Human Papilloma Virus in Retinoblastoma Tissues from Korean Patients

PURPOSE: Recent reports suggest the association of human papilloma virus (HPV) with retinoblastoma. This study was performed to elucidate whether HPV infection is related to retinoblastoma among Koreans. METHODS: A total of 54 cases diagnosed with retinoblastoma were enrolled from Seoul National University Children's Hospital and Seoul Metropolitan Government-Seoul National University Boramae Medical Center. Presence of human papilloma viral DNA was detected by in situ hybridization in formalin-fixed paraffin-embedded retinoblastoma tissues using both probes against high- and low risk HPV types. RESULTS: The mean age at diagnosis was 22.0 months (range, 1.1 to 98.0 months), and the mean age at enucleation was 27.8 months (range, 1.5 to 112.7 months) among the 54 patients with retinoblastoma. HPV was not detected in any of the retinoblastoma samples using either high risk or low risk HPV probes. CONCLUSIONS: Our study, being the first study in the Korean population, proposes that HPV infection may have no causal relationship with retinoblastoma in Koreans.

Retinoblastoma in patients with regressed retinopathy of prematurity.

Retinopathy of prematurity (ROP) is a well-known clinical entity in premature babies. We report two patients (1 and 2) with regressed ROP who later presented with retinoblastoma (RB). To the best of our knowledge, there is only one such report in the literature so far. Two unrelated patients 1 and 2, born at 32 weeks gestation were screened for ROP at 34 weeks gestation. This showed Zone II Stage II ROP which regressed by 38 weeks of gestation on follow-up. Both patients were lost to follow-up by 40 weeks of gestation. They presented at four years of age with white reflex in the eye. Patient 1 was found to have unilateral and patient 2 bilateral RB. The occurrence of RB in these patients with regressed ROP is probably coincidental.

In vivo growth of retinoblastoma in a newborn infant.

Retinoblastoma is a rare malignancy of the retina seen exclusively in children. It is known to cause rapid growth inside the eye and hence treatment should be started as soon as it is diagnosed. We report a case in a five-day-old infant in whom treatment (chemotherapy) was delayed by a month due to high bilirubin levels secondary to physiological jaundice, which gave us the unique opportunity to measure the growth of the tumor over a month. This case emphasizes that immediate treatment is warranted once this rare disease is diagnosed.

Descolamento da retina após tratamento do retinoblastoma / Rhegmatogenous retinal detachment after succesful treatment of retinoblastoma

OBJETIVO: Avaliar a apresentação de descolamento de retina nos pacientes tratados de retinoblastoma no Centro Infantil Dr. Domingos Boldrini - Campinas (SP), Brasil. MÉTODOS: Estudo retrospectivo de 220 pacientes submetidos a tratamento clínico e cirúrgico de retinoblastoma, no período de janeiro de 1978 a dezembro de 2008. RESULTADOS: Encontramos dois pacientes com descolamento de retina após tratamento de retinoblastoma sem atividade tumoral, corrigidos com sucesso pelo método cirúrgico de cerclagem+criopexia. CONCLUSÃO: É possível a correção de descolamento secundário de retina nos pacientes portadores de retinoblastoma, sem atividade tumoral.

PURPOSE: To evaluate the characteristics and evolution of the retinal detachment developed after successful treatment of retinoblastoma. METHODS: A retrospective analysis of 220 consecutive patients treated for retinoblastoma from january 1978 to december 2008 disclosed two patients who developed retinal detachment during the follow-up. RESULTS: Retinal detachment was found in two patients several months after the treatment of retinoblastoma. Both were managed by surgery with restoration of their monocular vision. CONCLUSION: Surgical management of retinal detachment is indicated to preserve visual function and quality of life in patients successfully treated for retinoblastoma.

Verteporfin photodynamic therapy of retinal capillary hemangioblastoma in von Hippel-Lindau disease.

An 18-year-old boy presented to us with bilateral retinal hemangioblastoma and von Hippel-Lindau disease with history of cerebral capillary hemangioblastoma and embryonic cell carcinoma of left testes. The vision in the right eye was already lost with development of secondary closed angle glaucoma, optic atrophy with subsequent development of bullous keratopathy. The multiple retinal angiomatous lesions in the seeing left eye were treated with various modalities like triple freeze thaw cryopexy, focal lasers and transpupillary thermo therapy in multiple sittings over a period of almost 20 years since detection. One particular angiomatous lesion in the left eye was showing resistance to all the above mentioned modalities and was finally successfully treated with verteporfin and photodynamic therapy to achieve complete regression without any post-treatment complication and with a sustained 20/20 vision till a follow-up of 15 months.

Bilateral metastasis to the retina, choroids and optic nerve from breast cancer: a clinicopathological case.

A 39-year-old Asian woman, with a known history of breast cancer, presented with a gradual onset of painless reduction in vision in both eyes. Examination revealed visual acuity of counting fingers in the right eye and light perception in the left. There were subretinal lesions in the posterior poles of both eyes and retinal detachment in the left eye. Later she developed left proptosis and restriction in left ocular movement, most likely to be caused by metastatic extraocular spread. She subsequently died from disseminated metastatic disease. Histopathological examination confirmed tumor cells infiltrating the choroids, retina and optic nerve in both eyes. The tumor cells were arranged in lobular fashion and stained positively with Periodic Acid Schiff, suggesting the primary to be lobular adenocarcinoma type. Choroidal metastatic disease is common but bilateral retinal and optic nerve involvement with extraocular spread from breast cancer is rare.