Table 2
Early or precursor perivascular epithelioid cell lesions reported in the uterus
Reference Age Sites of early or precursor PEC lesions / size Histopathology of early or precursor PEC lesions Immunohistochemistry Associated pathologic findings and important clinical features
Fadare 2004 [11] 41 (1) Myometrium, small bowel lamina propria, and ovarian hilum. Aggregates of epithelioid cells with eosinophilic cytoplasm and vacuolated cytoplasm in an occasional perivascular distribution, no cytologic atypia. Positive for HMB-45, Melan-A, SMA, desmin, and PR. (1) Cervical PEComa.
(2) < 1 mm. (2) Associated with tuberous sclerosis.
(3) No recurrence or metastasis at 35 months’ follow-up.
Liang 2008 [12] 59 (1) Myometrium, cervical wall, and ovarian hilum. Bland-looking epithelioid clear cells (1) Positive for HMB-45, Melan-A, SMA, and myogenin. (1) Uterine malignant PEComa and LAM of pelvic lymph nodes.
(2) 1-5 mm. (2) Negative for desmin, ER, and PR. (2) Associated with tuberous sclerosis;
(3) No follow-up data.
Clay 2010 [22] 46 (1) Myometrium. Epithelioid cells in close approximation with lymphatic-type vessels, clear to granular and eosinophilic cytoplasm. (1) Positive for HMB-45, Mart-1, SMA, and desmin. (1) Early LAM
(2) <2 mm. (2) Negative for CD10. (2) No tuberous sclerosis.
(3) No follow-up data.
The present case 29 (1) Endometrium of adenomyosis, pelvic endometriosis, ovarian endometriotic cyst, and the endometrium of the uterine cavity. Aggregates of spindle-shaped and epithelioid cells in a perivascular distribution, light eosinophilic cytoplasm, no cytologic atypia (1) Positive for HMB-45, SMA, CD10 and PR. (1) Uterine PEComa, sclerosing PEComa, and LAM.
(2) < 1 mm. (2) Negative for desmin and ER (2) Associated with tuberous sclerosis
(3) No recurrence or metastasis at 168 months’ follow-up

Fang et al. Diagnostic Pathology 2012 7:117   doi:10.1186/1746-1596-7-117

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