2020 Volume 25 Issue 2 Pages 312-316
An endodermal cyst (EC) is typically a benign tumor that can affect the craniospinal axis. A 43‒year‒old man presented with sensory disturbance in an upper extremity and occipitalgia. Magnetic resonance imaging (MRI) revealed a multicystic tumor at the C2 level with enhancement along the ventral surface of the spinal cord. Blood tests showed an abnormal elevation of serum carbohydrate antigen 19‒9 (CA 19‒9). Systemic positron emission tomography‒computed tomography showed normal findings. We performed a total tumor resection, and the tumor was diagnosed pathologically as EC. The patient sustained double vision, hearing loss, and swallowing difficulty on postoperative day 70. MRI taken on day 86 revealed extensive leptomeningeal dissemination. The serum CA 19‒9 level elevated continuously and finally reached 1,515.0 U/ml. On day 108, the patient died of respiratory failure. The autopsy did not reveal any abnormalities in the abdominal or chest organs. Microscopic examination of the leptomeningeal disseminated foci revealed an adenocarcinoma. Both the surgical and autopsy specimens stained positively for CA 19‒9. Spinal ECs may lead to malignant transformation and leptomeningeal dissemination that causes an abnormal elevation of the serum CA 19‒9 level.