OBJECTIVE: To describe the clinical and ultrasound characteristics of ovarian pure clear cell carcinoma. METHODS: This was a retrospective study involving data from 11 ultrasound centers. From the International Ovarian Tumor Analysis (IOTA) database, 105 patients who had undergone preoperative ultrasound examination by an experienced ultrasound examiner between 1999 and 2016 were identified with a histologically confirmed pure clear cell carcinoma of the ovary. An additional 47 patients diagnosed with pure clear cell carcinoma between 1999 and 2016 and with available complete preoperative ultrasound reports were identified retrospectively from the databases of the departments of gynecological oncology in the participating centers. The ultrasound images of all tumors were described using IOTA terminology. Clinical and ultrasound characteristics were analyzed for the whole group, and separately, for patients with and those without histologically confirmed endometriosis, and for patients with evidence of tumor developing from endometriosis. RESULTS: Median age of the 152 patients was 53.5 (range, 28-92) years and 92/152 (60.5%) tumors were FIGO Stage I. Most tumors (128/152, 84.2%) were unilateral. On ultrasound examination, all tumors contained solid components and 36/152 (23.7%) were completely solid masses. The median largest diameter of the lesion was 117 (range, 25-310) mm. Papillary projections were present in 58/152 (38.2%) masses and, in most of these (51/56, 91.1%), vascularized papillary projections were seen. Information regarding the presence, site and type of pelvic endometriosis at histology was available for 130/152 patients. Endometriosis was noted in 54 (41.5%) of these. In 24/130 (18.6%) patients, the tumor was judged to have developed from endometriosis. Patients with, compared to those without, evidence of tumor developing from endometriosis were younger (median 47.5 vs 55.0 years, respectively), and ground-glass echogenicity of cyst fluid was more common in pure clear cell cancers developing from endometriosis (10/20 vs 13/79 (50.0% vs 16.5%), respectively). CONCLUSIONS: Ovarian pure clear cell carcinoma is usually diagnosed at an early stage and typically appears as a large unilateral mass with solid components. Patients with clear cell carcinoma developing from endometriosis are younger than other patients with clear cell carcinoma, and clear cell cancers developing from endometriosis more often manifest ground-glass echogenicity of cyst fluid. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Clinic of Obstetrics and Gynecology University of Milan Bicocca San Gerardo Hospital Monza Italy

Department of Development and Regeneration KU Leuven Leuven Belgium

Department of Gynecologic Oncology IRCCS National Cancer Institute Milan Italy

Department of Obstetrics and Gynecology S Orsola Malpighi Hospital University of Bologna Bologna Italy

Department of Obstetrics and Gynecology University Hospitals Leuven Leuven Belgium

Departments of Obstetrics and Gynecology at Karolinska University Hospital Stockholm Sweden

Dipartimento Scienze della Salute della Donna e del Bambino Fondazione Policlinico Universitario A Gemelli IRCCS Rome Italy

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

Institute of Histopathology Catholic University of the Sacred Heart Rome Italy

Istituto di Ginecologia e Ostetricia Università Cattolica del Sacro Cuore Rome Italy

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

Skåne University Hospital Malmö Lund University Malmö Sweden

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ESGO/ISUOG/IOTA/ESGE Consensus Statement on pre-operative diagnosis of ovarian tumors