Key findings:

The abstract discusses the rarity and aggressive nature of primary angiosarcomas of the breast, comprising only 0.05% of breast carcinomas. It reports a case of a 44-year-old female presenting with a right breast lump diagnosed as angiosarcoma. Mastectomy was performed, followed by chemotherapy, highlighting the challenging diagnosis and treatment of this malignancy.

What is known and what is new?

The abstract highlights the rarity and aggressiveness of primary angiosarcomas of the breast, constituting only 0.05% of all breast carcinomas. The challenging diagnosis and poor survival rates are well-documented. However, the new aspect is the presentation of a case involving a 44-year-old female undergoing mastectomy and subsequent chemotherapy, underscoring the importance of multidisciplinary management strategies.

What is the implication, and what should change now?

The case highlights the rarity and aggressive nature of primary angiosarcomas of the breast, necessitating improved awareness and diagnostic strategies for early detection. Enhanced multidisciplinary approaches are crucial for effective management, including prompt surgical intervention followed by adjuvant therapies like chemotherapy. Continuous research into more targeted treatments is imperative to improve outcomes for patients with this challenging condition.

Angiosarcoma is a vascular tumor involving endothelium of blood or lymphatic vessels. They can be present at various sites like scalp, breast and extremities[1]. They constitute overall about 0.0005-0.05% of all breast carcinoma and 1% of all sarcomas. They affect women of the age group 30-40 years[2]. Angiosarcomas of the breast are of two types, primary and secondary. Primary angiosarcoma of breast (PAB) develops for the first time, while secondary angiosarcomas develop after previous cancer treatment with radiation therapy[3]. They are characterized as being highly malignant with high chances of local recurrence and visceral metastasis. The patients have poor outcomes and survival. Median survival is only 24 months[4]. The diagnosis of angiosarcoma breast is mainly by histology of excised specimens[5]. Treatment mainstay is surgery[6]. Role of chemotherapy and radiation therapy is very limited owing to the rarity of disease and lack of effective data.

A 44 year old patient presented to us with awareness of a lump in right breast for about 2 months. When she noticed the lump for the first time it was a small nodule in right breast. It progressed rapidly in two months to become a hard mass involving the whole breast. It was a painless mass. There were no other remarkable features in the patient's medical history. There is no history of previous breast or chest wall irradiation. For these complaints she visited the surgical department of our hospital. She had a mass in right breast of size 6x5cm involving upper outer and lower quadrants and nipple areola complex. It was not fixed to underlying muscle or overlying skin. There were no palpable nodes in the axilla or neck region. A tru cut biopsy was done suggestive of angiosarcoma breast. She was then operated at the surgical department. A modified radical mastectomy was done. The gross pathological examination revealed a 6.5x5.5x5cm gray brown mass with hemorrhagic foci involving retro areolar region and upper and inner quadrants of right breast. It was 2 cm from the deep resection margin and 1 cm from the skin of the nipple. Microscopic examination revealed foci of tumor cells infiltrating adipose and fibrous tissue of breast. Tumor cells are slender to spindle shaped and epithelioid having hyperchromatic nuclei with free chromatin, inconspicuous nuclei and eosinophilic cytoplasm. These cells are lining vascular channels separated by thin fibrous stroma and extravasated red blood cells. Margins were free. No lymphatic spread was present. On immunohistochemistry the cells stained positive for CD31, CD34 and Ki67 index of 20%. A diagnosis of high grade PAB was made and the patient was referred to our center. Patient is now receiving chemotherapy at our center.
 

Angiosarcomas of both primary and secondary constitute about 0.0005-0.05% of all breast malignancies and 1% of all sarcomas[1]. Primary Angiosarcoma of Breast constitute about 0.05% of all mammary tumors and 8% of all breast sarcomas [2]. They affect females of age group 30-40 years [3]. In our patient the age was 44 years. 

PAB are characterized as highly malignant tumors with high chances of local recurrence and visceral metastasis. The median survival is 24 months and five year recurrence free survival rate of only 33% [4].

The factors involved in carcinogenesis are exposure to vinyl chloride, arsenic and thermostat, foreign body-induced chronic irritation and the notion of localized trauma [5]. The primary breast angiosarcoma must be differentiated from radiotherapy induced secondary angiosarcoma. In our patient there was no history of any previous radiation. No other risk factor was present to the patient.[6]

Angiosarcomas usually are asymptomatic at first, and later present as a painless nodule that grows rapidly. Bluish red or purple discoloration of skin may be present [6]. The age and clinical presentation of patients were compatible with PAB.

Diagnosis of PAB is by histology using fine needle aspiration cytology or a core needle biopsy [7]. Radiologic diagnosis of PAB is difficult as there are no defined radiologic features for PAB. Magnetic Resonance Imaging of breast is helpful for diagnosing PAB. T1 and T2 scans show heterogeneous low and high intensity mass respectively. Sometimes, T1 scans can also show high intensity areas corresponding to hemorrhagic zones [8]. 

The PAB are classified into three grades I, II, III. Type I is low grade, Type II intermediate and Type III high grade tumor [9]. On Immunohistochemistry vascular markers CD34, CD31 are expressed. In addition, as these are vascular tumors developing from endothelial cells over expression of vascular endothelial growth factor VEGF-A and VEGFR-1 are seen [10].In our patient vascular markers CD34 and CD31 were expressed.

The standard treatment is mastectomy. Axillary lymph node dissection is not done routinely as lymphatic spread is rare. It is done only in cases of palpable axillary lymph nodes [11]. Role of adjuvant therapy is not well defined as PAB is a rare disease with no effective trials done so far. Kaklamanos et al., found tumor size, grade and margin status are the most important prognostic factors for survival [12 ]. In our patient the disease was a high grade tumor. She after surgery is now receiving adjuvant chemotherapy at our center. 

PAB is a rare entity. Its diagnosis is difficult as there are not many studies regarding the histology, clinical or radiologic features of the disease. It is a very poor prognostic disease and requires early detection and rapid treatment. More studies are warranted in future to establish the role of adjuvant therapies whether radiotherapy, chemotherapy or immunotherapy for effective treatment of PAB.

Funding: No funding sources.

Conflict of interest: None declared.

Ethical approval: The study was approved by the Institutional Ethics Committee of Shri Lal Bahadur Shastri Medical College.

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