OBJECTIVE: To describe the clinical and ultrasound characteristics of three types of rare malignant ovarian germ cell tumor: embryonal carcinoma, non-gestational choriocarcinoma and malignant mixed germ cell tumor. METHODS: This was a retrospective multicenter study. From the International Ovarian Tumor Analysis (IOTA) database, we identified patients with a histological diagnosis of ovarian embryonal carcinoma, non-gestational choriocarcinoma or malignant mixed germ cell tumor, who had undergone preoperative ultrasound examination by an experienced ultrasound examiner between 2000 and 2020. Additional patients with the same histology were identified from the databases of the departments of gynecological oncology in the participating centers. All tumors were described using IOTA terminology. Three examiners reviewed all available ultrasound images and described them using pattern recognition. RESULTS: One patient with embryonal carcinoma, five patients with non-gestational ovarian choriocarcinoma and seven patients with ovarian malignant mixed germ cell tumor (six primary tumors and one recurrence) were identified. Seven patients were included in the IOTA studies and six patients were examined outside of the IOTA studies. The median age at diagnosis was 26 (range, 14-77) years. Beta-human chorionic gonadotropin levels were highest in non-gestational choriocarcinomas and alpha-fetoprotein levels were highest in malignant mixed germ cell tumors. Most tumors were International Federation of Gynecology and Obstetrics (FIGO) Stage I (9/12 (75.0%)). All tumors were unilateral, and the median largest diameter was 129 (range, 38-216) mm. Of the tumors, 11/13 (84.6%) were solid and 2/13 (15.4%) were multilocular-solid; 9/13 (69.2%) manifested abundant vascularization on color Doppler examination. Using pattern recognition, the typical ultrasound appearance was a large solid tumor with inhomogeneous echogenicity of the solid tissue and often dispersed cysts which, in most cases, were small and irregular. Some tumors had smooth contours while others had irregular contours. CONCLUSIONS: A unilateral, large solid tumor with inhomogeneous echogenicity of the solid tissue and with dispersed small cystic areas in a young woman should raise the suspicion of a rare malignant germ cell tumor. This suspicion can guide the clinician to test tumor markers specific for malignant germ cell tumors. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Department of Clinical Science and Education Karolinska Institutet and Department of Obstetrics and Gynecology Sodersjukhuset Stockholm Sweden

Department of Clinical Sciences Malmö Lund University Malmö Sweden

Department of Development and Regeneration KU Leuven Leuven Belgium; Department of Obstetrics and Gynecology University Hospitals Leuven Leuven Belgium

Department of Obstetrics and Gynecology IRCCS San Raffaele Scientific Institute Milan Italy

Dipartimento Scienze della Salute della Donna del Bambino e di Sanità Pubblica Fondazione Policlinico Universitario Agostino Gemelli IRCCS Rome Italy

Dipartimento Scienze della Vita e Sanità Pubblica Università Cattolica del Sacro Cuore Rome Italy

Gynecological Oncology Center Department of Obstetrics and Gynecology Charles University Prague Czech Republic

Preventive Gynecology Unit Division of Gynecology European Institute of Oncology IRCCS Milan Italy

Skåne University Hospital Malmö Malmö Sweden

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