. Intraosseous Hemangioma. PORTNotes. In: OrthopaedicsOne - The Orthopaedic Knowledge Network. Created Feb 16, 2009 16:50. Last modified Mar 26, 2015 12:21 ver.11. Retrieved 2016-05-27, from http://www.orthopaedicsone.com/x/HQEjAQ.
DEFINITION AND PATHOGENESIS
- Benign lesion with thin-walled capillary to cavernous vessels within bone, occasionally associated with a soft tissue mass which probable represents a hamartomatous condition
- Variable biologic activity resulting in little or no sxs to an aggressive clinical picture necessitating differentiation from angiosarcoma
- Classification of vascular anomaliesaccording to the International Society for the Study of Vascular Anomalies (ISSVA)
- Infantile hemangioma
- Epithelioid hemangioma
- Spindle cell hemangioma
- Hemangioendothelioma NOS
- Epithelioid hemangioendothelioma
- Other tumors
- Arteriovenous malformation
- Capillary-venous malformation
- Capillary-lymphatic-venous malformation
- Lymphatic-venous malformation
- Capillary-arteriovenous malformation
- Capillary-lymphatic-arteriovenous malformation
- 75% between 20-60 years
- M:F = 1.2
- 1% of biopsied primary bone tumors
- Skull and mandible (47%) and spine (28%), also femur, humerus
- 1-7cm in size
- Incidental finding (10.7% autopsied spines), spinal cord compressive sxs with epidural extension, loosening of teeth and bleeding
- Complete paraplegia has been reported, with near complete recovery with decompression and fixation
- Preoperative embolization has been utilized
- "Corduroy cloth", "jailhouse", or "honeycombing" pathognomonic pattern
- "Polka dot" sign on CT
- Spine lesions centered in body, may extend to arch or disk
- Contiguous spinal bones or ribs may be involved
- Extradural mass from tumor or hemorrhage ? soft tissue phleboliths
- En face:
- Round to oval focus of lucency
- Honeycombed reticulated pattern
- "Sunburst" pattern
- Tangential projection:Dome shaped outer table
- "Sunray"/"spokewheel" pattern
- Inner table may bulge inward
- En face:
- Long Bones
- ? bony expansion
- Mixed sclerotic, lucent lesion
- Both T1- and T2-weighted images with ? uptake due to intralesional fat and blood
- Honeycombed trabecular arrangement in spine and long bones, and "sun ray" bone pattern on cut surface of the bone in skull lesions
- Gross hemorrhage, cystic areas
- Blood filled vascular channels/vessel proliferation; - anaplasia
- Residual bone trabeculae along lines of stress (spokes of a wheel in the skull)
- Capillary, cavernous, mixed, cellular, histiocytoid
- Special stains:
- Factor VIII +
- Weibel-Palade bodies
- Sclerosing hemangioma associated with hypophosphatemic rickets
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Fibrous dysplasia
- Meningioma (skull lesions)
- Malignant hemangioendothelioma
- R/O other causes of pain, i.e., HNP
- Metastatic bone disease
- Lymphoma of bone in densely sclerotic vertebral lesions
- Paget's disease
DISEASE COURSE AND TREATMENT
- Requires accurate dx, usually no tx unless associated with fx or soft tissue mass compressing cord
- Plain radiographs, MRI, or CT usually diagnostic without further w/u
- Pathologic fx may complicate diagnostic w/u and may necessitate bx
- Resection of a thoracic vertebral hemangioma under deep hypothermia and circulatory arrest has been reported (resection under routine measures usually adequate when neurologic deficit or pathologic fx has occurred)
- XRT has been advocated for spinal lesions in the past, although complete resection and decompression has been recently used
- Embolization to ? intraoperative bleeding (avoid the artery of Adamkiewicz!)
- Brown-Sequard syndrome has been reported after intralesional absolute alcohol injection into a T8 vertebral hemangioma
- When confined to bones, affects a region, such as multiple vertebrae
- May be associated with Osler-Weber-Rendu Disease
- Tumor of the glomus body (probably regulates blood flow in response to temperature)
- Often occurs subungually eroding into bone and causing a geographic lucent lesion, an innervated AV anastomotic lesion of the perivascular muscular glomus cell
- Unusual locations include middle phalanx, proximal ulna, sacrum, coccyx
- Highly vascular with round to cuboidal cells in sheets or nests
- Immunoreactive for (nerve fiber) substance p
- Curettage curative
PERIOSTEAL GLOMUS TUMOR
- Has been reported in a periosteal location of a long bone (femur)
PERIOSTEAL (SURFACE) and INTRACORTICAL HEMANGIOMA
- May appear radiographically identical to osteoid osteoma or Brodie's abscess
- Cortical thickening or erosion around a small lucent region; uptake on bone scan. MR with intermediate signal on T1 and T2
- Tibia 45%, fibula 36%, femur 9%, ulna 9%
- May be associated with significant pain and excision of the soft-tissue component (not the sclerosed bone) has been advocated
- Vascular proliferation of the medullary cavity and at least one other type of tissue
- Usually, multiple bones are involved
- Extraskeletal sites include GI tract, mediastinum, lung, spleen, paraspinal soft tissue, soft tissue adjacent to bone
- Histologic patterns:
- Cavernous lymphangioma
- Cavernous hemangioma
- Arteriovenous hemangioma
DISAPPEARING BONE DISEASE OF GORHAM
- (Gorham-Stout syndrome/disease, vanishing bone disease, acute spontaneous absorption fo bone, phantom bone)
- Unpredictably progressive disease affecting children and young adults related to hemangioma whcih radiographically casues bony disappearance, even crossing joints
- Histologcial findings of # of stimulated osteoclasts
- LR is the rule despite curettage, bone grafting, and multiple modalities reported
- Death may occur when disease localization leads to respiratory failure, airway obstruction, or spinal cord compression
- An intermediate (locally aggressive, rarely metastasizing) vascular tumor of bone
- Long tubular bones (40%)
- Short tubular bones of the distal lower extremity (18%)
- Flat bones (18%)
- Vertebrae (16%)
- Small bones of the hands (8%)
- Contiguous bone involvement has been reported
- Left posterior 10th rib and T9-10 reported
- Intralesional resection, laminectomy, and spinal fusion with stable disease at 3 years reported
- Left posterior 10th rib and T9-10 reported
- Radiologic evaltion
- Radiographs reveal well marginated lucency
- Successfully txd with curettage or marginal en bloc excision
- Disease course
Albrecht S, Zbieranowski I: Incidental glomus coccygeum: when a normal structure looks like a tumor. Am J Surg Pathol 14:922-4,1990.
Bansal VP, Singh R, Grewal DS, : Haemangioma of the patella. J Bone Joint Surg 56B:139-41,1974.
Bednar DA, Essess SI: Double hemangioma of the spine with paraparesis. Spine 15:1377-9,1990.
Ben Romdhane K, Khattech R, Ben Othman M. Epithelioid hemangioma of bone. Am J Surg Pathol. 1994 Dec;18(12):1270-1. Pubmed
Bjorkengren AG, Resnick D, Haghighi P, Sartoris DJ: Intraosseous glomus tumor: report of a case and review of the literature. Am J Roentgenol 147:739-41,1986.
Blankstein A, Spiegelmann R, Shacked I, Schinder E, Chechick A: Hemangioma of the thoracic spine involving multiple adjacent levels: case report. Paraplegia 26:186-91,1988.
Bruder E, Perez-Atayde AR, Jundt G, Alomari AI, Rischewski J, Fishman SJ, Mulliken JB, Kozakewich HP. Vascular lesions of bone in children, adolescents, and young adults. A clinicopathologic reappraisal and application of the ISSVA classification. Virchows Arch. 2009 Feb;454(2):161-79. Pubmed
Buckley SL, Burkus JD: Intraosseous limpoma of the ilium: a case report. Clin Orthop 228:297-301,1988.
Chan CW: Intraosseous glomus tumor: a case report. J Hand SUrg 6A:368-9,1981.
Devaney K, Tuyethoa NV, Sweet D: Surface-based hemangiomas of bone - a review of 11 cases. Clin Orthop 300:233-9,1994.
Djindjan M, Nguyen JP, Gaston A, Pavlovitch JM, Poirier J, Awad IA: Multiple vertebral hemangiomas with neurological signs: case report. J Neurosurg 76:1025-8,1992.
Dorfman HD, Steiner GC, Jaffe HL: Vascular tumors of bone. Hum Pathol 2:349-76,1971.
Downey EFJ, Brower AC, Holt RB: Case report 243: intraosseous lipoma of the femur. Skeletal Radiol 10:189-91,1983.
Drape JL, Idy-Peretti I, Goettmann S, : Subungual glomus tumors: evaluation with MR imaging. Radiolgoy 195:507- ,1995.
Duncan L, Halverson J, DeSchryver-Kescskemeti K: Glomus tumor of the coccyx. A curable cause of coccygodynia. Arch Pathol Lab Med 115:78-80,1991.
Eisenstein S, Spiro F, Browde S, Lewer Allen CM, Grobler L: The treatment of symptomatic vertebral hemangioma by radiotherapy. Spine 11:640-2,1985.
El-Atta MA, Ivancovich SM, Braunstein EM: Intraosseous lipoma. Arthritis Rheum 40:978-9,1997.
Enzinger FM, Weiss SW: Benign tumors and tumorlike lesions of blood vessels. In Soft Tissue Tumors. FM Enzinger and SW Weiss (Ed), 2nd ed., C. V. Mosby Co., St. Louis, 1988, pp. 489-532.
Enzinger FM, Weiss SW: Glomus tumor. In Soft Tissue Tumors. FM Enzinger and SW Weiss (Ed), 2nd ed., C. V. Mosby Co., St. Louis, 1988, pp. 581-595.
Evans HL, Raymond AK, Ayala AG. Vascular tumors of bone: A study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and high-grade angiosarcoma. Hum Pathol. 2003 Jul;34(7):680-9. Pubmed
Floris G, Deraedt K, Samson I, Brys P, Sciot R. Epithelioid hemangioma of bone: a potentially metastasizing tumor? Int J Surg Pathol. 2006 Jan;14(1):9-15; discussion 16-20.
Fox M, Onofrio BM: The natural history and management of symptomatic and asymptomatic vertebral hemagiomas. J Neurosurg 78:36-45,1993.
Freiberg RA, Air GW, Glueck CJ, : Multiple intraosseous lipoma with Type IV-hyperlipoproteinemia: a case report. J Bone Joint Surg 56A:1729-32,1974.
Gonzalez JV, Stuck RM, Steit N: Intraosseous lipoma of the calcaneus: a clinicopathologic study of three cases. J Foot Ankle Surg 36:306-10,1997.
González-Llanos F, López-Barea F, Isla A, Fernández-Prieto A, Zubillaga A, Alvarez F: Periosteal glomus tumor of the femur. Clin Orthop 380:199-203,2000.
Graham JJ, Yang WC: Vertebral hemangioma with compression fracture and paraparesis treated with preoperative embolization and vertebral resection. 9:97-101,1984.
Greenspan A, Raiszadeh K, Riley GM, : Intraosseous lipoma of the calcaneus. Foot Ankle Int 18:53-6,1997.
Hall F, Goldberg R, Kasdon E, White AA: Case report 131. Skeletal Radiol 5:275-8,1980.
Hart JAL: Intraosseous lipoma. J Bone Joint Surg 55B:624-32,1973.
Ide C, Gangi A, Rimmelin A, Beaujeux R, Maitrot D, Buchneit F, Sellal F, Dietmann JL: Vertebral haemangiomas with spinal cord compression: the place of preoperative percutaneous vertebroplasty with methyl methacrylate. Neuroradiology 38:585-9,1996.
Idy-Peretti I, Cermakova E, Dion E, Reygagne P: Subungual glomus tumor: diagnosis based on high-resolution MR images. Am J Roentgenol 159:1351- ,1992.
Johnson DL, Kuschner SH, Lane CS: Intraosseous glomus tumor of the phalanx: a case report. J Hand Surg 18A:1026-8,1993.
Keel SB, Rosenberg AE. Hemorrhagic epithelioid and spindle cell hemangioma: a newly recognized, unique vascular tumor of bone. Cancer. 1999 May 1;85(9):1966-72. Pubmed
Kenan S, Abdelwahab IF, Klein M, Lewis MM: Hemangiomas of the long tubular bone. Clin Orthop 280:256-60,1992.
Kenan S, Bonar S, Jones C, Lewis MM: Subperiosteal hemangioma - a case report. Clin Orthop 232:279-83,1988.
Kindblom-Meis JM, Enzinger FM: Color Atlas of Soft Tissue Tumors. Mosby-Wolfe, Portland, 1996, pp 74-105, 138-178.
Lamovec J, Bracko M. Epithelioid hemangioma of small tubular bones: a report of three cases, two of them associated with pregnancy. Mod Pathol. 1996 Aug;9(8):821-7. Pubmed
Laredo JD, Assouline E, Gelbert F, Wybier M, Merlan JJ, Tubiana JM: Vertebral hemangiomas; fat content as a sign of aggressiveness. Radiology 177:467-72,1990.
Laredo JD, Reizine D, Bard M, Merland JJ: Vertebral hemangiomas: radiologic evaluation. Radiology 161:183-9,1986.
Lee S, Hadlow AT: Extraosseous extension of vertebral hemangioma: a rare cause of spinal cord compression. Spine 24:2111-4,1999.
Levin MF, Vellet AD, Munk PL, : Intraosseous lipoma of the distal femur: MRI appearance. Skeletal Radiol 25:82-4,1996.
Lewis VO, Montag AG, Simon MA. Epithelioid hemangiomas of bone: spontaneous clinical and radiographic remission. Clin Orthop Relat Res. 2003 Feb;(407):167-72. Pubmed
Ling S, Rafii M, Klein M. Epithelioid hemangioma of bone. Skeletal Radiol. 2001 Apr;30(4):226-9. Pubmed
Liu CL, Yang DJ: Paraplegia due to vertebral hemangioma during pregnancy: a case report. Spine 13:107-8,1988.
Loxley S, Thiemeyer J, Ellsasser J: Periosteal hemangioma - a report of two cases. Clin Orthop 85:151-4,1972.
Luna JT, DeGroot H 3rd. Five-year follow-up of structural allograft reconstruction for epithelioid hemangioma of the talus and navicular: a case report and review of the literature. Foot Ankle Int. 2007 Mar;28(3):379-84.
Mackenzie DH: Intraosseous glomus tumours. Report of two cases. J Bone Joint Surg 44B:648-51,1962.
Möller G, Priemel M, Amling M, Werner M, Kuhlmey AS, Delling G: The Gorham-Stout syndrome (Gorham's massive osteolysis). J Bone Joint Surg 81B:501-6,1999.
Newmark J, Jones HR, Jr, Thomas CB, Aretz HT, Freiberg SR, Baker RA: Vertebral haemangioma causing acute recurrent spinal cord compression. J Neurol Neurosurg Psychiatry 54:471,1991.
Nielsen GP, Srivastava A, Kattapuram S, Deshpande V, O'Connell JX, Mangham CD, Rosenberg AE. Epithelioid hemangioma of bone revisited: a study of 50 cases. Am J Surg Pathol. 2009 Feb;33(2):270-7. Pubmed
O'Connell JX, Kattapuram SV, Mankin HJ, Bhan AK, Rosenberg AE. Epithelioid hemangioma of bone. A tumor often mistaken for low-grade angiosarcoma or malignant hemangioendothelioma. Am J Surg Pathol. 1993 Jun;17(6):610-7. Pubmed
Pena JM, Calone JA, Ortega F, Marco A, Martinez A, Saez F: Periosteal hemangioma of the left fibula. Case report 324. Skeletal Radiol 14:133-5,1985.
Ross JS, Masaryk TJ, Modic MT, Cartar JR, Mapstone T, Dendel FH: Vertebral hemangiomas: MR imaging. Radiology 165:165-9,1987.
Rozmaryn LM, Sadler AH, Dorfman HD: Intraosseous glomus tumor in the ulna. A Case report. Clin Orthop 220:126-9,1987.
Ryoppy S, Poussa M, Heiskanen O, Leijala M, Peltola K: Resection of a thoracic vertebra for hemangioma--operation under deep hypothermia and circulatory arrest: a case report. J Bone Joint Surg 72A:1245-9,1990.
Sar C, Eralp L: Double thoracic vertebral hemangioma causing complete paraplegia. Am J Orthop 33:81-4,2004.
Schajowicz F, Rebecchini AC, Bosch-Mayol G: Intracortical hemangioma simulating osteoid osteoma. J Bone Joint Surg 61B:94-5,1979.
Schwartz DA, Nair S, Hersey B, Winkelman AC, Finkelstein SD: Vertebral arch hemangioma producing spinal cord compression in pregnancy. Spine 14:888-90,1989.
Simmons TJ, Bassler TJ, Schwinn CP, Forrester DM: Case report 749: Primary glomus tumor of bone. Skeletal Radiol 21:407-9,1992.
Siegel MW: Intraosseous glomus tumor: a case report. Am J Orthop 9:68-9,1967.
Siegal T, Siegal T: Current considerations in the management of neoplastic spinal cord compression. Spine 14:223-8,1989.
Siegal T, Siegal T: Neoplastic epidural spinal cord compression: pathophysiology and prognostic factors. Semin Spine Sur 7:269-76,1995.
Sirikulchayanonta V, Jinawath A, Jaovisidha S. Epithelioid hemangioma involving three contiguous bones: a case report with a review of the literature. Korean J Radiol. 2010 Nov-Dec;11(6):692-6. Pubmed
Sugiura I: Intr-osseous glomus tumour. A case report. J Bone Joint Surg 58B:245-7,1976.
Sugiura I: Tibial periosteal hemangioma. Clin Orthop 106:242-4,1975.
Sun ZJ, Zhang L, Zhang WF, Liu B, Li ZB, Zhao YF. A possible hypoxia-induced endothelial proliferation in the pathogenesis of epithelioid hemangioma. Med Hypotheses. 2006;67(5):1133-5.
Sung MS, Kim YS, Resnick D. Epithelioid hemangioma of bone. Skeletal Radiol. 2000 Sep;29(9):530-4. PubmedVerbeke SLj, Bovée JV. Primary vascular tumors of bone: a spectrum of entities? Int J Clin Exp Pathol. 2011 Aug 15;4(6):541-51. Pubmed
Weiner SD, Muniyappa P: Surface hemangiomas presenting as bone lesions. Orthopedics 22:539-41,1999.
Willinsky RA, Rubenstein JD, Cruickshank B: Case report 216: intracortical hemangioma of the tibia. Skeletal Radiol 9:137-9,1982.
Yao L, Lee JK: Case report 494: Hemangioma of the surface of the ulna with prominent sclerosis. Skeletal Radiol 17:378-81,1988.
Yazici M, Iyigun OL, Gulman B, Rakunt C, Cizmeli O: Vertebral hemangioma presenting with intermittent claudication. Eur Spine J 5:131-3,1996.
Yochum TR, Lile RL, Schultz GD, Mick TJ, Brown CW: Acquired spinal stenosis secondary to an expanding thoracic vertebral hemangioma. 18:299-305,1993.