Pathologic diagnosis, origin, and natural history of pseudomyxoma peritonei.
Pathologic diagnosis, origin, and natural history of pseudomyxoma peritonei.
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DOI：
10.1200/edbook_am.2013.33.221
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发表时间：
2013-01-01
期刊：
影响因子：
--
通讯作者：
Gwin, Katja
中科院分区：
文献类型：
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作者：
Buell-Gutbrod, Rebecca;Gwin, Katja
粘液性腹水和腹腔内的粘液池与肿瘤性粘液上皮相关是腹膜假粘液瘤（PMP）的特征性特征。临床表现为腹胀和胶状腹水。在女性患者中，可以看到盆腔肿块。计算机断层扫描的影像学表现包括肝、脾边缘的扇形改变和致密腹水。在外科手术中，PMP表现为腹膜腔中肉眼可见的粘蛋白。阑尾外、右下象限及更远处的粘蛋白的存在是重要的诊断发现。阑尾可能扩张或破裂。在女性中，通常有双侧卵巢表面受累，提高了原发性卵巢肿瘤的鉴别诊断;然而，这些是极罕见的PMP原因。由于阑尾病变与卵巢转移性粘液性肿瘤之间存在联系，因此在任何粘液性腹膜或卵巢病变的情况下进行阑尾切除术可能是谨慎的，即使阑尾大体正常。胃肠道，特别是肝胰胆管系统，也需要由外科医生进行评估。病理上，PMP几乎完全来自阑尾的低度或高度粘液性肿瘤。这些肿瘤必须与罕见的良性粘液性腹水和非腹膜原发性肿瘤相鉴别。PMP的临床病程较长，伴有进行性纤维粘连和阻塞性疾病;据报道，采用温热腹腔化疗的积极手术和细胞减灭治疗可改善临床结局。
Mucinous ascites and pools of mucin within the peritoneal cavity associated with neoplastic, mucinous epithelium are the characteristic features of pseudomyxoma peritonei (PMP). Clinically, PMP presents with abdominal distension and gelatinous ascites. In female patients, pelvic masses can be seen. Radiologic findings on computed tomography include scalloping of the hepatic and splenic margins and dense ascites. Surgically, PMP is encountered as grossly visible mucin in the peritoneal cavity. The presence of mucin outside of the appendix, in the right lower quadrant, and beyond is an important diagnostic finding. The appendix may be distended or ruptured. In women, there is often bilateral surface involvement of the ovaries, raising the differential diagnosis of primary ovarian neoplasms; however, these are extremely rare causes of PMP. Because of the association between appendiceal lesions and metastatic mucinous neoplasms of the ovary, appendectomy in the setting of any mucinous peritoneal or ovarian process may be prudent, even if the appendix is grossly normal. The gastrointestinal tract, especially the hepatopancreato biliary system, also needs to be assessed by the surgeon. Pathologically, PMP arises almost exclusively from low- or high-grade mucinous neoplasms of the appendix. These neoplasms must be distinguished both from rare benign causes of mucinous ascites and from nonappendiceal primary tumors. PMP has a protracted clinical course with progressive fibrous adhesions and obstructive disease; aggressive surgical and cytoreductive therapy with hyperthermic intraperitoneal chemotherapy has been reported to improve clinical outcomes.