Pure pediatric ovarian immature teratomas: The French experience

Abstract

Objective To describe characteristics and outcome of pediatric ovarian immature teratomas (IT) to better define the place of chemotherapy. Methods Children with ovarian IT enrolled in TGM95 and TGM2013 studies were analyzed. Norris grading and International Federation of Gynecology and Obstetrics staging system were used. Results Thirty‐six cases were identified with a median age of 11 years (range = 1‐18): 35 of 36 stage I (17 stage IA, 13 stage IC, and 5 stage IX), including seven patients with gliomatosis peritonei (GP), and 1 stage IIIB (IT peritoneal implants). Centrally reviewed Norris grading was performed in 31 cases: 14 grade I and 17 grade II/III tumors. All patients underwent upfront surgery: 19 unilateral oophorectomy, 14 unilateral adnexectomy, 2 unilateral cystectomy, and 1 bilateral cystectomy. No extensive GP surgery was performed. Six patients received adjuvant vinblastin, bleomycin, and cisplatinum because of tumor rupture (n = 5, including two patients with GP) or stage III (n = 1). After a median follow‐up of 39.5 months (range = 6‐238), two events occurred 10 and 11 months after diagnosis: one bilateralization (initial stage IX, grade I) and one IT peritoneal relapse (initial stage IA, grade II), respectively. Both were successfully rescued by platinum‐based chemotherapy and delayed surgery. No stage IC patients treated without adjuvant chemotherapy relapsed (four grade I and three grade III). None of the seven patients with GP progressed. Five‐year event‐free survival and overall survival were 94% (95% CI = 81‐98%) and 100%. Conclusions The current series confirms the excellent prognosis of pediatric ovarian IT, arguing for conservative surgical approach in GP and against systematic adjuvant chemotherapy, even in ruptured tumors.

Surgical management and outcomes for stage 1 malignant ovarian germ cell tumours: A UK multicentre retrospective cohort study

“…There were excellent outcomes in the nine patients undergoing ovarian cystectomy for IT, although notably cystectomy was predominantly performed for small tumours with a low pre-operative AFP level. A retrospective study of 43 patients similarly found excellent long-term outcomes, with no cases of disease recurrence in 14 patients undergoing ovarian cystectomy [12]. However, in this group, 57% of ovarian cystectomies were followed by adjuvant therapy, whereas we found that despite only an 11% rate of adjuvant chemotherapy following cystectomy, there were no recurrences or deaths.…”

Adjuvant chemotherapy does not improve outcome in children with ovarian immature teratoma: A comparative analysis of clinical trial data from the Malignant Germ Cell International Consortium

“…French study of ovarian IT, in which there was no statistically significant difference in EFS and OS based on grade. 8 In a study by Gobel et al, grade was only a risk factor in incompletely resected tumors. 20 Newton et al showed that grade was not a significant risk factor; however, 92% of patients were stage I.…”

“…It therefore appears that grade is not a risk factor in completely resected or stage I tumors, but may be prognostic in incompletely resected and/or higher stage patients. 8,17,20 Finally, due to ongoing controversy regarding optimal clinical management, biological study of this highly underinvestigated tumor type is urgently required. 21 A recent small study of 10 ovarian IT cases (pediatric and adult) using whole-exome sequencing revealed near-diploid genomes with extensive loss of heterozygosity and an absence of mutations or oncogenic variants.…”

“…[1][2][3][4] In pediatric patients treated in the United States and United Kingdom, surgery has been the cornerstone of treatment for ovarian IT, regardless of stage or grade. [5][6][7][8] In order to gain insight into and compare the different management strategies, the Malignant Germ Cell International Consortium (MaGIC) previously compared adult and pediatric clinical trial data from the United States and United Kingdom. 9 One-hundred seventynine patients were included (98 children and 81 adults).…”

Role of Adjuvant Chemotherapy in Stage I Pure Ovarian Immature Teratoma: A Systematic Review and Meta-Analysis

“…Additionally, in pediatric POIT, surveillance in stage I of any grade is recommended and has been proven to be safe as well as to reduce chemotherapy-related adverse events [ 7 , 8 , 9 ]. Recently, a series of studies proposed that active surveillance may be acceptable in stage IA-IC POIT of any grade, regardless of whether patients are pediatric or adult [ 10 , 11 , 12 , 13 , 14 , 15 ]. Nonetheless, most of these studies have evaluated pediatric and adult patients together or included patients with different pathological subtypes of MOGCTs [ 12 , 13 , 15 ].…”