Acute Deterioration in Pleomorphic Xanthoastrocytoma in a Child

Pleomorphic Xanthoastrocytoma (PXA) occur in less than 1 % of cases and usually present in the second decade. They present with seizures, focal deficits, intratumoral bleed and rarely with malignant transformation. The few cases that have been reported with acute deterioration in PXA have been secondary to intratumoral bleed. We present a case of PXA in a child that deteriorated secondary to high intracranial pressure which was managed successfully.


Introduction
P leomorphic xanthoastrocytoma (PXA) is a rare low-grade astrocytoma (WHO II) which occurs in children and young adults 1,2,3 .They present with seizures, focal defi cits, intratumoral bleed and rarely with malignant transforma on 4,5 .The few cases that have been reported with acute deteriora on in glioma have done secondary to intratumoral bleed.We present a case of PXA in a child that deteriorated secondary to high intracranial pressure which was managed successfully.

The Case
A 13-year male child presented to the emergency with history of altered behavior, reduced vision and severe headache for 1 month.The pain had increased over the last one week and was associated with repeated vomi ng.There was no history of fever, trauma or seizure.On examina on the child was conscious but sick looking and reluctant to obey commands.His visual acuity was fi nger coun ng (FC) both eyes and had bilateral papilloedema.There was no obvious cranial nerve, motor or sensory defi cit except for generalized weakness.The CT showed a mul cys c lesion in the tempo-parietal region with severe midline shi and perilesional edema.He was planned for MRI but he suddenly deteriorated with abnormal posturing, bilateral fi xed pupils and respiratory distress.Emergency burr hole was done and the superfi cial cyst aspirated with full neurological recovery in few hours.
The MRI was done next day which showed a large mul cys c lesion with an enhancing solid nodule in the inferolateral cyst sugges ve of PXA (Figure 1).There was gross midline shi and compression of the ipsilateral ventricles with edema.Elec ve craniotomy was done and microscopic total excision of the tumor was done.Intraopera vely there was a mul cys c tumor with an ill-defi ned fi rm nodule which was vascular.The cyst was lined with an ill-defi ned membrane adherent to the surrounding brain.The cysts contained straw colored fl uid.The nodule was excised and mul ple cysts fenestrated into single cavity (Figure 2).Postopera ve contrast CT did not reveal any residual tumor or mass eff ect.On follow-up at four months he is fi ne with improved vision and no neurological problems.

Discussion
PXA occur in less than 1% of cases and usually present in the second decade.PXA was originally described by Kepes et al. in 1979 and recognized by World Health Organiza on as a separate disease in 1993 6 .The majority are located in the supratentorial compartment although case reports in the cerebellum and spinal cord have been reported.They present with chronic headache, seizure, visual abnormali es or acutely with intratumoral bleed or rapid increase in cyst 7 .The tumors are slow to grow and are low grade histologically (Grade II) with rare malignant transforma on.The diagnosis is usually made with help of contrast CT or be er with MRI.The la er shows a tumor enhancing nodule with iso to hypo-intense in T1 along with leptomeningeal involvement seen in over 70% of cases.On T2 it is iso to hyper-intense with minimal surrounding vasogenic oedema.The diff eren als include ganglioglioma and oligodendroglioma.Pathologically there is cellular pleomorphism comprising of mononucleated/ mul nucleated giant cells, spindle cells and granular bodies in a re culin-rich background with few mitoses; necrosis is usually absent.
The op mal treatment is near total or complete removal surgically if possible 9 .Even if the histologic appearance may be an aggressive tumor, the overall survival is 70%-80% a er gross total excision.The role of the use of adjuvant therapy (radia on or chemotherapy) is controversial with no diff erence in overall survival rate between the 2 groups at 15 years 10 .These tumors are slow growing and usually present secondary to seizures or visual abnormali es although rapid deteriora on has been reported secondary to intratumoral bleed.This case was unusual that there was acute deteriora on without any intratumoral bleed sugges ng a possible sudden increase in the cyst fl uid.Emergency burr hole aspira on relieved the intracranial pressure with full recovery of symptoms.This suggests that simple tapping of the cyst can help to relieve the intracranial pressure and can save lives if neurosurgical facility is not available in the centre or if it takes me to refer the pa ent.This is in consistent with other cys c lesions of the brain with raised intracranial pressure.The staples were removed on the 8 th day and he was discharged on the 10 th day.The histopathology was consistent with PXA (Figure 3).

Conclusion
PXA are low grade tumors that usually slow growing.The usual presenta on is with seizure, headache, visual abnormality and some mes acute deteriora on.Emergency cyst aspira on can help relieve the intracranial pressure and thus save lives in such cys c tumors.

Fig 1 :
Fig 1: MRI coronal contrast showing a large mul cys c tumor with enhancing nodule and gross midline shi .Note the air in the lateral cyst secondary to the previous burr hole aspira on.

Fig 2 :
Fig 2: Intraopera ve picture showing the tense bluish subcor cal cy c lesion.The mural nodule cannot be seen separate from the normal brain parenchyma.

Fig 3 :
Fig 3: Photomicrograph showing H &E stain x 400 hpf showing atypical cells with prominent nucleoli, hyperchromasia and eosinophilic lipidized cytoplasm.Few tumor giant cells also seen.