Table 2 Early or precursor perivascular epithelioid cell lesions reported in the uterus

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