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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1-TFE3 fusion.
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Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1-TFE3 fusion.

机译：通过特征性ASPCR1-TFE3融合证实了脑膜肺泡软部分肉瘤。

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摘要

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft part sarcoma (ASPS) is a rare but well-studied subtype of sarcoma. A 39-year-old man presented with seizures due to a left temporal meningeal-enhancing lesion with striking brain edema on MRI. The patient underwent neurosurgical resection for suspected meningioma. Histology showed large tumor cells clustering and forming small nests, in places with pseudoalveolar pattern. Diastase-resistant periodic acid-Schiff revealed very rare granular and rod-like cytoplasmic inclusions. Immunohistochemistry showed convincing positivity only with vimentin and smooth muscle actin. The histological features were strongly suggestive of ASPS. At the molecular level RT-PCR and sequencing analysis demonstrated ASPCR1-TFE3 fusion confirming the histological diagnosis of ASPS. There was no evidence of primary extracranial tumor by physical examination and on chest and abdominal CT scan 11 months after presentation. ASPS typically arise from the soft tissues of the extremities and develop multiple metastatic deposits usually with a long clinical course. This case may represent primary meningeal ASPS although metastatic deposit from an undiscovered primary site cannot be entirely excluded.
机译：肉瘤转移到大脑是罕见的，很少作为肿瘤的初始表现发生。肺泡软部分肉瘤（ASPS）是一种罕见但经过充分研究的肉瘤亚型。一名39岁男子因MRI出现左脑颞叶增强病变并伴有明显脑水肿而出现癫痫发作。该患者因怀疑脑膜瘤而接受了神经外科手术切除。组织学显示，在假肺泡模式的地方，大的肿瘤细胞聚集并形成小巢。耐抗消化酶的高碘酸-席夫（Schiff）显示出非常罕见的颗粒状和棒状细胞质内含物。免疫组织化学显示仅波形蛋白和平滑肌肌动蛋白具有令人信服的阳性。组织学特征强烈提示ASPS。在分子水平上，RT-PCR和测序分析表明ASPCR1-TFE3融合证实了ASPS的组织学诊断。入院后11个月，体检以及胸部和腹部CT扫描均未发现原发性颅外肿瘤。 ASPS通常起源于四肢的软组织，通常在漫长的临床过程中会形成多个转移性沉积物。尽管无法完全排除未发现的原发部位的转移性沉积物，但该病例可能代表原发性脑膜ASPS。

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