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目的探讨非阑尾起源的腹膜假黏液瘤(PMP)临床病理学特征及免疫组化表型。方法对8例明确非阑尾起源的PMP临床病理资料进行回顾性分析,对具有代表性的切片进行CK7、CK20、villin、CDX2和MUC-2免疫组化染色。结果 8例非阑尾起源的PMP中,男性3例,女性5例,年龄36~75岁,平均年龄为52岁。主要临床症状有腹胀、腹痛、纳差、腹围增大、腹腔积液。8例PMP的原发肿瘤,3例为结肠黏液腺癌,3例为畸胎瘤(2例腹膜后畸胎瘤,1例卵巢畸胎瘤),1例起源于脐尿管黏液腺癌,1例起源于肠重复的低级别黏液性肿瘤。3例结肠及1例脐尿管黏液腺癌、1例腹膜后畸胎瘤所发生的腹膜种植播散为高级别腹膜假黏液瘤,1例腹膜后畸胎瘤及1例肠重复低级别黏液性肿瘤所发生的腹膜种植播散为低级别腹膜假黏液瘤,1例卵巢畸胎瘤伴发的腹膜病变为无细胞性黏液池。除1例既往有腹膜后畸胎瘤病史,其余病例免疫组化均为CK7(-)/CK20(+)、villin和MUC-2(+)。结论非阑尾起源的PMP较为罕见,诊断需多取材以除外阑尾黏液性肿瘤,并结合临床所见综合分析。原发部位为结直肠及脐尿管的腹膜假黏液瘤通常为高级别,伴有黏液性肿瘤成分的性腺及性腺外畸胎瘤可发生低级别或高级别PMP,大多数情况取决于畸胎瘤中黏液性肿瘤的性质。PMP原发病变还应与肠重复畸形的黏液性肿瘤鉴别。免疫组化染色对判断腹膜假黏液瘤的起源没有特别的帮助。
Objective To investigate the clinicopathological features and immunohistochemical phenotypes of non-appendiculomatous pseudomyxoma (PMP). Methods The clinicopathological data of 8 cases with definite non-appendix origin were retrospectively analyzed. The representative sections were stained with CK7, CK20, villin, CDX2 and MUC-2 immunohistochemistry. Results In 8 cases of non-appendix PMP, there were 3 males and 5 females, aged from 36 to 75 years with a mean age of 52 years. The main clinical symptoms are bloating, abdominal pain, anorexia, abdominal circumference increased, ascites. 8 cases of primary tumor of PMP, 3 cases of colonic mucinous adenocarcinoma, 3 cases of teratoma (2 cases of retroperitoneal teratoma, 1 case of ovarian teratoma), 1 case originated in urachal mucinous adenocarcinoma, One case had low-grade mucinous tumors that originated in the gut duplication. 3 cases of colon and 1 case of urate mucinous adenocarcinoma, 1 case of retroperitoneal teratoma occurred peritoneal dissemination of disseminated high-grade peritoneal pseudomyxoma, 1 case of retroperitoneal teratoma and 1 case of intestinal low-grade mucus duplication Peritoneal dissemination occurred in sexual tumors spread to low-grade peritoneal pseudomyxoma, 1 case of ovarian teratoma with peritoneal lesions of acellular mucus pool. Except for one case of previous history of retroperitoneal teratoma, the remaining cases were immunohistochemically CK7 (-) / CK20 (+), villin and MUC-2 (+). Conclusion The non-appendix originated PMP is relatively rare, the diagnosis should be taken in order to exclude appendix mucinous tumors, combined with the clinical findings of a comprehensive analysis. Peritoneal pseudomyxomas with primary sites of colorectal and urachal catheters are usually of high grade, with low-grade or high-grade PMP in gonads and extragonadal teratomas with mucinous tumor components, most often depending on teratology The nature of myxomas in tumors. PMP primary lesions should also be associated with intestinal deformity mucinous tumor identification. Immunohistochemistry is not particularly helpful in judging the origin of peritoneal pseudomyxoma.