A pure extradural hemangioblastoma mimicking a dumbbell nerve sheath tumor in cervical spine: illustrative case

Abstract

BACKGROUND Spinal extradural hemangioblastomas (HBs) are quite uncommon, with most reported cases involving the thoracic and lumbar areas. Therefore, the presence of a dumbbell-shaped pure extradural cervical HB is exceptional, making preoperative diagnosis particularly challenging. OBSERVATIONS The authors report a case of a 27-year-old woman who presented to their outpatient clinic with progressive cervicobrachialgia and numbness in the left arm. Magnetic resonance imaging showed a C5–6 intradural extramedullary lesion, and, despite some atypical features, the diagnosis of a possible neurogenic tumor was made. A multidetector computed tomography scan and angiography confirmed the expansion and remodeling of the left neural foramen as well as the highly vascularized nature of the mass. Preoperative embolization of the lesion was performed. Complete tumor resection was accomplished, followed by a C5–6 posterior fusion. Histopathology and immunohistochemistry revealed an HB. LESSONS HBs should be considered among the differential diagnosis of cervical extradural tumors. Exhaustive preoperative workup and surgical planning are decisive in order to attain gross-total resection with favorable outcomes.

Case Report of a Hemangioblastoma Originating from the T1 Spinal Nerve Treated with in Toto Resection, Hemicorporectomy and Pedicle Screw Stabilization

“…The tentative pre-operative diagnosis in the current case was a schwannoma which is the most common spinal nerve sheath tumor – to differentiate HB from a schwannoma is difficult based on MRI findings [ 7 ]. In contrast to schwannoma, an extradurally HB located at the spinal nerve root is extremely rare [ 1 , 2 ].…”

Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt

“…Although the literature reports entirely extradural or extra- and intradural hemangioblastomas, they are considered to be a very rare feature, making this diagnosis particularly challenging [ 2 , 6 ]. To our knowledge, there are few cases described in the literature reporting extradural foraminal hemangioblastoma in a patient with Von Hippel-Lindau disease, showing prominent vessels and mimicking spinal dural arteriovenous fistula and schwannoma [ 7 ]. Aytar et al [ 8 ] and Mariniello et al [ 9 ] reviewed the literature, and the majority of extradural hemangioblastomas were located in the thoracic spine (40%), followed by the lumbar (33%) and cervical spine (13%).…”

Spinal cervical extradural hemangioblastoma

“…Spinal cervical hemangioblastomas extending extradurally into the vertebral foramina and in the paravertebral space are exceptional, with only nine reported cases in the English literature. [ 8 10 11 12 13 14 15 16 ] The data of the 11 patients (including 2 of ours own) are summarized in Table 1 . The patients where the data were completely available were 6 females and 3 males, with age ranging from 24 to 80 years (median 41 years).…”

“…The MRI appearance of a dumbbell intra-extraspinal tumor mass associated to enlargement of the intervertebral foramen first suggests a neurogenic tumor, such as schwannoma or neurofibroma. [ 10 13 15 16 ] Hemangioblastoma shows intense and often irregular contrast enhancement due to the presence of prominent tumor vessels with dilated arteries and veins; on the other hand, schwannoma enhances intensely and fairly homogeneously. However, because of the very rare occurrence of dumbbell intra-extraspinal hemangioblastomas, the correct diagnosis is suspected only in patients with VHLD.…”

“…In three reviewed cases. [ 12 13 16 ] the preoperative embolization of the highly vascular tumor mass was necessary. However, this procedure is not free of complications, including extravasion of embolic particles and bleeding.…”