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Inflammatory myofibroblastic tumor of the uterus: a clinicopathological, immunohistochemical, and molecular analysis of 13 cases highlighting their broad morphologic spectrum.

13例子宫炎性肌纤维母细胞瘤的临床病理、免疫组化和分子分析:重点突出其形态学谱系

Bennett JA,Nardi V,Rouzbahman M,Morales-Oyarvide V,Nielsen GP,Oliva E
阅读:2479 Modern PathologyOct 2017; 30 (10): 1336 - 1512:1489-1503 

Abstract

Inflammatory myofibroblastic tumors of the uterus are rare, and although most have a favorable prognosis, a small subset exhibit extrauterine disease, recur, or cause death. In this study, we evaluated the morphology and immunoprofile of 13 uterine inflammatory myofibroblastic tumors, including four with aggressive behavior. ALK rearrangements were detected by fluorescence in situ hybridization and fusion partners by anchored multiplex assay. Patients ranged from 8 to 63 (mean 39) years and tumors from 2.5 to 20 (mean 7.4) cm. Myxoid, compact, and hyalinized patterns were noted in 13, 12, and 2 tumors, ranging from 1 to 100%, 5 to 99%, and 0 to 5%, respectively. Nuclear atypia was mild in six (46%), moderate in five (38%), and severe in two (15%), with ganglion-like cells in two tumors. Mitoses ranged from 0 to 24 (mean 5) per 10 high-power fields. Inflammation was mild in five (38%), moderate in three (23%), and marked in five (38%), consisting of a lymphoplasmacytic infiltrate that was lymphocyte-predominant in six (46%). Lymphovascular invasion was noted in two (15%) and necrosis in eight (62%). All but one tumor were ALK-positive by immunohistochemistry, with granular cytoplasmic staining in nine (82%). ALK rearrangements (tested in 10) were detected in eight and was absent in one. The remaining tumor showed an isolated green 5' ALK signal. Fusion partners were identified in 10 (77%) and included THBS1 (n=3), IGFBP5 (n=2), DES (n=2), SEC31 (n=1), TPM3 (n=1), and TIMP3 (n=1). Size ≥8 cm was predictive of aggressive behavior (P<0.01), with increased mitoses (≥7 per 10 high-power fields), lymphovascular invasion, and compact-predominance approaching statistical significance. These data show that inflammatory myofibroblastic tumors of the uterus are morphologically heterogenous with frequent ALK expression and a variety of ALK fusion partners. Recognition of this rare mesenchymal neoplasm is crucial as those with aggressive behavior can potentially be treated with tyrosine kinase inhibitors.

摘要

子宫炎性肌纤维母细胞性肿瘤比较罕见,尽管预后良好,但有一小部分可以表现出宫外疾病、复发甚至导致死亡。本研究中,我们对13例子宫炎性肌纤维母细胞性肿瘤的形态和免疫组化特征进行了评估,包括具有侵袭性生物学行为的四例。采用荧光原位杂交和融合荧光原位杂交法检测了ALK重排。病人从8岁到63岁不等(平均39岁),肿瘤从2.5cm到20cm不等(平均7.4cm)。其中粘液样、致密、玻璃样变分别见于13例、12例、2例,其比例分别为1~100%、5~99%、0~5%。6例有轻微的核异型性(46%),5例中度异型性(38%),2例重度异型性(15%),两个肿瘤中有神经节状细胞。核分裂的范围为0~24(平均5)/10个高倍镜。炎症有5例(38%)较轻,3例(23%)中度,5例明显(38%),三例有淋巴细胞为主淋巴-浆细胞浸润(46%)。两例有(15%)淋巴管侵犯,八例有坏死(62%)。除1例肿瘤外,其余免疫组化均呈阳性,9例(82%)呈颗粒状细胞质染色。ALK重排(受检10例)检测到8例,一例缺乏。另一例显示了一个孤立的绿色5,端ALK信号。有10例(77%)中出现融合,包括THBS1(n =3)、IGFBP5(n=2)、DES(n=2)、SEC31(n=1)、TPM3(n=1)、TIMP3(n=1)。大小≥8厘米(P<0.01),核分裂(≥7/10高能量场),淋巴细胞浸润和致密为主的生长模式对于预测侵袭性生物学行为有统计学意义。这些数据表明,子宫炎性肌纤维细胞瘤多见ALK表达、多种形式的ALK融合。识别这种罕见的间充质肿瘤意义重大,因为对其中具有侵袭性行为者可以用酪氨酸激酶抑制剂治疗。

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