Bunt A H, Bensinger R E
Ophthalmology. 1981 Dec;88(12):1339-46. doi: 10.1016/s0161-6420(81)34855-6.
An 11-month-old white female with a three-month history of swelling of soft tissue around the right eye presented with a proptotic globe and marked limitation of motility. A CT scan showed a soft-tissue mass in the orbit, which was biopsied and examined by light and electron microscopy. The specimen consisted of large polygonal and cuboidal cells of uniform appearance arranged in pseudo-alveoli around capillaries. The nuclei were pale and ovoid with prominent nucleoli and rare mitoses. The cytoplasm was packed with lipid droplets and the paranuclear region contained clusters of periodic acid-Schiff (PAS)-positive, diastase-resistant granules and needle-shaped crystals, which showed a linear periodicity of 100 nm by electron microscopy. These cytoplasmic crystals, which were diagnostic for alveolar soft-part sarcoma, were also found to be acid phosphatase-positive and, therefore, components of the lysosomal system. A lid-sparing exenteration of the right orbit was performed, and subsequent radiation therapy by cesium implant delivered 24,000 rads to the operative site. The patient is alive at 22 months following initial biopsy with no evidence of tumor recurrence.
一名11个月大的白人女性，右眼周围软组织肿胀3个月，出现眼球突出和明显的活动受限。CT扫描显示眼眶内有一个软组织肿块，对其进行活检并通过光镜和电镜检查。标本由外观均匀的大的多边形和立方形细胞组成，围绕毛细血管排列成假腺泡状。细胞核淡染、呈卵圆形，有明显核仁，罕见有丝分裂。细胞质内充满脂滴，核旁区域有成群的过碘酸希夫（PAS）阳性、抗淀粉酶的颗粒和针状晶体，电镜显示其具有100nm的线性周期。这些细胞质晶体是肺泡软组织肉瘤的诊断依据，还发现其酸性磷酸酶阳性，因此是溶酶体系统的组成成分。对右眼眶进行了保留眼睑的眶内容摘除术，随后通过铯植入进行放射治疗，手术部位接受了24000拉德的辐射剂量。初次活检后22个月，患者仍存活，无肿瘤复发迹象。
