Case reportJ Neurosurg spine 25:379–382, 2016

Spindle cell hemangioendothelioma, alternately re-ferred to as spindle cell hemangioma, is a vascular tumor mainly affecting young adults. Initially de-

scribed by Weiss and Enzinger in 1986,12 these tumors typically appear in the distal extremities.10 In this report we describe the case of a 48-year-old man who presented with signs of myelopathy and was found to have an intra-medullary spinal cord spindle cell hemangioma, the first such tumor reported. This case report brings attention to a rare diagnosis for an intramedullary mass and discusses the biological behavior of the spinal spindle cell heman-gioma.

Case reportHistory and Presentation

A 48-year-old man presented with gait ataxia, bilateral lower extremity numbness, and subacute urinary retention. He did not have a history of sarcoma or any prior malig-nancy. A neurological examination revealed diminished strength in the left iliopsoas muscle (4/5 on the Medical Research Council scale).5 A sensory level at approximate-ly the level of T-10 was apparent. The patient exhibited clinical findings consistent with American Spinal Injury Association (ASIA) Grade D.1 In addition, deep tendon re-flexes were subtly enhanced symmetrically in the legs, and a Babinski sign was present on the left.

MRI of the thoracic spine with intravenous Gd demon-

strated an enhancing intramedullary lesion that measured approximately 0.7 × 0.6 × 1.7 cm eccentric to the left side of the cord at the T6–8 level (Fig. 1). Moreover, extensive T2 cord signal abnormalities extended superiorly and infe-riorly from the enhancing lesion. At the time of diagnosis, the initial concern was that the patient had an intramedul-lary astrocytoma or hemangioblastoma.

OperationFollowing a T6–8 laminectomy and midline dural

opening, a red-purple lesion was apparent on the spinal cord surface and was concerning for a possible vascular lesion (Fig. 2). A cleavage plane was identified between the tumor and the spinal cord permitting a gross-total resec-tion. Intraoperative bleeding was readily controlled. Small motor-evoked potentials (MEPs) were present throughout in the right abductor hallucis muscle, but there were no consistent MEPs in any of the other monitored leg mus-cles. Posterior tibial nerve somatosensory evoked poten-tials were also not adequate for intraoperative monitoring. Therefore, an epidural electrode was placed caudal to the tumor for recording of D-waves during tumor resection. These were less well-formed following right hemisphere stimulation, but aside from one change related to reposi-tioning of the recording electrode, they remained stable during tumor resection (Fig. 3).

Postoperatively, the patient experienced mild lower ex-

abbreviatioNs ASIA = American Spinal Injury Association; MEP = motor-evoked potential; SMA = smooth muscle actin; VIM = vimentin.sUbMitteD May 31, 2015. aCCepteD November 9, 2015.iNClUDe wheN CitiNg Published online April 8, 2016; DOI: 10.3171/2015.11.SPINE15641.

Intramedullary spindle cell hemangioma: case reportrani Nasser, MD,1 Kimberly ashayeri, ba,2 alan D. legatt, MD, phD,3,4 and John K. houten, MD5

Departments of 1Neurological Surgery, 3Neurology, and 4Neuroscience, Albert Einstein College of Medicine/Montefiore Medical Center; 2Albert Einstein College of Medicine, Bronx, New York; and 5Marcus Neuroscience Institute, Boca Raton, Florida

The authors describe the case of a 48-year-old man found to have the first reported intramedullary spinal cord spindle cell hemangioma. Previous research indicates that spindle cell hemangiomas are rarely found in the spine. Only 3 previ-ous cases exist, all in the intradural, extramedullary space. In the present case, gross-total resection of the tumor was possible with no loss of function from baseline. This report presents the successful resection of the first reported intra-medullary spindle cell hemangioma and reports 4-month follow-up, demonstrating the biological behavior of this rare tumor.http://thejns.org/doi/abs/10.3171/2015.11.SPINE15641Key worDs spindle cell hemangioma; intramedullary tumor; immunohistochemical analysis; gross-total resection; oncology

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tremity weakness, but was ambulatory (ASIA Grade D). However, the patient recovered to his neurological base-line within several days postoperatively, during a 7-day taper off steroids. An immediate postoperative MR image was obtained and revealed no evidence of residual tumor (Fig. 4).

Histological AnalysisUpon microscopic inspection, the lesion had endothe-

lial-lined channels of variable size. Histological analysis was positive for both CD31 and CD34. Moreover, many cells expressed vimentin (VIM). Furthermore, smooth muscle actin (SMA)-stained cells adjacent to the endo-thelial cells were consistent with pericytes and smooth muscle cells of the vascular wall. The final pathological analysis was consistent with spindle cell hemangioma.

Postoperative CourseAt the 2-month follow-up evaluation, the patient con-

tinued to improve clinically with resolution of his urinary retention and marked improvement in gait. The patient at this time was found to have only very mild weakness in left foot dorsiflexion. At the 4-month follow-up, the patient had full strength in all extremities and had complete reso-lution of his numbness. The patient exhibited improve-ment from ASIA Grade D to ASIA Grade E. Postopera-tive MRI at the 4-month interval showed no recurrence of the tumor (Fig. 5). Unfortunately, the patient was lost to follow-up after the 4-month office visit.

DiscussionSpindle cell hemangiomas are vascular epithelioid

Fig. 1. Preoperative MRI demonstrating an intradural intramedul-lary mass at T6–8. a: Sagittal T1-weighted precontrast (upper) and axial T1-weighted postcontrast (lower) images. b: Sagittal postcontrast image. C: Sagittal T2-weighted image.

Fig. 2. Intraoperative photographs of the vascular lesion on the spinal cord surface. Figure is available in color online only.

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intramedullary spindle cell hemangioma

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tumors that rarely occur in the spine, with only 3 cases previously reported, all in the intradural, extramedullary space.6,9,10

These tumors are genetically associated with syn-dromes such as Ollier disease, Klippel-Trenaunay syn-drome, and Maffucci syndrome.8,11 The patient presented did not have a genetic predisposition toward developing such a lesion. Histologically, spindle cell hemangiomas demonstrate many dilated vascular channels with inter-spersed spindle cells. Immunohistochemical analysis is significant for positive staining for VIM, SMA, and CD34.10

Although the natural history of this tumor is not fully understood, spindle cell hemangiomas are regarded as in-dolent in nature.7 In the present case, there is no evidence of recurrence. Ding et al. described these tumors as arising from abnormal vasculature,2 while others implicate varia-tions in blood flow creating alternating areas of expansion and collapse.3,11 Although the lesion is of vascular etiol-ogy and preoperative embolization has been described for spindle cell hemangiomas in the vertebral column,13 our experience in this case was that intraoperative bleed-ing was readily controlled and preoperative embolization would have been unnecessary.

Gross-total resection remains the preferred treatment modality.6 Postoperatively, there is a 60% recurrence rate according to Fukunaga et al.4 Ideally, patients with these tumors should be followed closely because of this high recurrence rate and the unknown tumor biology they may exhibit.12 Unfortunately in the present case, the patient

was lost to follow-up after 4 months. Radiosurgery has been used by some authors, especially for postoperative tumor recurrence.6 However, this would not likely be ap-propriate for an intramedullary lesion. Mahdavi et al. de-scribed the use of alpha interferon therapy in instances of recurrence.6

Spindle cell hemangiomas are vascular epithelioid tu-mors that occur very rarely in the spine. In this paper we report the first instance of an intramedullary spindle cell hemangioma in the thoracic spinal cord. Intraoperative

Fig. 4. Immediate postoperative sagittal (upper) and axial (lower) MR images, demonstrating gross-total resection of an intradural, intramedul-lary mass.

Fig. 3. Successive D-waves to single-pulse transcranial electrical stim-ulation (TCES), recorded from an epidural electrode caudal to the tumor during tumor resection. TCES preferentially stimulates the cortex under the anode. Each waveform is the average of the responses to 50 stimuli delivered at a rate of 12.2 per second.

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appearance was similar to hemangioblastoma, and gross-total resection was possible. Follow-up showed excellent recovery of neurological function with no progression of the tumor at the 4-month mark.

acknowledgmentsWe would like to thank Dr. Jonathan Nakhla for his hard work

and diligence.

references 1. American Spinal Injury Association/International Medical

Society of Paraplegia: International Standards for Neuro-logical and Functional Classification of Spinal Cord In-jury Patients. Chicago: American Spinal Injury Association, 2000

2. Ding J, Hashimoto H, Imayama S, Tsuneyoshi M, Enjoji M: Spindle cell haemangioendothelioma: probably a benign vas-cular lesion not a low-grade angiosarcoma. A clinicopatho-logical, ultrastructural and immunohistochemical study. Vir-chows Arch A Pathol Anat Histopathol 420:77–85, 1992

3. Enjolras O, Wassef M, Merland JJ: [Maffucci syndrome: a false venous malformation? A case with hemangioendo-thelioma with fusiform cells.] Ann Dermatol Venereol 125:512–515, 1998 (Fr)

4. Fukunaga M, Ushigome S, Nikaido T, Ishikawa E, Nakamori K: Spindle cell hemangioendothelioma: an immunohisto-chemical and flow cytometric study of six cases. Pathol Int 45:589–595, 1995

5. MacAvoy MC, Green DP: Critical reappraisal of Medical Research Council muscle testing for elbow flexion. J Hand Surg Am 32:149–153, 2007

6. Mahdavi Z, Grafe MR, Ostrup R, Kormanik P, Chamberlain MC: Spindle cell hemangioendothelioma of the spinal cord. J Neurooncol 27:231–234, 1996

7. Murakami I, Sarker AB, Teramoto N, Horie Y, Taguchi K, Akagi T: Spindle cell hemangioendothelioma: a report of two cases. Acta Pathol Jpn 43:529–534, 1993

8. O’Hara CD, Nascimento AG: Endothelial lesions of soft tis-sues: a review of reactive and neoplastic entities with empha-sis on low-grade malignant (“borderline”) vascular tumors. Adv Anat Pathol 10:69–87, 2003

9. Roncaroli F, Scheithauer BW, Krauss WE: Hemangioma of spinal nerve root. J Neurosurg 91 (2 Suppl):175–180, 1999

10. Talan-Hranilović J, Vucić M, Sajko T, Bedek D, Tomić K, Lupret V: Spinal spindle cell haemangioma: an atypical loca-tion. Acta Neurochir (Wien) 149:325–328, 2007

11. Tosios K, Koutlas IG, Kapranos N, Papanicolaou SI: Spindle-cell hemangioendothelioma of the oral cavity. A case report. J Oral Pathol Med 24:379–382, 1995

12. Weiss SW, Enzinger FM: Spindle cell haemangioendothe-lioma: a low-grade angiosarcoma resembling a cavernous hemangioma and Kaposi’s sarcoma. Am J Surg Pathol 10:521–530, 1986

13. Winter A, Siu A, Jamshidi A, Malawer M, Sherman JH: Spindle cell hemangioendothelioma of the sacrum: case re-port. J Neurosurg Spine 21:275–278, 2014

DisclosuresThe authors report no conflict of interest concerning the materi-als or methods used in this study or the findings specified in this paper.

author ContributionsConception and design: Nasser, Houten. Acquisition of data: Nasser, Legatt, Houten. Analysis and interpretation of data: Ashayeri, Nasser. Drafting the article: Ashayeri, Nasser, Houten. Critically revising the article: all authors. Reviewed submitted version of manuscript: Ashayeri, Legatt, Houten. Approved the final version of the manuscript on behalf of all authors: Ashayeri. Study supervision: Houten.

CorrespondenceKimberly Ashayeri, Montefiore Medical Center, Department of Neurosurgery, 3316 Rochambeau Ave., Bronx, NY 10467. email: kimberly.ashayeri@gmail.com.

Fig. 5. Four-month postoperative sagittal (upper) and axial (lower) MR images showing no evidence of recurrent tumor.

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