Primary spinal cord glioblastoma multiforme: a single-center experience.

INTRODUCTION: Primary spinal glioblastoma (GBM) are very rare tumors of the spinal cord, with dismal prognosis and their exact management is controversial. We attempt to formulate treatment guidelines for these extremely rare tumors based on our institutional experience and a comprehensive review of the literature. MATERIALS AND METHODS: In this retrospective study from 2008 to 2020, all the patients diagnosed with primary spinal GBM who underwent surgery at our institution were included. Clinical data were retrieved from case files, outpatient records and telephonic follow-up. Data on postoperative chemoradiation was noted in all the patients. The final diagnosis of spinal GBM was confirmed as per the histopathology reports. Patients who could not be followed up and those with prior history of cranial GBM were excluded from the study. RESULTS: Nine patients were followed up and a median survival of 11 months was noted. Chemotherapy with TMZ and radiotherapy to the whole craniospinal axis significantly improved survival in these patients. The extent of surgical resection was not shown to be significant. Intracranial metastasis was the leading cause of mortality in such patients. Three patients developed documented intracranial metastasis during the course of the disease. CONCLUSIONS: Low threshold must be kept in mind in diagnosing patients with high-grade spinal cord intramedullary tumors in view of the rapidly progressing nature of the disease. In case of positive histopathological diagnosis of spinal GBM, the whole craniospinal axis should be imaged and any cranial metastasis which was originally missed during initial workup could be given appropriate radiotherapy.

A Century With Craniopagus Twin Separation Surgeries: Nihilism to Optimism.

Craniopagus conjoined twins are extremely rare, reported 1 in 2.5 million live births. To date, 62 separation attempts in 69 well-documented cases of craniopagus twins have been made. Of these, 34 were performed in a single-stage approach, and 28 were attempted in a multistage approach. One or both twins died of massive intraoperative blood loss and cardiac arrest in 14 cases. We report our surgical experience with conjoined craniopagus twins (JB) with type III total vertical joining and shared circumferential/circular sinus with left-sided dominance. A brief review of the literature is also provided. In our twins, the meticulous preoperative study and planning by the multidisciplinary team consisting of 125-member, first-staged surgical separation consisted of creation of venous conduit to bypass part of shared circumferential sinus and partial hemispheric disconnection. Six weeks later, twin J manifested acute cardiac overload because of one-way fistula development from blocked venous bypass graft necessitating emergency final separation surgery. Unique perioperative issues were abnormal anatomy, hemodynamic sequelae from one-way fistula development after venous bypass graft thrombosis, cardiac arrest after massive venous air embolism requiring prolonged cardiopulmonary resuscitation, and return of spontaneous circulation at 15 minutes immediately after separation. This is the first Indian craniopagus separation surgery in a complex total vertical craniopagus twin reported by a single-center multidisciplinary team. Both twins could be sent home, but one remained severely handicapped. Adequate perioperative planning and multidisciplinary team approach are vital in craniopagus twin separation surgeries.

Optic Nerve Hemangioblastoma: Review.

BACKGROUND: Hemangioblastomas of the optic nerve are very rare tumors. They occur in association with Von Hippel-Lindau (VHL) syndrome; however, sporadic occurrences have been reported. We describe here a case of optic nerve hemangioblastoma in the absence of VHL and review the pertinent literature. CASE DESCRIPTION: A 33-year-old woman presented with gradually progressive vision loss in the right eye. On examination, the visual acuity on the right was hand movement close to face in all quadrants. Color discrimination was impaired. Fundoscopy revealed optic atrophy and no other retinal pathology. There was relative afferent pupillary defect in the right eye. No neurocutaneous markers were found. Imaging revealed lesion isointense on T1, hyperintense on T2/fluid-attenuated inversion recovery, and showing relatively homogenous enhancement on postcontrast study. Multiple flow voids were seen in the intracranial part of the lesion. The proximal part of the intraorbital right optic nerve was enlarged and tortuous with distended optic nerve sheath. A right single-piece fronto-orbital craniotomy was done. A reddish lesion seen involving the right optic nerve just proximal to the chiasm with multiple vessels and a distinct feeding vessel was seen supplying the tumor. The lesion was excised and the optic nerve was sacrificed approximately 1 cm proximal to the chiasm. The postoperative course was uneventful. CONCLUSIONS: Conclusions: Optic nerve hemangioblastoma is a rare occurrence and a high level of suspicion is required preoperatively in the absence of VHL syndrome.

Intraventricular Meningioma Presenting as Lateral Posterior Choroidal Artery Stroke.

BACKGROUND: Transient tumor attack is a rare but well-known phenomenon. Described by Ross in 1983, it demonstrated a transient ischemic attack-like picture in patients with intracranial mass lesions. Usually these attacks were recognized at sites anatomically away from the primary lesion that were not explained by primary mass effect of the lesion. The exact pathophysiology of such transient tumor attacks is postulated to be due to either a vascular steal phenomenon or compression of a vessel or localized prothrombotic state. CASE DESCRIPTION: Here we describe a case who was being evaluated for a lacunar stroke involving the lateral geniculate body, and a surprising finding of left intraventricular meningioma was detected. CONCLUSIONS: We try to shed some light on the pathophysiology of this unusual phenomenon.