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Myxoid Leiomyosarcoma of the Uterus: A Clinicopathologic Analysis of 30 Cases and Review of the Literature With Reappraisal of Its Distinction From Other Uterine Myxoid Mesenchymal Neoplasms

发布日期:2017/2/7 0:03:28 文章来源: 作者: 点击次数:1760

 

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Am J Surg Pathol. 2016 Mar;40(3):285-301.

Myxoid Leiomyosarcoma of the Uterus: A Clinicopathologic Analysis of 30 Cases and Review of the Literature With Reappraisal of Its Distinction From Other Uterine Myxoid Mesenchymal Neoplasms

Parra-Herran C1, Schoolmeester JK, Yuan L, Dal Cin P, Fletcher CD, Quade BJ, Nucci MR.

1*Department of Pathology, University of Ottawa and The Ottawa Hospital, Ottawa, ON, Canada †Mayo Clinic, Rochester, MN ‡Brigham and Women's Hospital, Harvard Medical School, Boston, MA.

 

Abstract: Myxoid leiomyosarcoma (mLMS) of the uterus is a rare neoplasm; thus, knowledge of its clinical behavior and morphology remains limited. We therefore reviewed 30 cases initially diagnosed as uterine mLMS to better characterize its clinicopathologic features. Diagnosis was confirmed in 25 subjects (average age 51.5 y), of whom 80% were stage 1 at presentation. The average tumor size was 10.8 cm. An infiltrative tumor border was observed microscopically in 24 cases (96%); the border in 1 case could not be assessed. Fourteen cases (56%) had >10 mitoses per 10 high-power fields, 8 (32%) had between 2 and 10, and 3 cases (12%) had <2 mitoses. Geographic tumor necrosis and moderate to severe nuclear pleomorphism were seen concurrently in 12 cases (48%). All tumors expressed smooth muscle markers. Estrogen receptor was expressed in 29.4% of cases. Eighteen of 21 cases (85.7%) were negative for ALK by immunohistochemistry. Follow-up information was available in 18 subjects: 8 died of disease, 6 were alive with local and/or distant recurrence, and 4 were alive with no evidence of disease. Individuals who died of disease tended to have tumors with >10 mitoses per 10 high-power fields. Among cases with ≥5-year follow-up, overall survival was 11.1%, significantly worse compared with reported survival rates for conventional LMS. The initial diagnosis of mLMS was revised in 5 cases. Four had a distinctive loose myxoid appearance, nuclei with vesicular chromatin, and ALK positivity by immunohistochemistry, suggesting, suggesting inflammatory myofibroblastic tumor. This diagnosis was confirmed by in situ hybridization in 2 cases. One additional myxoid tumor lacked smooth muscle or myofibroblastic features and could not be classified further. mLMS is an aggressive neoplasm characterized by infiltrative tumor borders and variability of other features (mitotic count, atypia, and necrosis). The differential diagnosis includes myxoid leiomyoma and inflammatory myofibroblastic tumor. Attention to distinguishing morphologic features and immunohistochemistry will aid in the interpretation. An illustrated algorithm with criteria for diagnosis is proposed.

 

子宫黏液性平滑肌肉瘤:30个病例临床病理分析及相关文献复习,以及与其他子宫黏液性间叶源性肿瘤的鉴别

摘要:子宫黏液性平滑肌肉瘤(mLMS)是一种少见的肿瘤,因此,关于其临床行为及形态学的知识是非常有限的。本文回顾复习了30例最初诊断为子宫mLMS,为了更好的了解其临床病理特征。其中25例被再次确诊(平均年龄51.5岁),80%病例处于1期。肿瘤的平均大小为10.8cm。在显微镜下可观察到的浸润性边界见于24例(96%);剩余1例的边界无法评估。有14个病例(56%)>10个核分裂/10HPF8例(32%)介于210个核分裂,3例(12%)小于2个核分裂。地图状肿瘤性坏死和中-重度核异型性可同时在12个病例(48%)中观察到。所有的肿瘤表达平滑肌的标记物。ER29.4%病例中表达。21个病例中18例(85.7%)的ALK免疫组化是阴性的。在18个可获取随访信息的病例中:8例死亡,6例带瘤生存,4例无疾病生存。死于疾病的个体倾向于肿瘤细胞>10个核分裂/10HPF。在超过5年随访的病例中,整体生存率仅为11.1%,相对于报道的经典型LMS生存率更差。在最初诊断mLMS的病例有5例被修正,其中4例有独特的疏松的黏液样外观,囊泡状胞核,同时ALK的免疫组化阳性,建议诊断为炎症性肌纤维母细胞肿瘤(IMT),其中2例经过原位杂交证实。剩余1例黏液样肿瘤缺乏平滑肌和肌纤维母细胞特征,而不能进一步分类。mLMS是一种侵袭性的肿瘤,具有浸润性的边界和其他不同的特征(核分裂数,异型性和坏死)。鉴别诊断主要包括黏液性平滑肌瘤和炎症性肌纤维母细胞肿瘤。鉴别主要依靠形态学特征和免疫组化。最后推荐出一个诊断标准的图表。(翻译:杨丽)

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