Nava-Castañeda Ángel, Tovilla-Canales José Luis, Zuazo Francisca, Rodríguez-Cabrera Lourdes, Garnica-Hayashi Lilia
Nepal J Ophthalmol. 2017 Jan;9(18):70-73. doi: 10.3126/nepjoph.v9i1.17538.
Alveolar Soft Part Sarcoma is a rare malignant tumor of uncertain histogenesis, representing 0.5-1% of all soft tissue tumors. It occurs predominantly in head and neck regions, especially the orbit and the tongue, in infants, causing no specific symptoms for an extended period of time.
A four year old girl was brought to consultation because of persistent swelling of her left upper and lower eyelid for the past six months. A 4 mm proptosis was noted on her left eye with significant upwards displacement of the globe. A firm, not pulsating and non tender mass was palpated, and motility examination revealed deficient abduction and infraduction. Visual acuity was 20/40 and 1/200 in the right and left eye, respectively. The computed tomography revealed an extraconal mass on the inferotemporal aspect of the left orbit, with no bony erosion or globe invasion. An excisional biopsy was made, finding a tumor with nests of clusters of large polygonal cells, separated by fibrous septa and a sinusoidal vascular channel. An Alveolar Soft Part Sarcoma was diagnosed. Our patient had a favorable post-operative follow up, and oncologic evaluations have not shown metastasis or local recurrences. Observations and Conclusions: Alveolar Soft Part Sarcoma is a rare malignant tumor of indolent course, but with propensity to distant metastases, making early diagnose and long term follow up necessary.
肺泡软组织肉瘤是一种组织发生不明的罕见恶性肿瘤，占所有软组织肿瘤的0.5 - 1%。它主要发生于婴儿的头颈部区域，尤其是眼眶和舌头，在较长一段时间内不产生特定症状。
一名4岁女童因过去6个月左上、下眼睑持续肿胀前来就诊。左眼有4毫米的眼球突出，眼球明显向上移位。触诊发现一个质地硬、无搏动且无压痛的肿块，活动度检查显示外展和下转功能受限。右眼视力为20/40，左眼视力为1/200。计算机断层扫描显示左眼眶颞下象限有一个眶锥外肿块，无骨质侵蚀或眼球侵犯。进行了切除活检，发现肿瘤由大的多边形细胞巢组成，被纤维间隔和窦状血管通道分隔。诊断为肺泡软组织肉瘤。我们的患者术后随访情况良好，肿瘤学评估未显示转移或局部复发。
肺泡软组织肉瘤是一种病程缓慢的罕见恶性肿瘤，但有远处转移倾向，因此早期诊断和长期随访很有必要。
