Capillary hemangiomas are benign vascular neoplasms. When associated with the spine, these growths frequently involve the vertebral body, but rarely have they been reported to occur as intradural lesions, while even more rarely occurring in a true intramedullary location. We report a rare case of an intramedullary capillary hemangioma of the thoracic spinal cord and a review of the literature

Blackburn, Spiros L

Kasukurthi, Rahul

Lusis, Eriks A

Ray, Wilson Z

Santiago, Paul

Rare Tumors

English

PubMed

Ray, Wilson Z.

Blackburn, Spiros L.

Lusis, Eriks A.

Digital Commons@Becker

Washington University School of MedicineDigital Commons@BeckerOpen Access Publications2009Intramedullary capillary hemangioma of thethoracic spine: Case report and review of theliterature.Rahul KasukurthiWashington University School of Medicine in St. LouisWilson Z. RayWashington University School of Medicine in St. LouisSpiros L. BlackburnWashington University School of Medicine in St. LouisEriks A. LusisWashington University School of Medicine in St. LouisPaul SantiagoWashington University School of Medicine in St. LouisFollow this and additional works at: https://digitalcommons.wustl.edu/open_access_pubsThis Open Access Publication is brought to you for free and open access by Digital Commons@Becker. It has been accepted for inclusion in OpenAccess Publications by an authorized administrator of Digital Commons@Becker. For more information, please contact engeszer@wustl.edu.Recommended CitationKasukurthi, Rahul; Ray, Wilson Z.; Blackburn, Spiros L.; Lusis, Eriks A.; and Santiago, Paul, ,"Intramedullary capillary hemangioma ofthe thoracic spine: Case report and review of the literature.." Rare Tumors.1,1. 26-28. (2009).https://digitalcommons.wustl.edu/open_access_pubs/5815[page 26] [Rare Tumors 2009; 1:e10]Intramedullary capillaryhemangioma of the thoracicspine: case report and reviewof the literatureRahul Kasukurthi,1 Wilson Z. Ray,2Spiros L. Blackburn,2 Eriks A. Lusis,2Paul Santiago21Division of Plastic and ReconstructiveSurgery, Washington University School ofMedicine, Saint Louis, MO, USA;2Department of Neurosurgery,Washington University School ofMedicine, Saint Louis, MO, USAAbstract Capillary hemangiomas are benign vascularneoplasms. When associated with the spine,these growths frequently involve the vertebralbody, but rarely have they been reported tooccur as intradural lesions, while even morerarely occurring in a true intramedullary loca-tion. We report a rare case of an intrame-dullary capillary hemangioma of the thoracicspinal cord and a review of the literature.IntroductionCapillary hemangiomas are benign vascularneoplasms typically encountered in the skin.They occur most frequently in childhood ascutaneous or subcutaneous lesions character-ized histologically by nodules of capillary-sizedvessels lined by flattened endothelium.1 As themost common primary tumor of the spine, cap-illary hemangiomas are frequently encoun-tered as interosseous lesions. The occurrenceof these tumors in the central nervous system,however, is exceedingly rare and they are evenless frequently encountered as intramedullarylesions.2 Despite the high prevalence of verte-bral body hemangiomas, extraosseous exten-sion causing neurological impingementremains uncommon.In the present case, we report an intra-medullary capillary hemangioma of the tho-racic spinal cord, an exceedingly rare presen-tation with few previously reported cases exist-ing in the literature.2-6Case ReportThe patient is a 47-year old right-handedman with several months of non-specific mid-back pain, lower extremity hyperesthesia, anda more recent two week history of gait difficul-ties.  His past medical history is significant fora prior left-sided intracerebral hemorrhagewith consequent hemiparesis three years ago.The patient’s baseline deficits associatedwith his prior left hemisphere stroke includeddysarthria, right facial weakness, and a denseright hemiparesis.  New deficits included 3-4/5strength of the left lower extremity and a sen-sory level just above the nipples consistentwith approximately a T3 spinal level. Reflexeswere 3+ in the bilateral lower extremities.Babinski reflex was present bilaterally.  MRI of the thoracic spine both before andafter the administration of intravenousGadolinium demonstrated what initiallyappeared to be a homogenously enhancingintradural, extramedullary lesion displacingthe cord anteriorly with significant cord defor-mation (Figure 1). Our differential diagnosisincluded: nerve sheath tumor, meningioma,and ependymoma.The patient was offered an excisional biop-sy of the lesion. T2 and T3 laminectomies wereperformed, and the lesion location was con-firmed using intra-operative ultrasound.Intradural exploration revealed a highly vascu-lar, partially encapsulated cherry-red lesionthat appeared to arise from the dorsal aspect ofthe spinal cord. In contrast to MRI findings, thetumor was found to be intramedullary in loca-tion. Overlying the tumor was a region ofthickened arachnoid, but no demonstratabledural attachment. Several involved nerve rootswere coagulated and divided. Further dissec-tion revealed an extremely friable exophytictumor arising from the region of the dorsalroot entry zone of T3. Intra-operative frozensection was consistent with a possible heman-giopericytoma. Post-operative MRI showed noevidence of residual tumor.  On histopathology, the tumor grossly con-sisted of a partially circumscribed lesion with abrownish-tan appearance. H&E stainingdemonstrated a hypercellular, vascular neo-plasm composed of cytologically bland spindledcells with minimal cytoplasm. These cells werearranged in vague lobules of short irregularspindle cells and dilated vascular spaces.Immunohistochemical findings are explainedin Figure 2. Further evaluation demonstrateddilated, compressed vascular channels andfocal extramedullary hematopoiesis. In total,the findings were consistent with capillaryhemangioma.Post-operatively the patient had good func-tional recovery. At three months follow-up thepatient had only minor residual lower extrem-ity dysesthesia with return of baseline motorfunction. The patient has remained stable 36months post resection.DiscussionCapillary hemangiomas involving the spinalcord are extremely rare, and are most fre-quently extramedullary in location.1-3 We areaware of only a handful of previously reportedcases of intramedullary spinal cord capillaryhemangiomas (Table 1). Notably, all but onepreviously reported case involved the thoracicspine or the conus.  MRI is the gold standard imaging modalityfor intramedullary lesions.13-16 Many suchlesions can be safely removed with properimaging, surgical planning and technique.  Inthe case of intramedullary capillary heman-giomas, however, on MRI the lesion can beinterpreted as extramedullary,5,8,10 and the orig-inal differential diagnosis often fails to includecapillary hemangioma,2,8,11 as was the case withour patient. As previously discussed byRoncaroli et al. and Nowak et al., the treatingsurgeon must be cognizant of intramedullarycapillary hemangiomas to avoid misdiagnosisand potential excessive treatment.1,2 Althoughextremely rare, capillary hemangiomas of thespinal cord have a benign clinical course. Glialbased tumors, such as ependymomas andastrocytomas, account for over 90% ofintramedullary tumors.17 The distinctive grossappearance of capillary hemangiomas intra-operatively often eliminates the more commonglial based lesions from the differential diag-nosis. The remaining possibilities includelesions ranging from: hemangiopericytoma,hemangioblastoma, cavernous angioma, heman-gioendothelioma, arteriovenous malforma-tions, venous angiomas, and capillary telang-iectasias. Fortunately, histopathological analy-sis reliably distinguishes these lesions fordiagnosis.Although related most closely to the catego-ry of vascular malformations, capillary heman-giomas that appear within the spinal cordparenchyma may have their origins elsewhere.Rare Tumors 2009; volume 1:e10Correspondence: Wilson Z. Ray, Washington University School of Medicine,660 S. Euclid Ave, Campus Box 8057,St. Louis, MO 63110, USAKey words: intramedullary capillary hemangioma,dysembryogenesis.Received for publication: 20 June 2009.Accepted for publication: 29 June 2009.This work is licensed under a Creative CommonsAttribution 3.0 License (by-nc 3.0)©Copyright R. Kasukurthi et al., 2009Rare Tumors 2009; 1:e10doi:10.4081/rt.2009.e10[Rare Tumors 2009; 1:e10] [page 27]Case ReportTable 1. Cases of intramedullary capillary hemangiomas previously reported in English.2,4-12umor or/ Patient Imaging TumorSize/ Symptoms/ Treatment Outcome RecurrenceAuthor/ Patient Imaging Tumor size/ Symptoms/ Treatment Outcome Recurrenceyear age/sex location deficitsRoncaroli 42 not size not reported; 1.5-year h/o abdominal surgery recovered none 2000 M reported T11 pain, leg weakness reported50 not size not reported; 1-year h/o LBP, proximal surgery little none M reported T11 leg weakness bilaterally, + radiotherapy improvement reportedL-1 sensory level, loss of 2 yearspatellar reflex post-op 53 not size not reported; 2 year h/o UMN, LMN surgery leg weakness none M reported conus signs bilateral lower 1.5 years post-op reported extremities64 not size not reported; 2 year h/o bilateral leg pain, surgery recovered none M reported T10 leg weakness reported Ianelli 3 months, CT: dilated size not reported; Abnormal head surgery neurologically intact none 2005 M ventricles. T 4-7 circumference; wide, full, 1 year post-op; reportedMRI: T4-7 tense anterior fontanel no focal deficitsintramedullary with diastatic sutures.enhancing lesion Mild irritability, lethargyapparent. No other neurological signsKelleher 57 MRI: T9 & T10 2.4x1.0x0.5 cm; 7-months h/o thoracic pain; dexamethasone spastic gait none 2005 M extramedullary T9-T10 2-week h/o bilateral lower and surgery 3 months post-op reportedenhancing lesion limb weakness, decreased lower limb DTRsMawk 1 month, CT: discoid mass in size not apraxia of legs, surgery rapid recovery none 1987 not stated R buttock. reported; conus hemangiomatous reportedMyelography: malformationvascular cord lesion involving the skin and with complete soft tissue of the buttockmyelographic block at L1-2Andaluz 41 MRI: intradural, 2x1 cm, 3 month h/o surgery recovered none 2002 M extramedullary conus medullaris LBP radiating to thighs. reportedenhancing mass Decreased L  flexor strength(4+/5), bilateral patellar andAchilles areflexiaNowak 63 MRI: intradural maximum diameter 3 year h/o intermittent surgery residual paresis none 2000 F extramedullary 1.0 cm; T12-L1 Lasègue’s sign; hypesthesia of  L tibialis anterior, reportedenhancing mass and activity related 14 months post-oplumbosciatalgia L thighAbe 65 not size not 2 month h/o lower limb surgery little improvement none2004 M reported reported; T5 weakness, paraparesis 7 years post-op reportedRoncaroli 74 MRI: enhancing  lower thoracic 9-month h/o bilateral surgery no changes 1 year no new2000 M nodules on cauda cord, conus, cauda leg weakness post-op hemangiomasequina, lower thoracic spinal cord, conus medullarisShin 66 MRI: intradural 1.3x2 cm; T8-T9 8-month h/o surgery recovered no new 2000 F mass at T8-T9; LBP, weakness of the lower hemangiomasintramedullary and limbs, paraparesis, extramedullary components sensory abnormalityHida 50 MRI: Mass not reported; tetraparesis, upper  surgery recovered not 1993 M from C3-T1 C3-T1 extremities hyporeflexia, to baseline reportedlower extremities flexion spasm, decreased pain/tactile sensation below C3, bladder/bowel dysfunctionAlthough several patients had imaging studies suggesting “extramedullary” lesions, they were ultimately found to have intramedullary capillary hemangiomas. h/o = history of; UMN = upper motor neuron; LMN = lower motor neuron; LBP = lower back pain; DTR = deep tendon reflexes; R = right; L = left.[page 28] [Rare Tumors 2009; 1:e10]Case ReportSimilar to intramedullary lipomas (whichaccount for 1% of intramedullary lesions), cap-illary hemangiomas of the spinal cord may rep-resent a form of dysembryogenesis. Ratherthan being a true neoplasm, these lesionsmore likely arise from inclusion of mesenchy-mal tissue into the neural tube during primaryneurulation.18-20 The available literature clearlydemonstrates the benign nature ofintramedullary capillary hemangiomas, andgross total resection appears to be curativewith good outcomes and no reported recur-rences (Table 1). Given the rarity of thislesion, its benign course and straightforwardtherapy, surgeon familiarity is important toensure patients receive appropriate diagnosisand treatment. References1. Nowak DA, Widenka DC. Spinal intraduralcapillary haemangioma: a review. EurSpine J 2001;10:464-72.2. Roncaroli F, Scheithauer BW, Krauss WE.Capillary hemangioma of the spinal cord.Report of four cases. J Neurosurg 2000;93:148-51.3. Holtzman RN, Brisson PM, Pearl RE,Gruber ML. Lobular capillary hemangiomaof the cauda equina. Case report. JNeurosurg 1999;90:239-41.4. Iannelli A, Lupi G, Castagna M, et al.Intramedullary capillary hemangiomaassociated with hydrocephalus in aninfant. J Neurosurg 2005;103:272-6.5. Kelleher T, Aquilina K, Keohane C,O'Sullivan MG. Intramedullary capillaryhaemangioma. Br J Neurosurg 2005;19:345-8.6. Mawk JR, Leibrock LG, McComb RD,Trembath EJ. Metameric capillary heman-gioma producing complete myelographicblock in an infant. Case report. J Neuro-surg 1987;67:456-9.7. Abe M, Tabuchi K, Tanaka S, et al.Capillary hemangioma of the central nerv-ous system. J Neurosurg 2004;101:73-81.8. Andaluz N, Balko MG, Stanek J, et al.Lobular capillary hemangioma of thespinal cord: case report and review of theliterature. J Neurooncol 2002;56:261-4.9. Hida K, Tada M, Iwasaki Y, Abe H.Intramedullary disseminated capillaryhemangioma with localized spinal cordswelling: case report. Neurosurgery1993;33:1099-101.10. Nowak DA, Gumprecht H, Stolzle A,Lumenta CB. Intraneural growth of a cap-illary haemangioma of the cauda equina.Acta Neurochir (Wien) 2000;142:463-7;discussion 7-8.11. Roncaroli F, Scheithauer BW, Deen HG.Multiple hemangiomas (hemangiomato-sis) of the cauda equina and spinal cord.Case report. J Neurosurg 2000;92:229-32.12. Shin JH, Lee HK, Jeon SR, Park SH. Spinalintradural capillary hemangioma: MR find-ings. AJNR Am J Neuroradiol 2000;21:954-6.13. Brotchi J, Dewitte O, Levivier M, et al. Asurvey of 65 tumors within the spinal cord:surgical results and the importance of pre-operative magnetic resonance imaging.Neurosurgery 1991;29:651-6.14. Rothwell CI, Jaspan T, Worthington BS,Holland IM. Gadolinium-enhanced mag-netic resonance imaging of spinaltumours. Br J Radiol 1989;62:1067-74.15. Scotti G, Scialfa G, Colombo N, Landoni L.Magnetic resonance diagnosis of intra-medullary tumors of the spinal cord.Neuroradiology 1987;29:130-5.16. Goy AM, Pinto RS, Raghavendra BN, et al.Intramedullary spinal cord tumors: MRimaging, with emphasis on associatedcysts. Radiology 1986;161:381-6.17. Miller DJ, McCutcheon IE. Hemangio-blastomas and other uncommonintramedullary tumors. J Neurooncol 2000;47:253-70.18. Muraszko K, Youkilis A. Intramedullaryspinal tumors of disordered embryogene-sis. J Neurooncol 2000;47:271-81.19. Tihan T, Chi JH, McCormick PC, et al.Pathologic and epidemiologic findings ofintramedullary spinal cord tumors.Neurosurg Clin N Am 2006;17:7-11.20. Warf BC, Scott RM, Barnes PD, HendrenWH. Tethered spinal cord in patients withanorectal and urogenital malformations.Pediatr Neurosurg 1993;19:25-30.Figure 1. (A) Sagittal T1-weighted MRimage with Gadolinium revealing a homo-geneously hyperintense mass involving theupper thoracic spine. (B) Axial T1-weight-ed MR image with Gadolinium demon-strating a centrally located lesion. Figure 2. (A) Photomicrograph of H&Estain shows a hypervascular tumor com-posed of thin and irregular capillary ves-sels and minimal cytoplasm. Magni-fication X 200.  (B) Immunohisto-chemi-cal staining revealed the tumor cells to bestrongly and diffusely immunoreactive forCD31, CD34, and CD99. Scattered S-100protein and BCL-2 positive cells werepresent.  Epithelial membrane antigen(EMA) and inhibin stains were negativeand MIB-1 labeling was brisk, but withrare mitoses. Magnification X 200. A ABB

Intramedullary capillary hemangioma of the thoracic spine: Case report and review of the literature.

Capillary hemangiomas are benign vascular neoplasms. When associated with the spine, these growths frequently involve the vertebral body, but rarely have they been reported to occur as intradural lesions, while even more rarely occurring in a true intramedullary location. We report a rare case of an intrame-dullary capillary hemangioma of the thoracic spinal cord and a review of the literature

Paul Santiago

Eriks A. Lusis

Wilson Z. Ray

Rahul Kasukurthi

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Intramedullary capillary hemangioma of the thoracic spine: case report and review of the literature

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