非阑尾起源的腹膜假黏液瘤8例临床病理分析  

Pseudomyxoma peritonei of non-appendiceal origin: a clinicopathologic analysis of eight cases

作　　者：李方[1] 齐长海[1] 卢一艳[1] 侯芳[1] 郝志红[1] 翟喜超[2] 庞少军[2] 

机构地区：[1]航天中心医院病理科,北京100049 [2]航天中心医院黏液瘤科,北京100049

出　　处：《诊断病理学杂志》2017年第2期86-91,共6页Chinese Journal of Diagnostic Pathology

基　　金：航天中心医院院级课题(YN201505)

摘　　要：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 clinicopathologic features and immunohistochemical phenotypes of pseudomyxoma peritonei（ PMP） derived from non-appendiceal primary. Methods Specimens from 8 patients with PMP of non-appendiceal origin were retrospectively analyzed using clinicopathological method. Representative slides of tumors were submitted for immunohistochemical staining. Antibodies used for immunohistochemical staining included CK7,CK-20,CDX-2,MUC-2. Results 8 cases of PMP were non-appendiceal origin,including three men and five women,ranging in age from 36 to 75 years,mean age was 52 years. These patients presented with abdominal distension,abdominal pain,anorexia, increased abdominal girth and ascites. The primary tumors, of which 3 cases were mucinous adenocarcinoma of the colon,3 cases of teratoma（ 2 cases of retroperitoneal teratoma,1 case of ovarian teratoma）,1 case of urachal mucinous adenocarcinoma,1 case of intestinal duplication containing low-grade mucinous neoplasm. Mucinous adenocarcinoma of colon（ 2 cases） and urachus（ 1 case）,and retroperitoneal teratoma（ 1 case） were associated with high grade PMP. One case of retroperitoneal teratoma and one cases of intestinal duplication containing low-grade mucinous neoplasm were associated with low grade PMP. One case of ovarian teratoma concomitant peritoneal lesion was acellular mucin. Immunohistochemical phenotypes were CK7- / CK20 ＋,Villin ＋,MUC- 2 ＋,except of one case of PMP with previous history of retroperitoneal teratoma. Conclusions Pseudomyxoma peritonei of non-appendiceal origin is rare,the diagnosis should be excluded appendiceal mucinous neoplasms based on the extensive sampling. Colorectal and urachal primaries are more likely to be associated with high grade PMP. Mucinous neoplasms in association with gonadal and extragonadal teratomas may progressed to low-grade or high-grade PMP,the majority depending on the types of mucinous neoplasms. Mucinous neoplasms arised in intestinal duplication should be also considered in

关 键 词：腹膜假黏液瘤 畸胎瘤 脐尿管癌 肠重复 

分 类 号：R735.5[医药卫生—肿瘤]