Rare pelvic cancers

Bladder cancer

Rhabdomyosarcomas are most common in paediatric patients. Urothelial cell carcinoma (UCC) is rare.

There is only a limited amount of information about the occurrence and properties of urothelial cell carcinoma in children and adolescents under the age of 20. It occurs predominantly in boys, and can develop as a secondary malignancy after pelvic radiotherapy for another type of tumour (usually sarcoma) or after a haemorrhagic cystitis treated with alkylating agents (cyclophosphamide, ifosfamide). It is usually unifocal, and clinical symptoms involve macroscopic haematuria and dysuria. Its behaviour is rather indolent, and recurrence is less common than in older patients. Radical resection with the aim of preserving the bladder is the choice of treatment.

Cervical and vaginal cancer

Cervical cancer does not occur at a pre-pubescent age, and is rare in pubescent and adolescent girls. Cervical cancers of mesenchymal origin (rhabdomyosarcoma) prevail in childhood and adolescence. The origin is usually unknown in cervical sarcomas, which only occur sporadically. The risk of precancerous lesions and carcinomas increases with age and  sexual activity. Infection with human papilloma virus (HPV) – particularly with high-risk types HPV 16 and HPV 18 – plays a key role. Clinical symptoms might involve bleeding or pelvic pain.

Cervical cancer treatment in adolescent girls depends on both type and extent of the disease. Treatment of sarcomas is comprehensive (surgery, chemotherapy +/– radiotherapy). Location of rhabdomyosarcoma in the area of cervix is considered to be prognostically good.

The prognosis of cervical cancer depends on its stage at the time of diagnosis. The five-year survival rate is almost 95% in localised carcinoma of stage IA, and more than 85% in stage IB. The survival rates are much lower for advanced stages of cervical cancer. The five-year survival rate of metastatic carcinoma is lower than 10%. Cervical cancer is one of few tumours preventable by a vaccine: HPV vaccination is recommended to be applied in adolescent girls before they start sexual relationships.

Non-germinal ovarian cancer

Non-germinal ovarian cancers are rather important in paediatric gynaecological oncology. They can arise from the stroma of ovary (juvenile granulosa cell tumour) or from the ovarian surface epithelium. Ovarian epithelial cancers in adolescents are usually localised at the time of diagnosis, and generally have a lower degree of malignancy and less aggressive behaviour when compared to carcinomas in older women. The five-year survival rates are up to 85%. Tumours of borderline malignancy (borderline ovarian tumours) account for up to 15% of all ovarian cancers, and most of them are diagnosed at stage I. These tumours have an indolent clinical behaviour, and their prognosis is very good: 95% of girls are alive 10 years after diagnosis. Early stage, serous histological type and young age are good prognostic factors. Unilateral adnexectomy is the choice of treatment in young girls. Adjuvant chemotherapy is not indicated, because it has not been proved to improve the overall survival even in the case of advanced cancer.

Invasive ovarian cancers are extremely rare in children and adolescents; their treatment and prognosis is the same as for adult women.