Intramedullary hemangioblastomas: surgical results in 16 patients

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>Hemangioblastomas are rare, benign, highly vascularized tumors that can be found throughout the neuraxis but are mainly located in the cerebellum and in the spinal cord. Spinal hemangioblastomas can present with motor and sensory deficits, whose severity varies according to the size and location of the tumor. Resection is the best treatment option to avoid neurological deterioration. The authors report surgical results in the treatment of intramedullary hemangioblastomas and discuss the technical nuances important to achieving total resection without adding new deficits.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>A consecutive series of patients with intramedullary hemangioblastomas operated on between 2000 and 2014 by the senior author (H.T.) is presented. The functional scale proposed by McCormick was used to evaluate the patients' neurological status before and after surgery.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>Sixteen patients were included in the study and underwent 17 surgeries. Follow-up was at least 6 months. Age at presentation varied from 13 to 58 years (mean 33.8 years). Ten patients (62.5%) were males and 6 patients (37.5%) were females. Seven (43.75%) of the 16 patients had associated von Hippel—Lindau syndrome, with hemangioblastomas also presenting in other locations. Three patients had multiple tumors in the same segment in the spinal cord, and 10 patients (62.5%) presented with cysts. According to the site of presentation, 11 tumors (68.75%) were localized at the cervical region (including the cervicomedullary junction) and 5 tumors (31.25%) at the thoracic level. Total resection was achieved in all cases, evidenced by postoperative MRI. Four patients had some functional worsening immediately after surgery. After 6 months, 1 patient had functional worsening compared with preoperative status, and 2 patients had clinical improvement. The majority of the patients remained clinically stable postoperatively.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>Adequate knowledge of anatomy and the correct use of microsurgical techniques allowed total resection of these tumors, with minimal morbidity and maximum functional recovery. Outcome seems to be directly related to the neurological status before surgery.</jats:p></jats:sec>

Remission after complete excision of an intramedullary hemangioma with an identifiable tumor plane in a dog

“…A temporary worsening of neurological signs, similar to that observed in the dog described here, occurs in about 6%‐34% of human patients surgically treated for intramedullary tumors . In 1 study, a deterioration was noted in 16 of 29 patients but was temporary in 15 of these cases .…”

“…Hemangioblastoma may represent a variation of hemangioma. Most human cases of hemangioma/−blastoma are characterized as hemangioblastoma . However, in 1 study, the number of hemangiomas and hemangioblastomas was roughly equal .…”

“…Most human cases of hemangioma/−blastoma are characterized as hemangioblastoma. 1,2,4,14 However, in 1 study, the number of hemangiomas and hemangioblastomas was roughly equal. 15 In contrast, hamartomas are characterized by an overgrowth of endothelial cells with surrounding vascular smooth muscle cells that can be interdigitated by neuroparenchyma.…”

“…26 A temporary worsening of neurological signs, similar to that observed in the dog described here, occurs in about 6%-34% of human patients surgically treated for intramedullary tumors. [1][2][3][4]14,22,[27][28][29] In 1 study, a deterioration was noted in 16 of 29 patients but was temporary in 15 of these cases. 3 Decline is most commonly sensory in nature, related to the proprioceptive pathways in the "posterior column" of man (known as the dorsal column-medial lemniscal system in dogs), and is most likely due to the dorsal myelotomy itself.…”

Phakomatoses and Their Tumors: Genetics and New Treatment Options

“…The correct use of microsurgical techniques and thorough understanding of the anatomy yields satisfactory results with minimal morbidity and maximum functional recovery. Outcome depends upon the neurological status before surgery, site, and size of lesion [90]. Favorable results can be achieved by careful dissection of the tumor and preoperative embolization to prevent hemorrhage.…”