891 - Pituitary and pineal germinoma with peritoneal metastasis

Case Summary

A 19-year-old man, known case of HIV infection, came to hospital with progressive headache for 2 months. Physical examination revealed impaired upward gaze and papilledema both eyes. MRI Brain revealed suprasellar and pineal region mass causing obstructive hydrocephalus. MRI whole spine showed leptomeningeal metastasis. Serum HCG was 3.1 mIU/ml, AFP was 1.12 ng/ml and LDH was 365 U/L. He was implanted ventriculoperitoneal shunt to decrease intracranial pressure. CSF HCG was 20.42 mIU/ml. After the operation, his clinical improved. MDT decided to perform craniospinal radiation. After 1 fraction of radiotherapy, his consciousness was deteriorated. CT brain showed seeding lesions in bilateral frontal horns of the lateral ventricle and pericollosal of bilateral frontal lobes and the fourth ventricle. Radiotherapy was withheld and chemotherapy (EP regimen) was commenced on March 2017. His clinical was obviously improved within a week after chemotherapy. After 3 cycles, MRI of the brain showed complete resolution of the intracranial masses. The 4^th cycle of EP regimen was continued and followed by craniospinal radiation which has completed in July 2017. In February 2018, the serum LDH was rising to 880 U/L. He was asymptomatic. MRI of the brain and whole spine wasn’t show residual or recurrence tumor. In May 2018, serum LDH was repeated and increased to 2574 U/L. The serum HCG increased to 12.09 mIU/ml. CT chest and whole abdomen revealed several peritoneal nodules, measured about 1.0-12.7cm. Salvage chemotherapy, TIP regimen, has been commenced from June 2018. The tumor markers were repeated before starting the second cycle. Serum LDH and serum HCG were 238 U/L and less than 0.1 mIU/ml, respectively. Standard treatment of pineal germinoma is craniospinal radiation with or without chemotherapy. This case has shown that in one who is not suitable for radiation, induction treatment with systemic chemotherapy may be considered. Furthermore chemotherapy, EP regimen, also shows excellent blood-brain barrier penetration leading to a good CNS response. Although extracranial metastasis of primary pineal germinoma is rare but is able to be found in one who performs VP shunt via CSF drop metastasis mechanism.

Editorial acknowledgement

Clinical trial identification