Rhabdomyosarcoma in Karachi 1998-2002.

The epidemiological features of rhabdomyosarcoma (RMS), an uncommon malignancy composed of cells with histopathologic features of striated muscle, were studied in Pakistan. Incident RMS cases recorded at the Karachi Cancer Registry during 1998 to 2004 were reviewed and to ensure maximum completeness of data, only those registered between 1998 and 2002 were considered for the present study. Two hundred and seventeen cases were reported to the Karachi Cancer Registry during this five-year period. One hundred and forty eight of the patients (60.4% males; 39.6% females) were residents of Karachi. The crude and standardized annual incidence rates/100,000 were 0.3 for males and 0.2 for females. The incidence was 0.5 in children below 15 years of age. The primary RMS sites in males were head and neck (28.1%), extremities (25.8%), genitourinary (GU) tract (17.9%), trunk (9.0%), orbit (7.9%), and retroperitoneum (3.4%). RMS occurred at other sites in 7.9% of the patients. Corresponding frequencies in females were head and neck (35.6%), extremities (16.9%), GU tract (16.9%), trunk (8.5%), orbit (8.5%) and other sites in 13.6%. Approximately 60% of the cases were childhood RMS and three fourths were below 21 years. The mean age of RMS cases all sites, males, was 18.5 years (95% CI 15.6; 21.4); for childhood RMS, 7.5 years (95% CI 6.0; 9.2); and for adult RMS 34.2 years (95% CI 28.3;40.2). In females, the corresponding figures were 18.2 (95% CI 13.7; 22.7); 6.6 (95% CI 5.0; 8.1) and 33.9 (95% CI 27.5; 40.5), respectively. One hundred cases were retraceable, and the mean survival time, RMS all sites and ages in both genders, was 1.5 years (95% CI 1.1; 1.9). The 5-year survival was 10%, and 3-year survival was 30% whereas 16.7% of the patients died within a year of diagnosis. The indicators of poor prognosis, a late presentation, rapid evolution, advanced disease, tumor burden (tumor size >5.cms) and regional lymph node involvement, are characteristic of RMS in Karachi. Recent advances in RMS multimodality treatment protocols have improved RMS prognosis in patients with limited disease. Pakistan should focus on early diagnosis and prompt treatment of malignancies. This requires health education for the general population to create awareness and training of health professionals at all levels to promote early diagnosis. An RMS group is required, which would monitor the treatment, recurrence, patient education and provide psychosocial support. Cytogenetic studies are advised for a better understanding of biologic differences in RMS cases in this population.

Epidemiology of ocular malignancies in Karachi.

The study was conducted with the objective of examining descriptive epidemiological characteristics of malignant ocular tumours in Karachi (1998-2002). The data for two hundred and forty two ocular malignancies registered at the Karachi Cancer Registry for Karachi Division during a 5-year period, from January 1(st) 1998 to December 31(st) 2002 were analysed. The age standardized incidence rate (ASIR) was 0.5/100,000 in males and 0.4/100,000 in females. The gender ratio (M:F) was 1.3. The mean age was 34.8 years (95% CI 30.1; 39.6) in males and 34.5 years (95% CI 28.0; 40.9) in females. A fourth of the malignancies were childhood tumours. The most common childhood malignancies were retinoblastomas and rhabdomyosarcomas, whereas the most common adult malignancies were conjunctival squamous cell carcinomas and melanomas. Approximately 97.0% of the tumours were histologically confirmed. The majority (62.5%) presented as low-grade (grade 1) lesions, and were localized to the eye (50%) at the time of diagnosis. The annual incidence rates remained stable during this period. The crucial importance of ocular malignancies is the high 5-year survival rates, associated disability following unilateral or bilateral enucleation and the implications as preventable components of Cancer Control Programs This article provides demographic statistics, which could be useful for the foundation, establishment and monitoring of a component of an effective cancer control program, the risk factors of ocular malignancies being well established. It is recommended that public health education to prevent ultraviolet light related ocular malignancies, information on preventative sun protection behavior, legislation for occupation related ocular cancers and genetic counseling for familial retinoblastoma should be essential primary components of all National Cancer Control Programs even in apparently low risk countries. In the long-term perspective, these efforts should further reduce the incidence - meanwhile stabilization of incidence rates could be achieved. Early detection and standardized treatment will reduce the associated morbidity and mortality.