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Hôpital Tenon Paris 75020, France
Hôpital Tenon Paris 75020, France
Hôtel Dieu de Paris Paris, France
Germ cell tumors of the ovary include all neoplasms derived from primordial germ cells of the embryonal gonad [1]. Five percent of germ cell tumors are malignant, representing 35% of all ovarian carcinomas. To our knowledge, we report the first case of pure primary ovarian choriocarcinoma in which preoperative diagnosis was based on imaging and biologic findings.
A 38-year-old nulliparous woman presented with a 13-week history of amenorrhea. A pelvic mass was found at physical examination. The plasma human chorionic gonadotropin (HCG) level was markedly elevated (2,460,000 mIU/mL).
Pelvic sonography showed a left-sided well-defined mixed adnexal mass (Fig. 1A). Intrauterine and ectopic pregnancy were ruled out.
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Unenhanced CT revealed a left laterouterine mass without fat. A large vascularized ovarian pedicle and irregular oversized arterial vessels were seen at the periphery of the mass during the arterial phase of dynamic CT (Fig. 1B). Significant contrast material uptake was observed in the peripheral solid portion of the mass. Abdominopelvic examination showed no extraovarian dissemination.
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Abnormal vessels and small cystic cavities in the irregular peripheral solid portion were displayed on T2-weighted MR images. High-signal-intensity foci in the solid portion and a large central area with high and intermediate signal intensities were suggestive of hemorrhage on T1-weighted images (Fig. 1C). Significant gadolinium uptake in the solid portion was suggestive of a highly vascularized tumor.
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Bilateral adnexectomy and hysterectomy were performed. Pathologic examination confirmed the diagnosis of left ovarian mass with normal left tube, and normal uterus without trophoblastic tissue. Macroscopically, multiple cystic cavities with hemorrhagic content were seen in the peripheral solid portion, and a large central necrotic and hemorrhagic area was found (Fig. 1D). Microscopic examination showed thick, highly vascularized fibrovascular septa. The proliferation was composed of cytotrophoblast and syncytiotrophoblast cells. The patient received postoperative chemotherapy and remains symptom-free 7 years later.
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Pure primary ovarian choriocarcinoma accounts for less than 1% of ovarian tumors [1]. Shiromizu et al. [2] recently studied 467 ovarian germ cell tumors and found only one choriocarcinoma. Choriocarcinoma is an aggressive tumor that may occur during or outside of pregnancy. Gestational choriocarcinoma of the ovary can be primary, associated with ovarian pregnancy, or metastatic, arising from a primary gestational choriocarcinoma in the uterus. Non-gestational choriocarcinoma of the ovary can be pure but is more frequently associated with other germ cell tumors [3].
Sonographic findings ruled out intrauterine and extrauterine pregnancy and revealed a left nonfunctional 10-cm adnexal mass. Images obtained during the arterial phase of dynamic CT were typical of a malignant tumor, although the presence of large vessels restricted to the periphery was unusual for a malignant epithelial tumor [4]. A peripheral irregular solid portion containing small cavities filled with hemorrhagic fluid and a large central portion with hemorrhagic and necrotic changes were suggestive of malignancy on MR imaging.
In summary, the highly vascularized nature of this mass, the presence of multiple cystic cavities in the solid portion, and central hemorrhagic and necrotic changes with high HCG level and an empty uterus are suggestive of ovarian choriocarcinoma.
References
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