ECE2009 Poster Presentations Clinical case reports and clinical reports (61 abstracts)
Double primary ovarian malignancies in a patient with bipolar disease complicating Swyer syndrome
Evangelia Zapanti 1 , Athanasia Giakioumi 1 , Konstantinos Terzidis 1 , Nikolaos Thomakos 2 , Alexandros Rodolakis 2 , P Grigori 3 & Maria Alevizaki 1
1Department of Clinical Therapeutics, Alexandra Hospital, Athens University School of Medicine, Athens, Greece; 2First Department of Obstetrics-Gynecology, Alexandra Hospital, Athens University School of Medicine, Athens, Greece; 3Department of Genetics, Alexandra Hospital, Athens, Greece.
Background: Swyer syndrome is a type of pure gonadal dysgenesis 46 XY caryotype in phenotypically female patients. A rare case of Swyer syndrome complicated by dysgerminoma and Retiform Sertoli-Leydig cell’s tumor is reported.
Case presentation: A 20-year-old phenotypically female patient presented to our department with primary amenorrhea. She was 1.68 m and weighed 82 kg (BMI 28). She presented with minimal breast development, sparse axillary and pubic hair and normal female external genitalia. Medical history included bipolar depression with two severe suicidal attempts. Endocrinological evaluation demonstrated hypergonadotropic hypogonadism. Abdominal imaging revealed hypoplastic uterus and streak gonads. Chromosomal analysis was performed and the karyorype proved to be 46 XY. The patient underwent diagnostic laparoscopy; this confirmed the ultrasonographic diagnosis and bilateral gonadectomy was performed.
Results: The histopathologic examination revealed dysgerminoma in the left gonad and Retiform Sertoli-Leydig cells tumor in the right gonad. Surgical staging was performed via bilateral pelvic lymphadenectomy accompanied by omentectomy. Peritoneal random biopsies and histology did not reveal metastatic disease. Two sessions of carboplatin chemotherapy were administered and treatment with estrogen was prescribed. Three months later the patient presented with deterioration of her psychiatric condition with frequent episodes of mania in spite of medical therapy.
Conclusion: Because the occurrence of malignancy in dysgenetic gonads is high, early diagnosis and prophylactic removal of the dysgenetic gonads is essential. Hormonal replacement therapy should be given with caution in patients with underlying bipolar disease because of the possible destabilizing effects of estrogens.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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