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.
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.
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.
