Ovarian Leydig cell tumor is classified as a pure sex cord tumor under sex cord-stromal tumors by current WHO tumor classification. It is a benign steroid cell tumor centered in and predominantly confined to the ovarian hilum. It affects adults between 25 - 84 years of age. The common clinical presentation of the affected patients is androgenic and rarely estrogenic manifestation. Ovarian Leydig cell tumor should not be confused with ovarian Sertoli-Leydig cell tumor which is classified as mixed sex core-stromal tumor.
Ovarian Leydig cell tumor is usually a well-circumscribed solid nodule composed of rounded epithelioid cells in cord, nests, and small sheets in a dense fibrous stroma. The tumor cells show abundant eosinophilic cytoplasm and typically round nuclei with a single prominent nucleolus. Nuclear-free zones of cytoplasm formed by cytoplastic fusion is a common characteristic feature. Cytoplasmic rod-shaped elongated eosinophilic crystals (Reinke crystals), and cytoplasmic lipochrome pigment are often seen. Fibrinoid material within blood vessel walls is also a diagnostic feature. Cytological atypia, necrosis, and mitoses are generally absent. Immunohistochemically, typically positive for inhibin, calretinin, and melan-A.
Feature Summary of Ovarian Leydig cell tumor
Well circumscribe solid nodule in ovarian hilum
The fusion of cytoplasm to form nuclear-free zones is a key feature
Cytoplasmic Reinke crystals and lipochrome pigments.
Fibrinoid material in blood vessels
Typically positive for inhibin, calretinin, and melan-A.
References
Hanley, Krisztina Z, and Marina B Mosunjac. “Practical Review of Ovarian Sex Cord-Stromal Tumors.” Surgical pathology clinics vol. 12,2 (2019): 587-620. doi:10.1016/j.path.2019.02.005 https://pubmed.ncbi.nlm.nih.gov/31097116/
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