Angiosarcoma presenting as recurrent breast cancer

Location: Grandball Room 1-2

Primary Presenter(s)

MH

Matthew J. Hamilton, DO

Kettering Health Dayton
Dayton, Ohio, United States

Introduction/Purpose: Angiosarcomas are an extremely rare malignancy of the breast, accounting for less than 0.04% of malignant neoplasms. Angiosarcoma of the breast may occur in primary or secondary form. Imaging and tissue sampling confer diagnostic assistance for the rare pathology. Surgical excision is the only curative-intent treatment currently available, with limited benefits provided by chemotherapy and radiation. Both primary and secondary have a malignant behavior and a poor prognosis.  There are many factors that differentiate the two forms including presentation, precipitating factors, and average age. Primary is usually seen in women in the 4th through 6th decades of life, and secondary is usually see in the upper 7th and lower 8th decades of life or about 10 years post radiotherapy.

Methods or Case Description: We present the case of an 81-year-old female with a past medical history of seizures, multiple sclerosis, basal cell carcinoma of the back, and left-sided breast cancer who presented with a 6-week history of fungating mass of the left breast. The mass was noted to be rapidly growing with foul-smelling odor. As a part of work-up, left-sided breast ultrasound was obtained with evidence of lobulated heterogeneous mass at the 3 o'clock position 7 cm from the nipple measuring approximately 7 x 3.2 cm. The axillary lymph nodes were also evaluated, without any suspicious findings. Ultrasound findings of the breast lesion were determined to be BI-RADS 5, highly suggestive for malignancy. Subsequently, left breast core biopsy was obtained with evidence of extensively necrotic and fibrotic tissue with head or chronic atypical cells, with low rate of mitotic figures. The sample was positive for P120, while estrogen receptor, progesterone receptor, HER2/neu receptor, E-cadherin negative. The atypical cells, however, did demonstrate a high proliferation rate with a Ki-67 of 60 to 70%. Further classification of cells is limited due to tissue samples. Therefore, the decision was made to pursue surgical excision for further characterization of the lesion. The patient did have a previous history of breast conservation therapy with lobectomy and radiation for left-sided malignancy. As a result, left-sided salvage mastectomy with creation of advancement flaps were pursued. On final pathology, the patient was noted to have an 11.2 cm angiosarcoma involving the skin as well as posterior and anteroinferior margin. The specimen was noted to be ERG positive.

Outcomes: This is an 81yo female who had her initial breast cancer in 1972 treated with lumpectomy followed by adjuvant radiotherapy. There was no recollection of adjuvant systemic therapy and records were unavailable for clarification. She was released from oncologic surveillance due to NED status for several years. Mammogram from May 2019 was unremarkable. She presented in March 2022 with rapidly enlarging, fungating left breast mass. Left breast ultrasound 3/2022 revealed a 7 cm lobulated heterogeneous mass 3 o'clock position 7 cm from the nipple. Biopsy 4/2022 revealed extensively necrotic tissue with focal clusters of atypical cells suspicious for malignancy. She underwent surgical resection 4/2022 with left-sided salvage mastectomy. Patient did pass away from unrelated complication of stroke 9/22.

Conclusion:

Angiosarcoma of the breast is an aggressive tumor with a notoriously poor prognosis. Due to the rarity of the malignancies, limited studies are present on definitive treatment and the role of adjuncts to surgery for the management of the neoplasm.

Angiosarcoma can be categorized into primary or secondary; primary predominantly affects young women, while secondary typically impacts in older population with a mean age of 70 years old . The incidence of radiation induced sarcoma after breast cancer (RIS-BC) is 0.07% at 5 years, 0.27% at 10 years, and 0.48% at 15 years