Key result
A 62-year-old male with primary adrenal leiomyosarcoma underwent radical adrenalectomy and developed aggressive metastatic disease, remaining alive on chemotherapy 31 months post-surgery.
Case Report (n=1)
Primary adrenal leiomyosarcoma is a rare and aggressive mesenchymal tumor that can present with early metastasis despite surgical resection.
Prolonged survival with chemotherapy possible despite early metastasis in adrenal leiomyosarcoma; leaves open optimal regimens for this rare tumor.
Adrenal leiomyosarcomas are rare mesenchymal tumors of the suprarenal region that are usually diagnosed after they have reached a large size. We report the case of a 62-year-old male with an incidentally found left adrenal mass. Magnetic resonance imaging of the retroperitoneal space showed a heterogeneously enhanced mass, measuring 10x8.2 cm, with characteristics suspicious of malignancy. The patient underwent left radical adrenalectomy after the hormonal evaluation of the tumor due to the high probability of adrenocortical carcinoma. However, microscopic examination of the tumor showed a spindle cell sarcoma. Immunohistochemically the neoplastic cells were found positive for desmin and smooth muscle actin and the diagnosis of a well differentiated adrenal leiomyosarcoma was established. During follow-up the patient presented an aggressive course as he developed bone, liver and pulmonary metastases early postoperatively, which were treated with radiation therapy and chemotherapy. The patient has progressive metastatic disease while on chemotherapy 31 months after surgery.
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Sakellariou et al. (2020) conducted a case report in Primary adrenal leiomyosarcoma (n=1). Left open radical adrenalectomy was evaluated. A 62-year-old male with primary adrenal leiomyosarcoma underwent radical adrenalectomy and developed aggressive metastatic disease, remaining alive on chemotherapy 31 months post-surgery.
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