Persistent mullerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism characterized by the presence of a uterus and fallopian tubes owing to failure of mullerian duct regression, which was shown in a genetically and phenotypically normal male patient. PMDS can occur at any age, preoperative diagnosis of PMDS is quite difficult, as most of them were discovered incidentally during surgery before adolescence. The co-existence of PMDS with seminoma has been rarely reported in the literature. It is necessary for people to grasp knowledge to diagnose this rare case. Therefore, we report a case of PMDS complicated with seminoma. A 28-yew-old-male who was made previous diagnosis of enorchia was found fallopian tubes and uterus during an exploratory laparotomy because of left abdominal pain.

Epithelioid angiomyolipoma (EAML) is a rare renal mesenchymal tumor with malignant potential and is frequently associated with tuberous sclerosis complex (TSC). As metastasis of the tumor cells occur early, EAML is considered a potentially malignant tumor type and intrigues further research on it. Under the microscope, we could find the tumor was composed of atypical polygonal cells sheet mixed with classic angiomyolipoma (AML) components such as blood vessels with notable thick vascular walls, smooth muscle-like cells and adipocytes. Immunohistochemical studies showed that epithelioid cells were focally positive for vimentin, melanocytic markers (HMB-45), myoid markers (α-smooth muscle actin), CD34 and CD68; negative for cytokeratin, epithelial membrane antigen, CD10, and S-100. And the Ki67 index showed approximately 3%. Here, we report the morphological and immunohistochemical features of clinically or histologically malignant renal EAML and discuss its diagnosis, differential diagnosis and the prognosis.