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LETTER TO THE EDITOR
Year : 2016  |  Volume : 53  |  Issue : 1  |  Page : 122-123
 

Angiosarcoma of pericardium presenting with recurrent pericardial effusion


Department of Pathology and Cardio-thoracic Center, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication28-Apr-2016

Correspondence Address:
N Kumar
Department of Pathology and Cardio-thoracic Center, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.180855

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How to cite this article:
Kumar N, Sharma S, Choudhary U K, Ray R. Angiosarcoma of pericardium presenting with recurrent pericardial effusion. Indian J Cancer 2016;53:122-3

How to cite this URL:
Kumar N, Sharma S, Choudhary U K, Ray R. Angiosarcoma of pericardium presenting with recurrent pericardial effusion. Indian J Cancer [serial online] 2016 [cited 2020 Jul 10];53:122-3. Available from: http://www.indianjcancer.com/text.asp?2016/53/1/122/180855


Sir,

Angiosarcomas are rare and highly malignant tumor of vascular origin. [1] It usually occurs in the skin, deep soft-tissue, head and neck, breast, spleen, liver, and heart. They are extremely rare in the pericardium and other serous membranes such as the pleura and peritoneum.[2] We report a case of pericardial angiosarcoma in a 56-year-old female who presented with multiple episodes of hemorrhagic pericardial effusion.

The present case was a 56-year-old female who presented recurrent history of dyspnea and chest pain since 6 months. X-ray and transthoracic echocardiography revealed presence of pericardial effusion; however, no obvious mass was seen either in the pericardium or in heart. Pericardiocentesis were performed three times and every time the color of pericardial fluid was hemorrhagic. The samples were sent for cytopathological examination. The smears prepared from centrifugated material did not show any clue of malignancy on morphology and the fluids were reported as hemorrhagic in nature. The patient underwent pericardiectomy operation and even at the time of intraoperative procedure no specific mass was found.

The pericardiectomy specimen was sent for histopathological examination. The specimen was grossly unremarkable except some focal areas of thickening and hemorrhagic discoloration. Multiple sections from representative areas were taken. Histopathological examination showed microscopic foci of a malignant tumor. The tumor cells were predominantly spindle in shape and showed nuclear pleomorphism along with mitotic figures. At few places the cells were forming irregular vascular channels. Immunohistochemical stains were performed and these cells were immunopositive for Cluster of Differentiation (CD) 31 (vascular marker). Hence, the overall findings were that of a high-grade angiosarcoma arising from pericardium [Figure 1]. The patient expired 3 days after the surgery due to post-operative complications.
Figure 1: Photomicrographs show features of a high-grade angiosarcoma. (a) The tumor cells are spindle in shape and arranged in short fascicles (H and E, ×40). (b) High power photomicrographs show pleomorphism and ill-defined vascular spaces (H and E, ×100). (c) (H and E, ×200). (d) Tumor cells are immunopositive for CD31 (IHC: CD31, ×200)

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Primary cardiac tumors are rare entities (0.0017-0.003% of routine autopsy studies, 25% malignant).[1] Angiosarcoma is the most frequent primary malignant cardiac tumor (31% of all the malignancies); although primary pericardial angiosarcoma is an uncommon.[2]

Angiosarcomas are traditionally associated with a poor prognosis. The biological behavior depends on the histological grade, site of origin and multifocality but generally has a rapidly progressive course. Surgical resection with or without adjuvant radiation or chemotherapy is the main treatment modality.[3],[4] in the index case there was no obvious visible lesion in the pericardium but there are multiple microscopic foci of tumors. The histology is also of high-grade.

We had a case of high-grade pericardial angiosarcoma who presented with recurrent hemorrhagic pericardial effusion. Hemorrhagic pericardial effusions as in the present case should prompt the clinician to search for a malignant etiology, even when the malignant cells are not identified on cytology of fluid. Earlier detection and targeted therapies may help improve survival in this otherwise almost uniformly fatal malignancy.


  Acknowledgment Top


We are grateful to the Department of Pathology, AIIMS for providing the facilities and resources.

 
  References Top

1.
Silverman NA. Primary cardiac tumors. Ann Surg 1980;191:127-38.  Back to cited text no. 1
[PUBMED]    
2.
Blondeau P. Primary cardiac tumors: French studies of 533 cases. Thorac Cardiovasc Surg 1990;38:192-5.  Back to cited text no. 2
    
3.
Herrmann MA, Shankerman RA, Edwards WD, Shub C, Schaff HV. Primary cardiac angiosarcoma: A clinicopathologic study of six cases. J Thorac Cardiovasc Surg 1992;103:655-64.  Back to cited text no. 3
    
4.
Terada T, Nakanuma Y, Matsubara T, Suematsu T. An autopsy case of primary angiosarcoma of the pericardium mimicking malignant mesothelioma. Acta Pathol Jpn 1988;38:1345-51.  Back to cited text no. 4
    


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