类似于卵巢性索肿瘤的子宫肿瘤的多种表型特征：12 例免疫组织化学研究。

Diverse phenotypic profile of uterine tumors resembling ovarian sex cord tumors: an immunohistochemical study of 12 cases.

机构信息

Department of Pathology, C.H.U. Sart-Tilman, Liège, Belgium.

出版信息

Am J Surg Pathol. 2010 Dec;34(12):1749-61. doi: 10.1097/PAS.0b013e3181f8120c.

DOI:10.1097/PAS.0b013e3181f8120c

PMID:21084963

Abstract

BACKGROUND

Uterine tumors resembling ovarian sex cord tumors (UTROSCTs) are rare neoplasms thought to be of putative endometrial stromal origin and solely composed of sex cord elements. Our study aimed to delineate the immunophenotype of these tumors and to verify whether their morphology reflects true sex cord-like differentiation.

DESIGN

Representative paraffin blocks from 12 UTROSCTs were selected after confirmation of the diagnosis. Cords and/or trabeculae were seen in all tumors, whereas tubules, diffuse areas, and a retiform pattern were present in 9, 6, and 2 cases, respectively. Tumors were stained for sex cord (inhibin, calretinin, WT1, and melan-A), epithelial (KL1 and epithelial membrane antigen), and smooth muscle markers (smooth muscle actin, desmin, smooth muscle myosin heavy chain, h-caldesmon, and histone deacetylase-8), CD10, HMB45, S100, and CD117. Intensity and percentage of staining were recorded.

RESULTS

Six out of 12 tumors were positive for sex cord markers (inhibin 3 of 12, calretinin 4 of 12, WT1 4 of 12, and melan-A 3 of 11) with 4 tumors coexpressing more than one marker. Half of the UTROSCTs showed positivity for KL1, with 2 tumors coexpressing epithelial membrane antigen. All but one tumor expressed one or more smooth muscle markers, with smooth muscle actin, desmin and histone deacetylase-8 being most commonly expressed. CD10 was positive in 6 of 12 tumors, CD117 in 4 of 12, and S100 in 2 of 11 tumors, whereas HMB45 was negative in 11 tumors tested.

CONCLUSIONS

UTROSCTs have a diverse immunohistochemical profile often coexpressing sex cord, epithelial, and smooth muscle markers. The expression of smooth muscle markers in these tumors does not imply a smooth muscle origin as endometrial and sex cord stromal tumors are not infrequently positive for these markers. Positivity for sex cord markers supports a true sex cord/steroid phenotype. Although the immunohistochemical profile of these tumors overlaps with that of endometrial stromal tumors with sex cord-like differentiation as well as ovarian sex cord stromal tumors, the origin of UTROSCT remains uncertain.

摘要

背景

类似于卵巢性索肿瘤的子宫肿瘤（UTROSCT）是一种罕见的肿瘤，被认为是潜在的子宫内膜基质来源，仅由性索成分组成。我们的研究旨在描绘这些肿瘤的免疫表型，并验证其形态是否反映真正的索样分化。

设计

在确认诊断后，从 12 例 UTROSCT 中选择了代表性的石蜡块。所有肿瘤均可见索状和/或小梁状结构，而管腔、弥漫区和网状结构分别见于 9、6 和 2 例。肿瘤染色用于检测性索（抑制素、钙视网膜蛋白、WT1 和黑素-A）、上皮（KL1 和上皮膜抗原）和平滑肌标志物（平滑肌肌动蛋白、结蛋白、平滑肌肌球蛋白重链、h-钙调蛋白和组蛋白去乙酰化酶-8）、CD10、HMB45、S100 和 CD117。记录染色的强度和百分比。

结果

12 例肿瘤中有 6 例（抑制素 3/12，钙视网膜蛋白 4/12，WT1 4/12，黑素-A 3/11）为阳性，4 例肿瘤同时表达多种标志物。一半的 UTROSCT 对 KL1 呈阳性，2 例肿瘤同时表达上皮膜抗原。除 1 例外，所有肿瘤均表达一种或多种平滑肌标志物，最常见的是平滑肌肌动蛋白、结蛋白和组蛋白去乙酰化酶-8。6 例肿瘤 CD10 阳性，4 例肿瘤 CD117 阳性，2 例肿瘤 S100 阳性，11 例肿瘤 HMB45 阴性。

结论

UTROSCT 具有多样化的免疫组织化学特征，常同时表达性索、上皮和平滑肌标志物。这些肿瘤中平滑肌标志物的表达并不意味着平滑肌起源，因为子宫内膜和性索基质肿瘤也经常对这些标志物呈阳性。性索标志物的阳性支持真正的性索/类固醇表型。尽管这些肿瘤的免疫组织化学特征与具有性索样分化的子宫内膜基质肿瘤以及卵巢性索基质肿瘤重叠，但 UTROSCT 的起源仍不确定。