Shaddix Kyle K, Fakhre G Peter, Nields William W, Steers Jeffery L, Hewitt Winston R, Menke David M
Department of Surgery, Mayo Clinic, Jacksonville, Florida 32224, USA.
Am Surg. 2008 Jan;74(1):43-6.
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.
腺泡状软组织肉瘤是一种血管丰富的软组织肿瘤，具有一致的恶性特征。它占所有软组织肉瘤的比例不到1%。腺泡状软组织肉瘤患者最常见的年龄为15至35岁，下肢软组织通常会受到影响。在儿童群体中，它最常发生在头颈部，尤其影响舌头和眼眶。腺泡状软组织肉瘤已被描述为发生于躯干、上肢和腹膜后的原发性病变；更新颖的发病部位包括纵隔、女性生殖道、胃、骨骼和喉部。众多病例报告描述了腺泡状软组织肉瘤在不同解剖部位的情况，但据我们所知，本报告是肝脏原发性腺泡状软组织肉瘤的首次记录。我们描述了一名有此表现的47岁女性。尽管进行了手术切除并采用了多种化疗方案，但该患者仍发生了广泛转移，并在确诊后约2年死亡。
