Late presentation of retinoblastoma in Malaysia.

AIMS: The aims of this study were to review the presenting features, treatment and outcome for Malaysian children with retinoblastoma currently. SUBJECTS AND METHODS: This was a prospective study, conducted at the General Hospital Kuala Lumpur from August 2001 until October 2007. Clinical data were collected at presentation and follow-up. RESULTS: One hundred five children were diagnosed to have retinoblastoma. There were 55 males and 50 females, ranging in age from 1 month to 14 years (median 20 months). Seventy-six children were Malay in ethnic origin (73%), 14 Chinese (13%), 12 Indian (11%), and other races (3%). Sixty-four children presented with leukocoria (61%), followed by 23 with proptosis (22%), 13 with squint (12%), and 3 with orbital cellulitis (3%). Thirty-three children (31%) deferred treatment for 6 months or more. Overall, 56 children had extraocular disease (55%), 52 at presentation, 4 later. Seventy-one children (68%) underwent primary enucleation, 76 received chemotherapy (72%), and 23 radiotherapy (22%). Fifty-seven children are alive (54%), of whom 3 are blind (5%). Twenty-seven children were lost to follow-up (26%) and 21 have died (20%). CONCLUSION: Retinoblastoma in Malaysia is still characterized by predominantly extraocular disease due to late presentation and high rates of abandonment.

Audit of pediatric hematology-oncology outpatients in Kuala Lumpur.

The aims of this study were to determine the types of cancers and hematological disorders in patients attending a pediatric hematology-oncology clinic. This was a prospective study at the Pediatric Institute, General Hospital Kuala Lumpur, Malaysia from June 2005-November 2006. During the 18-month study, 803 patients attended the clinic, 730 had oncological problems and 73 had hematological problems. The age range was from 2 months to 28 years (median 6 years). The patients were Malay (66%), Chinese (23%), Indian (10%) and other races (1%). Of the oncological patients, 51% had either leukemia (n=293) or lymphoma (n=77). The other most common diagnoses were retinoblastoma, followed by Wilm's tumor and germ cell tumors. Six patients (0.8%) developed a second malignant neoplasm. Of the hematological patients, 60% had platelet disorders, most commonly chronic immune thrombocytopenic purpura. Twenty-four per cent had bone marrow failure and 16% had red cell disorders.

Pediatric cancer deaths: curative or palliative?

AIMS: The aims of this study were to review the deaths of Malaysian pediatric oncology patients in order to determine the major causes and the proportion of patients who received palliative care. SUBJECTS AND METHODS: This was a retrospective review from 2001-2007 of deaths at the Pediatric Institute, General Hospital Kuala Lumpur. Clinical data such as age, gender, disease, cause, and place of death were collected. The patients were divided into two groups: those who received palliative care and those who received curative treatment. RESULTS: Two hundred forty-seven patients were included. There were 148 males and 99 females. The age ranged from 2 months to 22 years (median, 4 years). One hundred thirty cases (53%) were still in the curative phase of treatment at the time of death. The most common cause of death was septicemia (62%), followed by hemorrhage (18%) and underlying cancer (14%). One hundred seventeen cases (47%) were in the palliative phase at the time of death. All palliative care deaths occurred in the oncology ward with one exception. CONCLUSION: Nearly half of all inpatient pediatric cancer deaths were palliative in nature. Septicemia and hemorrhage were the major causes of death in the others.

Chemoreduction for intraocular retinoblastoma in Malaysia.

In the last decade, chemotherapy in combination with focal therapy (chemoreduction) has been increasingly used in intraocular retinoblastoma to avoid enucleation and radiotherapy. The aim of this study was to assess the feasibility and outcome of chemoreduction in Malaysian children with retinoblastoma. This was a prospective study from August 2001 to January 2006. Twenty children (25 eyes) were given 4 cycles of chemoreduction, after which the response was assessed. Fourteen eyes showed a complete response, 10 eyes showed a partial response, and 1 eye had progressive disease. Twelve eyes developed progressive disease later, 9 after an initial complete response and 3 after a partial response. Overall, progressive disease occurred in 52%. There were 2 treatment failures, in Reese-Elsworth groups 3 and 4. Both eyes required enucleation. One eye in group 5 required second line chemotherapy to achieve a complete response. No eyes were irradiated. Five children (25%) defaulted follow-up, one of whom returned with disseminated disease. In conclusion, 4 cycles of chemoreduction achieved a durable complete response in only 12% of eyes. Chemoreduction is feasible in Malaysia but requires good patient compliance and close follow-up.