Primary Alveolar Soft-Part Sarcoma of the Liver: Anomalous Presentation of a Rare Disease
Primary Alveolar Soft-Part Sarcoma of the Liver: Anomalous Presentation of a Rare Disease
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DOI：
10.1177/000313480807400109
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发表时间：
2008-01
期刊：
影响因子：
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通讯作者：
K. K. Shaddix-K.;G. P. Fakhre;W. Nields;J. Steers;W. Hewitt;D. Menke
中科院分区：
文献类型：
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作者：
K. K. Shaddix-K.;G. P. Fakhre;W. Nields;J. Steers;W. Hewitt;D. Menke
腺泡状软组织肉瘤是一种血管丰富的软组织肿瘤，均为恶性。它占所有软组织肉瘤的比例不到 1%。肺泡软组织肉瘤患者最常见的年龄为 15 至 35 岁，下肢软组织通常受到影响。在儿科人群中，它最常发生在头部和颈部，尤其影响舌头和眼眶。腺泡状软肉瘤被描述为躯干、上肢和腹膜后的原发性病变；更新奇的部位包括纵隔、女性生殖道、胃、骨头和喉。许多病例报告描述了不同解剖位置的肺泡软肉瘤，但据我们所知，这份报告是肝脏原发性肺泡软肉瘤的第一份文献。我们描述了一位47岁的女性有这样的表现。尽管进行了手术切除和多种化疗方案，该患者仍出现广泛转移，并在诊断后约 2 年后死亡。
Alveolar soft-part sarcoma is a highly vascular soft-tissue tumor that is uniformly malignant. It comprises less than 1 per cent of all soft-tissue sarcomas. Patients with alveolar soft-part sarcoma most frequently are aged 15 to 35 years, and the soft tissues of the lower extremities typically are affected. In the pediatric population, it most frequently occurs in the head and neck and particularly affects the tongue and orbit. Alveolar soft-part sarcoma has been described as a primary lesion in the trunk, upper extremities, and retroperitoneum; more novel locations include the mediastinum, female genital tract, stomach, bone, and larynx. Numerous case reports describe alveolar soft-part sarcoma in diverse anatomic locations, but this report is, to our knowledge, the first documentation of primary alveolar soft-part sarcoma of the liver. We describe a 47-year-old woman with such a manifestation. Despite surgical resection and numerous chemotherapeutic regimens, this patient had widespread metastasis and died approximately 2 years after the diagnosis was established.