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Presented by Saeid Movahedi, M.D. and prepared by Shien Micchelli, M.D.
Case 1: 38 y/o female who presents with an 8 cm uterine mass.
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Question 1 of 1
1. Question
Week 238: Case 1
38 y/o female who presents with an 8 cm uterine mass./images/9_12_05case1a.jpg
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/images/9_12_05case1e.jpgCorrect
Answer: Epithelioid leiomyoma
Histology: This is a variably cellular neoplasm consisting of rounded cells with slightly eosinophilic cytoplasm and vesicular nuclei with small nucleoli. The cells are arranged in cords, forming small to medium sized “cystic” structures within an edematous matrix. Mitotic figures are rare (1 MF per 10 HPF) and no tumor cell necrosis is identified.
Discussion: Epithelioid smooth muscle tumors (ESMTs) are rare and are composed predominantly of rounded or polygonal cells, with or without a minor spindle cell component. A higher proportion of these tumors is clinically malignant compared with typical spindle cell smooth muscle tumors, ranging from 10 to 40% of cases. The gross appearance of these neoplasms may resemble typical leiomyomas or these lesions may have worrisome gross features such as a more fleshy appearance or areas of hemorrhage. Benign tumors usually lack these features. Nuclear atypia, mitotic index (mitotic figures per 10 high power fields [MF/10 HPF]), and presence of tumor cell necrosis are features used to predict behavior.
images/esmt_table.jpg
ESMTs usually express muscle markers, but this can be diminished compared with expression in typical spindled smooth muscle tumors; focal cytokeratin expression can also be seen.
Differential diagnosis includes true epithelial tumors (carcinomas, which usually have more diffuse expression of cytokeratins and lack expression of muscle markers), PEComas (which express HMB-45 and CD99), endometrial stromal nodules (which usually exhibit diffuse expression CD10 and have focal or absent expression of muscle markers), and uterine tumors resembling ovarian sex cord tumors (these can have expression of inhibin).
Incorrect
Answer: Epithelioid leiomyoma
Histology: This is a variably cellular neoplasm consisting of rounded cells with slightly eosinophilic cytoplasm and vesicular nuclei with small nucleoli. The cells are arranged in cords, forming small to medium sized “cystic” structures within an edematous matrix. Mitotic figures are rare (1 MF per 10 HPF) and no tumor cell necrosis is identified.
Discussion: Epithelioid smooth muscle tumors (ESMTs) are rare and are composed predominantly of rounded or polygonal cells, with or without a minor spindle cell component. A higher proportion of these tumors is clinically malignant compared with typical spindle cell smooth muscle tumors, ranging from 10 to 40% of cases. The gross appearance of these neoplasms may resemble typical leiomyomas or these lesions may have worrisome gross features such as a more fleshy appearance or areas of hemorrhage. Benign tumors usually lack these features. Nuclear atypia, mitotic index (mitotic figures per 10 high power fields [MF/10 HPF]), and presence of tumor cell necrosis are features used to predict behavior.
images/esmt_table.jpg
ESMTs usually express muscle markers, but this can be diminished compared with expression in typical spindled smooth muscle tumors; focal cytokeratin expression can also be seen.
Differential diagnosis includes true epithelial tumors (carcinomas, which usually have more diffuse expression of cytokeratins and lack expression of muscle markers), PEComas (which express HMB-45 and CD99), endometrial stromal nodules (which usually exhibit diffuse expression CD10 and have focal or absent expression of muscle markers), and uterine tumors resembling ovarian sex cord tumors (these can have expression of inhibin).
