eMJA: Hysterectomy in a phenotypic male with advanced gonadal malignancy and intersex

Notable Cases

Hysterectomy in a phenotypic male with advanced gonadal malignancy and intersex

Jim L Parker, Deborah L Ekman and Lawrence J Hayden

MJA 2009; 190 (11): 644-646

Clinical record

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Discussion

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Acknowledgements

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Competing interests

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Author details

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References

Disorders of sex development (DSD), previously termed intersex, are uncommon, and are usually, but not always, diagnosed at birth. Issues of gender assignment, psychosexual development and the potential for malignant change in a dysgenetic gonad need to be considered. Here, we report a rare presentation of advanced malignancy in an abdominal gonad associated with the formation of a uterus in an adult male with a previously undiagnosed DSD.

Clinical record

A 59-year-old man presented with haematuria and gynaecomastia, and was subsequently found to have a large pelvic mass. Examination of his left breast identified a 3–4 cm mobile mass. Ultrasonography of the breast confirmed a mass, but could not distinguish between glandular tissue and a breast tumour. Computed tomography (CT) of the abdomen and pelvis showed a 10 cm complex pelvic mass that was thought to extend to the seminal vesicles. A core biopsy showed mixed cytological and immunohistochemical features of a possible granulosa cell tumour, but the precise diagnosis was limited by the small amount of material present for examination. A provisional diagnosis of gonadal malignancy in a dysgenetic gonad was considered.

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