Lax S F
Institut für Klinische Pathologie und Molekularpathologie, LKH Graz II, Standort West, Akademisches Lehrkrankenhaus der Medizinischen Universität Graz, Göstinger Straße 22, 8020, Graz, Österreich.
Pathologe. 2019 Feb;40(1):36-45. doi: 10.1007/s00292-019-0567-6.
Benign leiomyomas are the most frequent mesenchymal tumors of the uterus. In contrast, uterine sarcomas are very rare. Leiomyosarcomas are the most frequent sarcomas followed by endometrial stromal sarcomas (ESS). Leiomyosarcomas are characterized by marked nuclear atypia and high mitotic count and may also show tumor cell necrosis and myometrial and vascular invasion. For cases of diagnostic uncertainty, the category of smooth muscle tumor of uncertain malignant potential (STUMP) may be considered but should be rarely used. Besides low-grade ESS and stromal nodules, a category of high-grade ESS was reconsidered by the WHO in 2014. High-grade ESS are characterized by fibromyxoid and round cell histology, myoinvasive growth, and immunoreactivity for cyclin D1 and BCOR and distinct gene fusions involving YWHAE and BCOR, respectively. The very rare undifferentiated uterine sarcomas need to be redefined due to overlap with high-grade ESS. Uterine tumors resembling ovarian sex cord tumors (UTROSCT) rarely behave malignant, but need to be distinguished from endometrial carcinomas. Mixed epithelial and mesenchymal tumors of the uterus are rare with carcinosarcomas occurring more frequently than adenosarcomas. For prognosis of adenosarcomas the recognition of sarcomatous overgrowth is crucial. Carcinosarcomas are histologically heterogeneous although genetically clonal; biologically they are considered as undifferentiated carcinomas. There will be an increasing importance of molecular pathology for the classification of rare and unusual mesenchymal uterine tumors.
良性平滑肌瘤是子宫最常见的间叶性肿瘤。相比之下，子宫肉瘤非常罕见。平滑肌肉瘤是最常见的肉瘤，其次是子宫内膜间质肉瘤（ESS）。平滑肌肉瘤的特征是核异型性明显和有丝分裂计数高，还可能出现肿瘤细胞坏死以及肌层和血管浸润。对于诊断不确定的病例，可考虑不确定恶性潜能的平滑肌肿瘤（STUMP）类别，但应很少使用。除了低级别ESS和间质结节外，世界卫生组织在2014年重新审视了高级别ESS类别。高级别ESS的特征是纤维黏液样和圆形细胞组织学、肌层浸润性生长、对细胞周期蛋白D1和BCOR免疫反应阳性以及分别涉及YWHAE和BCOR的独特基因融合。由于与高级别ESS重叠，非常罕见的未分化子宫肉瘤需要重新定义。子宫性索样肿瘤（UTROSCT）很少表现为恶性，但需要与子宫内膜癌相鉴别。子宫混合性上皮和间叶性肿瘤很少见，癌肉瘤比腺肉瘤更常见。对于腺肉瘤的预后，识别肉瘤过度生长至关重要。癌肉瘤在组织学上具有异质性，尽管在基因上是克隆性的；在生物学上它们被视为未分化癌。分子病理学在罕见和不寻常的子宫间叶性肿瘤分类中将变得越来越重要。
