Renee A Cowan1, Erin N Haber2, Fabio R Faucz3, Constantine A Stratakis3, Veronica Gomez-Lobo4. 1. Children's National Medical Center, MedStar Washington Hospital Center, Washington, DC. Electronic address: cowanr@mskcc.org. 2. MedStar Georgetown University Hospital, Washington, DC. 3. Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics & Pediatric Endocrinology Inter-Institute Training Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland. 4. Children's National Medical Center, MedStar Washington Hospital Center, Washington, DC.
Abstract
STUDY OBJECTIVE: Mucinous cystadenomas (MCAs) are benign epithelial ovarian tumors that occur rarely in children and adolescents. Because children and adolescents typically have their childbearing years ahead of them, conservative therapy is indicated. However, there is concern that ovarian cystectomy might be associated with significant recurrence risk in patients with MCA. Furthermore, guanine nucleotide binding protein, alpha stimulating (GNAS) gene mutations are associated with McCune-Albright syndrome, which is associated with cystic ovaries. We sought to evaluate the outcomes of children and adolescents with MCA treated conservatively. A subset of patients underwent GNAS gene testing. DESIGN, SETTING, PARTICIPANTS, AND INTERVENTIONS: After institutional board review approval, the pathology database of a large urban children's hospital was queried to identify adolescents with MCA between the years 2008 and 2014. Fourteen patients, aged 8-18 years (median, 14), were identified. A buccal swab for genetic testing was obtained from a subset of consenting patients. MAIN OUTCOME MEASURES: MCA recurrence; ovarian return to normal size; GNAS gene variants. RESULTS: Two patients underwent oophorectomies, and the remaining 12 underwent cystectomies. Follow-up ultrasound examination revealed slow return of ovary to normal size. Of the 10 patients with available follow-up data, there were no recurrences at a median of 225 days from surgery. Four patients consented to a buccal swab for genetic testing, and the GNAS gene was noted to have rare variants in 2 patients. CONCLUSION: This series supports the use of ovary-sparing surgery in the treatment of MCA. Further research exploring possible genetic variants such as the GNAS gene in children and adolescents diagnosed with MCA is warranted.
STUDY OBJECTIVE: Mucinous cystadenomas (MCAs) are benign epithelial ovarian tumors that occur rarely in children and adolescents. Because children and adolescents typically have their childbearing years ahead of them, conservative therapy is indicated. However, there is concern that ovarian cystectomy might be associated with significant recurrence risk in patients with MCA. Furthermore, guanine nucleotide binding protein, alpha stimulating (GNAS) gene mutations are associated with McCune-Albright syndrome, which is associated with cystic ovaries. We sought to evaluate the outcomes of children and adolescents with MCA treated conservatively. A subset of patients underwent GNAS gene testing. DESIGN, SETTING, PARTICIPANTS, AND INTERVENTIONS: After institutional board review approval, the pathology database of a large urban children's hospital was queried to identify adolescents with MCA between the years 2008 and 2014. Fourteen patients, aged 8-18 years (median, 14), were identified. A buccal swab for genetic testing was obtained from a subset of consenting patients. MAIN OUTCOME MEASURES: MCA recurrence; ovarian return to normal size; GNAS gene variants. RESULTS: Two patients underwent oophorectomies, and the remaining 12 underwent cystectomies. Follow-up ultrasound examination revealed slow return of ovary to normal size. Of the 10 patients with available follow-up data, there were no recurrences at a median of 225 days from surgery. Four patients consented to a buccal swab for genetic testing, and the GNAS gene was noted to have rare variants in 2 patients. CONCLUSION: This series supports the use of ovary-sparing surgery in the treatment of MCA. Further research exploring possible genetic variants such as the GNAS gene in children and adolescents diagnosed with MCA is warranted.
Authors: Robert E Bristow; Andrea C Nugent; Marianna L Zahurak; Victor Khouzhami; Harold E Fox Journal: J Adolesc Health Date: 2006-07-10 Impact factor: 5.012