ABSTRACT
Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a recently described potentially lethal but treatable disorder that often occurs as a paraneoplastic manifestation of ovarian teratomas. We report three women with this disorder who presented with subacute onset of delirium, seizures and autonomic instability. Anti-NMDA receptor antibodies were detectable in the serum or cerebrospinal fluid of each patient. Ovarian masses were detected in two patients, and subsequently excised. In the third patient, an empirical bilateral salpingo-oophorectomy was performed and revealed a microscopic neoplasm. All patients experienced slow reversal of the neurological symptoms following surgery and immunotherapy. Our experience suggests that prompt syndrome recognition followed by tumor removal and immunotherapy usually results in neurological recovery.
Subject(s)
Ovarian Neoplasms/pathology , Paraneoplastic Syndromes, Nervous System/physiopathology , Receptors, N-Methyl-D-Aspartate/immunology , Teratoma/pathology , Adult , Autoantibodies/blood , Autoantibodies/cerebrospinal fluid , Female , Humans , Immunotherapy , Magnetic Resonance Imaging , Middle Aged , Ovarian Neoplasms/immunology , Ovariectomy , Paraneoplastic Syndromes, Nervous System/immunology , Paraneoplastic Syndromes, Nervous System/surgery , Salpingostomy , Teratoma/immunology , Tomography, X-Ray Computed , Treatment Outcome , Young AdultABSTRACT
OBJECTIVE: Ten percent of ovarian cancer is attributed to hereditary syndromes, most commonly to mutations in the BRCA1 or BRCA2 genes. These cancers are characterized by a prolonged sensitivity to platinum agents in spite of presentation at advanced stages. We hypothesized that women with BRCA-associated ovarian cancer would also show a high response rate to pegylated liposomal doxorubicin (Doxil). METHODS: A retrospective cohort study was conducted to compare the response rate, progression-free, and overall survival among women with BRCA-associated or sporadic ovarian cancer who were treated with Doxil. RESULTS: A response to Doxil was seen in 13 of 23 patients with BRCA mutations (56.5%; 3 by RECIST criteria and 10 by CA125 levels) compared with only 8 of 41 women with non-hereditary cancers (19.5%; 2 by RECIST criteria and 6 by CA125 levels; p=0.004). This was associated with an improved progression-free and overall survival as measured from the time of Doxil administration. Notably, platinum sensitivity did not directly correlate with a response to Doxil. CONCLUSIONS: Women with BRCA-associated ovarian tumors demonstrate a greater sensitivity to cytotoxic therapy with Doxil than has previously been reported in unselected cases.
