Definition
Malignant neoplasm of the breast exhibiting blood vascular differentiation
Diagnostic Criteria
All grades are characterized by infiltration of breast parenchyma by cells exhibiting vascular differentiation
Low grade tumors
Prominent freely anastomosing vascular channels Papillary growth and endothelial tufting minimal to absent
Cytologic atypia may be difficult to identify even after extensive sampling
Intermediate grade tumors
Freely anastomosing vascular channels
Papillary growth and endothelial tufting May have focal solid areas with polygonal or spindle cells
High grade tumors
Prominent solid areas of clearly malignant cells
Polygonal and spindled cells
Blood lakes and necrosis are common Vascular channels may be difficult to identify
May require extensive sampling, especially at edge of lesion
Demonstration of vascular nature may require immunohistology
Awareness of the varying patterns exhibited by the above grades is useful for diagnostic purposes but it is not clear that it has any predictive value
Richard L Kempson MD
Original posting:: May 27, 2006
Supplemental studies
Immunohistology
CD31 is the most specific vascular endothelial marker
Carcinomas and stromal cells are negative
Reaction with histiocytes may be confused with sheets of neoplasm
Most sensitive marker in high grade cases
CD34 is relatively specific
Carcinomas are negative
Frequent, extensive benign stromal cell reactivity may make interpretation difficult
Less sensitive than CD31 in high grade cases, but exceptions to this rule do occur
Factor VIII related antigen
Less sensitive than CD31 and CD34 in neoplasms
Differential Diagnosis
Angiosarcoma of the Breast Perilobular Hemangioma
Almost always a hemorrhagic mass >2 cm
Microscopic, incidental lesion
Diffuse, infiltrating
Localized
Complex anastomoses
No complex anastomoses
Intermediate and high grade tumors are cytologically atypical
No significant cytologic atypia
Frequent papillae or tufting
No papillae or tufting
Complete excision and examination are required to insure accurate diagnosis of an apparently benign vascular lesion in the breast
Angiosarcoma of the Breast Hemangioma of the Breast
Diffuse
Circumscribed
Infiltrates and destroys lobules
Adjacent to or surrounds lobules
Rarely <2 cm
Rarely >2 cm
No fibrous septa
Fibrous septa forming lobules of angioma
No feeder vessels
May have feeder vessels
Complex anastomoses
Few anastomoses, not complex
Intermediate and high grade tumors are cytologically atypical
Focal atypia at most
Frequent papillae or tufting
No papillae or tufting
May have hemorrhage and necrosis
No hemorrhage or necrosis
No muscular walls
Venous hemangioma may have muscular walls
Complete excision and examination are required to insure accurate diagnosis of an apparently benign vascular lesion in the breast
Angiosarcoma of the Breast Angiomatosis of the Breast
Variable patterns: channels, papillae, spindle cells
Uniform pattern of vessels
Usually a t least focal cytologic atypia
Cytologically bland
Invades lobules
Does not invade lobules
Frequent papillae and tufting
No papillae or tufting
Complete excision and examination are required to insure accurate diagnosis of an apparently benign vascular lesion in the breast
Angiomatosis is an extremely rare lesion; it is a diagnosis that requires consultation
Low Grade Angiosarcoma Pseudoangiomatous Stromal Hyperplasia
Endothelial lined spaces
No endothelial lining of spaces
Red blood cells in some spaces
No red blood cells in spaces
Infiltrates fat
No infiltration of fat
Infiltrates lobules
Merges with lobular stroma
Lining is CD31+
Lining is CD31 negative
Both contain anastomosing spaces containing CD34+ cells
High Grade Angiosarcoma
Undifferentiated or Poorly Differentiated Malignant Neoplasm
May have peripheral vascular differentiation
Lacks any vascular differentiation
CD34 or CD31 positive
Positive for other specific lineage markers
Angiosarcoma Atypical Vascular Lesion
Usually no history of radiation
Almost all have received radiation
Most within the breast but may be in the skin in irradiated patients
Usually in the skin of the breast
Most often a hemorrhagic mass > 2 cm
Small non-hemorrhagic lesion
Note: Atypical vascular lesions may evolve into angiosarcoma. The morphologic features that separate the two are poorly defined.
Clinical
Mean age mid-thirties
Lower age associated with higher grade
Approximate 5 year survival:
Low grade 76%
Intermediate grade 70%
High grade 15%
A recent report found that grading does not have predictive value (Nascimento 2008)
Grading / Staging / Report
Grading
Low Grade
Intermediate Grade
High Grade
Anastomosing channels
Prominent
Prominent
Frequently inconspicuous
Papillary growth
Rare to absent
Prominent
Variable
Endothelial tufting
Rare to absent
Prominent
Variable
Cytologic atypia
Mild
Mild to prominent
Prominent
Mitotic figures
Rare
Frequent
Frequent
Solid areas
Absent
Focal or absent
Prominent
Necrosis
Absent
Absent
Present
Blood lakes
Absent
Absent
Often present
Awareness of the varying patterns exhibited by the above grades is useful for diagnostic purposes but it is not clear that it has any predictive value
Staging
Evaluation of margins may be extremely difficult as peripheral neoplastic vessels may be cytologically and architecturally quite bland
Contralateral disease is generally evidence of metastasis rather than a second primary
Lists
Breast Tumors with Vascular (and Pseudovascular) Differentiation
Bibliography
Rosen PP, Oberman HA. Tumors of the Mammary Gland, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 7, 1993
Rosen PP, Kimmel M, Ernsberger D. Mammary angiosarcoma. The prognostic significance of tumor differentiation. Cancer. 1988 Nov 15;62(10):2145-51.
Donnell RM, Rosen PP, Lieberman PH, Kaufman RJ, Kay S, Braun DW Jr, Kinne DW. Angiosarcoma and other vascular tumors of the breast. Am J Surg Pathol. 1981 Oct;5(7):629-42.
Vorburger SA, Xing Y, Hunt KK, Lakin GE, Benjamin RS, Feig BW, Pisters PW, Ballo MT, Chen L, Trent J 3rd, Burgess M, Patel S, Pollock RE, Cormier JN. Angiosarcoma of the breast. Cancer. 2005 Dec 15;104(12):2682-8.
Farina MC, Casado V, Renedo G, Estevez L, Martin L, Requena L. Epithelioid angiosarcoma of the breast involving the skin: a highly aggressive neoplasm readily mistaken for mammary carcinoma. J Cutan Pathol. 2003 Feb;30(2):152-6.
Nascimento AF, Raut CP, Fletcher CD. Primary angiosarcoma of the breast: clinicopathologic analysis of 49 cases, suggesting that grade is not prognostic. Am J Surg Pathol. 2008 Dec;32(12):1896-904.
