Primary angiomyolipomas of the ovary which are not associated with genetical systemic diseases, particularly tuberous sclerosis, are extremely rare and are more likely to pose diagnostic challenges. This tumor, particularly of epithelioid variant, is more likely to be malignant and can carry a high possibility of metastasizing. Use of immunohistochemistry consisting of melanocytic biomarkers (HMB45 and Melan A) helps to ascertain their presence.
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