Unexplained recurrent pericardial effusion in a young adult male: Think beyond tuberculosis

Anshuman Darbari; Barun Kumar; Augustine Jose; Ajit Kumar

doi:10.4103/rcm.rcm_39_21

CASE REPORT

Year : 2021 | Volume : 10 | Issue : 3 | Page : 88-90

Unexplained recurrent pericardial effusion in a young adult male: Think beyond tuberculosis

Anshuman Darbari¹, Barun Kumar², Augustine Jose³, Ajit Kumar⁴
¹ Department of CTVS, AIIMS, Rishikesh, Uttarakhand, India
² Department of Cardiology, AIIMS, Rishikesh, Uttarakhand, India
³ Department of General Medicine, AIIMS, Rishikesh, Uttarakhand, India
⁴ Department of Anaesthesia, AIIMS, Rishikesh, Uttarakhand, India

Date of Submission	05-Jul-2021
Date of Decision	15-Aug-2021
Date of Acceptance	17-Aug-2021
Date of Web Publication	03-Nov-2021

Correspondence Address:
Dr. Anshuman Darbari
Department of CTVS, AIIMS, Rishikesh - 249 203, Uttarakhand
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/rcm.rcm_39_21

Abstract

Pericardial effusion is usually caused by infection, fluid overload states, connective tissue disorders, heart surgery, aortic dissection, and malignancy. When a patient presents with recurrent isolated pericardial effusion accompanied by a nonspecific history and negative laboratory tests, it can pose a diagnostic dilemma to the clinician. Primary malignant tumors of the pericardium are sporadic, and most primary malignant pericardial tumors are mesotheliomas. We report the case of a young adult male with recurrent pericardial effusion and no specific clinical clues enabling an early diagnosis, which later turned out to be caused by a primary angiosarcoma of the pericardium.

Keywords: Angiosarcoma, cardiac tumors, pericardial effusion, pericardium, tamponade

How to cite this article:
Darbari A, Kumar B, Jose A, Kumar A. Unexplained recurrent pericardial effusion in a young adult male: Think beyond tuberculosis. Res Cardiovasc Med 2021;10:88-90

How to cite this URL:
Darbari A, Kumar B, Jose A, Kumar A. Unexplained recurrent pericardial effusion in a young adult male: Think beyond tuberculosis. Res Cardiovasc Med [serial online] 2021 [cited 2023 Feb 7];10:88-90. Available from: https://www.rcvmonline.com/text.asp?2021/10/3/88/329846

Introduction

Neoplastic involvement of the pericardium most commonly occurs in the setting of an extrapericardial malignancy by direct extension or metastatic deposits. Primary malignant tumors of the pericardium are very rare. It is extremely rare to find a primary angiosarcoma originating from the pericardium, though; this is the most frequent differentiated sarcoma arising in the pericardium. Angiosarcoma is a malignant tumor with a vascular origin, found in skin, soft tissue, solid organs, and head-and-neck region. It has high malignant potential and is invariably associated with poor prognosis and short survival.^[1]

Case Report

A 26-year-old male, who was previously healthy, presented to our hospital with progressive exertional dyspnea and heaviness in the chest of 2-month duration. He does not have any associated chest pain, palpitations, cough, or hemoptysis. He was diagnosed as a case of pericardial effusion on the basis of clinical examination and an enlarged cardiac silhouette with sharply defined margins on the plain chest radiograph [Figure 1]. Transthoracic echocardiogram revealed a large pericardial effusion [Figure 2] and early diastolic collapse of the right ventricle and right atrium with tamponade physiology. He underwent an emergent pericardial catheter insertion for diagnostic and therapeutic purposes, under fluoroscopic guidance, and about 1 l of hemorrhagic fluid was removed. Fluid analysis revealed a monomorphic predominant exudative effusion without any abnormal cells. Gram stain was negative, and culture was sterile.

Figure 1: Plain chest radiograph showing an enlarged cardiac shadow with sharply defined borders

Click here to view

Figure 2: Transthoracic echocardiogram showing a large pericardial effusion. PE: Pericardial effusion; RV: Right ventricle; LV: Left ventricle; Ao: Aorta; LA: Left atrium

Click here to view

On detailed clinical examination, he was afebrile, moderately built without pallor, lymphadenopathy, jaundice and hepatosplenomegaly, sternal or bony tenderness, joint involvement, rashes, external markers of tuberculosis, or any features pointing to the connective tissue disorders.

He did not have associated fever, night sweats, significant weight loss, or contact with a tuberculosis case, nor was there any historical evidence suggestive of hypothyroidism or connective tissue disorder. Hemogram, peripheral blood picture, liver function tests, renal function tests, inflammatory markers, and thyroid function status were within normal limits. Mantoux test was negative, and antinuclear antibodies were not detectable.

A contrast-enhanced computed tomography (CE-CT) scan of the thorax was done, which did not reveal any evidence of pulmonary tuberculosis or thoracic malignancy and pericardial thickening or enhancement. On repeat deterioration, an echocardiogram done after 7 days showed the development of massive pericardial effusion with tamponade physiology again. Echo-guided pericardiocentesis was done again. At this time, polymerase chain reaction (PCR) analysis of pericardial fluid was positive for nontuberculous mycobacteria without any other abnormality. He was started on empirical antituberculous therapy and oral prednisolone.

He was followed up with serial echocardiograms, which revealed again increasing fluid accumulation within 5 days, so he was taken for pericardiectomy or pericardial window. The left anterior thoracotomy under general anesthesia was done. After pericardiotomy, multinodular pericardial lesion of about 2 cm × 2 cm size on the right ventricular diaphragmatic border and a 3 cm × 5 cm firm-hard multinodular cystic lesion over the ascending aorta and aortopulmonary window region [Figure 3] was found. A probable diagnosis of cardiac neoplasm was made [Video 1]. After putting left pleuropericardial drain, pericardium with overlying fat removal was done. The postoperative period was uneventful. After stabilization for 1 week, he was taken for wide excision and reconstruction under cardiopulmonary bypass but unfortunately succumbed 1 day after the reconstructive procedure. The histopathological examination of excised specimen confirmed this as high-grade pericardial angiosarcoma.

Figure 3: Intraoperative photograph showing a nodular lesion in the pericardium (marked by black arrow)

Click here to view

Discussion

The secondary involvement of the pericardium by local extension of malignancy is a more common phenomenon than a primary pericardial tumor. Most commonly, lung and breast malignancies involve the pericardium. Other sources of secondary pericardial involvement include leukemia, non-Hodgkin's lymphoma, and malignancies of gastrointestinal tract, genitourinary and gynecological tumors, and sarcomas of extracardiac origin.^[2],[3] Primary neoplasms arising from the pericardium can be benign or malignant. The common benign pathologies include lipoma, pericardial cyst, paraganglioma, and hemangioma, whereas the malignant neoplasms are mesothelioma, lymphoma, angiosarcoma, synovial sarcoma, liposarcoma, and germ cell tumor. Primary pericardial angiosarcoma is extremely rare, and very few case reports can be found in the literature. Occurrence is described in the 3^rd to 5^th decade of life and is more common in men. Other conditions producing a pericardial lesion are Erdheim-Chester disease ^{More Details}, a nonLangerhans cell histiocytosis, and IgG4-related pericardial infiltration, and thickening, which occurs in a background of pericarditis.^[4] A malignant process involving the pericardium can produce different hemodynamic effects including pericardial effusion and tamponade, myocardial extension and impaired contractile function, and pericarditis. If pericardial involvement is diffuse, it can produce constrictive physiology. In general, symptoms are very vague. Effusion is often hemorrhagic due to the vascular origin of the tumor and is prone to progress early into tamponade.^[5],[6]

Echocardiography does not contribute significantly to the evaluation of pericardial tumors except in identifying and monitoring the secondary effects produced by tumor growth and activity such as pericardial effusion and tamponade. However, it may reveal pericardial thickening, nodules, or mass rarely. A CE-CT scan characterizes many pathologies of the pericardium, but it is not consistent in identifying a primary pericardial malignancy. Angiosarcoma shows avid postcontrast enhancement owing to the vascular nature of the tumor.^[7],[8] Magnetic resonance imaging (MRI) is relatively more yielding in this aspect, though it requires validation studies. MRI-T1 sequences may show heterogeneous enhancement due to hemorrhage and necrosis. Enhancement is also noted in MRI-T2 sequences. Despite the availability of multimodality imaging, a surgical pericardiectomy is still the best way to deal with a pericardial malignancy and when done with wide margins, provides both diagnostic and therapeutic purposes.^[9]

Angiosarcomas have high malignant potential and are associated with poor outcomes even after surgical resection, which is often a palliative procedure. The tumor shows characteristic spindle-shaped neoplastic cells on histopathological examination and demonstrates positivity for CD31, CD34, and Factor VIII on immunohistochemical analysis. They usually have a rapidly progressing course, influenced by the histological grade, multifocality, hemodynamic effects, and site of origin. Various chemotherapeutic agents which have been tried in the treatment of pericardial angiosarcoma are vincristine, etoposide, doxorubicin, paclitaxel, etoposide, and pazopanib. Multimodal therapy has been shown to produce radiological regression and longer survival time in isolated case reports only.^[10]

In our patient, the pericardial fluid culture for bacteria and acid-fast bacilli was negative, as was the examination for malignant cells, initially. However, the presence of nontuberculous mycobacterium on PCR misled us to use empirical antituberculous therapy, which did not work well.

Conclusion

In patients with recurrent and rapidly developing hemorrhagic pericardial effusions without any evidence of infective origin, suspicion of malignancy should also be considered. These patients should undergo thorough evaluation with multimodal imaging, which should include CE-CT scan with MRI and if required, early operative exploration for diagnostic and therapeutic purposes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the parents have given his consent for images and other clinical information to be reported in the journal. The parents understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Silverman NA. Primary cardiac tumors. Ann Surg 1980;191:127-38.
2.	Gupta RK, Kenwright DN, Fauck R, Lallu S, Naran S. The usefulness of a panel of immunostains in the diagnosis and differentiation of metastatic malignancies in pericardial effusions. Cytopathology 2000;11:312-21.
3.	Butany J, Leong SW, Carmichael K, Komeda M. A 30-year analysis of cardiac neoplasms at autopsy. Can J Cardiol 2005;21:675-80.
4.	Kupsky DF, Newman DB, Kumar G, Maleszewski JJ, Edwards WD, Klarich KW. Echocardiographic features of cardiac angiosarcomas: The Mayo Clinic Experience (1976-2013). Echocardiography 2016;33:186-92.
5.	Leduc C, Jenkins SM, Sukov WR, Rustin JG, Maleszewski JJ. Cardiac angiosarcoma: Histopathologic, immunohistochemical, and cytogenetic analysis of 10 cases. Hum Pathol 2017;60:199-207.
6.	Fatima J, Duncan AA, Maleszewski JJ, Kalra M, Oderich GS, Gloviczki P, et al. Primary angiosarcoma of the aorta, great vessels, and the heart. J Vasc Surg 2013;57:756-64.
7.	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.
8.	Ma GT, Liu JZ, Miao Q, Cui QC. Angiosarcoma of the pericardium: A case report. Int J Clin Exp Pathol 2015;8:13568-70.
9.	Chen FF, Jiang SF, Dong C, Che Y, Du LY, Li ZY, et al. Case report: Thromboembolism and hemorrhagic pericardial effusion – The Janus face of primary pericardial angiosarcoma. Front Cardiovasc Med 2020;7:618146.
10.	Li Y, Wang B, Zhang L, Yang Y, Wang J, Lv Q, et al. Rare case of pericardial angiosarcoma. Circ Heart Fail 2018;11:e005342.