Primary angiosarcoma of breast: A case report

Abstract

INTRODUCTION

Primary breast angiosarcoma is a rare form of breast cancer, accounting for only 0.04% of all malignant breast tumours.

PRESENTATION OF CASE

A 28-year-old woman presented with a progressive lump in the right breast for one month. Fine needle aspiration cytology (FNAC) was highly suspicious of angiosarcoma, and this diagnosis was confirmed on core needle biopsy. Modified radical mastectomy was performed as sole treatment.

DISCUSSION

Primary breast angiosarcoma is a rare type of breast malignancy, usually occurring in the third to fourth decade and only reported in women. The histological features of angiosarcoma of the breast are conventionally graded I, II or III. Total mastectomy appears to be the only treatment conferring benefit, chemotherapy and radiation therapy being of little proven value to date. The 5-year disease-free survival for grade I tumours can be as high as 76%, and up to 70% for grade II tumours whereas for grade III tumours it is reportedly about 15%.

CONCLUSION

Primary angiosarcoma of the breast is a rare malignancy with a poor prognosis, even after complete resection. Surgery is the mainstay of treatment with a limited role for chemotherapy and radiotherapy.

1. Introduction

Malignant breast lesions that arise from stromal tissues are extremely rare, accounting for <1% of all malignant breast tumours. Most mammographically and clinically evident intraparenchymal vascular tumours prove to be malignant angiosarcomas. Angiosarcoma is the most common sarcoma to occur in the breast, but is relatively rare. Angiosarcoma usually presents as a palpable mass, but 17% of cases may present with a bluish discolouration or bruising of the overlying skin. This malignant tumour occurs primarily in young women. Ultrastructural examination and immunostaining for factor VIII-related antigen helps with the diagnosis of angiosarcoma of the breast, a neoplasm which carries poor prognosis.

2. Case report

2.1. Presentation of case

A 28-year-old female presented with a lump in the right breast for a month, rapidly progressive. There was no history of breast surgery or breast irradiation. On examination the 5 cm × 5 cm mass in the upper inner quadrant of the right breast was firm, non tender and not attached to the skin or deep structures, and there was no evidence of nipple retraction, skin thickening or axillary lymphadenopathy. Routine blood investigations and a chest X Ray were within normal limits, as were a serum CEA and serum CA 15-3. Mammography of the right breast showed an ill defined, non spiculated and non-calcified lobulated mass, 5 cm in diameter. USG was not performed. FNAC was highly suspicious of angiosarcoma and the diagnosis was confirmed on core needle biopsy. A modified radical mastectomy was performed. The cut surface of the tumour appeared spongy and haemorrhagic. Microscopic examination of the mass demonstrated many dilated vascular spaces lined and surrounded by clusters of spindle cells suggestive of grade II angiosarcoma. On immunohistochemistry the tumour was positive for CD31. At two-month follow-up the patient was free of any evidence of recurrence.

3. Discussion

Angiosarcomas are the most common sarcoma of the breast but are still relatively rare. The neoplasm is of vascular origin. The frequency of this rare tumour is 0.04% of primary breast tumours¹ and approximately 8% of breast sarcomas. Angiosarcoma can occur in the breast as a primary lesion or as a secondary lesion as in chronically lymphedematous arms after axillary treatment for carcinoma (Stewart–Treves syndrome) and as a complication of breast radiation treatments.

Primary angiosarcomas usually occur in young women (20–50 years).^2,3

Angiosarcoma occurs almost exclusively in the female breast, with only five cases of male breast angiosarcoma reported in the literature.

Angiosarcoma may have an insidious clinical onset, presenting as a painless often discrete palpable mass that grows rapidly.^4,5 Some patients complain of a painful mass with tenderness. Approximately 2% of patients may present with diffuse enlargement of the breast. However, a bluish red discolouration of the overlying skin may be there.⁵ Nipple retraction, discharge, or axillary node enlargement are generally absent. In most reported cases, the tumour size is >4 cm in diameter.⁶ Most reports indicate that tumour size does not correlate with survival. The tumour size at discovery can reach 4 cm in diameter. Bilateral tumours have been reported and several cases have been diagnosed in the postmenopausal women.

On mammograms, angiosarcomas appear as an ill-defined mass and lack the spiculations often seen in breast carcinomas. Rarely, the tumour may show a soap bubble appearance with a mean size of about 4.5 cm. They rarely manifest coarse non-branching microcalcifications.⁷

Sonography usually shows a solid mass that may have well-defined or lobulated margins, with both hypoechoic and hyperechoic appearance. There is often no acoustic shadowing.⁷

Magnetic resonance imaging (MRI) of angiosarcoma shows a mass with low signal intensity on T1-weighted images, but high signal intensity on heavily T2-weighted images. The latter suggests the presense of vascular channels containing slowflowing blood.^7,8

Preoperative diagnosis, by FNAC and biopsy, may be difficult. Chen et al. reported that the false negative rate of percutaneous biopsy was 37%.⁹ The differential diagnoses of this rare malignancy include benign haemangioma, cystosarcoma phyllodes, stromal sarcoma, metaplastic carinoma, fibrosarcoma, liposarcoma, and reactive spindle cell proliferative lesions.^6,10 Large-core biopsies might facilitate the correct diagnosis as they provide a larger sample,¹¹ but such a macrobiopsy is often difficult to perform due to the vascular nature of these tumours. Surgical resection and microscopic examination of sufficient sampling of the tumour are often necessary to render a final diagnosis (Figs. 1 and 2).

Fig. 1.

Microscopic photograph of angiosacoma of breast stained by H & E stain under low magnification.

Fig. 2.

Microscopic photograph of angiosacoma of breast stained by H & E stain under high magnification.

The histologic features of angiosarcoma of the breast are classified into grades I, II and III. Angiosarcomas have a wide range of histological appearances from well-differentiated grade I tumours consisting infiltrating bland vascular channels to poorly differentiated grade III tumours with a sarcomatous spindle cell pattern. Large amounts of blood are often present. Papillary clusters of tumours cells, which can be mistaken for ductal carcinoma in situ, may also occur. Factor VIII and CD31 immunostaining confirms the diagnosis.

Primary angiosarcoma of the breast tends to metastasise haematogenously to lungs, liver, bones, skin and the contralateral breast (from the other breast).

As angiosarcomas of the breast are very rare, there is no established standard treatment. Mastectomy is the mainstay of treatment. Although some individuals seem to benefit from chemotherapy, it is of minimal benefit for patients with disseminated disease. Paclitaxel has been shown to produce excellent response in a number of studies in patients with primary angiosarcoma of breast.¹² Immunotherapy may also play a part in treating this rare type of breast cancer. Recently, an approach to attacking a proliferative endothelium has been extensively investigated. New agents against angiogenesis, such as bevacizumab¹³ or rapamycin,¹⁴ might also be useful against this tumour.

According to Rosen's study, the 5 years disease free survival rate for low grade tumours can be as high as 76% and up to 70% for intermediate grade tumours. Whereas 5 years survival rate for high grade tumours is about 15%.¹⁵

4. Conclusion

The importance of this case report is that primary breast angiosarcoma is a rare disease which can develop without prior exposure of the breast to either surgery or irradiation. A mass that shows heterogeneous hyperechogenicity on ultrasound with an associated architectural distorted appearance and typical malignant characteristics should alert the radiologist to a possible diagnosis of angiosarcoma. Core cut biopsy is the most important method of making the diagnosis of primary breast angiosarcoma. Total mastectomy remains the most effective mode of treatment. Skin thickening should be evaluated thoroughly and if necessary biopsied to exclude malignant infiltration. Although no clinical trial has yet proven any benefit from adjuvant chemotherapy or radiation therapy in managing angiosarcoma, both therapies should be considered in patients at high risk of recurrence.

Conflict of interest statement

There is no conflict of interest.

Funding

None.

Ethical approval

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Author contributions

All authors contributed equally.