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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 4  |  Issue : 3  |  Page : 185-187

Huge malignant mediastinal hemangiopericytoma compressing the heart and mediastinum


Department of Surgery, College of Medicine and Medical Sciences, Taif University, Taif 21947; Department of Surgery, King Abdul-Aziz Specialist Hospital, Taif 10127, Saudi Arabia

Date of Web Publication9-Dec-2015

Correspondence Address:
Majed Al-Mourgi
Department of Surgery, College of Medicine, Taif University, P.O. Box: 888, Taif 21947
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0521.171439

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  Abstract 

Hemangiopericytoma is a hypervascular tumor, originating from the pericytes of the capillary vessels and occurs anywhere in the body, but it is a rare mediastinal tumor. In this case report, I will present a 45-year-old male patient with huge mediastinal hemangiopericytoma compressing the heart and occupying most of the anterior mediastinum. To my knowledge, it was the first case reported in the Middle East and the largest in the English literature. Complete excision was performed but unfortunately, the patient becomes arrested and died at the end of the first postoperative day. Though mediastinal hemangiopericytoma is a rare mediastinal mass, it must be considered in differential diagnosis, and if it is suspected radiologically, the Tru-cut needle biopsy must be avoided and the tumor hypervascularity can be reduced by preoperative embolization or radiotherapy to facilitate its resection.

Keywords: Cardiac compression, hemangiopericytoma, mediastinal


How to cite this article:
Al-Mourgi M. Huge malignant mediastinal hemangiopericytoma compressing the heart and mediastinum. Saudi J Health Sci 2015;4:185-7

How to cite this URL:
Al-Mourgi M. Huge malignant mediastinal hemangiopericytoma compressing the heart and mediastinum. Saudi J Health Sci [serial online] 2015 [cited 2019 May 20];4:185-7. Available from: http://www.saudijhealthsci.org/text.asp?2015/4/3/185/171439


  Introduction Top


Hemangiopericytoma is derived from the vascular pericytes and represents about 1% of the vascular tumors.[1],[2] The mediastinum is a rare site of the tumor, and the most common sites are the subcutaneous soft tissues, muscles of the extremities, and retroperitoneum.[3] Hemangiopericytoma is usually discovered incidentally as a noncalcified solitary mass on chest X-ray, with no specific clinical or radiographic features.[4] Herein, a huge mediastinal hemangiopericytoma with marked cardiac and mediastinal compression is presented.


  Case Report Top


A 45-year-old Nigerian male patient presented to a hospital at Taif city with a mild right sided chest pain of 3 months duration associated with shortness of breath, dry cough, and intermittent low-grade fever. The case was diagnosed as mediastinal mass (examination report and investigations are not available), and a Tru-cut biopsy was done, however, after performing the biopsy the patient conditions deteriorated and they transferred him to our hospital. The patient was dyspneic with facial edema, engorged neck veins, pulse were 105/min, blood pressure 130/80, respiratory rate 30/min, and oxygen saturation was 70% on room air. Resuscitation was performed followed by plain radiography [Figure 1] and computed tomography [Figure 2] examination of the chest where a 30 cm × 30 cm anterior mediastinal mass was detected shifting the heart and the great vessels to the left and extending to the right side to occupy about 60% of right hemothorax. Laboratory findings were within normal. Improvement of the patient was not complete, and a decision of exploration was made. A right anterolateral thoracotomy was done, and exploration revealed a huge anterior mediastinal mass shifting the heart and mediastinum to the left and extending to the right hemithorax. The mass was capsulated with highly vascular surface, and the sites of the Tru-cut needles were oozing blood. The hematoma was explored where the capsule was opened; blood was evacuated, and bleeding was stopped after taking a biopsy. The first postoperative week was uneventful, but the chest tightness was not improved. The biopsy result showed a malignant hemangiopericytoma. At the end of the 1st week, the patient started to have chest tightness, tachypnea, and his oxygen saturation dropped again to 70% on room air. Echocardiography showed; a left ventricular dilatation with the akinetic anterior wall, tricuspid regurge, the pulmonary artery systolic pressure was 85 mm Hg, and the ejection fraction was 20%. After the meeting of the local oncology board had decided that chemotherapy and radiotherapy are almost ineffective, and surgical excision is the only measure, especially in the presence of a marked cardiac compression, a decision of resection of the tumor was done after counseling the patient and taking a high risk-informed written consent. The chest was opened through an anterolateral thoracotomy; the mass was highly vascular but without obvious feeding vessels, with extensive areas of infarction, however, thorough dissection of the capsulated mass from the right lung, chest wall, and diaphragm was done [Figure 3]. Hard vascular adhesions were found with the heart and the mediastinum, the hemostasis was very difficult, and the patient had been transfused with 12 units of packed red blood cells to maintain the central venous pressure within normal limits, however, the tumor was completely excised, and the chest was closed. Postoperatively, the patient's cardiac conditions were unstable, and, unfortunately, he was arrested at the end of the first postoperative day and did not return.
Figure 1: Preoperative chest X-ray

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Figure 2: Preoperative chest computed tomography scan

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Figure 3: Specimen after resection

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  Discussion Top


Hemangiopericytoma is a rare potentially malignant tumor originating from pericytes that surround the basement membrane of capillaries and small venules within the lung parenchyma or mediastinal structures.[3] The present case was an anterior mediastinal hemangiopericytoma, which is extremely rare, and few case reports were recorded in the literature whereas the intrapulmonary hemangiopericytoma is relatively more common.[1],[4],[5],[6],[7],[8]

A hemangiopericytoma usually affects elderly patients with no specific clinical or radiographic features, and usually presents as an asymptomatic, noncalcified solitary mass on chest X-ray.[4] In this case, the patient was younger than recorded, and the presentation was late with marked respiratory and cardiac manifestations.

These tumors are composed of aggregated spindle cells with prominent vascular channels and the presence of the characteristic stag horn cells, and malignant form is recognized by its increased mitotic rate, large tumor size and foci of hemorrhage and necrosis.[3] Immunohistochemically, hemangiopericytomas are known to show a positive response to antibodies against vimentin and Type IV collagen and a negative response to Type VIII-related antigen, S-100 protein, neuron-specific enolase, carcinoembryonic antigen, desmins, laminin, and cytokeratins.[9] These findings coincide with that in our case.

Preoperative histological diagnosis is usually difficult and is not mandatory if high vascularization is suspected on imaging techniques and when a mass appears to be radiologically resectable.[10],[11] Unfortunately, this patient presented to our hospital after having a Tru-cut needle biopsy followed by the development of progressive hematoma with cardiac and mediastinal compression.

Most of the investigators found that it is necessary to consider all hemangiopericytomas to be malignant and to perform extended radical excision; they also recommended looking for an intrathoracic spread with manual examination with resection of any suspected tissues.[12] In the present case; the patient presents in an advanced stage, but surgical resection was elected to relieve cardiac and mediastinal compression. Complete excision was done in spite of the marked hypervascularity of the tumor, but unfortunately, the patient died at the end of the first postoperative day after developing ventricular fibrillation.

Some authors recommend adjuvant chemo and radiotherapy, though, is considered to be almost ineffective.[3] However, it was reported that adriamycin as a single therapy or in combination therapy was effective against metastases.[13] Other studies verified that preoperative radiotherapy of large hemangiopericytomas significantly reduced the vascularity of the tumor and made complete resection much easier.[14] In the present case due to the presence of marked mediastinal compression preoperative irradiation had been contraindicated, in addition, embolization was not available.

A proposed innovative treatment protocol includes percutaneous embolization of hypervascular mediastinal tumors followed by radical surgical resection with intraoperative and postoperative radiotherapy.[14],[15],[16] For local tumor recurrence or superior vena cava obstruction, many investigators recommend palliative radiotherapy.[13],[14],[16]

The prognosis of intrathorathic hemangiopericytoma is poor and the relative 5 years survival is <35% versus 85% in other sites.[12]


  Conclusion Top


Though mediastinal hemangiopericytoma is a rare mediastinal mass, it must be considered in differential diagnosis, and if it is suspected radiologically, the Tru-cut needle biopsy must be avoided and the tumor hypervascularity can be reduced by preoperative radiotherapy or embolization to facilitate its resection.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Kulshreshtha P, Kannan N, Bhardwaj R, Batra S, Gupta S. Primary mediastinal hemangiopericytoma treated with preoperative embolization and surgery. Ann Thorac Surg 2014;97:335-8.  Back to cited text no. 1
    
2.
Madala J, Guttikonda VR, Korlepara R. Lipomatous hemangiopericytoma in a child: A case report with immunohistochemical evaluation. Journal of Oral and Maxillofacial Pathology: JOMFP 2015; 19: 92-6.  Back to cited text no. 2
    
3.
Simonton SC, Swanson PE, Watterson J, Priest JR. Primary mediastinal hemangiopericytoma with fatal outcome in a child. Arch Pathol Lab Med 1995;119:839-41.  Back to cited text no. 3
    
4.
Hayashi A, Takamori S, Tayama K, Mitsuoka M, Tamura K, Shirouzu K, et al. Primary hemangiopericytoma of the superior mediastinum: A case report. Ann Thorac Cardiovasc Surg 1998;4:283-5.  Back to cited text no. 4
    
5.
Mori M, Nakanishi N, Furuya K. Hemangiopericytoma of the mediastinum causing spontaneous hemothorax. Ann Thorac Surg 1994;58:1525-7.  Back to cited text no. 5
    
6.
Gómez Fiñana MS, Payá Pérez L, Paredes Osado JR, Aranda López I, Massuti Sureda B, Talavera Sánchez J. Hemangiopericytoma of the soft palate and mediastinum: A case report. Acta Otorrinolaringol Esp 1994;45:465-8.  Back to cited text no. 6
    
7.
Fukunaga M, Shimoda T, Nikaido T, Ushigome S, Ishikawa E. Soft tissue vascular tumors. A flow cytometric DNA analysis. Cancer 1993;71:2233-41.  Back to cited text no. 7
    
8.
Chnaris A, Barbetakis N, Efstathiou A, Fessatidis I. Primary mediastinal hemangiopericytoma World J Surg Oncol 2006;4:23.  Back to cited text no. 8
    
9.
Yoshida M, Morita M, Kakimoto S, Kawakami M, Sasaki S. Primary hemangiopericytoma of the trachea. Ann Thorac Surg 2003;76:944-6.  Back to cited text no. 9
    
10.
Hansen CP, Francis D, Bertelsen S. Primary hemangiopericytoma of the lung. Case report. Scand J Thorac Cardiovasc Surg 1990;24:89-92.  Back to cited text no. 10
    
11.
Ching Ong C, Teo LLS. Imaging of anterior mediastinal tumours. Cancer Imaging 2012;12:506-15.  Back to cited text no. 11
    
12.
Espat NJ, Lewis JJ, Leung D, Woodruff JM, Antonescu CR, Shia J, et al. Conventional hemangiopericytoma: Modern analysis of outcome. Cancer 2002;95:1746-51.  Back to cited text no. 12
    
13.
Jalal A, Jeyasingham K. Massive intrathoracic extrapleural haemangiopericytoma: Deployment of radiotherapy to reduce vascularity. Eur J Cardiothorac Surg 1999;16:378-81.  Back to cited text no. 13
    
14.
Mira JG, Chu FC, Fortner JG. The role of radiotherapy in the management of malignant hemangiopericytoma: Report of eleven new cases and review of the literature. Cancer 1977;39:1254-9.  Back to cited text no. 14
[PUBMED]    
15.
Jha N, McNeese M, Barkley HT Jr, Kong J. Does radiotherapy have a role in hemangiopericytoma management? Report of 14 new cases and a review of the literature. Int J Radiat Oncol Biol Phys 1987;13:1399-402.  Back to cited text no. 15
[PUBMED]    
16.
Nair S, Gobin YP, Leng LZ, Marcus JD, Bilsky M, Laufer I, et al. Preoperative embolization of hypervascular thoracic, lumbar, and sacral spinal column tumors: technique and outcomes from a single center. Interventional Neuroradiology 2013;19:377-85.  Back to cited text no. 16
    


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