Oliva E, Clement P B, Young R H
Department of Pathology, Harvard Medical School, Boston, Massachusetts, USA.
Adv Anat Pathol. 2000 Sep;7(5):257-81. doi: 10.1097/00125480-200007050-00001.
Endometrial stromal tumors are reviewed with emphasis on their wide morphologic spectrum and problems in differential diagnosis, highlighting issues that have received particular attention in the recent literature. These neoplasms are divided into two major categories--endometrial stromal nodules and endometrial stromal sarcomas--a distinction made on the basis of the lack of significant infiltration at the periphery of the former. The division of endometrial stromal sarcomas into low-grade and high-grade categories has fallen out of favor and the designation endometrial stromal sarcoma is now considered best restricted to neoplasms that were formally referred to as "low-grade" stromal sarcoma. Endometrial sarcomas without recognizable evidence of a definite endometrial stromal phenotype, designated poorly differentiated "endometrial sarcomas," are almost invariably high grade and often resemble the mesenchymal component of a malignant mullerian mixed tumor. Two features of endometrial stromal tumors that may cause confusion are smooth muscle differentiation and epithelial patterns. Cases in the former category often have a characteristic "starburst" pattern of collagen formation. The most common epithelial patterns resemble those seen in ovarian sex-cord stromal tumors. Much less common is endometrioid gland differentiation. Some endometrial stromal tumors have a prominent fibrous or myxoid appearance and the myxoid tumors should be distinguished from myxoid leiomyosarcoma. Other unusual features of endometrial stromal tumors are also discussed. Lesions in the differential diagnosis of uterine endometrial stromal neoplasms include highly cellular leiomyoma, cellular intravenous leiomyomatosis, adenomyosis with sparse glands, metastatic carcinoma, and lymphoma. Endometrial stromal sarcomas at extrauterine sites may be primary or metastatic from a uterine tumor, the latter sometimes being occult and difficult to definitively establish, particularly if there is a history of a remote hysterectomy for "leiomyomas." Endometrial stromal sarcomas of the ovary, whether primary or metastatic, may be difficult to distinguish from ovarian sex-cord stromal tumors. Extragenital endometrial stromal sarcomas may be confused with diverse lesions such as gastrointestinal stromal tumors, hemangiopericytoma, lymphangiomyomatosis, or mesenchymal cystic hamartoma of the lung. Immunohistochemistry may play a role in evaluating these tumors and in some instances establishing the diagnosis although conventional light microscopic analysis suffices in the majority of cases. The unusual tumor, the "uterine tumor resembling an ovarian sex-cord tumor," is also considered in this review as it is almost certainly of endometrial stromal derivation in many cases. These neoplasms may have a striking resemblance to granulosa cell tumors or Sertoli cell tumors, including those with a retiform pattern, and have recently been shown to be frequently inhibin positive.
对子宫内膜间质肿瘤进行综述，重点关注其广泛的形态学谱及鉴别诊断中的问题，突出近期文献中特别受关注的问题。这些肿瘤分为两大类——子宫内膜间质结节和子宫内膜间质肉瘤，前者依据其周边缺乏明显浸润来与后者区分。将子宫内膜间质肉瘤分为低级别和高级别已不再常用，目前认为“子宫内膜间质肉瘤”这一名称最好仅限于过去被称为“低级别”间质肉瘤的肿瘤。没有明确子宫内膜间质表型证据的子宫内膜肉瘤，即所谓低分化“子宫内膜肉瘤”，几乎均为高级别，且常类似于恶性苗勒管混合瘤的间叶成分。子宫内膜间质肿瘤可能造成混淆的两个特征是平滑肌分化和上皮样形态。前一类病例通常有特征性的胶原形成“星芒状”模式。最常见的上皮样形态类似于卵巢性索间质肿瘤所见。子宫内膜样腺体分化则少见得多。一些子宫内膜间质肿瘤有显著的纤维样或黏液样外观，黏液样肿瘤应与黏液样平滑肌肉瘤相鉴别。还讨论了子宫内膜间质肿瘤的其他不寻常特征。子宫子宫内膜间质肿瘤鉴别诊断中的病变包括富于细胞性平滑肌瘤、细胞性静脉内平滑肌瘤病、腺体稀疏的子宫腺肌病、转移癌和淋巴瘤。子宫外部位的子宫内膜间质肉瘤可能是原发性的，也可能是子宫肿瘤转移所致，后者有时隐匿且难以明确诊断，尤其是在有因“平滑肌瘤”行子宫切除术病史的情况下。卵巢的子宫内膜间质肉瘤，无论是原发性还是转移性的，可能难以与卵巢性索间质肿瘤相鉴别。生殖器外的子宫内膜间质肉瘤可能与多种病变混淆，如胃肠道间质肿瘤、血管外皮细胞瘤、淋巴管平滑肌瘤病或肺间叶性囊性错构瘤。免疫组织化学在评估这些肿瘤及某些情况下确立诊断方面可能发挥作用，不过在大多数病例中，传统光镜分析就足够了。本综述还讨论了不寻常的肿瘤“类似卵巢性索肿瘤的子宫肿瘤”，因为在许多情况下它几乎肯定起源于子宫内膜间质。这些肿瘤可能与颗粒细胞瘤或支持细胞瘤有惊人的相似之处，包括那些具有网状模式的肿瘤，并且最近显示其常常抑制素阳性。
