Germinoma in a 16-month old baby: A case report with brief review of the literature

Abstract

A 16-month old Saudi boy was admitted to the Emergency Room (ER) of King Faisal University Hospital; he was drowsy and extremely ataxic and was vomiting. The mother noticed that the child had progressive unsteady gait and was crying from headaches and vomiting for a month prior to his admission to this hospital.On admission, he was found to have marked bilateral papilloedema. The right pupil was slightly more dilated than the left. Both pupils showed sluggish reaction to light. Responses were extensor bilaterally. Emergency CT scan showed an enhancing mass compressing the cerebellum from the right side. The scan did not reveal evidence of tumour elsewhere intracranially. The fourth ventricle was distorted with associated hydrocephalus (Fig. 1 A).Emergency operation was performed on August 3, 1989 for seemingly total removal of a posterior fossa malignant tumour, through suboccipital craniectomy and atlas laminectomy. At operation, the tumour was very adherent to the dura; the dura was dissected. The tumour was dark greyish brown in colour, relatively vascular and unencapsulated. There was a clear line of cleavage between the tumour and the compressed cerebellum. External drainage was left in the lateral ventricle through a separate burr hole. The patient showed marked improvement. Nine days later, VA shunt was inserted. As the patient developed meningitis and ventriculitis, a few days later, the shunt was replaced by external drainage. The patient's condition deteriorated and he became unconscious. He was responding to pain stimuli by flexion withdrawal. He had cardiac arrest twice during two weeks.After the infection subsided, bilateral ventricular shunts were inserted. On 12.9.1989, he became unable to breath and was bradycardic. CT scan showed a large recurrence of the tumour. At an emergency operation done then, the tumour was found infiltrating the cerebellum, vermis, and the brain stem. Only partial removal was possible because of the infiltration of the tumour into the brain stem. A follow-up CT scan done 10 days later showed reduction in the size of the tumour. Two weeks later, the patient was decerebrate, unable to breath, and hypothermic. CT scan revealed a huge growth of the tumour, supra and infratentorially (Fig. 1 B). The patient died on 26.10.1989. Autopsy unfortunately was not permitted. Pathological Features."The tumour is composed of atypical bizarre cells, with large hyperchromatic granular nuclei and prominent nucleoli encore-

Atypical location and clinical behavior of a subset of intracranial germ cell tumors in children younger than 3 years of age

“…In our review, germinomas in this young age group appear to be highly aggressive. Only 1 of the 4 patients with germinoma (Case 7) 18 responded to chemotherapy, and all 4 patients, 4,13,18 including Patient 1 from our series, died of their malignancy, with a median survival of 19.5 weeks (range 10-44 weeks). This dismal survival is in contrast to that in older children with germinoma, which showed a greater than 90% response rate (complete and partial response) to chemotherapy 17,19 and a 5-year survival rate exceeding 90%.…”