A rare case of infantile cerebellar pilocytic astrocytoma and thrombocytopenia presenting with intratumoral hemorrhage.

Incidence of gliomas presenting with hemorrhage is around 3.7-7.2%. Low-grade gliomas account for <1% tumor with hemorrhage. Infants presenting with cerebellar pilocytic astrocytomas (PAs) and hemorrhage with thrombocytopenia have not been reported. We report an interesting case of a 9-month-old infant who presented to the emergency department in a drowsy state with recurrent vomiting. Laboratory investigations showed anemia, thrombocytopenia, and coagulopathy. Radiological evaluation showed a large PA with bleed. The patient was treated with retromastoid suboccipital craniotomy and tumor excision and improved postoperatively. Cerebellar PA with bleed and coagulopathy in infants has not been reported in literature till date. Their presentation seems to be acute in nature, and high index of suspicion is required for the diagnosis of these posterior fossa tumors, which can deteriorate rapidly in infants.

Introduction

Spontaneous intracerebral hemorrhage is an uncommon but recognized initial presenting sign of both primary and metastatic brain tumors. The rate of tumor-related intracranial hemorrhage is variably reported from <1% to 14.6%.[1] Hemorrhage in primary gliomas occurs in 3.7–7.2% of gliomas, mainly in glioblastoma multiforme and oligodendroglioma with low-grade astrocytomas accounting for <1%. Hemorrhage associated with pilocytic astrocytomas (PAs) is only sporadically reported.[2] It is even rarer in infants. Here, we report an interesting case of hemorrhage in cerebellar region in infants' PA with associated low platelet counts and coagulopathy.

Case Report

A 9-month-old infant presented to the emergency department in a drowsy state. The child improved following the administration of cerebral decongestants. Routine blood tests revealed thrombocytopenia and deranged prothrombin time. Computed tomography (CT) [Figure 1a and b] of the head demonstrated right cerebellar nonenhancing solid cystic mass lesion with hemorrhage, midline shift with obstructive hydrocephalus. Magnetic resonance imaging plain and contrast [Figure 2a–d] confirmed the CT findings and the lesion was not enhancing on contrast.

Figure 1

Plain computed tomography images demonstrating cerebellar tumor with bleeding. (a) Computed tomography axial view (b) computed tomography coronal view

Figure 2

Magnetic resonance imaging of the brain demonstrating right cerebellar tumor with bleeding. (a) Magnetic resonance imaging T1-weighted image, (b) magnetic resonance imaging T2-weighted image, (c) apparent diffusion coefficient image, (d) contrast image

Under fresh frozen plasma, platelet-rich plasma cover, retromastoid craniotomy was performed and tumor decompression done. At surgery, there was a solid-cystic right cerebellar tumor with an abnormal leash of blood vessels. Cystic component was filled with blood. Gross total excision of tumor was done.

Histopathology [Figure 3a–d] revealed PA with hemorrhage. Interspersed in the cells was hemorrhagic component. The child recovered well postoperatively without the need for any cerebrospinal fluid diversionary procedure.

Figure 3

Histopathology images demonstrating sheets of pilocytic cells and blood vessels and hemorrhage. (a and b) Dense sheets of elongated bipolar cells with fibrillary process, (c and d) neoplastic astrocytes with foci of bleeding

Discussion

The reported rate of hemorrhage in primary gliomas has been around 3.7–7.2% (mainly in glioblastoma and oligodendroglioma) while the low-grade astrocytomas account for <1% of cases.[2] White et al.[1] reported that the rate of spontaneous hemorrhages in histologically proven cases of PAs was 8%. They concluded that there was no particular location susceptible for hemorrhage; however, no bleeding occurred in cerebellum in their series. The symptomatic hemorrhages associated with cerebellar PAs are extremely rare.[345]

The etiology of intratumoral hemorrhage in PAs is unclear. Endothelial proliferation, rupture of an encased aneurysm, and dysplastic capillary beds have been factors hypothesized as potential causes for bleeding.[67] In our case, an abnormal leash of blood vessels was seen at the surgery. Contributing to this was the deranged coagulation profile and thrombocytopenia (whether cause or effect could not be ascertained) which could have led to a vicious cycle leading to symptomatic hemorrhage.

In all the above series, the association of infantile cerebellar PAs with symptomatic hemorrhage with association of thrombocytopenia and deranged coagulation profile has not been reported, making this case a rare and interesting one.

Conclusion

PAs, although benign, can present with hemorrhage. Cerebellar PA with bleeding and coagulopathy in infants has not been reported in literature. Their presentation seems to be acute in nature, and high index of suspicion is required for the diagnosis of these posterior fossa tumors, which can deteriorate rapidly in infants.

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Conflicts of interest

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