ObjectiveTo describe the outcome of intraocular tumor resection by partial transscleral sclerouvectomy (PTSU) combined with micro-invasive vitrectomy and reconstruction of the eyeball (MVRE) in Asian patients.Design, Methods and ParticipantsThis retrospective, interventional cohort study included 366 patients who underwent PTSU combined with MVRE for intraocular tumors both in adult and pediatric age groups. The medical records of these patients were reviewed for clinical, operative, and histopathological features.Main Outcome MeasuresGlobe salvage, best corrected visual acuity (BCVA), surgical side effects, tumor control, and tumor-related metastasis and death.ResultsThe mean follow-up duration was 87 months (median, 66; range, 1-303 months). Among the 366 patients, the mean age was 8.5 years (median, 7; range, 1-19 years) in the 37 pediatric patients, and was 43 years (median, 42; range, 20-51) in 329 adult patients. The tumor mainly involved the ciliary body (n=136; 37.2%) and choroid (n=86; 23.5%). The common pathologic diagnosis of the 366 patients was as follows. In the pediatric age group, histopathologic examination revealed positive tumor margins in 37 patients mainly including ciliary body medulloepithelioma (8/37), ciliary body melanocytoma (13/37) and uveal melanoma (5/37). In the adult group, the pathological diagnosis mainly included melanoma (195/329), RPE adenoma (21/329), amelanotic melanoma (13/329), ciliary body adenoma of nonpigmented epithelium (19/329), schwannoma/neurilemmoma (11/329), melanocytoma (24/329), and leiomyoma (9/329). The globe salvage rate was 81.1% in the pediatric age groups (<20 years), and 93.6% in the adult group (≥20 years), respectively. Of the 338 salvaged eyes, final BCVA was 20/20 to 20/40 in 16 (4.7%), 20/40 to 20/80 in 58 (17.2%), 20/80 to 20/200 in 160 (47.3%), and ≤ 20/200 in 104 (30.8%). Early side effects included corneal edema in 28 (7.7%) patients, hyphema in 46 (12.6%), and vitreous hemorrhage in 76 (21%) patients. Postoperative side effects included proliferative vitreoretinopathy (PVR) in 67 (18.3%), late cataract in 42 (11.5%), and glaucoma in 18 (5%) patients. Local tumor recurrence was detected in 20 patients (5.5%) at a mean interval of 23.6 months, including melanoma (n=19) and medulloepithelioma (n=1). Enucleation was necessary in 28 (7.7%) cases owing to recurrence in 15 (53.6%), eye prophylaxis with high-grade malignancy in 5 (17.8%), and blind painful eye in 8 (28.6%) cases. Kaplan-Meier estimated for 5, 10-year metastasis rate and metastasis-related death rate (95%CI) in 213 UM patients were 3.2% (1.4%-7.0%), 6.9% (3.8%-12.3%); and 3.5% (1.6%-7.6%), 7.6% (4.2%-13.5%), respectively.ConclusionsAs a surgically challenging procedure, PTSU combined with MVRE offers several theoretical advantages over enucleation and radiotherapy. It can achieve control of most intraocular tumors, preserve useful vision, and maintain a cosmetically normal eye.
PurposeTo report six Asian adult patients with retinoblastoma (RB).DesignRetrospective and observational small case series.ParticipantsSix patients with a white dome-shaped tumor of the retina were evaluated from May 10, 1995, to September 10, 2021.Main Outcome MeasuresInitial tumor and associated fundus features, pathology, gene mutation, treatment, tumor course on follow-up, and salvage globe outcome.ResultsThe six affected Asian patients consisted of three men and three women. The mean age at the time of diagnosis was 36.5 years (median: 31 years, range: 20-55 years). All patients were unilateral. In all cases, the tumors were white, dome-shaped, with full-thickness retinal involvement, and mushroom-like protrusions into the vitreous cavity. The mean tumor thickness measured by ultrasonography was 4.5 mm (median: 3.2 mm, range: 3.2-6.8 mm). Associated characteristic symptoms included dilated retinal feeding artery and draining vein (100%), surrounding subretinal infiltration (83%), exudative retinal detachment (83%), and vitreous seeds (67%). Local tumor resection was performed in three patients, I-125 plaque brachytherapy combined with transpupillary thermotherapy (TTT) and intravitreous injection of melphalan (combination treatment) in one patient, I-125 plaque brachytherapy in two patients, and enucleation in one (20%) patient. RB1 gene testing was carried out on four patients and pathological diagnosis on five patients. Genetic analysis revealed that the RB1 mutation was a mosaic c.709dupG (p.Glu237GlyfsTer4) duplication in one patient, a mosaic c.763C>T(p.Arg255Ter) mutation in another patient, while the remaining two patients were RB1 negative. At the end of the follow-up, none of the patients had developed tumor-related metastasis or died. The findings were consistent in all patients who had an adequate follow-up. This study focused on this rare lesion to distinguish it from other intraocular white lesions in adults, including choroidal osteoma, vitreoretinal lymphoma, and retinal capillary hemangioma, all of which are different clinical entities.ConclusionIn adults, RB is typically a white, full-thickness retinal mass that is unilateral, often combining with retinal feeding vessels, subretinal infiltration, and vitreous seeds. Genetic studies on adult-onset RB are essential and still require elucidation. Despite RB being a malignant tumor, patient survival was minimally affected.
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