Bilateral adrenal leiomyosarcoma treated with multiple local therapies

Abstract

A 62-year-old woman presented with right flank pain, and computed tomography (CT) showed bilateral adrenal tumors (right, 8 cm; left, 4 cm). There were no abnormal findings on physical examination, and adrenal hormone levels in serum and urine were within normal limits. Radiological studies showed a right adrenal tumor with intratumoral hemorrhage, a cystic tumor in the left adrenal gland, and no sign of distant metastasis. Because laboratory tests showed normal levels of adrenal hormones, we considered the tumors to be nonfunctioning adrenal tumors. The right adrenal tumor was surgically removed because it was thought to be malignant, and histological examination revealed that it was a leiomyosarcoma. Postoperative CT showed a pleural metastasis in the right chest wall. The patient received combination chemotherapy with cyclophosphamide, vincristine, adriamycin, and dacarbazine. The metastasis was also treated with radiofrequency ablation (RFA). Because the left adrenal tumor grew rapidly despite two courses of the chemotherapy, it too was surgically removed. After the operation, metastasis in the right iliac bone was treated with RFA and radiotherapy. Positron emission tomography detected bilateral femoral metastases, and these were treated with radiotherapy in combination with a low dose of cisplatin. A liver metastasis and a small metastasis in the left kidney were treated with RFA and a metastasis in the pancreatic tail was removed surgically. Sixteen months after the right adrenalectomy, the patient died due to systemic spread of the disease. Multiple local treatments including RFA, radiotherapy, and resection were effective for the local control of metastatic lesions of adrenal leiomyosarcoma.

Primary Adrenal Leiomyosarcoma

“…Most of the tumors have been unilateral; only 2 patients had bilateral tumors. 12,17 Tumors were larger than 8 cm in diameter in 63.2% of cases; there have only been a few cases where the tumors were 3 cm and presented as a mass in the abdomen or flank. 14 There are no tumor markers or imaging characteristics that allow a preoperative diagnosis, and all cases have been diagnosed after surgical resection or at necropsy.…”

Recurrent Primaty Adrenal Leiopyosarcoma: A Complete Literature Review and Presentation of a Rare Adrenal Tumor

“…The efficacy of chemotherapy is limited, and only a few patients have responded to agents such as gemcitabine and taxanes (11,(13)(14). Hamada et al (17) used the combination of cyclophosphamide, vincristine, Adriamycin, and dacarbazine in a case of bilateral adrenal leiomyosarcomas. On the other hand, radiotherapy alone is reportedly not an effective therapy for adrenal leiomyosarcomas (20).…”

Three uncommon adrenal incidentalomas: a 13-year surgical pathology review

“…Primary mesenchymal adrenal tumors are rare, and more often adrenal leiomyosarcoma is metastatic or an extension from the retroperitoneum [35]. Only 18 cases of primary adrenal leiomyosarcoma were reported in the literature in 2009 [36]. The source of these rare tumors remains unknown, with origin being suggested from the smooth muscle layer of the adrenal blood vessel [37].…”

“…Immunocompromised patients including transplant recipients, those on chemotherapy and those with HIV/AIDS are predisposed to the development of smooth muscle tumors (SMT). They are also reported to have an increased risk for developing adrenal leiomyosarcoma [36]. In this context, Epstein-Barr virus infection has been reported to be involved in the pathobiogenesis of these SMTs [39,40].…”