Ronnett B M, Shmookler B M, Sugarbaker P H, Kurman R J
Department of Pathology, Johns Hopkins University, Baltimore, Maryland, USA.
Anat Pathol. 1997;2:197-226.
For many years the clinical syndrome of PMP has been enigmatic. Based on recent studies reevaluating the condition, tumors previously designated PMP can now be viewed as two pathologically and prognostically distinct disease processes. Disseminated peritoneal adenomucinosis is characterized by copious mucinous ascites (the classical clinical syndrome of PMP) and histologically bland peritoneal mucinous tumors. The condition can be attributed to a ruptured appendiceal mucinous adenoma in the vast majority of cases. It has an indolent clinical course when surgically treated but may recur over months to years. Peritoneal mucinous carcinomatosis is characterized by abundant peritoneal mucinous tumor, similar to the clinical presentation of adenomucinosis. However, microscopically, the peritoneal tumors have the architectural and cytologic features of carcinoma, are derived from gastrointestinal mucinous adenocarcinomas, and are associated with a significantly worse prognosis than cases of adenomucinosis. A third group of tumors displays intermediate or discordant histologic features but manifests a clinical course very similar to cases of pure peritoneal carcinomatosis. Women often have concomitant ovarian mucinous tumors that suggest primary ovarian neoplasia. Morphologic, immunohistochemical, and molecular studies support the interpretation that the ovarian tumors are secondary and that adenomucinosis is of appendiceal origin in women as well as men. The recognition that the ovarian tumors in nearly all of the cases of DPAM and mucinous carcinomatosis are secondary seriously calls into question the existence of a borderline group of ovarian mucinous tumors. Therefore, primary ovarian mucinous tumors should be classified as either benign or malignant. Tumors exhibiting the features currently interpreted as borderline should be included in the benign group and designated atypical proliferative mucinous tumors.
多年来，腹膜假黏液瘤（PMP）的临床综合征一直令人困惑。基于最近对该疾病的重新评估研究，以前被诊断为PMP的肿瘤现在可被视为两种在病理和预后上截然不同的疾病过程。播散性腹膜腺黏液瘤的特征是大量黏液性腹水（PMP的经典临床综合征）和组织学上温和的腹膜黏液性肿瘤。在绝大多数情况下，这种情况可归因于阑尾黏液性腺瘤破裂。手术治疗后临床病程较为缓慢，但可能在数月至数年内复发。腹膜黏液性癌的特征是大量腹膜黏液性肿瘤，类似于腺黏液瘤的临床表现。然而，在显微镜下，腹膜肿瘤具有癌的结构和细胞学特征，源自胃肠道黏液腺癌，且与腺黏液瘤病例相比，预后明显更差。第三组肿瘤表现出中间或不一致的组织学特征，但临床病程与单纯腹膜癌病例非常相似。女性常伴有卵巢黏液性肿瘤，提示原发性卵巢肿瘤。形态学、免疫组织化学和分子研究支持这样的解释：卵巢肿瘤是继发性的，并且在女性和男性中，腺黏液瘤均起源于阑尾。几乎所有播散性腹膜腺黏液瘤和黏液性癌病例中的卵巢肿瘤都是继发性的这一认识，严重质疑了卵巢交界性黏液性肿瘤组的存在。因此，原发性卵巢黏液性肿瘤应分为良性或恶性。表现出目前被解释为交界性特征的肿瘤应归入良性组，并命名为非典型增生性黏液性肿瘤。
