1. Low-Grade Ovarian Serous Neoplasms (Low-Grade Serous Carcinoma and Serous Borderline Tumor) Associated With High-Grade Serous Carcinoma or Undifferentiated Carcinoma
- Author
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W. Glenn McCluggage and Clinton Boyd
- Subjects
Adult ,Oncology ,medicine.medical_specialty ,Pathology ,endocrine system diseases ,Serous carcinoma ,medicine.disease_cause ,Pathology and Forensic Medicine ,Pathogenesis ,PAX8 Transcription Factor ,Internal medicine ,Biomarkers, Tumor ,medicine ,Carcinoma ,Humans ,Paired Box Transcription Factors ,Neoplasm ,WT1 Proteins ,Aged ,Ovarian Neoplasms ,business.industry ,Cell Differentiation ,Cell Dedifferentiation ,Middle Aged ,medicine.disease ,Immunohistochemistry ,female genital diseases and pregnancy complications ,Cystadenocarcinoma, Serous ,Serous fluid ,Treatment Outcome ,Cystadenoma ,Female ,Surgery ,Neoplasm Grading ,Tumor Suppressor Protein p53 ,Anatomy ,PAX8 ,Carcinogenesis ,business - Abstract
Recent literature has suggested a dual pathway of ovarian serous carcinogenesis, with most serous carcinomas falling into 1 of 2 categories, low grade and high grade. These are considered to represent 2 distinct tumor types with a different underlying pathogenesis and associated with different molecular events, clinical behavior, and prognosis. Low-grade serous carcinoma is thought to evolve in many instances from a preexisting serous borderline tumor and cystadenoma. Given the distinct pathogenesis and different molecular events, it is expected that the coexistence of low-grade and high-grade serous carcinoma would be rare or may even be mutually exclusive; moreover, there are very few reported examples in the literature. We report a series of 7 cases in patients aged 34 to 78 years in whom ovarian low-grade serous carcinoma (4 cases, including 3 with associated serous borderline tumor), serous borderline tumor (2 cases), or seromucinous borderline tumor (1 case) was associated with a high-grade carcinoma, either high-grade serous (5 cases) or undifferentiated carcinoma (2 cases). The low-grade and high-grade components coexisted in the original neoplasm in 4 cases, and the high-grade component was present only in recurrence in 3 cases. In both instances, the undifferentiated carcinoma had a focal rhabdoid morphology, and alternative primary sites of tumor were excluded by a combination of clinical, radiologic, and pathologic parameters. We illustrate that low-grade serous carcinoma or serous borderline tumor ("low-grade" serous neoplasms) may rarely be associated with, and probably give rise to, a high-grade carcinoma, either high-grade serous or undifferentiated carcinoma. The coexistence of a low-grade serous neoplasm and undifferentiated carcinoma can be regarded as a form of dedifferentiation. p53 was diffusely positive in 4 of 6 high-grade carcinomas, which raises the possibility that secondary Tp53 mutation is important in high-grade transformation in some of these cases. WT1 was negative in the 2 undifferentiated carcinomas, and PAX8 was positive in 1, suggesting that the latter marker is more useful in helping to confirm a Mullerian origin in dedifferentiated low-grade serous neoplasms.
- Published
- 2012
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