Lujan Mario G, Hoang Mai P
Department of Pathology, The University of Texas Southwestern Medical Center, Dallas, Tex 75390-9073, USA.
Arch Pathol Lab Med. 2003 Jan;127(1):e32-5. doi: 10.5858/2003-127-e32-PLOTA.
Primary leiomyosarcomas arising in the adrenal gland are exceedingly rare, with only 3 cases reported in the literature. We present the clinical, morphologic, and immunohistochemical features of a pleomorphic leiomyosarcoma, a variant of leiomyosarcoma that has not been described in the adrenal gland. A 63-year-old man presented with a 1-year history of enlarging right upper quadrant mass and pulmonary nodule. A diagnosis of metastatic pulmonary carcinoma to the adrenal gland was rendered on a needle biopsy specimen. Preoperative chemotherapy reduced only the pulmonary mass but not the adrenal mass, which continued to enlarge. Documented by computed tomography and confirmed at surgery, the tumor had completely replaced the right adrenal gland, invading into both the posterior aspect of the right liver and the superior pole of the right kidney. Histologic sections showed a diffuse proliferation of pleomorphic, large, and polygonal neoplastic cells with prominent nucleoli. Many bizarre mitotic figures were present. The neoplastic cells were strongly positive for desmin, calponin, and vimentin. Approximately 80% of the neoplastic cells were positive for the proliferation marker Ki-67. They were negative for smooth muscle actin, muscle-specific actin, myoglobin, myogenin, CD117, cytokeratins, carcinoembryonic antigen, epithelial membrane antigen, chromogranin, CD34, CD31, S100 protein, and HMB-45.
原发于肾上腺的平滑肌肉瘤极为罕见，文献中仅报道过3例。我们报告了1例多形性平滑肌肉瘤的临床、形态学及免疫组化特征，多形性平滑肌肉瘤是平滑肌肉瘤的一种变异型，此前尚未见在肾上腺发生的报道。一名63岁男性，有右上腹肿物及肺结节增大1年的病史。针吸活检标本诊断为肾上腺转移性腺癌。术前化疗仅使肺肿物缩小，肾上腺肿物未缩小且持续增大。经计算机断层扫描证实并在手术中确认，肿瘤已完全取代右肾上腺，侵犯至右肝后侧及右肾上极。组织学切片显示多形性、大的多边形肿瘤细胞弥漫性增生，核仁明显。可见许多怪异的有丝分裂象。肿瘤细胞结蛋白、钙调蛋白及波形蛋白呈强阳性。约80%的肿瘤细胞增殖标记物Ki-67呈阳性。平滑肌肌动蛋白、肌肉特异性肌动蛋白、肌红蛋白、肌细胞生成素、CD117、细胞角蛋白、癌胚抗原、上皮膜抗原、嗜铬粒蛋白、CD34、CD31、S100蛋白及HMB-45均为阴性。
