BACKGROUND Alveolar soft part sarcoma (ASPS) is a clinically and morphologically distinct malignant soft tissue tumour. It occurs mostly in the lower extremities in adults. The purpose of our study was to describe the related clinicopathologic factors, treatment and prognosis of recurrent orbital ASPS in children. CASE PRESENTATION Three children aged from 1 to 12 years presented with unilateral proptosis, restricted ocular motility and impaired visual acuity of the affected eye. Periodic acid Schiff (PAS) -positive diastase-resistant crystalline granules were found in all cases. Immunostaining of TFE-3, INL1 and Ki67 was positive in the tumour cells of two patients. The time until local recurrence of primary tumor in patient 2 and patient 3, who only underwent tumour resection, was shorter than that of patient 1, who underwent tumour excision and postoperative radiotherapy. The recurrent masses were successfully treated with complete tumour excision followed by adjuvant radiotherapy. Patient 1 presented metastasis at 11 years after radiotherapy. CONCLUSIONS Orbital ASPS in children is easily misdiagnosed due to its rare occurrence and atypical clinical findings. Early diagnosis with multidisciplinary, complete surgical resection combined with adjuvant radiotherapy is essential for achieving long-term disease-free survival in orbital ASPS patients.

中文翻译：

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长期随访中小儿眼眶软组织肉瘤复发：3例报告并复习文献。

背景技术肺泡软部分肉瘤（ASPS）是临床上和形态上不同的恶性软组织肿瘤。它主要发生在成年人的下肢。我们研究的目的是描述儿童复发性眶ASPS的相关临床病理因素，治疗和预后。病例介绍3名1至12岁的儿童表现为单侧眼球突出，眼球运动受限和患眼的视力受损。在所有情况下均发现高碘酸希夫（PAS）阳性的耐扩张酶晶体颗粒。TFE-3，INL1和Ki67的免疫染色在两名患者的肿瘤细胞中呈阳性。仅进行了肿瘤切除的患者2和患者3中直到原发肿瘤局部复发的时间比患者1短。谁做了肿瘤切除和术后放疗。完全切除肿瘤，然后进行辅助放疗，成功治疗了复发的肿块。患者1在放疗后11年出现转移。结论由于其罕见的发生和非典型的临床发现，儿童眼眶ASPS容易被误诊。多学科，完整的手术切除结合辅助放疗的早期诊断对于在眼眶ASPS患者中实现长期无病生存至关重要。结论由于其罕见的发生和非典型的临床发现，儿童眼眶ASPS容易被误诊。多学科，完整的手术切除结合辅助放疗的早期诊断对于在眼眶ASPS患者中实现长期无病生存至关重要。结论由于其罕见的发生和非典型的临床发现，儿童眼眶ASPS容易被误诊。多学科，完整的手术切除结合辅助放疗的早期诊断对于在眼眶ASPS患者中实现长期无病生存至关重要。