학술논문

Germ Cell Tumor Presenting with Precocious Puberty in a Girl in United Arab Emirates
Document Type
article
Source
Dubai Medical Journal, Pp 1-5 (2020)
Subject
mixed germ cell tumor
embryonal cancer
yolk sac tumor/endodermal sinus tumor
precocious puberty
multidisciplinary team
Medicine
Language
English
ISSN
2571-726X
Abstract
We herein report a rare case of malignant mixed germ cell tumor comprising both an embryonal carcinoma (60%) and a yolk sac tumor (40%). A 7-year-old girl presented to the emergency department with persistent vaginal bleeding, abdominal pain, and vomiting. A CT scan of the pelvis and abdomen revealed a huge pelvic mass, 16 × 16 × 8 cm, arising from the left ovary and extending into the upper abdomen and displacing bowel loops to the sides. The mass was lobulated and heterogeneously enhancing. Initial investi­gations revealed raised values of beta-human chorionic gonadotrophin (B-hCG) and alpha-fetoprotein (AFP). She underwent a laparotomy and a left salphingo-oophorectomy. Histopathology of the excised mass revealed a malignant mixed germ cell tumor comprising both an embryonal carcinoma (60%) and a yolk sac tumor (40%). Following surgery, she was started on adjuvant chemotherapy based on the MAKEI 96 protocol and was given 4 cycles of PEI chemotherapy (cisplatin, etoposide, and ifosfamide). Multidisciplinary team management consisting of careful initial surgery with adequate staging biopsies followed by combination chemotherapy and endocrinological evaluation and treatment can greatly improve the prognosis of these patients.