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

ABSTRACT Objective: Leiomyosarcomas are mesenchymal tumors that very rarely present as a primary adrenal malignancy. Methods: We present the complete clinical presentation, imaging, and anatomicopathologic features of a unique case of recurrent primary adrenal leiomyosarcoma (PAL) after initial surgery. This case is accompanied by an updated literature review. Results: A 63-year-old Hispanic female referred to our clinic 4 months after having right adrenalectomy. Eight months prior to this encounter, she started to notice bilateral lower extremity edema with skin changes. Computed tomography of the abdomen revealed a large 6.8 × 4.4 cm adrenal mass that appeared to be encroaching in the inferior vena cava with hypodense areas, irregular borders, and a necrotic center. A preoperative hormonal evaluation failed to reveal evidence of a functional adrenal tumor. Microscopic examination revealed spindle cell neoplasia. Immunohistochemistry of the surgical specimen revealed positive staining for desmin, smooth muscle actin, and focal myogenin (MYF-4). S-100 protein and inhibin were negative. The diagnosis of intermediate-grade leiomyosarcoma was confirmed. Two months after surgery, surveillance positron emission tomography scan revealed another right adrenal mass measuring 5.6 × 5 cm, with a standardized uptake value of 2.1. Conclusion: PAL is excessively rare. To our knowledge, this is only the second case of PAL with documented recurrence and the 19th case reported in the current medical literature. Surgical removal of the tumor is the first step in treatment. Adjuvant therapies are still not well standardized based on the low incidence of cases. Abbreviations: CT computed tomography PAL primary adrenal leiomyosarcoma SMA smooth muscle actin