Paediatric dysgerminoma: Results of three consecutive French germ cell tumours clinical studies (TGM-85/90/95) with late effects study

Paediatric dysgerminoma: Results of three consecutive French germ cell tumours clinical studies (TGM-85/90/95) with late effects study
Dysgerminoma is an ovarian germ cell tumour and a rare type of childhood cancer. Treatment for dysgerminoma consists of surgery followed by adjuvant radiation therapy in early years and adjuvant chemotherapy in more recent years.
In this prospective cohort study located in France, patients ≤ 18 years with ovarian dysgerminoma were treated according to three consecutive treatment protocols: TGM-85, 90 and 95. Treatment consisted of primary unilateral or bilateral oophorectomy followed by a) prophylactic radiation therapy 1985 – 1998) or b) wait-and-see strategy for localized disease or platinum-based chemotherapy for advanced disease (1998 - 2005). Patients were assessed for late effects either by a clinical follow-up visit or by a questionnaire with emphasis on fertility, renal function, and hearing impairment.
This study includes 48 patients who had a median age of 12.8 years (range 7 – 18.7) at diagnosis and a median follow-up time of 14.7 years (range 1.4 – 28.4). Twelve patients had a bilateral oophorectomy.

Follow-up data were available for 27/48 patients (14 clinical visits and 13 questionnaires). 13/17 patients who attempted pregnancy were successful - 5 requiring assisted reproduction technologies. 3/17 had Brock grade I otoxicity and two patients Brock grade IV otoxicity. 24/25 patients assessed had normal Renal function. Five-year EFS was 91.4% (three recurrences, one second neoplasm). One patient developed melanoma 16 years after therapy. Overall survival was 100%.
In this study, dysgerminoma treatment was heterogeneous due to three different treatment protocols and therefore associations between treatment exposure and late effects are difficult to draw. Besides, only half of the patient populations was evaluated for late effects, therefore the prevalence of late effects might be biased. The very small numbers limit generalizability but it may be difficult to recruit larger numbers in this rare disease.
Ovarian dysgerminoma is a rare childhood cancer with an excellent prognosis even in advanced disease. Treatment for dysgerminoma significantly affects fertility, but ototoxicity and renal impairment are rare late effects. The numbers are small in this study and other information on platinum toxicity must be taken into account clinically.