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Washington University Experience | NEOPLASMS (GLIAL) | Astrocytoma, pilocytic - Gross Pathology | 4A0 Case 4 History
Case 4 History ---- The patient was a 10 year old boy with a history of a suprasellar pilocytic astrocytoma, Noonan's syndrome, congenital deafness and blindness, and developmental delay. The patient presented in May 2008 with a chief complaint of headaches and abnormal gait. An MRI of the brain and brainstem performed at SLCH at the end of May showed a sellar and suprasellar mass with extensive extension along the prepontine region and down to the level of the pontomedullary junction. At surgery it was determined at this time that the brain mass was a nonresectable pilocytic astrocytoma of the optic nerve. Rather than undergoing a primary resection of the tumor, the patient underwent chemotherapy, which he did not tolerate well due to extensive pancytopenia. The patient then underwent radiation therapy for this mass which showed no treatment effect. At the end of June 2009. he presented with status epilepticus and labored breathing, was treated and discharged but was later admitted in November 2009 with a chief complaint of one month history of seizures and gradual decreasing mental status and evidence of enlarged ventricles visible on head CT. Left ventriculoperitoneal shunt malfunctioned, for which he underwent a shunt revision. The shunt became infected in December 2009, with intraoperative findings of thick frankly purulent material around the valve and beneath the wound. The patient needed a second shunt revision in January 2010 and became increasingly irritable and went into status epilepticus that prompted his transfer to the Pediatric ICU. His seizure activity was terminated with benzodiazepine administration. Within a few hours of this seizure, the patient suffered respiratory depression and associated hypertension, and was intubated. His serum sodium level was checked revealing a level of 132. The patient developed severe brain edema, went into status epilepticus, rapidly deteriorated and died. ---- At autopsy the weight of the unfixed brain was 1440g and appeared markedly edematous. Coronal sections of the cerebral hemispheres and brainstem show a large hemorrhagic area involving the midbrain, pons and hypothalamus. The mass compressed and distorted the upper brainstem. The lateral ventricles were compressed secondary to parenchymal edema. ---- Sections of the brainstem show a large area of fresh and organizing hemorrhage. Admixed within these hemorrhagic necrotic areas were nests of piloid tumor cells, abundant Rosenthal fibers and hyalinized blood vessels. Overall, the findings were consistent with residual pilocytic astrocytoma with extensive necrosis and superimposed hemorrhage. A GFAP immunostain was strongly and diffusely positive within these areas. The adjacent brain parenchyma shows marked piloid gliosis, collections of macrophages, some laden with hemosiderin, scattered chronic inflammatory cells, a rare foreign body giant cell, mineralized neurons and scattered axonal spheroids. There is very little viable tumor left consistent with the patient's reported history of therapy.
