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Rhabdomyosarcoma in Karachi 1998-2002
Yasmin Bhurgri1, Asif Bhurgri, Rehan Puri
1Karachi Cancer Registry, Karachi.
Asian Pacific Journal of Cancer Prevention : APJCP
|September 18, 2004
Summary
Rhabdomyosarcoma (RMS) in Pakistan shows a higher incidence in males and children, with head and neck being the most common primary site. Early diagnosis and prompt treatment are crucial for improving survival rates in this rare cancer.
Area of Science:
- Oncology
- Epidemiology
- Pediatric Oncology
Background:
- Rhabdomyosarcoma (RMS) is a rare malignancy of striated muscle cells.
- Understanding the epidemiological features of RMS is crucial for public health planning and targeted interventions.
- Pakistan faces challenges in managing rare cancers like RMS due to factors like late presentation.
Purpose of the Study:
- To describe the epidemiological characteristics of rhabdomyosarcoma (RMS) in Pakistan.
- To analyze incidence rates, primary tumor sites, age distribution, and survival patterns of RMS patients in Karachi.
- To identify prognostic indicators and recommend strategies for improving RMS outcomes in the region.
Main Methods:
- Retrospective review of incident RMS cases recorded at the Karachi Cancer Registry from 1998 to 2002.
- Analysis of patient demographics, primary tumor sites, age at diagnosis, and survival data.
- Calculation of incidence rates and survival statistics (mean survival time, 3-year, and 5-year survival).
Main Results:
- A total of 217 RMS cases were identified between 1998 and 2002.
- The incidence rate was higher in males (0.3/100,000) than females (0.2/100,000), with 0.5/100,000 in children under 15.
- Head and neck (28.1% in males, 35.6% in females) and extremities (25.8% in males, 16.9% in females) were the most common primary sites. Approximately 60% of cases were childhood RMS.
- Mean survival time was 1.5 years, with a 5-year survival rate of only 10%.
- Late presentation, advanced disease, and lymph node involvement were identified as poor prognostic indicators.
Conclusions:
- Rhabdomyosarcoma in Pakistan predominantly affects children and young adults, with a higher incidence in males and common primary sites in the head/neck and extremities.
- Survival rates for RMS in Pakistan are low, emphasizing the need for early diagnosis and improved treatment strategies.
- Recommendations include enhanced public health education, training for healthcare professionals, and the establishment of a dedicated RMS support group for monitoring and psychosocial support.