Uterine perivascular epithelioid cell tumors (PEComa) and the accuracy of proposed classification systems in predicting the malignant versus non-malignant behavior.
Gynecol Oncol
; 188: 35-43, 2024 Sep.
Article
em En
| MEDLINE
| ID: mdl-38905754
ABSTRACT
OBJECTIVE:
To compare the accuracy of available classification systems (Folpe, modified Folpe, Bennet, and Schoolmester) in predicting the behavior of uterine Perivascular Epithelioid Cell tumors (PEComas).METHODS:
We reviewed the pathology registry to identify all uterine PEComas treated at our center. We conducted a systematic literature review searching electronic databases from inception to November 2023. We included all references reporting at least one case of uterine PEComa; cases associated with tuberous sclerosis complex were excluded. Patient-level data were extracted by identified records. Survival analysis was used to assess the accuracy of all proposed classification systems to classify uterine PEComas as malignant versus non-malignant.RESULTS:
Six uterine PEComas were treated at our center. The literature search identified 101 uterine PEComas from 32 studies. Eighty-five out of 107 PEComas (28 studies and our series) reported enough follow-up data and details to apply all four classifications. The modified Folpe classification demonstrated the highest hazard ratio (HR) for relapse (HR8.63; 95% confidence interval [CI] 2.06-36.1) and death due to PEComa (HR6.8, 95%CI0.89-51.6) for malignant versus non-malignant PEComas. Changing the cut-off of PEComa size to ≥8 cm and mitotic figures per 50 high power fields to ≥5, the HR for recurrence lowered (HR6.26; 95% CI 2.20-17.80), but HR for death increased (HR10.3; 95% CI 1.35-77.80).CONCLUSIONS:
The modified Folpe classification was the most accurate in predicting the PEComa behavior. Changing the cut-off of PEComa size and number of mitotic figures may improve the accuracy in predicting death due to disease.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Neoplasias Uterinas
/
Neoplasias de Células Epitelioides Perivasculares
Idioma:
En
Ano de publicação:
2024
Tipo de documento:
Article