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Immune-Mediated Encephalitis and Virilization in Association with a Mature Cystic Ovarian Teratoma in an Adolescent Girl

2009; Karger Publishers; Volume: 72; Issue: 4 Linguagem: Inglês

10.1159/000236087

1663-2826

Aruna Poduval, Z. Antal, T. Lee, Antonia Bär, Josep Dalmau, Radhika Muzumdar,

Herpesvirus Infections and Treatments

<i>Background:</i> Mature cystic teratomas are the most common form of ovarian tumor in children and adolescents. These tumors are mostly benign and non-secreting. Virilization from an ovarian teratoma is exceptionally rare in pediatrics. Equally rare is the association of ovarian teratomas with auto-immune encephalitis. <i>Methods:</i> We describe the case of a 15-year-old girl with menstrual abnormalities and virilization, who had a past medical history of encephalitis of an unknown etiology 16 months prior to presentation. <i>Results:</i> Endocrine evaluation revealed an elevated serum testosterone and 17-hydroxy progesterone. A large left ovarian tumor was seen on a CT scan. Surgical excision revealed a mature cystic teratoma containing 6 liters of clear fluid with high androgen levels. Antibodies to the N-methyl-<i>D</i>-aspartate receptor of the hippocampus were detected in pre-operatively archived serum, but undetectable 6 months postoperatively. Immunohistochemistry studies on the tumor sections revealed that the antibodies in the patient’s serum reacted with areas of the tumor expressing the N-methyl-<i>D</i>-aspartate receptor. Postoperatively, the patient’s menstrual cycles became regular and her behavioral problems resolved. Her testosterone levels fell precipitously as well. <i>Conclusion:</i> Both virilizing mature cystic teratomas and teratoma-associated encephalitis are extremely rare in the pediatric population. We report on the first instance of these 2 rare entities occurring in the same patient.