Klippenstein K A, Wesley R E, Glick A D
Department of Ophthalmology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Ophthalmic Surg Lasers. 1999 Jul-Aug;30(7):579-83.
Patients with the inherited, bilateral form of retinoblastoma have an increased incidence of osteogenic sarcoma such that the mortality from the secondary tumor exceeds that of the initial bilateral retinoblastoma. We report a 29-year-old male survivor of bilateral retinoblastomas originally diagnosed at 8 months of age, whose treatment eventually included bilateral enucleation, bilateral orbital radiation, and systemic chemotherapy. At age 26, a tumor removed from his right maxillary sinus was diagnosed as fibroma. At age 29, he developed an inferior orbital mass that extended into the right maxillary sinus. A biopsy and comparison with the previous maxillary sinus mass revealed both lesions to be leiomyosarcoma. Both light and electron microscopy supported the diagnosis. The patient has survived treatment with orbital exenteration and maxillectomy combined with postoperative radiation to the right orbital-maxillary area. This appears to be the fourth case of leiomyosarcoma in the third decade of life in a male patient with a previously irradiated orbit after enucleation for bilateral retinoblastoma. Leiomyosarcoma appears to be another orbital tumor associated with bilateral retinoblastoma.
患有遗传性双侧视网膜母细胞瘤的患者发生骨肉瘤的几率增加，以至于继发肿瘤导致的死亡率超过了最初的双侧视网膜母细胞瘤。我们报告了一名29岁男性，他8个月大时被诊断为双侧视网膜母细胞瘤，最终接受了双侧眼球摘除术、双侧眼眶放疗和全身化疗。26岁时，从他右上颌窦切除的肿瘤被诊断为纤维瘤。29岁时，他出现了一个延伸至右上颌窦的眶下肿物。活检并与之前的上颌窦肿物对比后发现，这两个病变均为平滑肌肉瘤。光镜和电镜检查均支持该诊断。该患者接受眼眶内容物剜除术和上颌骨切除术，并结合术后对右侧眶上颌区域进行放疗后存活下来。这似乎是第四例在摘除双侧视网膜母细胞瘤眼球后眼眶接受过放疗的男性患者在30岁时发生平滑肌肉瘤的病例。平滑肌肉瘤似乎是另一种与双侧视网膜母细胞瘤相关的眼眶肿瘤。
