The case presented here illustrates a 75 year old female patient who underwent surgical resection of a right adrenal mass of uncertain nature. The ?nal histological diagnosis was consistent with leiomyosarcoma arising from the adrenal anatomic site. Primary leiomyosarcoma of the adrenal gland is a very rare malignant mesenchymal neoplasm: to our knowledge, this is only the twelfth case reported in literature. We describe the clinical course and a brief review of clinical and histological features, biologic behaviour, diagnostic approaches and therapeutic strategies.
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