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Breast malignant, males, children

Sarcoma

Angiosarcoma


Reviewer: Monika Roychowdhury, M.D. (see Reviewers page)
Revised: 29 September 2012, last major update September 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

Definition
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● Tumor composed of malignant cells with endothelial features

Terminology
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● Also called lymphangiosarcoma, less preferred term is “malignant hemangioendothelioma”

Epidemiology
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● Rare, < 0.2% of primary breast tumors (Ann Diagn Pathol 2009;13:147), 3-9% of breast sarcomas
● (a) young women with no prior history
● (b) women 5-10 years post-radiation therapy for breast carcinoma (in skin or breast, (Cancer 2009;115:4055), or
● (c) skin of chronically edematous arm after axillary lymph node dissection (Stewart-Treves syndrome, incidence has decreased dramatically due to use of more conservative surgery, Am J Surg Pathol 1983;7:329)
● Note: post-radiation and post-axillary dissection cases have more prominent cutaneous involvement

Clinical features
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● Clinically forms bulky mass associated with bruise-like cutaneous changes
● Estimated incidence of 0.14% after breast conserving therapy (J Am Acad Dermatol 2006;54:499)
● Precursor lesion is “atypical vascular lesion”, which occurs after surgery and radiation of the breast (Am J Surg Pathol 2008;32:943)
● May resemble recurrent breast carcinoma clinically (Clin Breast Cancer 2008;8:94)

Xray
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● Solitary mass on mammography without calcifications or skin thickening, at site of prior surgical incision

Case reports
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● Tumor post-radiation therapy #1 (Arch Pathol Lab Med 2002;126:989), #2 (Univ Oklahoma), #3 (Int Semin Surg Oncol 2006;3:26), #4-low grade tumor (Hum Pathol 1992;23:710), #5 - bilateral tumor with Paget’s disease (Mod Pathol 1995;8:1)
● 26 year old woman with epithelioid angiosarcoma (Am J Surg Pathol 1997;21:599)

Treatment and prognosis
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● Treatment is complete excision
● Post-radiation cases have shorter latency period and no edema, compared to Stewart-Treves syndrome cases (Am J Surg Pathol 2004;28:781)
● Tumors have similar behavior with or without prior radiation therapy (Cancer 2005;104:2682)
● Median survival of 3-6 years (Am J Clin Oncol 2009;32:582)
● Often recurs locally
● Grading may not have prognostic significance (Am J Surg Pathol 2008;32:1896, but see Am J Surg Pathol 1983;7:53)

Clinical images
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Hemorrhagic lesion

Post-radiotherapy

Erythema and ulceration

51 year old woman with high grade tumor

Gross description
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● Soft, spongy, hemorrhagic
● May have blue nodules at site of prior scar
● Mean 5cm
● Usually poorly circumscribed

Gross images
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Well circumscribed mass (AFIP)

Infiltrative mass with hemorrhage

Intermediate grade tumor with hemorrhage

High grade tumor

Contributed by anonymous pathologist:

Mastectomy post radiation and chemotherapy for ductal carcinoma

Microscopic description
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● Anastomosing vascular channels lined by atypical endothelial cells with pleomorphic, hyperchromatic nuclei
● Infiltrative margins
● Microscopic heterogeneity common within same tumor, so sample thoroughly
● Variable mitotic activity
● Usually no fibrous septae, no epithelial component
● Post-radiation tumors are typically high grade

Grade I (well differentiated):
● Anastomosing vascular channels dissect interlobular stroma
● Vessels have wide lumina with red blood cells
● Endothelium lining vessels have prominent and hyperchromatic nuclei, may resemble benign vascular tumors

Grade II (moderately differentiated):
● 75% of tumor is well differentiated, but solid foci are also found (Am J Surg Pathol 1981;5:629)

Grade III (poorly differentiated):
● Interanastomosing vascular channels are intermingled with solid endothelial or spindled areas, with necrosis and mitotic figures
● At least 50% of tumor is composed of solid or spindled areas without vascular channels

Micro images
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Neoplastic vascular channels

Solid areas and neoplastic vascular channels

Markedly pleomorphic tumor cells, mitotic figures and apoptotic cells

Focal spindle cells

Various images

Fig 1: Infiltrating vascular lesion with spindle cell proliferation in dermis
Fig 2/3: Pleomorphic spindle cells with “blood lakes” and brisk mitotic activity


       

Low grade tumor recurring as high grade tumor

Low grade angiosarcoma:
       

   

           

Low grade angiosarcoma (contributed by anonymous pathologist)

Intermediate grade angiosarcoma:

Dilated vascular spaces contain endothelial cells and red blood cells

Focal solid neoplastic vascular proliferation in fat

Focal spindle cell and small vessel proliferation

Focal complex capillary and endothelial cell proliferation

Spindle cells and mitotic figure

High grade angiosarcoma:

Irregular spaces resemble adenocarcinoma

Anastomosing vascular channels

   
High grade tumor

Anastomosing vascular channels with spindle and papillary foci

Solid growth and hemorrhagic necrosis

Spindle cells with indistinct vascular spaces

Plump spindle cells with mitoses

Blood lakes (foci of hemorrhagic necrosis

Post-mastectomy (AFIP):

Atypical vascular proliferation in dermis and subcutis and changes of chronic lymphedema

Vascular channels in superficial dermis with endothelial atypia

Anastomosing vascular channels with papillary proliferation of atypical endothelial cells

Focal solid pattern of atypical endothelial cells

Post-radiotherapy:
   

Skin tumor

Stains:
   
H&E, Ki-67 and Skp2

   
Left: CD31; right: Factor VIII-related antigen

Cytology description
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● Moderate to highly cellular with variable cellular aggregates
● Cells are epithelioid and spindled
● May have subtle angioformative changes (Cancer 2005;105:145)

Positive stains
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● Vimentin, Factor VIII
● CD31 and CD34 (well differentiated areas)
● High Ki-67 (Arch Pathol Lab Med 2007;131:538)
● ER (variable)

Negative stains
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● Keratin

Electron microscopy description
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● Prominent pinocytic vesicles and cell-cell junctions

Differential diagnosis
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Metaplastic carcinoma
Acantholytic squamous cell carcinoma: has at least focal areas of squamous differentiation, keratin+, negative for vascular markers
Hemangioma: well circumscribed, no atypia
Atypical vascular lesions: more circumscribed, less atypia than low grade angiosarcoma
Pseudoangiomatous stromal hyperplasia

Additional references
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Stanford University

End of Breast malignant, males, children > Sarcoma > Angiosarcoma


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