Intramedullary hemangioblastoma. Case report

Abstract

Hemangioblastomas are benign tumors that develop from the vessels of the central nervous system and can be a manifestation of autosomal dominant von Hippel-Lindau disease. Statistically, they account for 1.5‒2.5% of all intracranial tumors and 2‒15% of spinal cord tumor lesions. There are very few publications on the intramedullary localization of these neoplasms.
The patient, 45 years old, a serviceman, presented with complaints of headache, slight unsteadiness of gait, as well as slight weakness in the right extremities, more pronounced in the right upper extremity, periodic numbness of the upper extremities, which progressed and made further service impossible. On neurological examination: pupils D=S, light reflexes were brisk, eye movement was fully preserved and horizontal nystagmus. BNI - PS - I, BNI - NS - I. HB - I. GR - I. Swallowing and phonation were fully preserved. There was a slight hemiparesis on the right. Hemihypesthesia on the right was more prominent in the upper extremity. Ataxia of mixed genesis. Pelvic organs function was preserved. Periodic constipation for up to 7 days. Magnetic resonance imaging revealed a multifocal brain lesion. Supratentorially, a cystic mass measuring 2.60×2.12×2.14 cm with a solid component up to 1.5 cm in the diameter was detected in the of the thickened corpus callosum. Intramedullary cystic-solid lesion of the medulla oblongata extending to the cervical spinal cord with conventional dimensions of the solid component 1.76×1.23×1.57 cm and the cystic component was 1.52×1.62×1.22 cm. Magnetic resonance imaging of the cervicothoracic region of the spinal cord revealed significant hydromyelitic expansion of the central canal from the C2 level to the Th3 level (up to 10mm in the diameter). Endovascular embolization of the neoplasm with a liquid embolic agent (Phil) and following microsurgical en bloc tumor resection were performed.
Hemangioblastomas with an intramedullary location are extremely difficult and risky for the surgical removal. The presence of a cystic component in the hemangioblastoma structure or perifocally gives a chance to remove such a neoplasm avoiding risk of functional deterioration. Preoperative endovascular obliteration of hemangioblastoma vascularity is considered as an effective measure, although it is associated with a risk of cerebral ischemia in corresponding brain structures. Another crucial issues of intramedullary hemangioblastoma surgery includes multimodal intraoperative neuromonitoring and maximum possible intraoperative magnification to minimize injury of adjacent brain structures. Present practice shows that multiple intramedullary cysts in the spinal cord commonly regressing after neoplasm removal and leads to improvement of neurological deficit within a relatively short period of time.
Conclusions. Hemangioblastomas with intramedullary growth and perifocal cyst can be surgically removed with a good functional result and comprehensive approach includes preoperative selective angiography with endovascular embolization, multimodal intraoperative neuromonitoring and appropriate microsurgical technique.