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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 3  |  Page : 427-429
Cytodiagnosis of angiosarcoma of breast


1 Department of Pathology, SCB Medical College, Cuttack, Orissa, India
2 Department of Surgery, SCB Medical College, Cuttack, Orissa, India

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   Abstract 

A 24-year-old female presented with swelling in the left breast for one-and-a-half years associated with pain for last 4 months. During this period, she was operated twice and once again there was local recurrence, the biopsy was interpreted as benign vascular lesion. The case was diagnosed by preoperative cytology as angiosarcoma of breast, after which, she underwent modified radical mastectomy. Angiosarcoma of breast is uncommon with extremely bad prognosis. Familiarity with the clinical and pathologic features is critical in avoiding underdiagnosis and delayed treatment.

Keywords: Angiosarcoma, breast, recurrence

How to cite this article:
Kar A, Mukhopadhyay D, Das SS, Swain NN, Das BM, Nayak M, Rath J, Satpathy S. Cytodiagnosis of angiosarcoma of breast. Indian J Pathol Microbiol 2008;51:427-9

How to cite this URL:
Kar A, Mukhopadhyay D, Das SS, Swain NN, Das BM, Nayak M, Rath J, Satpathy S. Cytodiagnosis of angiosarcoma of breast. Indian J Pathol Microbiol [serial online] 2008 [cited 2019 Dec 11];51:427-9. Available from: http://www.ijpmonline.org/text.asp?2008/51/3/427/42551



   Introduction Top


Angiosarcoma of breast is a rare tumor, accounting for approximately one in 1700-2000 primary malignant tumors of the breast. [1],[2] Two hundred and nineteen cases are diagnosed till date since the first case reported by Schmidt in 1887. [3] These tumors rapidly grow to a large size with blue-red discoloration of skin. Angiosarcoma is seen in slightly younger females than mammary carcinoma. In spite of the large size, the classic signs of ordinary breast carcinoma, such as skin retraction, nipple discharge and auxiliary node enlargement, are absent. Preoperative diagnosis of angiosarcoma of breast by aspiration cytology and biopsy is often difficult. It has a high mortality rate, with only 10-20% of patients remaining disease-free after 5 years. [4] The grim prognosis, to some extent, is attributable to underdiagnosis of the tumor in initial biopsy. We report this case because of its rarity and its misinterpretation as benign lesion due to the not uncommonly deceptive benign histologic appearance.


   Case history Top


A 24-year-old lady developed a painless swelling over outer quadrant of the left breast, which gradually increases to a size of 3 cm 3 cm. The patient consulted the local doctor and underwent excisional biopsy in October 2006. The histopathological picture was interpreted as vascular hamartoma. After 3 months of operation, there was a swelling again at the same site in January 2007. Then lumpectomy was done and the biopsy report came out to be capillary hemangioma. After 2 months, there was a second recurrence and she noticed a small red-colored swelling in the upper portion of breast. The mass rapidly grew into a globular mass of size of 7 cm 5 cm of variegated consistency. The overlying skin was erythematous with blistering lesions. After 15 days, she developed another superficial swelling in the lower portion of the same breast [Figure 1]. Both the swellings were fixed to the skin, but mobile over chest wall. Her left axillary nodes were also enlarged. Fine needle aspiration cytology of the mass was done and aspiration was bloody on repeated attempts. On thorough searching, syncytial clusters of spindle-shaped cells, forming interconnecting channels on an hemorrhagic background, were found [Figure 2]. A preoperative cytologic impression of "possibly angiosarcoma" was given. She was planned for modified radical mastectomy. Gross received was mastectomy specimen with nipple and areola measuring 15 8 5 cm. Behind the nipple, there was a cavity containing blood clot and friable papillary structures measuring 7 cm in diameter [Figure 3]. Multiple sections were taken from the mass, papillary portion, resected margins and lymph nodes. Histopathological study revealed normal lobular structures of breast with numerous anastomosing vascular channels infiltrating into breast tissue in a dissecting plane with areas showing marked endothelial proliferation. Some papillary projections into the vascular lumina are also present [Figure 4]. Areas of hemorrhage and necrosis were discernible. Sections from axillary nodes and resected tumor margins did not show tumor dissemination. On immunohistochemistry, the tumor cells were immunoreactive for CD31 [Figure 5].


   Discussion Top


Angiosarcoma is a malignant neoplasm of endothelial origin, either originating from lymphatics (lymphangiosarcoma) or blood vessels (hemangiosarcoma). It is a rare tumor, comprising fewer than 1% of all sarcomas. [5] Angiosarcoma arising in breast (de novo) account for about less than 1% of all malignant neoplasms in the breast. It manifests as a rapidly growing mass with a blue-red discoloration of overlying skin. In the present case, in the first two instances, the mass was unremarkable but in the second recurrence, it presented as a breast lump with erythematous skin. Angiosarcoma of the breast occurs mainly during third and fourth decades of life, in contrast to breast carcinoma that generally arises in a later age. It also differs from a classic breast carcinoma in many aspects like the mass is accompanied by edema and hyperemia of overlying skin without regional node involvement, absence of classic signs like skin retraction and nipple discharge, negativity for epithelial markers such as CEA, CA 15-3, CA 19-9, presence of cystic components in breast magnetic resonance imaging, immunoreactivity for endothelial markers such as VIII-related antigens, CD31, CD34 and Ulex europaeus. Preoperative diagnosis of angiosarcoma of breast by aspiration cytology and biopsy is often difficult, with a false negative rate of 37%. [6] Review of the literature revealed that in 32 out of 87 cases, the malignant nature of the lesion was not recognized in the initial biopsy specimens. [6] For cytological diagnosis, multiple passes into different sites of the mass should be done, so that solid areas can be sampled. While scanning the smears, the tumor cells may be few in numbers and missed in low-grade angiosarcoma. In this case, particularly finding of syncytial clusters of pleomorphic spindle-shaped cells with vascular channels gave the clue to diagnosis. In biopsy, the primary tumor was diagnosed as benign vascular tumor because it was composed of well-formed vascular channels lined by endothelial cells without solid area. However, in angiosarcoma, on close scrutiny, one can find features unequivocally suggestive of the diagnosis like free anastomosis of vascular channels, scattered hyperchromatic endothelial cells, occasional areas of intraluminal tufting comprising of 2-3 tumor cells [Figure 4] and dissecting into breast tissue leaving behind intact breast acini and lobules. The delay in the correct diagnosis was in some cases up to 2 years (within 1 year in the said case). [7] Therefore, when examining an angiomatous breast lesion, it is important to include angiosarcoma in the differential diagnosis. Another fact one should keep in mind is that benign vasoformative lesions of breast rarely present as a palpable mass or diffuse swelling of breast. [8]

The treatment of choice is surgical with simple mastectomy being the appropriate surgical procedure when findings, such as axillary lymph node enlargement or chest wall invasion, are absent. The planned surgery here was modified radical mastectomy because of axillary lymph node enlargement, which of course did not reveal tumor dissemination. Irradiation and chemotherapy play limited role as adjuvant therapy. Angiosarcomas are the most malignant of all breast tumors. The median survival period is 14.5-34 months with a 5-year survival rate of approximately 15%. Death is usually due to metastasis to lungs, skin and bone and some patients pass away with massive bleeding from metastatic lesions.

 
   References Top

1.McClanaham BJ, Hogg L. Angiosarcoma of the breast. Cancer 1954;7:586-94.  Back to cited text no. 1    
2.McDivitt RW, Stewart FW, Berg JW. Tumours of the breast. Atlas of tumour pathology, Fascicle 2, 2nd Series. Washington DC: Armed Forces Institute of Pathology; 1966.  Back to cited text no. 2    
3.Schmidt GB. Ueber das Angiosarcoma der mamma. Arch Klin Chir 1887;36:421-7.  Back to cited text no. 3    
4.Rosen PR, Kimmel M, Ernsberger D. Mammary angiosarcoma. Cancer (Medicine) 1988;62:2145-51.  Back to cited text no. 4    
5.Montgomery E. Soft tissue tumours. In Silverberg's principles and practice of surgical pathology and cytopathology. In : Silverberg SG, editor. 4th ed, Elsevier: Churchill Livingstone; 2006.  Back to cited text no. 5    
6.Chenk KT, Kirkegaard DD, Bocian JJ. Angiosarcoma of the breast. Cancer 1980;46:368-71.  Back to cited text no. 6    
7.Kessler E, Kozenitzky IL. Haemangiosarcoma of the breast. J Clin Pathol 1971;18:81-4.  Back to cited text no. 7    
8.Saha SP, Thompson R, Still R. Angiosarcoma of the breast. South Med J 1971;64:1376-85.  Back to cited text no. 8    

Top
Correspondence Address:
Asaranti Kar
C/o Dr. Tushar Kar, Qrs. No. JO - I, Medical Campus, S.C.B. Medical College, Cuttack - 753 007, Orissa
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.42551

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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    Abstract
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