Challenges in Treating Patients with Down's Syndrome and Testicular Cancer with Chemotherapy and Radiotherapy: The Royal Marsden Experience

Abstract 

Aims

With a life expectancy similar to the general population, greater numbers of patients with Down's syndrome are being diagnosed with testicular cancer. Learning difficulties and medical co-morbidity are common in this patient population and may lead to non-standard oncological treatment. We aimed to identify and discuss management challenges in the treatment of these patients with chemotherapy and radiotherapy and report their clinical outcome.

Materials and methods

The Royal Marsden Hospital urology database was searched from 1982 to 2005 to identify all cases of patients with Down's syndrome and histologically confirmed testicular cancer who were referred for consideration of chemotherapy or radiotherapy.

Results

Nine patients were identified, of whom eight received chemotherapy or radiotherapy. Two patients had bilateral tumours and four had crypto-orchidism. At the time of diagnosis, the patients were 21–50 years of age. Of the 11 tumours identified, nine were seminomas and two were malignant teratoma undifferentiated. Five patients presented with stage I disease, of whom three received carboplatin and one received para-aortic radiotherapy as adjuvant treatment. Three patients presented with stage II disease, of whom two were treated with carboplatin and one received combination chemotherapy followed by radiotherapy. One patient with stage IV disease was treated with carboplatin. Five of nine patients relapsed within 30 months, of whom three were successfully salvaged with radiotherapy and one with combination chemotherapy.

Conclusion

After standard and non-standard therapy for seminoma, the relapse rate for patients in our cohort was high. Since relapsed disease is much more difficult to manage with combination chemotherapy on account of respiratory, cardiac and renal co-morbidity, adequate initial treatment is advised. Consideration of psycho-social issues and the multiple treatment strategies available is vital in delivering optimal care to patients with Down's syndrome and testicular cancer.

Key words: Clinical relapse, germ cell tumour, radical treatment, trisomy 21