Abstract：
BACKGROUND: Primary uterine alveolar soft part sarcoma (ASPS) is a rare, indolent mesenchymal malignancy with less than 40 patients documented in the literature.
METHODS: We report an example of ASPS in a 61-year-old postmenopausal woman. Macroscopically, the uterus showed multiple nodular masses. Microscopic examination revealed tumor arranged in nests and alveolar pattern. The tumor cells were moderately to markedly pleomorphic, epithelioid to polygonal, with eccentrically placed nuclei, vesicular chromatin, prominent macro-nucleoli, and moderate to abundant eosinophilic cytoplasm. PAS-positive and diastase-resistant intracytoplasmic crystals were also seen in some tumor cells. On immunohistochemistry, the tumor cells showed diffuse positivity for vimentin and nuclear positivity for TFE3, a surrogate marker for ASPS. These were negative for SMA, desmin, CD10, h-caldesmon, cyclin D1, EMA, Melan A, and CD34. SMARCB1 expression was retained. Based on the histopathology and IHC, a final diagnosis of uterine ASPS was rendered.
CONCLUSIONS: Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare tumors. Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare sarcoma in an uncommon site with an unusual age.
METHODS: We report an example of ASPS in a 61-year-old postmenopausal woman. Macroscopically, the uterus showed multiple nodular masses. Microscopic examination revealed tumor arranged in nests and alveolar pattern. The tumor cells were moderately to markedly pleomorphic, epithelioid to polygonal, with eccentrically placed nuclei, vesicular chromatin, prominent macro-nucleoli, and moderate to abundant eosinophilic cytoplasm. PAS-positive and diastase-resistant intracytoplasmic crystals were also seen in some tumor cells. On immunohistochemistry, the tumor cells showed diffuse positivity for vimentin and nuclear positivity for TFE3, a surrogate marker for ASPS. These were negative for SMA, desmin, CD10, h-caldesmon, cyclin D1, EMA, Melan A, and CD34. SMARCB1 expression was retained. Based on the histopathology and IHC, a final diagnosis of uterine ASPS was rendered.
CONCLUSIONS: Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare tumors. Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare sarcoma in an uncommon site with an unusual age.
摘要：
背景：原发性肺泡软组织肉瘤（ASPS）是一种罕见的，文献中记录的少于40例患者的惰性间充质恶性肿瘤。
方法：我们报告了一个61岁绝经后妇女ASPS的例子。宏观上,子宫显示多发结节。显微镜检查显示肿瘤呈巢状和肺泡状排列。肿瘤细胞中度到明显多形性，上皮样到多边形，原子核偏心放置，囊泡染色质，突出的宏观核仁，和中等至丰富的嗜酸性细胞浆。在某些肿瘤细胞中也观察到PAS阳性和耐淀粉酶的胞浆内晶体。关于免疫组织化学，肿瘤细胞对波形蛋白呈弥漫性阳性,对ASPS的替代标志物TFE3呈细胞核阳性.这些对SMA是阴性的，desmin,CD10,h-caldesmon,细胞周期蛋白D1，EMA，MelanA,CD34SMARCB1表达得以保留。根据组织病理学和免疫组化，最终诊断为子宫ASPS。
结论：了解特征性组织病理学和免疫组织化学特征有助于准确诊断此类罕见肿瘤。对特征性组织病理学和免疫组织化学特征的了解可以帮助在不寻常的年龄的罕见部位准确诊断这种罕见的肉瘤。
方法：我们报告了一个61岁绝经后妇女ASPS的例子。宏观上,子宫显示多发结节。显微镜检查显示肿瘤呈巢状和肺泡状排列。肿瘤细胞中度到明显多形性，上皮样到多边形，原子核偏心放置，囊泡染色质，突出的宏观核仁，和中等至丰富的嗜酸性细胞浆。在某些肿瘤细胞中也观察到PAS阳性和耐淀粉酶的胞浆内晶体。关于免疫组织化学，肿瘤细胞对波形蛋白呈弥漫性阳性,对ASPS的替代标志物TFE3呈细胞核阳性.这些对SMA是阴性的，desmin,CD10,h-caldesmon,细胞周期蛋白D1，EMA，MelanA,CD34SMARCB1表达得以保留。根据组织病理学和免疫组化，最终诊断为子宫ASPS。
结论：了解特征性组织病理学和免疫组织化学特征有助于准确诊断此类罕见肿瘤。对特征性组织病理学和免疫组织化学特征的了解可以帮助在不寻常的年龄的罕见部位准确诊断这种罕见的肉瘤。
