. Intraosseous Angiosarcoma. PORTNotes. In: OrthopaedicsOne - The Orthopaedic Knowledge Network. Created Feb 16, 2009 16:50. Last modified Nov 04, 2016 05:49 ver.7. Retrieved 2017-09-24, from http://www.orthopaedicsone.com/x/IQEjAQ.
DEFINITION AND PATHOGENESIS
- (Malignant hemangioendothelioma, hemangiosarcoma)
- Solitary and multifocal malignant tumor of blood vessels occuring within a bone
- Endothelial differentiation
- Rare lesion which because of contiguous and multicentricity of some lesions, require careful evaluation and awareness of neoplasm behavior
- < 1% of primary bone malignancies
- 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
- 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
- Cavernous or in very aggressive lesions, a solid intramedullary mass, hemorrhagic, poorly defined margins
- Typically 2-10 cm
- 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
- + 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
- 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)
- 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
- Have a better prognosis than the adult form
- Ulceration and bleeding from the mass may be life-threatening
- (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
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