Case 983-- Unexpected Death of a 9-Year-Old Girl

Contributed by CI Herbst1, MBChB (US), FC For Path (SA), JJ Dempers1, MBChB, (US), FC For Path (SA), SD Zaharie2, MBChB, (MD EFN)
Division of Forensic Medicine and Pathology1, University of Stellenbosch and Western Cape Forensic Pathology Service, and
Division of Anatomical Pathology2, University of Stellenbosch and National Health Laboratory Service, Cape Town, South Africa.


CLINICAL HISTORY

A 9 year old female child was discovered dead in bed by her caregiver. For two months prior to death, the child had been complaining of right-sided body weakness. She was treated at a local hospital with Prednisone after being diagnosed with Bell's Palsy, as a right-sided cranial nerve VII palsy was observed during clinical examination. No imaging was conducted at this hospital visit. This weakness progressively worsened to involve the left side of the body. On the day of death, the child had vomited once and complained of headache before falling asleep. Due to the death being sudden and unexpected, a medico-legal autopsy was indicated. No injuries or signs of trauma were present. Internal examination revealed a swollen brain with a large tumor mass in the pontine area.

GROSS AND MICROSCOPIC PATHOLOGY

Macroscopically, a tumor with a nodular appearance was seen extending from the pons overgrowing the basilar artery with extension towards the infundibular area. (Figure 1a) This infundibular area of the tumor showed small hemorrhagic spots. The brain and upper cervical spinal cord were fixed and retained for formal neuropathological examination.

The H&E stained sections revealed a diffuse infiltrating tumor with a fibrillated background containing pleomorphic cells (Figures 1b and 1c). Mitotic figures were frequent (Figure 1d). Sections taken from the upper cervical spine show no tumor infiltration. GFAP was positive in the tumor cells (Figure 1e). The tumor cells were negative with synaptophysin. Ki67 could not be done on the post-mortem tissue. What is your diagnosis?

FINAL DIAGNOSIS


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