Journal of Cancer Research and Therapeutics

: 2015  |  Volume : 11  |  Issue : 3  |  Page : 655-

Primary breast angiosarcoma associated with abnormalities in international normalized ratio platelet count and anemia

Pejva Soleymanzadeh1, Mostafa Hosseini2, Sima Karbalaei-Esmaeili2, Hamidreza Alizadeh-otaghvar2,  
1 Department of Surgery, Rassoul-e-Akram Hospital; Core Facilities, Iran University of Medical Sciences, Tehran, Iran
2 Core Facilities, Iran University of Medical Sciences, Tehran, Iran

Correspondence Address:
Pejva Soleymanzadeh
Hazrat-e-Rasoul Hospital, Iran University of Medical Sciences, Tehran


Angiosarcomas are rare malignancies with poor prognosis and could affect any organ in the body. Breast angiosarcomas mostly affect adolescent and young adult females. Here, we present the case of a 19-year-old female with low grade primary breast angiosarcoma associated with international normalized ratio (INR) abnormalities, thrombocytopenia and anemia. Patient experienced severe bleeding on biopsy. INR and count of blood cells turned to normal levels after mastectomy. Patient refused chemotherapy. Three months later, computed tomography showed liver metastasis.

How to cite this article:
Soleymanzadeh P, Hosseini M, Karbalaei-Esmaeili S, Alizadeh-otaghvar H. Primary breast angiosarcoma associated with abnormalities in international normalized ratio platelet count and anemia.J Can Res Ther 2015;11:655-655

How to cite this URL:
Soleymanzadeh P, Hosseini M, Karbalaei-Esmaeili S, Alizadeh-otaghvar H. Primary breast angiosarcoma associated with abnormalities in international normalized ratio platelet count and anemia. J Can Res Ther [serial online] 2015 [cited 2022 Nov 30 ];11:655-655
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Breast angiosarcoma is a rare condition, which makes it difficult to be studied in controlled trials. Therefore, evidences from case reports may provide valuable evidences on diagnosis, complications, patient care and outcomes. [1] This is a highly malignant neoplasm, which is divided into two types: Primary breast angiosarcoma, which affects women without prior history of cancer or radiotherapy and secondary breast angiosarcomas, which happen secondary to radiotherapy and affect those who have history of breast cancer diagnosis. [2],[3] It is caused by uncontrolled division of endothelial cells lining blood vessels. It may happen in different tissues including breast. Breast angiosarcomas comprise about 8% of cases of angiosarcoma, the most common type after skin angiosarcomas. [4]

The disease usually have poor prognosis, as it usually is diagnosed after the disease has spread throughout the body, and this late diagnosis decreases the chance of cure. [2] They are mostly highly aggressive and fast-growing, but in some cases there are low grade less aggressive tumors with slow growth. Platelet deficiency and prothrombin time deficiencies are common in advanced angiosarcomas. Disseminated intravascular coagulation (DIC) with characteristic consumptive coagulopathy is common in angiosarcoma patients. Consumptive coagulopathy is thought to be responsible for coagulopathies. [5],[6],[7] This Kasabach-Merritt syndrome usually affects younger patients suffering from benign vascular tumors. [8]

Here, we present the case of a 19-year-old female with low grade primary angiosarcoma associated with international normalized ratio (INR) abnormalities, which was corrected spontaneously after mastectomy.


Patient was a 19-year-old female, which referred to the hospital due to a palpable mass in outer upper quadrant of the right breast. The mass was emerged 3 weeks ago and have had size increment until reference to the hospital. Patient had no history of medicine consumption, background disease, radiation, and familial background of lung cancer. She has had regular menstruation cycle to the date of reference.

In breast examination, a 3 cm × 4 cm fixed mass without pain, warmness, redness and discharge was found. No sign of ecchymosis or hematoma was observed in overall body examination. Melena and rectorrhagia was not observed. No especial pathology was observed in pelvic and abdominal ultrasonography, and the size of liver and the spleen was normal. Computed tomography (CT) scan using contrast agent showed a highly vascular mass [Figure 1].{Figure 1}

Liver function tests were normal. Complete blood count showed thrombocytopenia (platelet count = 70,000/μl). Coagulation tests showed prothrombin time (thrombin time) = 24, partial thromboplastin time (PTT) = 24 and INR = 3.2. To take biopsy, patient was transferred to operating room and biopsy was taken for pathology testing. Patient went on severe uncontrollable bleeding during biopsy and lost about 2 l of blood. After hematologic assessment and consultation and bone marrow aspiration, no specific pathologic indication was found for thrombocytopenia and hemostatic complications. Immunohistochemistry results were positive for CD31 and negative for cyclin-dependent kinase. The final diagnosis was low grade angiosarcoma.

Patient was mastectomized to remove breast tissue, nipple, areola and skin, but pectoralis major muscle left intact [Figure 2]. Skin defect was grafted with the skin flap from the right femoral region. She was then referred to chemotherapy ward.{Figure 2}

Patient's thrombocytopenia was restored and the platelet count reached 175,000/μl. Coagulation factors returned to normal level (PT = 4, PTT = 35 and INR = 1.1). The patient was referred to the chemotherapy ward, but she refused to receive chemotherapy. Three months later she referred again to the hospital. CT scan imaging showed liver metastasis [Figure 3]. She was alive when we were writing this report and then she lost to follow-up due to changing location.{Figure 3}


Uncontrolled division of vascular endothelial cells give rise to a rare malignancy named angiosarcoma. These sarcomas comprise about %0.04 of primary breast tumors and 8% of sarcomas of the breast tissue. Therefore, like many other rare conditions, data from case reports may have substantial impact on understanding the clinical manifestations and symptoms, etiology and management of the patients. The prognosis is poor and patients usually die shortly after diagnosis of the disease. The disease mainly affects adolescents and young adults. [5],[9]

Some coagulation disorders have been reported in advanced stages of the disease which has been mainly attributed to the consumption of coagulation factors of the blood in DIC events. [5] Platelet deficiency and anemia has been reported in some cases. Consumptive coagulopathy depletes the platelet and coagulation factors in the circulation. This results in increased bleeding on traumat, surgery and similar events. Treatment of these complications is usually necessary to prevent hemorrhage, which could be serious most of the times. Infusions of fresh frozen plasma (FFP) may be useful in bleeding control. However, in this case the patient went on serious bleeding and lost considerable amount of blood. FFP infusions were not effective and we had to control bleeding by gauze-packing the bleeding area. Interestingly, during a short time after mastectomy the patient's coagulation tests returned to normal levels and INR corrected.

As these kinds of tumors are composed of highly vascular masses formed as a result of uncontrolled vascular endothelial cells proliferation, they usually attract a massive amount of blood flow. [10] DIC event in this flow will deplete the coagulation factors in the blood reservoir. [5] It appears that removing breast through mastectomy, eradicated these vascular masses, and finally decreased the DIC events. This in turn lead into correction of the level of coagulation factors in blood and returning the results of coagulation tests into normal level. [5] This is a first report of its kind, which describes a case of primary breast angiosarcoma, which is associated with INR abnormalities and correction of these abnormalities spontaneously after surgical resection of the tumor tissue.

Here, we described an unusual case of rare low grade primary breast angiosarcoma with INR abnormalities, thrombocytopenia and anemia which returned to normal level shortly after surgical removal of the tumor tissue. Our report adds the primary breast angiosarcoma to the possible disorders, which may cause increase INR level and reduced platelet count. However, it is reasonable to test these patients for blood coagulation disorders and associated complications. Further studies on effective management of this condition may help prevent subsequent hemorrhagic outcomes.


We appreciate Dr. Fazel Gorjipour for reviewing our paper before sending out for publication.


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