Pediatric lynch-associated melanoma arising in ovarian teratoma: a case report.
5 affiliations
- 1University Hospitals Cleveland Medical Center - Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, United States of America.
- 2University Hospitals Rainbow Babies and Children's Hospital, United States of America.
- 3University Hospitals Cleveland Medical Center - Dept of Pathology, United States of America.
- 4University Hospitals Cleveland Medical Center - Dept of Radiology, United States of America.
- 5Case Western Reserve University School of Medicine, Cleveland, OH, United States of America.
Abstract
Background
Mature teratomas of the ovary are a benign entity. However, rare secondary malignant transformation of germ cell components may occur and give rise to a teratoma of malignant transformation (TMT). While reports of this pathology have been described in peri- and postmenopausal patients, this represents the first case of teratoma-associated malignant melanoma in a pediatric patient with Lynch syndrome. There is a lack of consensus recommendations regarding management of this rare phenomenon.Case presentation
We present the case of a 12-year-old premenarchal female with a pelvic mass who underwent surgical resection with final pathology consistent with a melanoma in a teratoma of malignant transformation. She was subsequently diagnosed with a germline MSH6 mutation during postoperative evaluation. She received adjuvant ipilimumab and nivolumab for 3 cycles complicated by grade 3 transaminitis responding to medical therapy. An interval laparoscopic ovarian cystectomy was performed on the contralateral ovary due to concern for recurrence, which was negative. The patient remains on single agent nivolumab without evidence of detectable disease almost 18 months after initial diagnosis.Conclusion
Melanoma arising from a teratoma of malignant transformation is an exceedingly rare pathological diagnosis, especially in the pediatric population. Surgical management is the mainstay of treatment with addition of systemic therapy including immunotherapy. Lynch syndrome in a pediatric patient presents additional considerations for future cancer risk and fertility. Finally, while ctDNA may have a role in the detection of recurrence and treatment planning, this remains investigational.
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BibTeX
@article{alcheikh2026pediatric,
title = {{Pediatric lynch-associated melanoma arising in ovarian teratoma: a case report.}},
author = {Rana Alcheikh and Jaime O Boyd and Shahrazad Saab and Pedro Albuquerque and Rachel Egler and Sarah K Lynam},
journal = {Gynecologic oncology reports},
year = {2026},
month = oct,
doi = {10.1016/j.gore.2026.102226},
url = {https://europepmc.org/article/MED/42923311},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Pediatric lynch-associated melanoma arising in ovarian teratoma: a case report.
AU - Rana Alcheikh
AU - Jaime O Boyd
AU - Shahrazad Saab
AU - Pedro Albuquerque
AU - Rachel Egler
AU - Sarah K Lynam
PY - 2026
DA - 2026/10/03
JO - Gynecologic oncology reports
DO - 10.1016/j.gore.2026.102226
UR - https://europepmc.org/article/MED/42923311
AB - Background Mature teratomas of the ovary are a benign entity. However, rare secondary malignant transformation of germ cell components may occur and give rise to a teratoma of malignant transformation (TMT). While reports of this pathology have been described in peri- and postmenopausal patients, this represents the first case of teratoma-associated malignant melanoma in a pediatric patient with Lynch syndrome. There is a lack of consensus recommendations regarding management of this rare phenomenon.Case presentation We present the case of a 12-year-old premenarchal female with a pelvic mass who underwent surgical resection with final pathology consistent with a melanoma in a teratoma of malignant transformation. She was subsequently diagnosed with a germline MSH6 mutation during postoperative evaluation. She received adjuvant ipilimumab and nivolumab for 3 cycles complicated by grade 3 transaminitis responding to medical therapy. An interval laparoscopic ovarian cystectomy was performed on the contralateral ovary due to concern for recurrence, which was negative. The patient remains on single agent nivolumab without evidence of detectable disease almost 18 months after initial diagnosis.Conclusion Melanoma arising from a teratoma of malignant transformation is an exceedingly rare pathological diagnosis, especially in the pediatric population. Surgical management is the mainstay of treatment with addition of systemic therapy including immunotherapy. Lynch syndrome in a pediatric patient presents additional considerations for future cancer risk and fertility. Finally, while ctDNA may have a role in the detection of recurrence and treatment planning, this remains investigational.
SN - 2352-5789
ER -