This contribution will cover a variety of uncommon tumors and tumor-like lesions of the ovary that can be associated with a myriad of diagnostic problems for the pathologist. A few of these lesions are not specific to the ovary and may be more common in the uterus or even extraovarian sites, but the mere knowledge that they also occasionally occur in, or involve, the ovary will facilitate their recognition by the pathologist. In contrast, the entity considered first, the highly malignant small cell carcinoma of hypercalcemic type, is an enigmatic tumor unique to the ovary.
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