Intradural Hemangiopericytoma of the Lumbar Spine: A Rare Entity

Abstract

SUMMARY:We report a case of a patient with an intradural hemangiopericytoma of the lumbar spine and the unusual MR angiography (MRA) and spinal angiography findings of arteriovenous shunting with spinal venous congestion. We highlight the concordance of the unusual MRA and angiographic findings and their relationship to combined endovascular and surgical therapy. H emangiopericytomas, initially described by Stout and Murray, 1 are vascular tumors derived from pericytes, the smooth muscle cells that control the diameter of the capillaries. The mass itself is composed of numerous capillaries with an intact basement membrane, with the neoplastic pericytes in the extravascular space.2 Hemangiopericytomas may be considered benign or malignant, have been identified in all age groups, and demonstrate no sexual predisposition.3 Primary spinal hemangiopericytomas are rare, with only 39 reported cases in the literature, those being primarily extradural.We describe the unique presentation of a lumbar intradural hemangiopericytoma that produced imaging findings consistent with an arteriovenous shunt, with congested spinal veins and concordant spinal MR angiography (MRA) and conventional angiographic findings. Endovascular embolization of the hemangiopericytoma was performed to treat the arteriovenous shunt and prevent excessive intraoperative blood loss. In addition, the enlarged radicular vein was ligated during surgery to definitively prevent any myelopathic complications. Case ReportA 54-year-old man presented to our emergency department with left paraspinal, buttock, and anterolateral thigh pain that radiated down the anterior shin and left foot. Initial noncontrast MR examination revealed a lesion in the left L4-L5 neural foramen, which was soft tissue signal intensity on all sequences. Serpiginous, intradural signal intensity voids were identified in the lumbar spinal canal without change in signal intensity in the cord (Fig 1). Contrast-enhanced spinal MRA and postcontrast images were recommended to elucidate the nature of the intradural flow voids and better define the neuroforaminal mass.The L4-L5 neuroforaminal mass enhanced with contrast (Fig 2). MRA (Fig 3, left) of the lumbar spine demonstrated prominent intradural vessels contiguous with the mass with congested pial veins, compatible with a spinal arteriovenous fistula or arteriovenous malformation. Spinal angiographic examination was recommended both to further elucidate the relationship between the soft tissue mass and the abnormal surface veins and for possible perioperative intervention.Spinal angiography (Fig 3, right) revealed a hypervascular mass in the left L4 recess with radicular venous drainage and filling of congested intradural veins ascending to the level of the conus, where congested anterior and posterior spinal veins were apparent. Devascularization of the tumor was performed without residual tumor blush and disappearance of the apparent arteriovenous shunting accounting for spinal venous congestion. At surgery, the L4-L5 nerve sheath was inc...

Surgical management of primary spinal hemangiopericytomas: an institutional case series and review of the literature

“…The first intradural case was reported in 1961 by Kruse [11]. Since then, 23 cases have been reported, including our four present cases [4][5][6][7][8][9][10][11][12][13][14]. The occurrence of HPCs has been reported throughout the cervical, thoracic and lumbar spine, but the cervicothoracic region appears to have the greatest prevalence ( Table 2).…”

“…Betchen et al [5] reported that extradural tumors are associated with more pain than intradural tumors. In a review of the literature, 5 out of the 19 patients had pain complaints involving intradural lesions [4,8,9].…”

“…Cases of spinal HPCs are extremely rare and are most often intradural. There have been only 19 reported cases of intradural tumors, most being extramedullary [4][5][6][7][8][9][10][11][12][13][14]. Only two prior case reports have been documented to have intramedullary involvement [6,10].…”

Intracranial Meningeal Hemangiopericytomas in Children and Adolescents: CT and MR Imaging Findings

“…4 Histologically, hemangiopericytoma arises from pericytes of Zimmerman or pericapillary cells and can occur anywhere where capillaries are found. 2,8,9 It has been classified as a mesenchymal tumor of the meninges and further subclassified into 2 histologic types, hemangiopericytoma and anaplastic hemangiopericytoma (corresponding to WHO grades II or III) by the WHO in 2007. 10 The literature on CT and MR imaging of intracranial meningeal hemangiopericytomas is currently limited.…”

Hemangiopericytoma of cervicothoracic spine: a case report and literature review.

“…HPCs in the spine may involve the structures of the vertebra and the spinal canal, including the extradural and intradural space. Our search of the literature showed that 140 cases of HPC in the spine had been reported (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(49)(50)(51)(52)(53)(54)(55)(56), and 64 cases were extradural (2, 4-6, 8-10, 15, 20-22, 24, 25, 27, 28, 31, 32, 34, 35, 37, 40-42, 44-46, 49, 51, 53-55) (Table I). Here we present a case of primary HPC invasion in the cervicothoracic spine, as well as presenting a review of the literature.…”