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- 我们是腹膜假性粘液瘤群体 请专家前来
| 姓 名： | ××× | 性别： | 女 | 年龄： | 58 |
| 标本名称： | 腹膜假性粘液瘤 | ||||
| 简要病史： | 3年前 阑尾粘液性囊腺瘤 | ||||
| 肉眼检查： | 果冻状 | ||||
我们这里是腹膜假性粘液瘤患者群体，因为这种病很少，而且没有标本，所以估计研究的很少，但是现在我们在这里已经很多了，这个月我们有8个人做了这个手术，希望这方面的专家能够及时看到，能够对标本进行研究，也希望群主或管理员帮着宣传一下，让更多的人了解一下这方面的情况。
这个病多发于阑尾，粘液瘤在腹腔内种植，并不侵入脏器，只是占有腹腔的有限空间，最后致肠梗、肠粘，他对静脉化疗不起作用，现在多进行减瘤术后的腹腔热灌注化疗。
麻烦了，群主，希望能宣传一下，有需要了解的 我的电话13563070850
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×参考诊断
Low-grade appendiceal mucinous neoplasms cause pseudomyxoma peritonei that tends to remain confined to the abdominal cavity for long periods, often with little or no evidence of lymphatic or hematogenous spread and minimal invasion of underlying viscera. Progressive loss of intestinal function occurs and intestinal obstruction is often the cause of death. In contrast, frankly invasive adenocarcinomas infiltrate the parenchyma of abdominal organs and are likely to exhibit lymphatic and hematogenous spread. Thus, the distinction between low-grade and high-grade carcinomas is of prognostic significance. Specific features that have been associated with a poor prognosis in appendiceal neoplasia include advanced stage, nonmucinous lesions, signet ring cell carcinoma, bulky peritoneal disease, abdominal distenstion, and the spread of mucus beyond the right lower quadrant of the abdomen (whether or not cells are identified within it). If extensive sampling fails to demonstrate neoplastic cells in the extra-appendiceal mucin, the prognosis is better.
Recent advances in therapy have involved cytoreductive surgery to extirpate all visible peritoneal tumor, accompanied by intraperitoneal chemotherapy. The overall 5-year survival of such a regimen was 57% in one recent series in another, the median survival was 9.8 years. A 20-year survival of 70% has been claimed, provided the mucinous neoplasm is minimally invasive and cytoreduction is complete, but if only palliative resection is possible, the median survival at 2 years was 57% in one study
Recent advances in therapy have involved cytoreductive surgery to extirpate all visible peritoneal tumor, accompanied by intraperitoneal chemotherapy. The overall 5-year survival of such a regimen was 57% in one recent series in another, the median survival was 9.8 years. A 20-year survival of 70% has been claimed, provided the mucinous neoplasm is minimally invasive and cytoreduction is complete, but if only palliative resection is possible, the median survival at 2 years was 57% in one study
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Because pseudomyxoma peritonei is essentially a clinically descriptive term, the pathologic diagnosis should indicate the histologic type and grade of the neoplasm and its origin—for example, “appendiceal mucinous adenocarcinoma, low-grade, with pseudomyxoma peritonei” or “mucinous adenocarcinoma of the urachus, well differentiated, with pseudomyxoma peritonei The pathologist should state whether the mucin is acellular or cellular, based on extensive sampling, because of the prognostic implications.
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Histologically, malignant cells may be scanty within this mucinous material, and widespread sampling may be required to demonstrate them. Sometimes, even extensive sampling fails to demonstrate epithelial cells in the mucin .This improves the prognosis provided sampling has been adequate. When found, they are seen as strips, small groups, acinar structures, or single cells, often with abundant intracellular mucin . Cytologic dysplasia is typically low grade and may be extremely subtle. The cells generally immunoexpress CDX-2 and CK20, and there is often coexpression of CK7. Signet ring morphology may be identified; these lesions should be classified as high-grade mucinous adenocarcinomas. Cytologic preparations of pseudomyxomatous material reveal abundant mucin, often with mesothelial cells, histiocytes, and fibroblast-like spindle cells. Epithelial cells with abundant intracellular mucin can be identified in the majority of cases if adequate material is available; they may be present as tight three-dimensional clusters, flat monolayers, strips with basally located nuclei, or single cells.
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Classification of Pseudomyxoma Peritonei
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