Outcomes of surgical resection of large solitary hemangioblastomas of the craniocervical junction with limitations in preoperative angiographic intervention: report of three cases.

Abstract

Introduction: Hemangioblastomas of the central nervous system may occur sporadically, or in association with von Hippel-Lindau (vHL) disease. The treatment of large solitary hemangioblastomas of the posterior cranial fossa mandates a combination of angiographic intervention and surgery. However, large tumors may derive their vascularity from major cerebellar vessels, which can make their embolization hazardous.

Aim: To describe the surgical outcomes of three cases of large hemangioblastomas with compression of the medulla oblongata, where the potential for preoperative embolization was extremely limited.

Cases: Three patients (all males; 68, 36 and 38 years) presented with a history of chronic headache and caudal cranial nerve deficiencies. Diagnostic imaging showed large vascular lesions (4 x 3, 4 x 5 and 5 x 5 cm) at the craniocervical junction, compressing the brainstem. There were no concomitant findings associated with vHL disease.

Treatment: Staged treatment was administered. Preoperative embolization was attempted at first. One patient (68 yrs) showed a PICA occlusion and associated cerebellar infarction after embolization; embolization was deemed hazardous in the other two. In the second phase, the lesions were removed via a midline suboccipital approach with resection of the arch of altas. Complete removal was possible in all three cases. POSTOPERATIVE COURSE AND FOLLOW-UP: The caudal cranial nerve deficiencies deteriorated soon after surgery in all three patients. A tracheotomy was required in two patients, which was removed uneventfully during the rehabilitation phase. Ventriculo-peritoneal shunts were implanted in two patients. MRI follow-up three (1 case) and four years (2 cases) after surgery showed no relapse. The Karnofsky Index scores were 80, 70 and 90 in the three patients aged 68, 36 and 38, respectively.

Conclusion: Total microneurosurgical removal of large hemangioblastomas at the craniocervical junction with limited preoperative embolization (associated with morbidity) should be seriously considered. Although the early outcome is not encouraging, the long-term outcomes seem favorable.