CASE N°22 – Primary epithelioid angiosarcoma of the breast

Clinical History:

Age: 35

Sex: Female

From about 2 months painless lump in the right breast. She was subjected to ultrasound : In the right breast presence of a cysts about 5cm in diameter with solid vegetation of major axis 3,5 cm. It has been suggested a cytological examination of the lesion

Cytological Images:

Cytological material: FNAC of the breast

Cytological Report:

Epithelioid cells with elongated nuclei and cytoplasm, irregular pleomorphic nuclei, nuclear hyperchromasia and prominent nucleoli, arranged in macroaggregates with fibrovascular axis or isolated, occasionally multinucleated. Background of blood  with scattered histiocytes. Cytological findings suspicious of malignancy. Histological verification needed.

Histological Images:

Histological material: Right mastectomy

 vimentin+

 CD31+

 CD34-

 CK Pool –

 CKMNF116-

 FactorVIII+

 S-100 –

 Mib1 (Ki67): 40%

Histological Report:

Histopathologic examination and immunohistochemistry revealed a diagnosis of primary epithelioid angiosarcoma of the breast. Vimentin(+), CD31(+), factorVIII(+), CD34(-), S100(-), CKpool(-), CKMNF116(-). Proliferation index: MIB1 = 40%.

Discussion:

Sarcomas represent fewer than 1% of primary breast malignancies (1,2).  The most commonly reported primary non-phyllodes sarcomas of the breast are angiosarcoma, fibrosarcoma, malignant fibrous histiocytoma, liposarcoma, and leiomyosarcoma (2). Angiosarcomas account for fewer than 0.05% of all malignant mammary tumours (3).

We report a rare and aggressive case of primary epithelioid angiosarcoma of the breast—a disease that can be misdiagnosed as carcinoma because of similar histopathology. An immunohistochemical panel including ck, vimentin, CD31, CD34 should be used to differentiate lesions with a similar histomorphology to reach a final diagnosis (4).  Mastectomy with adjuvant radiotherapy and chemotherapy appears to be the best treatment modality. Thalidomide appears to be a promising drug in the management of angiosarcoma. Exploration of newer agents is warranted to improve survival.

References:

1. McGowan TS, Cummings BJ, O’Sullivan B, Catton CN, Miller N, Panzarella T. An analysis of 78 breast sarcoma patients without distant metastases at presentation. Int J Radiat Oncol Biol Phys 2000;46:383–90.

2. Blanchard DK, Reynolds CA, Grant CS, Donohue JH. Primary nonphylloides breast sarcomas. Am J Surg 2003;186:359–61.

3. 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;32:1896–904.

4. S. Muzumder, P. Das, M. Kumar, S. Bhasker, C. Sarkar, K. Medhi V.K. Iyer, and G.K. Rath Primary epithelioid angiosarcoma of the breast masquerading as carcinoma Current Onco logy Volume 17; Number1: 64-69

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