurrence after debulking surgery along with an uncertain natural history prompt a re-evaluation of prior treatment recommendations. No prior long-term clinical study of these patients is available. methods: The experience with five cases of cystic peritoneal mesothelioma, four females and one male, are reviewed. All of these patients were treated with cytoreductive surgery with peritonectomy procedures and heated intraoperative intraperitoneal chemotherapy. CT, pathology and current status were investigated in order to learn more about the natural history of this disease. RESULTS: All patients were symptomatic from abdominal distention and three of the four complained of severe pain. female patients complained of long periods of recurrent abdominal and pelvic pain poorly managed by oral analgesics. In one patient prolonged conservative management over ten years resulted in transition to an invasive process with extensive lymph nodal metastases. Her prognosis for long-term survival is guarded because of mesothelioma extension into the chest. Disease control of both ascites and pain in the abdomen and pelvis was achieved in all five patients treated with cytoreductive surgery plus intraperitoneal chemotherapy. CONCLUSIONS: Cystic peritoneal mesothelioma should no longer be referred to as "benign" cystic mesothelioma. An aggressive approach with complete disease eradication is the correct goal of treatment. From our experience, cytoreductive surgery to remove all visible tumor and intraperitoneal chemotherapy to control microscopic residual disease will help patients with peritoneal cystic mesothelioma to remain symptom- and disease-free over an extended time period with a single surgical intervention. disease eradication may prevent the transition to an aggressive and fatal disease process.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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