Exponential Increase in Size of Pineal Region Teratoma in Short Duration following Ventriculoperitoneal Shunt

Abstract

A 4-year-old male child presented with complaints of generalized tonic–clonic seizures for one and half years. Magnetic resonance imaging brain was suggestive of pineal region germ cell tumor with obstructive hydrocephalus. Patient had exponential increase in size of lesion following ventriculoperitoneal shunt for which patient underwent emergency craniotomy and tumor excision. Postoperative biopsy showed immature teratoma. Early diagnosis and complete resection of intracranial immature teratoma are must to have a favorable outcome, as delay in surgery may cause resection of tumor incomplete due to rapid increase in size of tumor following cerebrospinal fluid diversion.

 

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Keywords

immature teratoma - pineal region mass - ventriculoperitoneal shunt

Case Presentation

A 4-year-old male child presented with complaints of generalized tonic–clonic seizures for one and half years. Patient was conscious and obeying commands. Bilaterally vision was finger counting at 1 m with papilledema with no motor or sensory deficits. Magnetic resonance imaging brain was suggestive of pineal region mass with obstructive hydrocephalus ([Fig. 1]) for which patient underwent ventriculoperitoneal (VP) shunt. Cerebrospinal fluid (CSF) analysis showed raised alpha fetoprotein (36.45 ng/mL) and β-human chorionic gonadotropin (2.30 mIU/mL) and normal alkaline phosphate level. Patient was planned for chemoradiotherapy but was deferred in view of no proven pathological diagnosis and was planned for tumor decompression.

Fig. 1 Magnetic resonance imaging brain (P + C): T2 heterogeneously hyperintense pineal region lesion that is heterogeneously contrast enhancing and has few areas of diffusion restriction.

Patient had sudden fall in Glasgow Coma Scale for which computed tomography (CT) head was done. CT head showed exponential increase in size of lesion in 3 weeks postoperatively following VP shunt ([Fig. 2]). Patient underwent left frontoparietal parasagittal craniotomy and gross total excision of lesion via transcortical transventricular approach ([Fig. 3]). Postoperative CT scan showed complete excision of lesion ([Fig. 3]). Patient was extubated on postoperative day 2. Biopsy was immature teratoma.

Fig. 2 Computed tomography head done at 3 weeks following ventriculoperitoneal (VP) shunt. There is exponential increase in size of lesion following VP shunt. Lesion is heterogenous with predominantly solid portion and involving entire third ventricle and extending into bilateral lateral ventricle.

Fig. 3 Intraoperative picture of a lesion and postoperative computed tomography head.

 

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Discussion

Intracranial immature teratoma is rare malignant tumor and has poor prognosis.[1] Due to the deep location of intracranial immature teratoma, surgery is challenging in these cases, and hence it results in incomplete resection and requires multimodalities treatment.[2] Early diagnosis and early resection of tumor may add in complete excision of tumor. This improves the overall survival and no adjuvant treatment is needed.

Literature has shown that there is rapid increase in size of intracranial immature teratoma following treatment of hydrocephalous.[3] We found the same findings in our case as there was massive increase in size of tumor following 3-week post-VP shunt. The exact reason for rapid increase in size of immature teratoma is not known in existing literature. The reason may be that following treatment of hydrocephalus, there is decrease in intracranial pressure and intracranial immature teratoma being highly malignant, may have increased exponentially. To know the exact pathophysiology, we need the larger studies.

It is better to excise tumor soon following CSF diversion. Due to rapid increase in size of tumor, patient may deteriorate and may have poor prognosis. Due to large size of tumor, complete resection of tumor may not be possible. This case scenario may be avoided if tumor is excised primarily and may undergo CSF diversion later if required.

 

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Conclusion

Early diagnosis and complete resection of intracranial immature teratoma are must to have a favorable outcome, as delay in surgery may cause resection of tumor incomplete due to rapid increase in size of tumor following CSF diversion.

 

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Conflict of Interest

None declared.

• References

• 1 Goyal N, Kakkar A, Singh PK. et al. Intracranial teratomas in children: a clinicopathological study. Childs Nerv Syst 2013; 29 (11) 2035-2042
• 2 Han JW, Koh KN, Kim JY. et al. Current trends in management for central nervous system germ cell tumor. Clin Pediatr Hematol Oncol 2016; 23: 17-27
• 3 Woo SB, Lee CY, Kim CH. et al. Rapid-growing intracranial immature teratoma presenting obstructive hydrocephalus and abducens nerve palsy: a case report and literature review. Brain Tumor Res Treat 2022; 10 (02) 117-122

Publikationsverlauf

Artikel online veröffentlicht:
25. Juni 2025