Evaluation of diagnostic criteria and behavior of ovarian intestinal-type mucinous tumors: atypical proliferative (borderline) tumors and intraepithelial, microinvasive, invasive, and metastatic carcinomas. 1999

M A Riopel, and B M Ronnett, and R J Kurman
Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.

Histologic criteria for the distinction of ovarian mucinous borderline tumors (MBTs) from invasive mucinous carcinomas (MUCCAs) and the definitions of intraepithelial (noninvasive) carcinoma and microinvasion are controversial. Accurate assessment of the behavior of these tumors has been obscured by inclusion of cases of pseudomyxoma peritonei (PMP), an entity of extraovarian origin, and misclassification of the ovarian tumors in PMP and metastatic mucinous carcinomas (METCAs) as either advanced-stage MBTs or primary ovarian MUCCAs. One hundred thirty-six intestinal-type ovarian mucinous tumors without PMP were evaluated for the presence of stromal invasion, marked cytologic atypia, epithelial stratification of more than three cell layers, and necrosis. Criteria for the diagnosis of MBT, MBT with intraepithelial carcinoma, MBT with microinvasion (MIBT), MUCCA, and METCA were established and correlated with behavior. Twenty-three (59%) of 39 patients whose tumors had stromal invasion of more than 5 mm died of disease. Stromal invasion of more than 5 mm was the sole feature that correlated with a poor prognosis. In the absence of this feature, marked cytologic atypia, epithelial stratification of more than three layers, microinvasion (<5 mm), or necrosis did not have an adverse effect on survival. Tumors were classified as MBT (n = 65; 48%) based on absence of stromal invasion, regardless of degree of cytologic atypia or epithelial stratification; of these, 28 (43%) qualified as intraepithelial carcinoma based on epithelial stratification of more than three cell layers or marked cytologic atypia. Tumors with stromal invasion of less than 5 mm were classified as MIBT (n = 8; 6%). Tumors with stromal invasion of more than 5 mm were classified as MUCCA (n = 24; 18%). Tumors with a nodular pattern of stromal invasion, morphology inconsistent with ovarian origin, or a primary site elsewhere were classified as METCA (n = 35; 26%). Four tumors could not be definitively classified. Of the 86 cases with follow-up (median, 33 months) all MBTs (n = 44) and MIBTs (n = 6) were stage I, with 5-year survival rates of 100%. MUCCAs (n = 17) that were stage I had a 5-year survival rate of 91%; all patients with advanced-stage MUCCA died of disease. Five-year survival rate for METCAs (n = 19) was 11%. METCAs were more common than MUCCAs but can mimic MUCCAs and MBTs clinically and histologically. In the absence of a primary site elsewhere, METCA should be strongly suspected when there is bilateral surface involvement and a characteristic nodular pattern of invasion. It is important to recognize this pattern because 5-year survival rate for METCA (11%) was substantially less than that of MUCCA (all stages, 51%) and MBT (100%). Because all MBTs, regardless of degree of atypia or stratification, were stage I and benign, we prefer to designate them as atypical proliferative mucinous tumors. Marked cytologic atypia, epithelial stratification of more than three layers, and microinvasion (<5 mm) had no effect on the behavior of MBT. Noninvasive mucinous tumors with marked cytologic atypia or excessive epithelial stratification can be interpreted as atypical proliferative tumors with intraepithelial carcinoma and those with microinvasion can be designated as atypical proliferative tumors with microinvasion; these tumors appear to represent transitional stages in ovarian mucinous carcinogenesis.

UI MeSH Term Description Entries
D010051 Ovarian Neoplasms Tumors or cancer of the OVARY. These neoplasms can be benign or malignant. They are classified according to the tissue of origin, such as the surface EPITHELIUM, the stromal endocrine cells, and the totipotent GERM CELLS. Cancer of Ovary,Ovarian Cancer,Cancer of the Ovary,Neoplasms, Ovarian,Ovary Cancer,Ovary Neoplasms,Cancer, Ovarian,Cancer, Ovary,Cancers, Ovarian,Cancers, Ovary,Neoplasm, Ovarian,Neoplasm, Ovary,Neoplasms, Ovary,Ovarian Cancers,Ovarian Neoplasm,Ovary Cancers,Ovary Neoplasm
D010190 Pancreatic Neoplasms Tumors or cancer of the PANCREAS. Depending on the types of ISLET CELLS present in the tumors, various hormones can be secreted: GLUCAGON from PANCREATIC ALPHA CELLS; INSULIN from PANCREATIC BETA CELLS; and SOMATOSTATIN from the SOMATOSTATIN-SECRETING CELLS. Most are malignant except the insulin-producing tumors (INSULINOMA). Cancer of Pancreas,Pancreatic Cancer,Cancer of the Pancreas,Neoplasms, Pancreatic,Pancreas Cancer,Pancreas Neoplasms,Pancreatic Acinar Carcinoma,Pancreatic Carcinoma,Acinar Carcinoma, Pancreatic,Acinar Carcinomas, Pancreatic,Cancer, Pancreas,Cancer, Pancreatic,Cancers, Pancreas,Cancers, Pancreatic,Carcinoma, Pancreatic,Carcinoma, Pancreatic Acinar,Carcinomas, Pancreatic,Carcinomas, Pancreatic Acinar,Neoplasm, Pancreas,Neoplasm, Pancreatic,Neoplasms, Pancreas,Pancreas Cancers,Pancreas Neoplasm,Pancreatic Acinar Carcinomas,Pancreatic Cancers,Pancreatic Carcinomas,Pancreatic Neoplasm
D011230 Precancerous Conditions Pathological conditions that tend eventually to become malignant. Preneoplastic Conditions,Condition, Preneoplastic,Conditions, Preneoplastic,Preneoplastic Condition,Condition, Precancerous,Conditions, Precancerous,Precancerous Condition
D002278 Carcinoma in Situ A lesion with cytological characteristics associated with invasive carcinoma but the tumor cells are confined to the epithelium of origin, without invasion of the basement membrane. Carcinoma, Intraepithelial,Carcinoma, Preinvasive,Intraepithelial Neoplasms,Neoplasms, Intraepithelial,Intraepithelial Carcinoma,Intraepithelial Neoplasm,Neoplasm, Intraepithelial,Preinvasive Carcinoma
D002288 Adenocarcinoma, Mucinous An adenocarcinoma producing mucin in significant amounts. (From Dorland, 27th ed) Carcinoma, Colloid,Carcinoma, Mucinous,Adenocarcinomas, Mucinous,Carcinomas, Colloid,Carcinomas, Mucinous,Colloid Carcinoma,Colloid Carcinomas,Mucinous Adenocarcinoma,Mucinous Adenocarcinomas,Mucinous Carcinoma,Mucinous Carcinomas
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005069 Evaluation Studies as Topic Works about studies that determine the effectiveness or value of processes, personnel, and equipment, or the material on conducting such studies. Critique,Evaluation Indexes,Evaluation Methodology,Evaluation Report,Evaluation Research,Methodology, Evaluation,Pre-Post Tests,Qualitative Evaluation,Quantitative Evaluation,Theoretical Effectiveness,Use-Effectiveness,Critiques,Effectiveness, Theoretical,Evaluation Methodologies,Evaluation Reports,Evaluation, Qualitative,Evaluation, Quantitative,Evaluations, Qualitative,Evaluations, Quantitative,Indexes, Evaluation,Methodologies, Evaluation,Pre Post Tests,Pre-Post Test,Qualitative Evaluations,Quantitative Evaluations,Report, Evaluation,Reports, Evaluation,Research, Evaluation,Test, Pre-Post,Tests, Pre-Post,Use Effectiveness
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective

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