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Intraosseous Angiosarcoma

DEFINITION AND PATHOGENESIS

  • (Malignant hemangioendothelioma, hemangiosarcoma)
  • Solitary and multifocal malignant tumor of blood vessels occuring within a bone
    • Endothelial differentiation

IMPORTANCE

  • Rare lesion which because of contiguous and multicentricity of some lesions, require careful evaluation and awareness of neoplasm behavior
    • < 1% of primary bone malignancies

CLINICAL FEATURES

  • Bx can be very bloody!
  • M:F = 2:1
  • Most common in 3rd-5th decades, mean age ~50 yrs
  • Local pain and swelling may be longstanding

RADIOLOGIC FEATURES

  • Solitary, multifocal, similar in appearance to lytic metastases
    • Up to 1/3 with multifocal bone involvement
  • Cortical destruction with a soft tissue mass
    • Poorly marginated lucency of bone
  • May occur at the site of a bone infarct (undifferentiated pleomorphic sarcoma >> osteosarcoma > fibrosarcoma > angiosarcoma in incidence), predominantly in M
  • Femur most common site
  • ± pathological fx

GROSS PATHOLOGY

  • Cavernous or in very aggressive lesions, a solid intramedullary mass, hemorrhagic, poorly defined margins
  • Typically 2-10 cm

HISTOLOGIC FEATURES

  • Complex anastomosing channels
  • Abnormalities in sizes and shapes of vascular channels
  • Abnormalities in organization of endothelial cells
  • Hobnail cells
  • Syncytial or papillary cell tufts
  • Shedding of individual and clusters of cells into the lumen of rudimentary vessels
  • Cellular atypia
    • Malignant spindled to epithelioid endothelial cells
  • Areas of solid sarcoma, loose fibroblastic stroma
  • Necrosis can be prominent
  • Immunohistochemistry
    • + factor VIII-related antigen
    • + CD-31 and CD-34 in vasoformative areas
    • + FLI-1
    • + keratin and EMA in epithelioid variant
      • Suspect intraosseous angiosarcoma if + keratin
  • EM
    • Weibel-Palade bodies specific for vascular endothelium: a single, membrane bound, rod-like body that contains parallel arrays of microtubules on EM
  • "Angiotropism" is intravascular spread

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Metastatic disease and undifferentiated sarcoma
  • Invasive PVNS
  • Cellular hemangioma
    • Epithelioid hemangioma
  • Epithelioid hemangioendothelioma

DISEASE COURSE AND TREATMENT

  • Wide resection if lesion is solitary
  • Chemotherapy has been tried in high grade lesions
  • Amputation may be necessary in contiguous extremity lesions, XRT in contiguous axial lesions
  • Prognosis related to histologic grade
  • 5 yr survival when associated with a bone infarct: (14% in osteosarcoma, 22% in undifferentiated pleomorphic sarcoma)

SPECIAL CONSIDERATIONS

 

  • Hemangioperictyoma

    • Composed of the pericytes of Zimmerman, occurring in adults, especially the thigh, pelvis, and retroperitoneum, and may be either benign or malignant, occurring in bone or soft tissue
  • Infantile Hemangiopericytoma

    • Have a better prognosis than the adult form
    • Ulceration and bleeding from the mass may be life-threatening
  • Hemangioendothelioma

    • (grade I and II; low-grade angiosarcoma)
    • Mean age is 35-40, presenting with a dull aching pain
    • Has been reported 30 yrs after IF of a tibial fx
    • Spinal lesions have been txd w/ XRT alone, curettage and XRT, anterior resection alone, and anterior resection and XRT (numbers too small to make recommendations, although the one pt txd w/ XRT alone developed pulmonary metastasis
    • Stabilization is recommended to diminish chronic back pain
    • Differentiate from epithelioid hemangioma of bone
  • ENDOVASCULAR PAPILLARY ANGIOENDOTHELIOMA (DABSKA TUMOR OF BONE)

    • 2 reported cases
    • Spindle cell proliferation with vascular spaces and papillary tufts of hobnail endothelial cells
    • Wide resection and aggressive curettage have been reported

REFERENCES

Abdelwahab IF, Klein MJ, Hermann G, Springfield D: Angiosarcomas associated with bone infarcts. Skeletal Radiol 27:546-51,1998.
Aflatoon K, Staals E, Bertoni F, Bacchini P, Donati D, Fabbri N, Boriani S, Frassica FJ: Hemangioendothelioma of the spine. Clin Orthop 418:191-7,2004.
Alpern MB, Thorsen MK, Kellman GM, Pojunas K, Lawson TL: CT appearance of hemangiopericytoma. J Comput Assist Tomogr 10:264-7,1986.
Arbelaez A, Castillo M, Williams L, : Vertebral hemangioendothelial sarcoma: MR findings. J Comput Assist Tomogr 23:981-3,1999.
Boutin RD, Spaeth HJ, Mangalik A, : Epithelioid hemangioendothelioma of bone. Skeletal Radiol 25:391-5,1996.
Campanacci M, Boriani S, Giunti A: Hemangioendothelioma of bone: a study of 29 cases. Cancer 46:804-14,1980.
Ellis TS, Schwartz A, Starr JK, : Epithelioid hemangioendothelioma of the lumbar vertebral column: case report and review of literature. Neurosurgery 38:402-7,1996.
Ferrari D, Pignatti G, Bertoni F, Bacchini P, Giunti A: Angioma of bone following intramedullary nail fixation. Orthopedics 24:795-7,2001.
Kleer CG, Unni KK, McLeod RA: Epithelioid hemangioendothelioma of bone. Am J Surg Pathol 20:1301-11,1996.
Marks DS, Thomas AM, Thompson AG, : Surgical management of haemangioendothelioma of the spine. Eur Spine J 4:186-90,1995.
McCaarthy EF, Lietman S, Argani P, Frassica FJ: Endovascular papillary angioendothelioma (Davska tumor) of bone. Skeletal Radiol 28:100-3,1999.
Nakayama T, Nishino M, Takasu K, Hayakawa K, Toguchida J, Tanaka C: Endovascular papillary angioendothelioma (Dabska tumor) of bone.
O'Connell JX, Kattapuram SV, Mankin HJ, : Epithelioid hemangioma of bone: a tumor often mistaken for low-grade angiosarcoma or malignant hemangioendothelioma. Am J Surg Pathol 17:610-7,1993.
Rong Y, Sato K, Yamamura S, Sugiura H, Katagiri H, Iwata H: Malignant hemangioendothelioma of the left calcaneus associated with fever and hematological abnormalities. Skeletal Radiol 26:64-6,1997.
Stout AP: Hemangio-endothelioma: a tumor of blood vessles featuring vascular endothelial cells. Ann Surg 118:445-64,1943.
Sybert DR, Steffee AD, Keppler L, : Seven-year followiup of vertebral excision and reconstruction for malignant hemangioendothelioma of bone. Spine 20:841-4,1995.
Templeton PA, Gordon DJ, O'Hara MD: Infantile haemangiopericytoma of the hand. J Hand Surg 21B:121-3,1996.
Yaghmai I: Angiographic manifestations of soft tissue and osseous hemangiopericytomas. Radiology 126:653-9,1978.
Weiss SW, Ishak KG, Dail DH, : Epithelioid hemangioendothelioma and related lesions. Semin Diagn Pathol 3:259-87,1986.
Wenger DE, Wold LE: Malignant vascular lesions of bone: radiologic and pathologic features. Skeletal Radiol 29:619-31,2000.
Wold LE, Unni KK, Beabout JW, : Hemangioendothelial sarcoma of bone. Am J Surg Pathol 6:59-70,1982.

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