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.

Retinoblastoma in Karachi, Pakistan.

The objective was to assess epidemiologic aspects of retinoblastoma development in Karachi, Pakistan. Incident cases, diagnosed clinically or microscopically and registered at Karachi Cancer Registry (KCR) during 1(st)January 1998 to 31(st) December 2002 were reabstracted, rechecked and reanalyzed for this purpose. One hundred and one cases of retinoblastoma were reported to KCR over the 5 years (1998-2002). Fifty-seven were residents of Karachi, 34 (59.6%) males and 23 (40.4%) females. The gender ratio (M:F) was 1.5. The mean age at diagnosis was 3.96 years (95% CI 2.92; 4.99) and 3.85 years (95% CI 2.72; 4.98) in males and females respectively. The annual crude incidence of retinoblastomas in Karachi was 4.0/100,000 and 2.4/100,000 in children under the age of 5 and 10 years respectively, the corresponding age standardized rates being 5.3/100,000 and 4.8/100,000. The age groups at risk of developing retinoblastoma, associated morbidity and possibility of almost 100% 5-year survival with available treatments, calls for ophthalmologic screening of all infants below 1 year, and high-risk children until the age of 7 years. In order to detect retinoblastoma, as early as possible, health education for parents and health providers, and improved training of ophthalmologists is essential. Genetic testing for siblings and children of retinoblastoma cases and identification of high-risk children would be helpful, but lacks financial feasibility in developing countries at present. Future health care planning should focus on capacity building for neonatal ophthalmologic screening, handling of parents'and children'emotional reactions and opportunities for education, occupational training and cosmetic rehabilitation for surviving retinoblastoma patients.

Incidence of cancer esophagus in Quetta and Karachi, Pakistan.

OBJECTIVE: To study the differences in the incidence of cancer esophagus in Karachi and Quetta, Pakistan. METHODS: Incident cases of cancer esophagus registered from January 1, 1995 to December 31, 2000 were included for Karachi South, and those registered from January 1, 1998 to December 31, 2000 were included for Karachi Division and Quetta. RESULTS: In Quetta, cancer esophagus had age-standardized incidence rate (ASIR) of 25.5/100,000 population in males and 23.4/100,000 population in females, and it was the commonest malignancy in both genders. In comparison, in Karachi South and Karachi Division, cancer esophagus ranked 7th among cancers in males (ASIR 6.2/100,000 and 5.0/100,000 population, respectively) and 5th in females (7.0/100,000 and 4.9/100,000 population, respectively). The ASIR was similar among males and females in all data sets. CONCLUSION: The incidence of cancer esophagus in Quetta is comparable to that in high-incidence regions, whereas the incidence in Karachi is similar to that in moderate-incidence zones. In contrast to other world regions, cancer esophagus was equally common in males and females in Pakistan. The high risk in Quetta warrants investigation for risk factors and a targeted cancer control program.

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.

Cancer of the oral cavity and pharynx in Karachi--identification of potential risk factors.

The objective of the study was to provide an overview of the demographics of cancer of the oral cavity and pharynx in Karachi South (1995-2001), and identify potential risk factors. Cases recorded for Karachi South, at Karachi Cancer Registry during 1(st) January 1995 to 31(st) December 2002 were analysed. For maximum completion of data cancer cases, recorded from 1(st) January 1995 to 31(st) December 2001 were included for final analysis. The age standardized incidence rates per 100000 population (ASIRs) for cancer of the oral cavity (excluding salivary gland) in Karachi South were 17.1 and 16.5 in males and females whereas the ASIRs for cancer of the pharynx (excluding nasopharynx) were 7.1 and 2.4 in males and females, respectively. The oral pharyngeal ratios were 2.4 and 6.9 for males and females and gender ratios (M F) were 1.04 for the oral cavity and 3.0 for the pharynx. The mean ages were 51 years (95% CI 49.6; 52.2) and 56.1 years (95% CI 54.4; 57.8) respectively. Cancer of the oral cavity ranked 2(nd) in Karachi in both genders. Cancer of the pharynx ranked 7(th) in males and 14(th) in females. Approximately 97% of the oral cavity and pharyngeal cancers were histologically confirmed. The majority of the oral (47.1%) and pharyngeal (51.9%) cancer cases presented as grade II lesions, and were discovered at advanced stages. Of the cancers reported during 1995-2001, 60.4% of the oral and 78.1% of the pharyngeal lesions had spread to a distant site at the time of diagnosis. Squamous cell carcinoma comprised 96.5% and 91.8% of the totals. The incidences of these cancers are comparable to the highest risk regions of the world. As distinct from other geographical areas oral cancer is as common in females as in males, which may reflect the pattern of exposure to known risk factors such as betal quid, arecanut and tobacco and the absence of alcohol as a risk factor in both genders. Apergillus contamination of arecanut could also be a risk factor but no confirmation studies or quantification is available. Despite the common risk factors, incidence of pharyngeal cancer is three times higher in men as compared with women. The keys to reducing the incidence and mortality due to oral and pharyngeal cancers are prevention and control, emphasizing cessation of tobacco use and cancer screening. However a targeted cancer and tobacco control program does not presently exist in Pakistan.