Thymoma: A rare presentation as recurrent pericardial effusion

Dipesh Jha; Prakash Tendulkar; Minakshi Dhar; Bishal Pratap Shah; Khusbhoo Bisht

doi:10.4103/jiag.jiag_2_23

CASE REPORT

Year : 2023 | Volume : 19 | Issue : 1 | Page : 73-75

Thymoma: A rare presentation as recurrent pericardial effusion

Dipesh Jha¹, Prakash Tendulkar², Minakshi Dhar², Bishal Pratap Shah², Khusbhoo Bisht³
¹ Department of Geriatric Medicine, AIIMS, Rishikesh, Uttarakhand, India
² Department of General Medicine, AIIMS, Rishikesh, Uttarakhand, India
³ Department of Clinical Pharmacology, AIIMS, Rishikesh, Uttarakhand, India

Date of Submission	11-Jan-2023
Date of Decision	16-Feb-2023
Date of Acceptance	21-Feb-2023
Date of Web Publication	17-Mar-2023

Correspondence Address:
Minakshi Dhar
Department of Geriatric Medicine, AIIMS, Rishikesh, Uttarakhand
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiag.jiag_2_23

Abstract

Recurrent pericardial effusion is always complicated for the primary care physician to diagnose, and it often goes undiagnosed. Thymoma is rare cancer, but it is the most common tumor in the anterior mediastinum. It can present in a variety of ways; it can be asymptomatic for an extended period of time and only rarely as pericardial effusion. A 68-year-old male presented to us with two episodes of pericardial effusion in the previous 2 years and was later diagnosed with thymoma.

Keywords: Cardiovascular, Masaka, pericardial effusion, recurrent pericardial effusion, thymoma

How to cite this article:
Jha D, Tendulkar P, Dhar M, Shah BP, Bisht K. Thymoma: A rare presentation as recurrent pericardial effusion. J Indian Acad Geriatr 2023;19:73-5

How to cite this URL:
Jha D, Tendulkar P, Dhar M, Shah BP, Bisht K. Thymoma: A rare presentation as recurrent pericardial effusion. J Indian Acad Geriatr [serial online] 2023 [cited 2023 Mar 22];19:73-5. Available from: http://www.jiag.com/text.asp?2023/19/1/73/371900

Introduction

The Thymus is a lymphoid organ located in the anterior mediastinum, above the pericardium, great vessels, and behind the sternum. Thymic neoplasms can manifest as benign or malignant lesions. Although uncommon, it is the most common malignancy of the anterior mediastinum. It has a variable clinical presentation; one-third can present with local compressive symptoms, one-third with myasthenia gravis, and another third can be asymptomatic and accidentally diagnosed on chest radiography. Thymoma with recurrent pericardial effusion is a rare disease manifestation. A 68-year-old male presented with recurrent pericardial effusion and was later diagnosed with thymoma, as described in this case.

Case Report

A 68-year-old male, a pharmacist by occupation, presented with complaints of exertional dyspnea and bilateral lower limb swelling, which was associated with low-grade fever and oro-genital ulcers for a 1-month duration in December 2019. He was admitted to the hospital with these complaints and found to have pericardial effusion. Seven hundred and fifty millilitres of hemorrhagic fluid was tapped and sent for investigations. Reports came negative for tuberculosis (Acid Fast bacilli [AFB] and Cartridge Based Nucleic Acid Amplification Test [CBNAAT]) and malignancy. Contrast computed tomography (CT) of the chest and abdomen was performed to evaluate the cause of the recurrent pericardial effusion. The contrast CT report was indicative of pericardial thickening and cavitation in bilateral lung fields and a few subcentric lymph nodes. Bronchoalveolar lavage was also performed, which came normal for AFB and CBNNAT. Biopsy was taken from the ulcer base and diagnosed with oro-genital herpes. His antinuclear antibody, HIV, and thyroid profile came negative. As tuberculosis is the most common cause of pericardial effusion in this area of the world, the patient was empirically started on antitubercular therapy along with steroids on clinical suspicion. The patient took antituberculous therapy for 9 months and was asymptomatic till June 2021. In June 2021, he again presented with exertional dyspnea but had no fever or chest pain.

Furthermore, echocardiography revealed cardiac tamponade. He underwent pericardiocentesis, which was hemorrhagic. Fluid analysis was again negative for AFB and CBNAAT. Renal function test and thyroid profile came normal. Contrast-enhanced CT (CECT) of the chest was done, which showed an ill-defined heterogeneous enhancing lesion of size 7.8 cm × 6.2 cm × 8.2 cm (AP × TR × CC) seen in the anterior and posterior mediastinum. No fluid/fat attenuation areas were seen. Posteriorly, it encased ascending aorta, main pulmonary artery, and right and left pulmonary arteries with mild compression. The mass was insinuating along the pericardial recesses. Anteriorly, it was abducting the adjacent right costal pleura with no extrapleural invasion [Figure 1]. CECT of the abdomen was suggestive of an ill-defined hypoenhancing lesion measuring 31 mm × 19 mm in segment VII of the liver in the subcapsular location, suggestive of metastasis. The biopsy was done from the mediastinal mass, which showed dense collagenous tissue with a scattered nest of bland epithelial cells with intermixed mature lymphocytes, suggestive of thymoma [Figure 2]. Immunohistochemistry markers came positive for PanCK and CD-5 [Figure 3]a and [Figure 3]b. Surgical resection was deferred due to the extensive spread of neoplasm and mass encasing major vessels such as ascending aorta and main pulmonary artery. According to the Masaoka^[1] staging system for thymoma, our patient was Stage VI (b) with hepatic metastasis. He was started on induction chemotherapy with cyclophosphamide, cisplatin, and doxorubicin (CAP regimen) due to the nonresectability of the tumor.

Figure 1: Well-defined heterogeneous mass encroaching in the mediastinum encasing the large vessels (red arrow) and right pleural effusion (yellow arrow)

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Figure 2: WHO type B thymoma showing dense collagenous tissue (yellow arrow) with mixed cortical epithelial cells (white arrow) and lymphocytes (white arrow heads)

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Figure 3: Immunohistochemistry (IHC) showing Pan-CK positive epithelial cells (a) and CD IHC stained T-lymphocytes for CD 5(b)

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Discussion

This case presents a rare clinical picture of thymoma with initial presentation as pericardial effusion. This is a rare presentation after a thorough review of the literature. The overall incidence of thymoma in the US is 0.13 per 100,000 persons-years. The incidence of thymoma rises in middle age and peaks in the seventh decade of life. Thymoma is usually an incidental finding identified on imaging in asymptomatic patients. Fifty percent of thymoma cases present with symptoms of myasthenia gravis.^[2] Pleural effusion is the most common manifestation of this disseminated disease. Thoracic symptoms can be the initial presentation. However, pericardial effusion presenting this early in thymoma without any evidence of primary disease is rare.^[3] The presentation was unique as the primary disease was quiescent for a long duration. However, the cause for recurrent pericardial effusion was clarified a little later, when cells had already metastasized.

According to the Masaoka staging system for thymoma, it was Stage IV (b) with hepatic metastasis. Histological classification based on the WHO criteria, it was type B2 thymoma with epithelial cells with intermingled abundant immature T-cells. TNM classification of the tumor was Stage IV B (T4, N2, M1b).^[4]

The prognosis of thymoma is determined by the stage of the tumor. The recurrence rate is to be determined in the lower stages (primary inpatient with resectable tumor), whereas the patient's survival is to be determined regardless of resection status in the higher stages.^[5] In this case, it falls into TNM and Masaoka Stage IV B, indicating a more advanced stage of the disease.

A retrospective study by Ströbel et al. concluded that the long-term outcome of thymic epithelial tumor patients is related to the WHO histotype, tumor stage, and completeness of surgical procedures removal and type of treatment. Masaoka Stage I and II patients had an excellent prognosis in their study. The prognosis was worse in the Masaoka Stage III patient after complete resection. Masaoka Stage IV patients had a 10-year survival rate of only 47%.^[6]

Thymoma is a rare malignancy, and presenting with hemorrhagic pericardial effusion is even rarer. Although it manifests variably, making an early diagnosis is a complex task. The disease extended and encased major vessels, which rendered the tumor nonresectable, and the modality of treatment was chemotherapy (CAP Regimen).^[7]

Conclusion

Thymoma is a rare malignancy, and its presentation with hemorrhagic pericardial effusion is even rarer. The possibility of thymoma should be considered in a patient who presents with a history of recurrent spontaneous pericardial effusion. Suspicion of thymoma in patients presenting with recurrent pericardial effusion expediates early diagnosis of thymoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients 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

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