罕見病例報告：原發性腎上腺平滑肌肉瘤及其相關文獻回朔
3亞洲大學健康管理學系
Rare care report: primary leiomyosarcoma of the adrenal gland and literature review
3Asia University Healthcare Administration, Taichung, Taiwan
Purpose:
The primary adrenal leiomyosarcoma is quite rare clinically. We report a 61 years old female coming to our hospital for right flank pain and the image revealed a right suprarenal advanced tumor lesion with liver invasion. The pathology revealed the rare tissue type. We would share the experience of the diagnosis, clinical course and a review of the associated literature.
Key Words : adrenal gland , leiomyosarcoma ,
Materials and Methods:
A 61 years old female came to our hospital for right intermittent flank pain. The physical exam just revealed mild Rt knocking pain and no palpated mass or nodule. The song exam disclosed the right suprarenal mass. So the computed tomography (CT) was arranged and one 4 x 5 cm adrenal tumor with liver invasion was suspected. Besides, the associated laboratory exams for endocrine function were normal. Therefore she was admitted for further surgical intervention. The radical adrenalectomy was done through tradition open method. The tumor with invaded hepatic tissue was resected. The patient was recovered soon and discharge 5 days after operation. The histopathological exam revealed the mass as leiomyosarcoma, FNCLCC grade, involving the periadrenal soft tissue and liver tissue. The tumor recurrence was noted through following exam 3 months later.
Results and Discussion
The leiomyosarcomas are not common type cancer and it is around 5-10% of soft tissue sarcomas. The leiomyosarcoma is mainly differentiated from smooth muscle, so it is most noted from uterus, gastrointestinal system, the vascular walls and the skin. Therefore the primary adrenal leiomyosarcoma is more rarely in the past record. In our case, the CT revealed an irregular margin mass, low attenuation on non-enhanced but heterogenous change on enhanced images. The histopathological exam reveal leiomyosarcoma, FNCLCC grade 2, with invasion with periadrenal soft tissue and liver tissue. The IHC stains reports : SMA(+), desmin(+), H-caldesmon(+), S100(-) and CD34(-). The Mitotic Rate is 18/10 high-power fields (HPF). Review of the recently associated literature, only 14 cases of primary leiomyosarcoma of the adrenal gland have been reported. The Kanthan et al. reported only one case in 94 cases of adrenal incidentaloma retrospectively. The etiology is not clear but a few studies have suggested that HIV and Epstein Barr virus may be etiologic roles. The adrenal leiomyosarcoma rarely metastasize to regional lymph nodes, but with metastases most frequently observed in the lungs and liver. The effects of radiation and chemotherapy are limited, so the radical surgical resection is the most effective treatment. In our case, it is advanced condition for liver invasion. The further treatment for local advanced or distal metastatic condition is necessary for more literature analysis.