Diagnostic difficulties of primary angiosarcoma of the breast: a case report

Abstract

BackgroundAngiosarcoma of the breast is a rare tumor, which may be primary or secondary to breast surgery or irradiation. It is characterized by polymorphic and nonspecific clinical and radiological features. A pathologist plays a key role in positive and differential diagnosis and in establishing the prognosis: only a histological examination can confirm the diagnosis, and the histologic grade is the most important prognostic factor. In fact, angiosarcomas of the breast constitute a very heterogeneous group and they are classified into three grades based on the degree of differentiation. We will illustrate diagnostic challenges through this new case of primary angiosarcoma of the breast. Microscopic findings were initially interpreted as a benign vascular tumor. We will also discuss the relevant medical literature.Case presentationA 56-year-old Arabian woman presented with a palpable right breast mass that had been enlarging for 2 months, measuring 5 cm, without axillary lymphadenopathy. She had no personal or family history of breast surgery or breast irradiation. A mammography showed no evidence of spiculation. No suspicious calcifications were seen. A needle core biopsy was performed. Microscopic findings were initially interpreted as a benign vascular tumor. However, as the mass measured 5 cm, the diagnosis of angiosarcoma was more appropriate, and mastectomy without axillary dissection was performed. Microscopic examination found mild to moderately scattered pleomorphic cells, and scattered mitotic figures. It also showed papillary formations, solid foci of spindle cells, and hemorrhagic necrosis. The margins of the tumor were infiltrative. The diagnosis of primary intermediately differentiated angiosarcoma of the breast (grade II) was made. No distant metastases were found. Our patient was lost to follow-up and further treatment after mastectomy until she developed local tumor progression 4 months later.ConclusionsThrough this case report, we emphasize the importance of clinicopathological confrontation in angiosarcoma of the breast.

Angiosarcoma primario de mama

“…Suele presentarse clínicamente como una masa mal definida, indolora y con rápido crecimiento. Puede manifestarse de forma bilateral y la afectación axilar no es común (0-5%) 5 . En tumores de gran tamaño se han descrito casos de trombocitopenia y clínica hemorrágica, denominado síndrome de Kasabach-Merritt 6 .…”

Primary and Secondary Angiosarcoma of the Breast: Report of Two Cases and Review of the Literature

“…For example, in the primary tumor, patients have a history of exposure to carcinogenic agents, such as vinyl chloride, arsenic, and thorium dioxide. On the other hand, about the development of secondary angiosarcoma, the main associated factor is a chronic inflammation of the tissues: radiotherapy, radical mastectomy and axilla dissection [3,8]. In other words, we can affirm that the frequency of the secondary tumor has increased in recent years due to the greater use of radiotherapy in breast-conserving surgery [4].…”

“…For this reason, immunohistochemical markers are a fundamental tool. The main stains are CD31, CD34 and factor VIII; the best is CD31, which identifies endothelial membrane proteins individually [6,8,12]. On the other hand, a functional classification from the histopathological point of view is Rosen's, which groups angiosarcoma of the breast into three grades according to their level of differentiation: low, intermediate, and high [4].…”

“…In the same sense, the histological grade is also related to overall survival and is directly proportional; the more dedifferentiated the neoplasm is, the lower the survival rate. For example, well-differentiated angiosarcomas have a 5-year survival of 76%, while for poorly differentiated tumors, it is only 15% [8]. Finally, it can be pointed out that secondary angiosarcomas are more aggressive since primary angiosarcomas can even reach survival rates of up to 93 months.…”

Breast Angiosarcoma Surveillance Study: UK national audit of management and outcomes of angiosarcoma of the breast and chest wall

“…The histopathological appearances are challenging and reinforce the need for specialised pathological review. Surgery remains the mainstay of treatment but guidance on extent is limited to exhortation to achieve wide margins even though determination of tumour absence at a margin is not straightforward 11 . The paucity of evidence-based advice emphasises the need for the management of breast angiosarcomas within specialist sarcoma multidisciplinary teams and the rigorous collection of all associated clinical data 18 .…”