Brain-stem hemangioblastomas: The seemingly innocuous lesion in a perilous location

• Published in: Neurology India Vol. 66; no. 3; pp. 779 - 796
• Main Authors: Joseph, Jeena, Behari, Sanjay, Gupta, Shruti, Bhaisora, Kamlesh, Gandhi, Anish, Srivastava, Arun, Jaiswal, Awadhesh
• Format: Journal Article
• Language: English
• Published: India Wolters Kluwer India Pvt. Ltd 01.05.2018; Medknow Publications and Media Pvt. Ltd; Medknow Publications & Media Pvt. Ltd
• Subjects: Adolescent; Adult; Analysis; Cerebellar Neoplasms - pathology; Cerebellar Neoplasms - surgery; Female; Health screening; Hemangioblastoma - pathology; Hemangioblastoma - surgery; Hemangioendothelioma; Humans; Kidney cancer; Male; Middle Aged; Neurosurgical Procedures - adverse effects; Neurosurgical Procedures - methods; Postoperative Complications - epidemiology; Retrospective Studies; Risk factors; Treatment Outcome; Tumors; Young Adult; India; management of abdominal masses and/or pheochromocytoma may be required. Draining veins of the tumour must be preserved until the majority of the tumour has been addressed; surgery Key Messages: Significant neurological improvement is achievable in patients following the successful excision of brain.stem hemangioblastomas. The patients may require significant perioperative management. In the presence of VHL disease; complication avoidance; Brain-stem hemangioblastoma; radiology; outcome; surgery
• ISSN: 0028-3886; 1998-4022
• DOI: 10.4103/0028-3886.232294

Introduction: Hemangioblastomas [75% sporadic, 25% with Von Hippel Lindau (VHL) disease] are highly vascular, benign lesions. The surgical nuances, management, and complication avoidance in brain-stem hemangioblastomas (BHs) have been studied. Material and Methods: Over 18 years, 27(mean age: 29 years; range 15-60 years) consecutive cases of BH underwent microsurgical excision. All patients were assessed clinico-radiologically for neurological deficits and screened for VHL disease. Outcome of the patients was based on Karnofsky Performance Status scale (KPS). Results: 12 out of 19 (70.4%) patients with hydrocephalus underwent a cerebrospinal fluid (CSF) diversion procedure. Lower cranial nerve palsy was present in 10 (37%) patients and motor weakness in 13 (48%). The tumours [mean size 3.34 ± 1.06 cm, range: 1.4-5.5 cm; 11 solid, rest solid-cystic; 18 (66.7%) subpial and 9 (33.33%) intramedullary] were divided into four categories based on size: A: <2 cm (n = 5,18.5%); B: 2-3 cm (n = 10,37%); C: 3-4 cm (n = 6,22.2%); D: >4 cm (n = 6,22.2%). Their location was at posterior cervicomedullary junction (n = 12); pontomedullary junction (n = 7); pons (n = 3), medulla (n = 3) and ponto-mesencephalic region (n = 2). Multiple flow voids were seen in >50% patients with tumour >2 cm. 5 patients had syringomyelia; and, 8 had diffuse cervical cord expansion. Two patients with a large vascular tumour underwent preoperative embolization. Six patients had VHL disease; one underwent bilateral adrenalectomy for refractory hypertension; and, the another, nephrectomy for renal cell carcinoma. Twenty-six patients underwent a midline suboccipital craniectomy; and, 1 with a cerebellopontine angle tumour, a retromastoid craniectomy. 15 patients underwent total excision; 10 patients, near-total (<10% remaining) excision, and 2 patients, a subtotal (>10% remaining)) excision. Three patients (2 with VHL disease) expired due to exsanguinating hemorrhage, spreading venous thrombosis and aspiration pneumonitis, respectively. At follow-up visit (median: 25 ± interquartile range 2-56months), 17 patients had improved KPS, 4 remained in same status and 3 (recently operated, on tracheostomy) had worsened KPS. Conclusions: Significant improvement is achievable in neurological status in patients following successful extirpation of a brain-stem hemangioblastoma, despite a turbulent perioperative period. Leaving tumour capsule adherent to the brain-stem often helps in preserving brain-stem function. Postoperatively, the patients should be monitored for their respiratory and lower cranial nerve status to prevent aspiration pneumonitis.