Objective: This study determined the clinical characteristics of retinoblastoma (RB) from 1998 to 2008 and compared the epidemiological and clinical patterns with those of the period from 1967 to 2001. Methods: We reviewed the clinical records of 152 patients with RB from 1998 to 2008 in terms of demographic and ophthalmological data and clinical staging or classification. Results: Sixty-three percent of cases were unilateral and 37% were bilateral. Three (3%) of 95 unilateral cases and 7 (12%) of 57 bilateral cases had family history of RB (p = 0.038). The mean age at onset was 17.8 months for unilateral and 7.4 months for bilateral cases, while the mean age at diagnosis was 26.4 months and 13.7 months respectively. The delay from onset to diagnosis was 69% in unilateral and 56% in bilateral RB groups. Financial cost (71.4%) was the leading reason for delay, followed by misdiagnosis (24.5%), and inaccessibility of medical facility (2.0%). The most common manifestations were leukocoria (77%), extraocular findings of orbital mass (9%), and proptosis (6%). Advanced intraocular stage was seen in 63 – 71.6% among those with unilateral and 56 – 60% in those with bilateral tumor. Conclusion The onset of disease had not changed over the years, but patients in general were brought earlier for consultation. Most cases presented in the advanced stage. Decreasing the occurrence of extraocular RB through early consultation and treatment can improve patient survival.
Retinoblastoma ; Epidemiology

Objective: This study reported the demographic profile, clinical presentation, treatment, and outcomes of retinoblastoma (RB) patients seen at Jose R. Reyes Memorial Medical Center (JRRMMC) from January 2011 to December 2020. Methods: This was a single-center, retrospective study. Medical records of patients diagnosed with RB at JRRMMC from January 2011 to December 2020 were reviewed. Descriptive statistics were used to summarize the characteristics of the participants. Results: A total of 31 confirmed RB cases were seen, with almost half residing outside Metro Manila (52%). The median age at diagnosis was 2 years, with a slight male predominance (55%). Majority (81%) of patients had unilateral presentation, with leukocoria as the most common sign (19%) prompting consult. Nearly half (43%) of 37 eyes were diagnosed as Group E using the International Classification of Retinoblastoma (ICRB) system. Majority were in the advanced stage; 57% of eyes underwent enucleation and 29, 3 and 3% of patients required additional treatments such as chemotherapy, laser, and radiotherapy, respectively. RB was confirmed in 20 eyes (54%) through histopathology. Survival outcomes showed that 4 patients (13%) were alive, with either completed or ongoing treatment, and 19 (61%) did not complete prescribed management or were lost to follow-up. There were 8 (26%) known deaths. Conclusion Majority of cases were unilateral and at an advanced stage needing enucleation which may indicate low levels of awareness and screening efforts. Survival rates are difficult to ascertain due to patients abandoning treatment that may be attributed to limited social service support. The study reflects the 10-year data prior to the establishment of a collaborative, multispecialty RB team in the institution and exposes various areas that need to be addressed to improve clinical outcomes.
Retinoblastoma ; Enucleation ; Chemotherapy ; Epidemiology

The authors reviewed 250 cases of tumors in the eye and its adnexa diagnosed histopathologirally at the Department of Ophthalmology, Seoul National University Hospital during the last 10 years (Jan. 1976 - Dec. 1985). The results were summarized as follows: 1. The sex distribution revealed 130 males (52%) and 120 females (48%). 2. The tumors of the eye and its adnexa involved the lid 73 cases (29.2%), epibulbar tissue 72 cases (28.8%), the orbit 56 cases (22.4%), and intraocula. tissue 49 cases (19.6%) in decreasing order. 3. There were 159 cases (63.6%) of benign tumors and 91 cases (36.4%) of malignant tumors. 4. The most frequent malignant tumor of the eye and its adnexa was retinoblastoma, and the most frequent benign tumor was nevus. 5. Of the intraocular malignant tumors, the incidence of retinoblastoma was impressively higher than that of malignant melanoma in Koreans. 6. Of the primary malignant lid tumors, the relative incidence of squamous cellcarcinoma and sebaceous gland carcinoma to basal cell carcinoma in Koreanswas high in comparison with that of Caucasians.
Carcinoma, Basal Cell/*epidemiology ; Carcinoma, Squamous Cell/*epidemiology ; European Continental Ancestry Group ; Eye Neoplasms/*epidemiology ; Eyelid Neoplasms/*epidemiology ; Female ; Humans ; Korea ; Male ; Melanoma/*epidemiology ; Nevus/*epidemiology ; Orbital Neoplasms/*epidemiology ; Retinoblastoma/*epidemiology

INTRODUCTIONRetinoblastoma is a very rare disease. There were 30 cases of retinoblastoma diagnosed and treated at National University Hospital (NUH).

MATERIALS AND METHODSA retrospective chart review was performed on the medical records of 30 patients who were diagnosed with retinoblastoma between 1995 and 2008 at the Department of Paediatrics, National University Hospital, Singapore.

RESULTSThe median age at diagnosis was 1.6 years (range, 0-5.9) with a median follow-up of 1.8 years (range, 0.1 to 11.6). The median time from presenting signs to the time of diagnosis was 5.2 months (range, 0-25.2). Common presenting signs of retinoblastoma were identified; the most common of which were leukocoria (50.0%), squinting (13.3%), poor vision (10.0%), strabismus (6.6%) and unknown (33.3%). Of the 30 patients, 10 were from Singapore whilst the other 20 patients were from the surrounding countries. Twelve patients had bilateral disease at the time of diagnosis, while 18 had unilateral disease. Staging information was available in 27 patients. Enucleation was performed in 25 of 30 patients. Radiation therapy was given in 3 patients in 1995 (bilateral disease), 2001 (bilateral disease) and 2003 (unilateral disease). At the time of analysis, 19 patients were alive with no evidence of disease. Overall 5-year survival for the cohort was 88.1% [95% confidence interval (CI), 88.0-100] and event-free survival for the whole cohort was 74.2% (95% CI, 55.8-92.6).

CONCLUSIONIn our limited experience, the importance of collaboration and standardisation of the staging system, raising awareness and education of primary healthcare providers and parents are strongly stressed.

Child ; Child, Preschool ; Confidence Intervals ; Eye Enucleation ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Pupil Disorders ; diagnosis ; epidemiology ; Retinal Neoplasms ; diagnosis ; epidemiology ; mortality ; surgery ; Retinoblastoma ; diagnosis ; epidemiology ; mortality ; surgery ; Retrospective Studies ; Singapore ; epidemiology ; Strabismus ; Survival Analysis ; Vision Disorders

PURPOSE: This study was carried to examine the temporal trend and geographical distribution of the childhood cancers in Taegu and Kyungpook province and to postulate an etiological hypothesis for development of the childhood cancer. METHODS: A total of 799 childhood cancer patients whose addresses were either Taegu or Kyungpook province were diagnosed at 5 major hospitals in Taegu from January 1982 to December 1996. The types, sexes, years, and frequencies of the childhood cancer and regional distributions were analyzed, based on the hospital records of these patients. RESULTS: The most common childhood cancer was leukemia that accounted for 49.2% of all childhood cancer cases and it was followed by CNS tumor (12.3%), lymphoma (8.4%), neuroblastoma (7.4%), Wilms tumor (3.9%), retinoblastoma (3.4%), rhabdomyo sarcoma (2.7%), bone tumor (2.4%), embryonal carcinoma (1.9%), hepatoblastoma (1.3%) and others (7.1%). Male to female ratio of the cases was 1.5:1. The changes of the annual incidence rates over 15 years in Taegu and Kyungpook area were not consistently increasing but rather variable. Cancer incidence rate of Taegu was significantly higher than that of Kyungpook province (P<0.005). The incidence rates of industrialized cities around Taegu were significantly higher than those of agricultural regions of northern Kyungpook (P<0.05). CONCLUSION: Geographical difference in cancer incidence rate suggested that certain environmental factors may be associated with the childhood cancer. To identify such factors an analytical epidemiologic study is warranted. For the analytical epidemiologic study, a detailed history of residential area and occupational history of parents should be recorded uniformly for all the new childhood cancer cases.
Carcinoma, Embryonal ; Daegu* ; Epidemiologic Studies ; Epidemiology ; Female ; Gyeongsangbuk-do* ; Hepatoblastoma ; Hospital Records ; Humans ; Incidence ; Leukemia ; Lymphoma ; Male ; Neuroblastoma ; Parents ; Retinoblastoma ; Sarcoma ; Wilms Tumor

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.
Child, Preschool ; DNA, Viral/*analysis ; Eye Infections, Viral/complications/diagnosis/*epidemiology ; Female ; Humans ; In Situ Hybridization ; Incidence ; Infant ; Male ; Papillomaviridae/*genetics ; Papillomavirus Infections/complications/diagnosis/*epidemiology ; Prevalence ; Prognosis ; Republic of Korea/epidemiology ; Retinal Neoplasms/complications/pathology/*virology ; Retinoblastoma/pathology/*virology

OBJECTIVETo investigate the safety of treatment with ophthalmic artery cannulation for intra-arterial chemotherapy (IAC) for children with intraocular retinoblastoma (RB).

METHODIn the RB Treatment Center of General Hospital of Armed Police Forces between January 2009 and September 2011, 42 patients who were diagnosed intraocular RB and treated with ophthalmic artery cannulation for IAC, 8 patients were treated 1 circle, 31 patients were treated 2 circles and 3 patients were treated 3 circles (total, 96 times). Each month had IAC once. The ophthalmic and the whole body evaluations were performed during IAC and after IAC for each circle, the blood cell count, alanine aminotransferase (ALT), serum creatinine (Scr), CK-MB content before and after IAC for 1 circle, 2 circles and 3 circles were determined.

RESULT(1) In 52 eyes of 42 patients, 44 eyes (84.6%) were in remission. (2) Successful IAC was achieved in all cases, no severe side effects occurred during IAC. (3) The main ophthalmic complications were eyelid edema and blepharoptosis after IAC, the incidence for 1 circle was 18% (2/11) and 9% (1/11); for 2 circles was 29% (11/38) and 21% (8/38); for 3 circles was all 100% (3/3). The rare complications were vitreous hemorrhage and heterotropia, the incidence was all 2% (1/42). The incidence of eyelid edema and blepharoptosis had no significant differences for 1 circle IAC compared with 2 circles (P > 0.05); the incidence of eyelid edema and blepharoptosis had significant differences for 3 circles IAC compared with 2 circles and 1 circle (P < 0.01). (4) No fever, septicemia and other systemic toxic effects occurred. (5) ALT of 19% patients (8/42) elevated temporarily and CK-MB of 24% patients (10/42) increased. The blood cell counts, ALT, Scr, and CK-MB content before IAC had no significant differences compared with that at 24 h after IAC for 1 circle, 2 circles and 3 circles (P > 0.05).

CONCLUSIONOphthalmic artery cannulation for IAC is a safe and effective method in treating intraocular stage retinoblastoma.

Antineoplastic Agents, Alkylating ; administration & dosage ; therapeutic use ; Catheterization ; methods ; Child, Preschool ; Female ; Humans ; Infant ; Infusions, Intra-Arterial ; Liver Function Tests ; Male ; Melphalan ; administration & dosage ; therapeutic use ; Neoplasm Staging ; Ophthalmic Artery ; Postoperative Complications ; epidemiology ; Retinal Neoplasms ; drug therapy ; pathology ; Retinoblastoma ; drug therapy ; pathology ; Retrospective Studies ; Treatment Outcome