Access Keys:
Skip to content (Access Key - 0)

Hemangioma

DEFINITION AND PATHOGENESIS

  • Capillary hemangioma
  • Cavernous hemangioma
  • Arteriovenous hemangioma
  • Venous hemangioma
  • Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia)
  • Pyogenic granuloma (granulation tissue type capillary hemangioma)
  • Intramuscular hemangioma
  • Synovial hemangioma
  • Hemangioma of peripheral nerve (perineural hemangioma)
  • Angiomatosis

IMPORTANCE

  • The most common tumor of childhood

CLINICAL FEATURES

  • 7% of all benign tumors, the most common tumor in infancy and childhood; may present as a painless mass, variation in size
  • 60% of synovial hemangiomas occur in the knee
    • Swelling and joint pain
    • Limitation of motion reported in hip location
    • Spontaneous hemarthrosis/monarthritis in children and adolescents
  • Overlying skin discoloration may be present
  • May become clinically apparent during pregnancy
  • Pulsatile or audible bruit rarely
  • Tender, spongy mass that may decrease in size with palpation or elevation
  • Contractures have been reported
  • Bilateral 1st dorsal compartment hemangiomas reported causing bilateral deQuervain's stenosing tenosynovitis
  • Locking of a MCPJ by a volar plate hemangioma reported
  • Neuropathies have been reported: PIN, ulnar nerve at the cubital tunnel
    • FDP flexor sheath location reported as a cause of CTS
  • Unusual location

RADIOLOGIC FEATURES

  • Mineralization types
  • Phleboliths (49%) (on radiographs or CT)
  • Nonspecific amorphous or curvilinear
  • Metaplastic ossification
  • ± cortical erosions or sclerosis in long-standing lesions
  • LLD has been reported
  • MRIreveals worrisome indistinct margins; MRA diagnostic
    • T1-weighted images reveal poorly marginated mass isointense with muscle
      • Some areas isointense with fat can be seen
    • T2-weighted images show marked ? signal
    • Serpiginous regions
    • Robust contrast enhancement
    • ± effusion in joint locations
  • High flow signal, vascular channels
  • The combination of lobulation, septation, and central low-signal-intensity dots (not seen on gadolinium-enhanced images) on MRI specific for hemangioma

GROSS PATHOLOGY

  • Tortuous blood vessels within subcutaneous or muscular tissues

HISTOLOGIC FEATURES

  • Depends on type, but with a myriad of dilatedvessels with plump nuclei within the cells
    • Cavernous
    • Lobular capillary
    • Arteriovenous
    • Venous
  • EM:
    • Weibel-Palade bodies

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Soft tissue sarcomas, esp angiosarcoma
  • Vascular malformation
  • Intravascular papillary endothelial hyperplasia
  • Hemangioendothelioma
  • Hemangiopericytoma
  • Kaposi's sarcoma
  • PVNS or synovial chondromatosis in the joint location
  • Synovial sarcoma

DISEASE COURSE AND TREATMENT

  • Observation in asymptomatic pts after adequate bx
  • Intramuscular hemangiomas must be suspected prior to bx or bleeding may be a problem
  • Large unresectable hemangioma tx is controversial
  • Percutaneous embolization may require several txs
  • LR often because of incomplete excision

SPECIAL CONSIDERATIONS

  • INFANTILE HEMANGIOMA

  • ANGIOMATOSIS

  • KASABACH-MERRITT SYNDROME

    • Thrombocytopenia purpura associated usually with a cavernous hemangioma of an extremity and trauma such as a femoral fx
    • Surgery can be complicated by bleeding due to platelet consumption from sequestration in sinusoids, secondary fibrinolysis, hypofibrinogenemia, ? fibrin split products, and DIC
    • Resuscitation includes FFP, cryoprecipitate, platelets
    • Right posterior triangle kaposiform hemangiomaextending to skull base resulting in respiratory compromise and spinal cord compression in an infant reported
      • Corticosteroids, medical managment, embolization and vincristine reported 
    • Hematology consultation!
  • MAFFUCCI'S SYNDROME

    • Multiple hemangiomas and enchondromas with a high rate of malignant degeneration of either type lesion
  • KLIPPEL-TRENAUNAY SYNDROME

    • Gigantism (bone and soft tissue hypertrophy with LLD), massive cavernous hemangiomas and varicosities (laterally in a 'lumbar-to-foot' pattern)
    • (Also at risk for Wilms' tumor)
    • High output cardiac failure can occur necessitating tx
    • Total circulatory arrest in infants for surgical resection has been described
    • May require amputation for functional or cosmetic reasons (pre-prosthetic consultations)
    • Thrombophlebitis, DVT/PE
    • 95% LE, 85% unilateral
  • EPIDURAL SPINAL CAVERNOUS HEMANGIOMA

    • Pure epidural lesion rare
    • Low to intermediate signal on T1-weighted and high signal on T2-weighted and proton density images
    • Laminectomy and microsurgical resection when symptomatically indicated

REFERENCES

Akgun I, Kesmezacar H, Ogut T, Dervisoglu S: Intra-articular hemagnioma of the knee. Arthroscopy 19:E17,2003.

Bieber EJ, Moore JR, Weiland AJ: Lipomas compressing the radial nerve at the elbow. J Hand Surg 11A:533-5,1986.
Boe S: Synovial hemangioma of the knee joint. Arthroscopy 2:178-80,1986.

Bonaga S, Bardi C, Gigante C, Turra S: Synovial involvement in hemangiomatosis. Arch Orthop Trauma Surg 123:102-6,2003.

Buetow PC, Kransdorf MF, Moser RP Jr, Jelink JS, Berrey BH: Radiologic appearance of intramuscular hemangioma with emphasis on MR imaging. AJR 154:563-7,1990.
Burrows PE, Mulliken JB, Fellows KE, : Chlidhood hemangiomas and vascular malformations: angiographic differentiation. AJR 141:483- ,1983.
Busa R, Adani R, Marcuzzi A, Caroli A: Acute posterior interosseous nerve palsy caused by a synovial haemangioma of the elbow joint. J Hand Surg 20B:652-4,1995.
Cai Q, Hodgson SF, Kao PC, Lennon VA, Klee GG, Zinsmiester AR, Kumer R: Brief report: inhibition of renal phosphate transport by a tumor product in a patient with oncogenic osteomalacia. N Engl J Med 330:1645-9,1994.
Chang E, Boyd A, Nelson CC, Crowley D, Law T, Keough KM, Folkman J, Ezekowitz RA, Castle VP: Successful treatment of infantile hemangiomas with interferon-alpha-2b. J Pediatr Hematol Oncol 19:237-44,1997.
Chatha DS, Rybak LD, Wittig JC, Desai P: Elbow mass in a 9-year-old girl. Clin Orthop 463:237-43,2007.

Cohen EK, Kressel HY, Perosio T, Burk DL, Jr, Dalinka MK, Kanal E, Schiebler ML, Fallon MD: MR imaging of soft-tissue hemangiomas: correlation with pathologic findings. AJR 150:1079-81,1988.
Cotten A, Flipo RM, Hervaux B, Gougeon F, Lecomte-Houcke M, Chastenet P: Synovial haemangioma of the knee: a requently misdiagnosed lesion. Skeletal Radiol 24:257-61,1995.

Das KM, Ahmed AM, Aljubab A, Alzoum MA. Multidetector computed tomography evaluation of cavernous haemangioma of the azygous vein. Interact Cardiovasc Thorac Surg. 2013 Aug;17(2):436-7. doi: 10.1093/icvts/ivt126.

DeFilippo JL, Yu JS, Weis L, Lucas J: Soft tissue hemangioma with adjacent periosteal reaction simulating a primary bone tumor. Skeletal Radiol 25:174-7,1996.

Demertzis JL, Kyriakos M, Loomans R, McDonald DJ, Wessell DE. Synovial hemangioma of the hip joint in a pediatric patient. Skeletal Radiol. 2013 Sep 24.

DePalma AF, Manler GG: Hemangioma of the synovial membrane. Clin Orthop 32:93-9,1964.

Devaney K, Vinh TN, Sweet DE: Synovial hemangioma: a report of 20 cases with differential diagnostic considerations. Hum Pathol 24:737-45,1993.
Doppman JL, Pevsner P: Embolization of arteriovenous malformations by direct percutaneous puncture. AJR 140:773-8,1983.
Engelstad BL, Gilula LA, Kyriakos M: Ossified skeletal muscle hemangioma: radiologic and pathologic features. Skeletal Radiol 5:35-40,1980.
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.
Fernandez GN: Locking of a metacarpo-phalangeal joint caused by a haemangioma of the volar plate. J Hand Surg 13B:323-4,1988.

George R, Lee K. Synovial angioma of the FDP flexor sheath. A rare cause of carpal tunnel syndrome. Open Orthop J. 2013;7:72-4. doi: 10.2174/1874325001307010072.

Goswamy J, Aggarwal R, Bruce IA, Rothera MP. Kasabach-Merritt syndrome in a child with upper airway compromise and spontaneous periorbital bruising. Ear Nose Throat J. 2013 Jun;92(6):E16.

Greenspan A, Azouz EM, Matthews J, Decarie JC: Synovial hemangioma: imaging features in eight histologically proven cases, review of the literature, and differential diagnosis. Skeletal Radiol 24:583-90,1995.
Greenspan A, McGahan JP, Vogelsang P, Szabo RM: Imaging strategies in the evaluation of soft-tissue hemangiomas of the extremities: correlation of the findings of plain radiography, angiography, CT, MRI and ultrasonography in 12 histologically proven cases. Skeletal Radiol 21:11-18,1992.

Halborg A, Hansen H, Sneppen HO: Haemangioma of the knee joint. Acta Orthop Scand 39:209-16,1968.
Hawnaur JM, Whitehouse RW, Jenkins JPR, Isherwood I: Musculoskeletal haemangiomas: comparison of MRI with CT. Skeletal Radiol 19:251-8,1990.

Holland KE, Drolet BA. Approach to the pateint with an infantile hemangioma. Dermatol Clin. 2013 Apr;31(2):289-301. doi: 10.1016/j.det.2012.12.006.

Johnson EW, Ghormley RK, Dockerty MB: Hemangiomas of the extremities. Surg Gynecol Obstet 102:531-8,1956.
Kaplan PA, Williams SM: Mucocutaneous and peripheral soft-tissue hemangiomas: MR imaging. Radiology 163:163-6,1987.
Kindblom-Meis JM, Enzinger FM: Color Atlas of Soft Tissue Tumors. Mosby-Wolfe, Portland, 1996, pp 74-105, 138-178.
Kline SC, Moore JR: Intraneural hemangioma: a case report of acute cubital tunnel syndrome. J Hand Surg 17A:305-7,1992.
Kudawara I, Yoshikawa H, Araki N, Veda T: Intramuscular haemangioma adjacent to the bone surface with periosteal reaction. J Bone Joint Surg 83B:659-62,2001.

Lenchik L, Poznanski AK, Donaldson JS, Sarwark JF: Case report 681:synovial hemangioma of the knee. Skeletal Radiol 20:387-9,1991.
Levin DC, Gordon DH, McSweeney J: Arteriography of peripheral hemangiomas. Radiology 121:625- ,1976.
Levine E, Wetzel LH, Neff JR: MR imaging and CT of extrahepatic cavernous hemangiomas. AJR 147:1299-304,1986.

Lightbody KA, Wilkie MD, Luff DA. Capillary haemangioma of the ethmoid sinus. BMJ Case Rep. 2013 Apr 10;2013. doi:pii: bcr2013008695. 10.1136/bcr-2013-008695.

Llauger J, Monill JM, Palmer J, : Synovial hemangioma of the knee: MRI imaging findings in two cases. Skeletal Radiol 24:578- ,1995.
Louis DS, Fortin PT: Perineural hemangioma of the upper extremity: report of four cases. J Hand Surg 17A:308-11,1992.
McNeill TW, Chan GE, Capek V, : The value of angiography in the surgical management of deep hemangiomas. Clin Orthop 101:176- ,1984.
Meyer JS, Hoffer FA, Barnes PD, Mulliken JB: Biological classification of soft-tissue vascular anomalies: MR correlation. AJF 157:559-64,1991.

Moon NF: Synovial hemangioma of the knee joint. Clin Orthop 90:183-90,1973.
Murphy MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS: From the archives of AFIP. Musculoskeletal angiomatous lesions: radiologic pathologic correlation. Radiographics 15:893- ,1995.
Nakamura I, Hoshino Y: Extraneural hemangioma: a case report of acute cubital tunnel syndrome. J Hand Surg 21A:1097-8,1996.
Narvaez JA, Narvaez J, Aguilera C, De Lama E, Portabella F: MR imaging of synovial tumors and tumor-like lesions. Eur Radiol 11:2549-60,2001.

Okahashi K, Sugimoto K, Iwai M, Tanaka M, Fujisawa Y, Takakura Y: Intra-articular synovial hemangioma: a rare cause of knee pain and swelling. Arch Orthop Trauma Surg 124:571-3,2004.

Patel CB, Tsai T-M, Kleinert HE: Hemangioma of the median nerve: a report of two cases. J Hand Surg 11A:76-9,1986.

Price NJ, Cundy PJ: Synovial hemangioma of the knee. J Pediatr Orthop 17:74-77,1997.

Ramseier LE, Exner GU: Arthropathy of the knee joint caused by synovial hemangioma. J Pediatr Orthop 24:83-6,2004.
Rauch RF, Silverman PM, Korobkin M, : Computed tomography of benign angiomatous lesions of the extremities. J Comput Assist Tomogr 8:1143- ,1984.

Rogalski R, Hensinger R, Loder R: Vascular abnormalities of the extremities: clinical findings and management. J Pediatr Orthop 13:9-14,1993.
Sooriakumaran S, Lal Landham T: The Klippel-Trenaunay syndrome. J Bone Joint Surg 73B(1):169-70,1991.
Suh JS, Hwang G, Hahn SB: Soft-tissue hemangiomas: MR manifestations in 23 patients. Skeletal Radiol 23:621-5,1994.
Teo E-LHJ, Strouse PJ, Hernandez RJ: MR imaging differentiation of soft-tissue hemangiomas from malignant soft-tissue masses. AJR 174:1623-8,2000.

Thacker MM. Benign soft tissue tumors in children. Orthop Clin North Am. 2013 Jul;44(3):433-44, xi. doi: 10.1016/j.ocl.2013.05.001.

Trias A, Dilenge D: A new approach to the treatment of cavernous hemangioma of skeletal muscle. J Bone Joint Surg 54B:770- , 1972.
Upton J, Mulliken JB, Murray JE: Classification and rationale for management of vascular anomalies in the upper extremity. J Hand Surg 10A:970-5,1985.

Vilanova JC, Barcelo J, Villalon M: MR and MR angiography characterization of soft tissue vascular malformations. Curr Probl Diagn Radiol 33:161-70,2004.
Wang Y-C, Jeng C-M, Wu D-Y, Chang C-Y, Resnick D: Giant ossified cavernous hemangioma of an extremity associated with an equinovarus deformity. Skeletal Radiol 27:522-4,1998.
Weinzweig J, Watson HK, Wiener BD, Genter BE: Hemangioma of the extensor pollicis brevis in the first dorsal compartment: an unusual cause of bilateral de Quervain's disease. J Hand Surg 21A:256-8,1996.
White CW, Wolf SJ, Korones DN, Sondheimer HM, Tosi MF, Yu A: Treatment of childhood angiomatous diseases with recombinant interferon alfa-2a. J Pediatr 118:59-66,1991.
Yuh WT, Kathol MH, Sein MA, Ehara S, Chin L: Hemangiomas of skeletal muscle: MR findings in five patients. AJR 149:765-8,1987.

Content

Download: PDF | EPUB

Famous Quote
"The eye too close to a picture has no right to telegraph criticism about the painter's purpose or skill. Stand where the painter intended." Waltbie Davenport Babcock
Figures
Related Content

Resources for Hemangioma and related topics on OrthopaedicsOne.