Stathopoulos Christina, Gaillard Marie-Claire, Puccinelli Francesco, Maeder Philippe, Hadjistilianou Doris, Beck-Popovic Maja, Munier Francis L
a Department of Ophthalmology , University of Lausanne, Jules-Gonin Eye Hospital, Fondation Asile des aveugles , Lausanne, Switzerland.
b Department of Radiology , Centre Hospitalier Universitaire Vaudois , Lausanne , Switzerland.
Ophthalmic Genet. 2018 Apr;39(2):242-246. doi: 10.1080/13816810.2017.1393826. Epub 2017 Nov 3.
To report the occurrence and management of secondary choroidal infiltration in two retinoblastoma (rb) patients.
Fundus examination and imaging with spectral domain optical coherence tomography (SD-OCT), B-scan ultrasonography (B-scan), and ultrasound biomicroscopy (UBM).
Case 1: A 19-month-old girl with multifocal unilateral group B rb pretreated with intravenous chemotherapy (IVC) was referred for further management. At 3.5 years of age, routine 3-Tesla magnetic resonance imaging (3T-MRI) revealed an asymptomatic pinealoblastoma that underwent resection and adjuvant intensive IVC. Concomitant ophthalmic follow-up revealed a recurrence 8.3 × 2.8 mm at the posterior pole nasally to the optic disc on B-scan, localized within the choroid on SD-OCT and 3T-MRI. With high dose IVC ongoing, total regression of the choroidal mass was confirmed on SD-OCT already after 3 weeks. At 6-month follow-up, choroidal and pineal tumors were in complete remission. Sadly, the child died of intravascular disseminated coagulation-like disease after the 5th IVC. Case 2: A heavily pretreated 20-month-old girl with bilateral rb was referred for persistent vitreous seeding in her remaining eye (OD). Three months after intravitreal chemotherapy and chemothermotherapy, a hemorrhagic mass was observed inferior to the primary tumor. Two weeks later, an underlying peripheral choroidal mass 16 × 6 mm was documented by UBM and confirmed by 3T-MRI. Complete resolution was achieved 3 weeks after combined intra-arterial chemotherapy (IAC) of melphalan-topotecan. No recurrence or metastasis was observed at 34-month follow-up.
Isolated massive choroidal invasion can be treated conservatively with IVC or IAC in selected cases. SD-OCT, UBM, and B-scan ultrasonography are instrumental in the detection and follow-up of choroidal lesions.
报告两例视网膜母细胞瘤（RB）患者继发性脉络膜浸润的发生情况及处理方法。
进行眼底检查，并采用光谱域光学相干断层扫描（SD-OCT）、B超扫描及超声生物显微镜（UBM）成像检查。
病例1：一名19个月大的女童，患有多灶性单侧B组RB，此前接受过静脉化疗（IVC），前来接受进一步治疗。3.5岁时，常规3特斯拉磁共振成像（3T-MRI）显示无症状松果体母细胞瘤，该肿瘤接受了切除术及辅助强化IVC治疗。同时进行的眼科随访发现，B超扫描显示视盘鼻侧后极部有一处8.3×2.8毫米的复发灶，SD-OCT及3T-MRI显示其位于脉络膜内。在持续进行高剂量IVC治疗的情况下，3周后SD-OCT证实脉络膜肿块完全消退。6个月随访时，脉络膜及松果体肿瘤完全缓解。遗憾的是，该患儿在第5次IVC治疗后死于类血管内弥散性凝血疾病。病例2：一名20个月大的女童，双侧RB，此前接受过大量治疗，因患眼（右眼）残留玻璃体种植前来就诊。玻璃体内化疗及温热化疗3个月后，在原发肿瘤下方观察到一个出血性肿块。两周后，UBM记录到一个16×6毫米的周边脉络膜肿块，并经3T-MRI证实。美法仑-拓扑替康联合动脉内化疗（IAC）3周后肿块完全消退。34个月随访时未观察到复发或转移。
在某些病例中，孤立性大量脉络膜浸润可采用IVC或IAC进行保守治疗。SD-OCT、UBM及B超扫描有助于脉络膜病变的检测及随访。
