Aggressive Intrathecal Adult Granulosa Cell Tumor Causing Cauda Equina Syndrome

Abstract

Granulosa Cell Tumor (GCT) are the most common malignant sex cord stromal tumors and contain both sex cord and stromal components. They account for approximately 3%-5% of all ovarian tumors. GCT are slow growing indolent tumors with a favorable prognosis. However recurrence is associated with high mortality. Metastasis is rare, they present with nonspecific symptoms of abdominal bloating and distention as well as symptoms due to hormonal imbalance due to estrogenic activity of the tumor. There are two classifications of GCT which include Juvenile and adult variants. High recurrence rate is a major factor responsible for mortality associated with these tumors. We report a case of locally aggressive GCT of the abdomen with infiltration into the bony lumbar vertebrae before invading the entire spinal canal resulting in cauda equina syndrome. To our knowledge such an extensive locally aggressive GCT with adjacent bony and intrathecal extension from the abdomen into the thecal sac has not been reported. Given the indolent course of this disease, early diagnosis and treatment are critically important for best outcome.