Department of Molecular and Cellular Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima 890-8544, Japan.
Diagn Pathol. 2013 Aug 23;8:142. doi: 10.1186/1746-1596-8-142.
Paratesticular liposarcoma is a rare neoplasm, described in single case studies or components of larger studies, as histologically well-differentiated liposarcoma (WDL) and dedifferentiated liposarcoma (DL). However, leiomyosarcomatous differentiation is an extremely rare occurrence in WDL and DL. We report a case of leiomyosarcomatous differentiation in a 77-year-old man. The patient presented with a painless right scrotal mass. Magnetic resonance imaging showed a large mass along the right spermatic cord. The resected mass, measuring 17.5 × 12 × 5 cm, was composed of a high-grade pleomorphic undifferentiated sarcomatous component with necrosis. Atypical smooth muscle differentiation was also detected. Additional tumor sampling revealed the presence of a WDL component. Immunohistochemical analysis of the pleomorphic sarcomatous component showed positive staining for MDM2 and CDK4, and negative staining for alpha smooth muscle actin (αSMA) and desmin. The smooth muscle component was positive for αSMA and desmin, and negative for MDM2 and CDK4. Extension from primary retroperitoneal sarcoma was not proved. We diagnosed of DL with leiomyosarcomatous differentiation.
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副睾脂肪肉瘤是一种罕见的肿瘤,在单病例研究或较大研究的组成部分中描述为组织学上分化良好的脂肪肉瘤 (WDL) 和去分化脂肪肉瘤 (DL)。然而,平滑肌肉瘤分化在 WDL 和 DL 中极为罕见。我们报告一例 77 岁男性的平滑肌肉瘤分化病例。患者表现为无痛性右侧阴囊肿块。磁共振成像显示右侧精索沿线上有一个大肿块。切除的肿块大小为 17.5×12×5cm,由高级别多形性未分化肉瘤成分伴坏死组成。还检测到非典型平滑肌分化。进一步的肿瘤取样显示存在 WDL 成分。多形性肉瘤成分的免疫组织化学分析显示 MDM2 和 CDK4 阳性染色,α 平滑肌肌动蛋白 (αSMA) 和结蛋白阴性染色。平滑肌成分 αSMA 和结蛋白阳性,MDM2 和 CDK4 阴性。未证明原发性腹膜后肉瘤的扩展。我们诊断为具有平滑肌肉瘤分化的 DL。
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