Do Glioblastomas with Syndromic Association Have Better Prognosis? A Case of Supratentorial Glioblastoma with Embryonal Tumor Differentiation in a Child with Multiple Enchondromatosis.

Glioblastoma (GBM) is an aggressive cancerous neoplasm of the brain that has numerous morphological subtypes. Primitive neuroectodermal differentiation (hereafter, referred to as embryonal tumor [ET] differentiation) in GBM is one of them and is known to occur in adults. Their presentation in pediatric population is rare and can be a source of diagnostic confusion. The dual pathology leads to doubts where one could ask whether it is ET differentiation in GBM specimen or glial differentiation in ET specimen. This histological discrimination has a bearing on the treatment regimens and prognosis. We report a case of a 10-year-old boy presenting with a supratentorial GBM, isocitrate dehydrogenase wild type with ET differentiation, and multiple benign bony lesions of both extremities. He underwent surgical excision for the brain neoplasm followed by radiotherapy and has shown prolonged survival with no recurrence. In this article, we discuss prognostic factors associated with long-term survival of these tumors.

Penetrating orbital trauma leading to trans-orbital brain herniation.

INTRODUCTION: Development of a posttraumatic herniation of brain parenchyma through the orbit is a rare complication of orbital roof fracture. Mostly, the injury is due to a direct impact to the frontal region resulting in orbital roof fracture with dural defect and herniation of cerebrospinal fluid or brain parenchyma. These patients present with acute or gradually progressive proptosis with impending risk of loss of vision and mandate surgical decompression of optic nerve with watertight closure of the dural defect. Bony reconstruction may be required to achieve normal contour of the orbit. MATERIALS AND METHODS: We encountered an interesting pediatric patient who presented to us with altered sensorium and progressive proptosis 3 days after a penetrating trauma to his left orbit by falling on the handle of a bicycle. The plain computerized tomography of the head (NCCT) showed a left orbital roof defect with herniation of brain matter into the orbit displacing the globe inferolaterally. A craniotomy was performed and watertight closure of the dural rent was achieved with use of autologous bone to cover the defect. CONCLUSIONS: The child improved gradually, and at 6 months follow-up, he had good cosmetic outcome with improvement of vision.

Spinal cord abscess secondary to infected dorsal dermal sinus in an infant: uncommon presentation of a known entity.

Infection along the congenital dermal sinus tract is well known. However, congenital dorsal dermal sinus presenting with intramedullary abscess is quite rare. The sinus tract usually presents in the midline and acts as a portal of entry for infection that may manifest as meningitis, extradural or subdural abscess and may further involve the cord. Surgical drainage of pus and complete excision of the sinus tract is the standard treatment. Here we describe an infant with an infected congenital dorsal dermal sinus with atypical presentation as large paracentral abscess in the upper back. We further highlight the importance of recognising and treating these skin dimples even when clinically silent to avoid catastrophic complications.