Brain metastasis secondary to primary vaginal clear cell adenocarcinoma not associated with diethylstilbestrol: a case report and a review of the literature

Vollständiger Abstract

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Background Cases of primary clear cell adenocarcinoma of the vagina (PCCAV) are infrequently encountered and account for only 5%–10% of all primary vaginal cancers 1. The most common associated risk factor for developing PCCAV is in utero exposure to diethylstilbestrol (DES). Patients with non-DES-related PCCAV are even scarcer and usually have a worse prognosis. PCCAV has been reported to metastasize to the lungs, supraclavicular lymph nodes, and the pelvis, while metastases to the liver, brain, and peritoneum are extremely rare. We present a rare case of a 17-year- old female patient with cerebral metastases from a non-DES-related PCCAV. Case report A 17-year-old female patient presented to the medical emergency unit at the Chris Hani Baragwanath Academic Hospital with a 2-month history of left-sided weakness, complicated by a 2-week history of generalized tonic–clonic seizures, vomiting, and headaches. Three months before this presentation, she had been experiencing menorrhagia associated with foul vaginal discharge, which was unresponsive to treatment received at peripheral hospitals. Further examination by the gynecology team revealed a vaginal mass which was subsequently biopsied. A CT scan was performed as part of her workup, and the results showed a right contrast-enhancing parietal mass with extensive vasogenic edema, causing significant midline shift. Results The histology from the vaginal mass favored a clear cell vaginal adenocarcinoma, while that from the parietal mass was determined to be a metastatic clear cell vaginal adenocarcinoma. Pulmonary metastases were also noted on CT staging. Conclusion Cerebral metastases from non-DES-related vaginal clear cell tumor are extremely rare. Our case adds to the existing body of knowledge, highlights the role of neurosurgery in its management, and demonstrates the need for the development of relevant standard management guidelines.