Germ Cell Tumor Presenting with Precocious Puberty in a Girl in United Arab Emirates

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.

Malignant Ovarian Germ Cell Tumors in Pediatric Age Group: A Clinicopathological Study over 21 Years in Eastern Rajasthan (India)

“…A few patients exhibit isosexual precocity (precocious pubertity) because of β-hCG production by tumor cells. [9] Many germ cell tumors posses the unique property of producing biomarkers [5,9]. These markers (if initially raised) are helpful in diagnosis, response to treatment and detection of subclinical recurrent disease in follow up.…”