ABSTRACT
Brain metastases are the most common type of brain tumor in adults, commonly arising from primary tumor sites of the lung, breast, skin (melanoma), colon, and kidney. Isolated central nervous system (CNS) metastasis arising from urothelial carcinoma (UC) is a rare presentation yielding a poor prognosis. A 71-year-old male patient with a history of urothelial carcinoma, treated one year prior with partial cystectomy and adjuvant gemcitabine and cisplatin (GC) therapy, presented with worsening neurological symptoms, including progressively worsening dizziness, shuffling gait, drifting, expressive aphasia, and confusion. MRI revealed a left frontal 4.0 x 3.6 cm brightly contrast-enhancing tumor with possible hemorrhage, extensive vasogenic edema, and moderate mass effect. An additional smaller right cerebellar lesion was also noted. Outpatient CT of his chest, abdomen, and pelvis revealed no evidence of other malignant sites. He ultimately underwent a left craniotomy with a total resection of his left frontal mass. Pathological examination revealed a urothelial primary. Post-operative MRI revealed complete resection of the left frontal mass and the patient was discharged with no neurologic deficits on exam. In many cases, brain metastases may present years later following initial therapy of UC as the CNS may act as a sanctuary site during systemic chemotherapy. Chemotherapeutics such as gemcitabine with better penetration of the blood-brain barrier may be beneficial in delaying the onset of these metastases.
ABSTRACT
BACKGROUND: Multifocal rosette-forming glioneuronal tumors (RGNTs) are challenging to manage. Gross total resection is often impossible, and data on adjunctive therapies are limited. We reviewed cases of multifocal RGNTs in the literature with special focus on dissemination patterns and management. METHODS: A literature review was conducted using PubMed and the key words "(multifocal OR multicentric OR satellite OR dissemination) AND glioneuronal." RESULTS: There were 21 cases of multifocal RGNTs identified. Follow-up was available in 18 cases at a median of 17 months. Progression-free survival and overall survival at 1 year were 84% and 94%, respectively. Of all cases, 43% had cerebrospinal fluid (CSF) dissemination, 48% had intraparenchymal spread, and 10% had both. The presence of CSF dissemination led to palliative care and/or death in 20% of cases (n = 2). None of the cases with intraparenchymal spread progressed. Radiotherapy was used in 50% of cases with CSF dissemination, chemotherapy was used in 20%, and CSF shunting was used in 36%. No tumors with intraparenchymal spread required adjunctive therapy or shunting. CONCLUSIONS: RGNTs with CSF dissemination are more likely to behave aggressively, and early adjunctive therapies should be discussed with patients. Tumors with intraparenchymal spread grow slowly, and maximal safe resection followed by observation is likely sufficient in the short term. Long-term behavior of multifocal RGNTs is still unclear.
