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首页> 外文期刊>Journal of cutaneous pathology >Cutaneous extrarenal rhabdoid tumor with myogenic differentiation.
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Cutaneous extrarenal rhabdoid tumor with myogenic differentiation.

机译：皮肤性肾外横纹肌样瘤具有肌源性分化。

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BACKGROUND: The rhabdoid phenotype is characterized by large epithelioid cells with abundant eosinophilic cytoplasm and paranuclear inclusions of intermediate filaments. Although originally described in tumors from pediatric kidneys, the rhabdoid phenotype has since been described in a variety of patient ages and extrarenal sites. Extraordinarily, the rhabdoid phenotype has emerged in cutaneous neoplasms, either as a pure extrarenal rhabdoid tumor or a composite phenotype coupled with another malignancy. Regardless of the clinical setting, the rhabdoid phenotype is uniformly associated with aggressive biological behavior. We report the findings from a rare and very aggressive primary extrarenal rhabdoid tumor of the skin with myogenic differentiation. CASE REPORT: A 53-year-old woman presented with an ulcerated nodule on her right medial calf and ipsilateral inguinal lymphadenopathy. Histological examination of the skin nodule as well as cytologic examination of a lymph node disclosed the characteristic rhabdoid phenotype. Electron microscopy demonstrated paranuclear globules of intermediate filaments; special stains suggested an underlying myogenic histogenesis. The patient was aggressively treated with chemotherapy but ultimately died of her disease 8 months after presentation. CONCLUSIONS: The rhabdoid phenotype is rarely seen in cutaneous neoplasms, but when present, portends a poor prognosis adding particular importance to its recognition.
机译：背景：横纹肌瘤表型的特点是上皮样细胞大，嗜酸性细胞质丰富，中间丝有副核包涵体。尽管最初在小儿肾脏的肿瘤中描述过，但此横纹肌瘤表型已在各种患者年龄和肾外部位进行了描述。异常的是，横纹肌瘤表型已在皮肤肿瘤中出现，要么是单纯的肾外横纹肌瘤，要么是复合表型，再加上另一种恶性肿瘤。无论临床情况如何，横纹肌瘤表型均与侵袭性生物学行为一致。我们报告发现与罕见的，侵略性的原发性肾上腺横纹肌样皮肤与肌源性分化的发现。病例报告：一名53岁的女性在右小腿内侧出现溃疡性结节和同侧腹股沟淋巴结病。皮肤结节的组织学检查以及淋巴结的细胞学检查揭示了特征性的横纹肌瘤表型。电子显微镜显示中间丝状的副核小球。特殊的染色提示潜在的肌源性组织发生。该患者接受了化学疗法的积极治疗，但在就诊后8个月最终死于疾病。结论：在皮肤肿瘤中很少见到横纹肌瘤表型，但是当存在时，预后很差，对其识别尤为重要。

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