罕見病例報告:原發性腎上腺平滑肌肉瘤癌及相關文獻討論

張君愷、溫聖辰

高雄醫學大學附設中和紀念醫院 泌尿科

Case report: Primary leiomyosarcoma of the adrenal gland and literature review

Chun-Kai Chang, Sheng-Chen Wen

Divisions of Urology, Kaohsiung Medical University Chung-Ho Memorial Hospital, Kaohsiung, Taiwan;

Primary leiomyosarcoma of the adrenal gland is extremely rare non adrenal hormone secreting tumor. Our case was a 48-year-old woman who suffered from left flank soreness and difficult urination for one month, went to local hospital for help. The physical examination didn’t reveal knocking pain or palpated mass. Ultrasonography and computed tomography revealed approximately 9.6 cm enhancing mass in the left suprarenal region. Blood biochemistry with adrenal hormone test showed within normal range. Then she was transferred to our hospital and admitted for surgery. Robotic assisted left adrenalectomy was smoothly done. One episode of fever was noted after operation but soon recovered and then discharge six days after operation. The pathology report showed histologic and immunohistochemical features of leiomyosarcoma with immunoreactive for Desmin, smooth muscle actin, and H-caldesmon. After four months of follow up, no tumor recurrence was noted so far.

Primary leiomyosarcoma of the adrenal gland is a rare tumor which was differentiated from smooth muscle that affects both genders and more common in the older age group but younger age group can also be affected especially patients who have acquired immunodeficiency syndrome and Epstein-Barr virus infection. Review the articles, there are only 30 cases of primary leiomyosarcoma of the adrenal gland have been reported to date. Patient may complain about pain or discomfort over abdomen, flank or loin area. If the tumor is bigger, palpable abdominal mass may also be noted. The blood biochemistry with adrenal hormone test tend to be within normal limit which revealed non adrenal hormone secreting tumor. Radiology imaging survey such as ultrasound, CT scan and MRI scan can be used to demonstrate the position of the adrenal tumor and to evaluate the advanced disease or possible cause of distant metastasis. The mainstay treatment for localized tumor was surgery. Advanced and metastatic tumor will be treated by surgery and chemotherapy. The short-term outcome of localized tumor was good. However, advanced and metastatic tumor even with chemotherapy after radical surgery don’t show good survival outcome. Our case was primary leiomyosarcoma of the adrenal gland without any evidence of invasion into any adjacent tissue or distant metastasis. The radical surgical resection without adjunctive therapies such as radiotherapy and chemotherapy may be effective treatment for localized disease but further literature analysis will be needed.