Angioleiomyomas are benign mesenchymal tumours commonly occurring in the subcutis of extremities. They are typically composed of interlacing fascicles of smooth muscle cells with intersecting vascular channels. Angioleiomyomas of the uterus are rare with only very few case reports available in literature. Herein, we report a case of this rare entity in a 47-year-old woman owing to its highly unusual features of cellular atypia, raised CA-125 levels, and pseudo Meigs syndrome.

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A 52-year-old Caucasian woman with Ehlers-Danlos syndrome (EDS) presented for mammography as part of the NHS Breast Screening Programme. A focus of dense calcifications was seen in the left breast. Core biopsy showed stromal calcification. The patient insisted on removal of the abnormal area. The wide local excision showed stromal calcification identical to that seen in the core biopsy. Clinicians should be aware of EDS patients presenting with breast stromal calcifications and this fact should be taken into account when investigating such lesions.