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CASE REPORT
Year : 2023 | Volume
: 9
| Issue : 1 | Page : 60-63
Immature mediastinal teratoma mimicking as pulmonary stenosis – A rare presentation
Shibsankar Sarkar, Biswajit Majumder, Debasish Mohapatra, Ratul Ghosh
Department of Cardiology, R.G. Kar Medical College, Kolkata, West Bengal, India
Correspondence Address:
Shibsankar Sarkar
BE Block, Sector 1, Salt Lake, Kolkata - 700 064, West Bengal
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/jpcs.jpcs_82_22
Anterior mediastinal teratoma can externally compress the pulmonary artery, which can present with symptoms of pulmonary stenosis rarely. We have reported a case of a 47-year-old male with immature mediastinal teratoma presenting as pulmonary stenosis. Initial physical examination revealed an ejection systolic murmur in the pulmonary area. With suspicion of valvular heart disease, the patient was referred to our cardiology department. Chest X-ray shows mass just above the left hilar zone. Two-dimensional echocardiography shows a mediastinal mass compressing the pulmonary trunk causing an obstruction. Contrast-enhanced computed tomography of thorax and magnetic resonance study confirmed the mediastinal mass. Fine-needle aspiration cytology of the mass suggestive of immature teratoma. The patient was transferred to the cardiothoracic vascular surgery department for further management. Mediastinal mass can mimic valvular heart disease with symptoms of cardiovascular pathology. Proper investigations for diagnostic clarity and prompt management are crucial in these conditions.
Keywords: Mediastinal mass, pulmonary stenosis, two-dimensional echocardiography
Germ cell tumors are more common in men and young adults. Teratomas are usually located in the anterior part of the mediastinum. They are slow-growing tumors. However, when they become large, they can compress adjacent organs. Such external compression can cause symptoms such as dyspnea, chest pain, or cough.[1],[2] We present the case of a 47-year-old man with a primary mediastinal immature teratoma that caused compression of the main pulmonary artery, mimicking pulmonary stenosis.
Case ReportA 47-year-old male, previously healthy presented with a history of atypical angina and dizziness on exertion for 6 months. The patient also experienced mild shortness of breath on lying down in a supine position which is partially relieved on the left lateral position and sitting up. There is no history of cough, palpitation, weight loss, or any other comorbidity. Routine blood investigations and electrocardiography were within normal limits. Primary care physician referred the patient to our department as a suspected case of valvular heart disease based on the clinical finding of a systolic murmur over the pulmonary area. Initial vital signs were pulse rate 80/min, blood pressure 126/82 mmHg, and respiratory rate 12/min. The patient was afebrile, and there was no palpable swelling over the chest or any lymphadenopathy on examination. On auscultation, a harsh grade 3/6 ejection systolic murmur was heard in the left second intercostal space along the sternal border. The chest roentgenogram revealed a round, smooth mass in the left upper border of the mediastinum [Figure 1]. Two-dimensional echocardiography shows narrowing at the level of the right ventricular outflow tract with a systolic gradient of 83 mmHg [[Figure 2], [Figure 3], [Figure 4] and Videos 1, 2]. Contrast-enhanced computed tomography (CECT) of the thorax shows a large lobulated heterogeneously enhancing soft-tissue density lesion in the anterior mediastinum [Figure 5] that mandates tissue sampling. Magnetic resonance imaging (MRI) of the thorax [Figure 6] shows a heterogeneous T2/STIR hyperintense SOL with irregular margin and areas of necrosis noted in the superior mediastinum of size 7 cm × 8 cm × 7 cm. The space occupying lesion (SOL) was compressing the brachiocephalic vessels, main pulmonary artery, and left pulmonary artery. Fine-needle aspiration cytology smears show discrete and few clusters of mature squamous epithelial cells, anucleate squames, a few degenerated inflammatory cells, and keratin flakes without any malignant cells suggestive of teratoma [Figure 7]. The patient was referred to the cardiothoracic vascular surgery department for surgical resection.
Figure 1: Chest X-ray PA view showing mediastinal mass. PA: Posteroanterior
Figure 2: 2D echocardiography short axis view showing extracardiac mass compressing pulmonary artery. 2D: Two-dimensional
Figure 3: Parasternal short-axis view showing turbulence in RVOT. RVOT: Right ventricular outflow tract
Figure 4: CW in RVOT showing a gradient of 83 mmHG. RVOT: Right ventricular outflow tract
Figure 5: CECT thorax showing mediastinal mass compressing pulmonary trunk. CECT: Contrast-enhanced computed tomography
Figure 6: MRI thorax showing mass in mediastinum. MRI: Magnetic resonance imaging
Figure 7: FNAC of mediastinal mass. FNAC: Fine-needle aspiration cytology Discussion Pulmonary stenosis is defined as the constriction of the right ventricular outflow tract below, above, or at the pulmonary annulus which leads to an increase in right ventricular pressure. The etiology is broadly divided into intrinsic (congenital) or extrinsic compression. The differential diagnosis of extrinsic pulmonary artery compression includes malignant tumors, infection, cysts, and other benign processes leading to fibrosis in the mediastinum.[3] Following malignant tumors may cause extrinsic pulmonary stenosis: Hodgkin's and non-Hodgkin's lymphoma, teratoma, lung carcinoma, pericardial sarcoma, thymoma, and chondrosarcoma of the sternum. Acquired pulmonary stenosis after childhood is rarely found. Pulmonary stenosis and right ventricular outflow tract obstruction by a tumor were rarely reported. Adults with isolated mild-to-moderate right ventricular outflow tract obstruction of any type are usually asymptomatic. The usual presentations of patients with severe right ventricular outflow tract obstruction are exertional fatigue, dyspnea, syncope, light-headedness, chest discomfort (right ventricular angina), and cyanosis.[4] Dyspnea or fatigue may be secondary to an inability to increase cardiac output adequately with exercise. Syncope or light-headedness may occur in the presence of severe pulmonic stenosis with decreased preload or dehydration. With the progression of the disease, as right ventricular compliance is reduced sufficiently, it elevates right atrial pressure. If the foramen ovale is patent, it allows right-to-left shunting causing cyanosis and increased risk of paradoxical embolism.[5],[6] Teratoma is a germ cell tumor that is composed of well-differentiated tissues derived from more than one of all three embryonic cell layers: ectoderm, mesoderm, and endoderm. It can involve either the gonadal organs or the extragonadal sites such as the mediastinum, pineal gland, and sacrococcygeal. Teratoma can be classified as either mature and immature or benign and malignant. Teratomas are commonly classified using the Gonzalez-Crussi grading system: 0, mature; 1, immature, probably benign; 2, immature, possibly malignant (cancerous); and 3, true malignant. In the case of the latter, additional cancer staging applies.[7] Mature teratoma of the mediastinum is generally benign and slow growing, rarely it can transform into a malignant one. The incidence of benign mediastinal teratoma has no sex predilection; however, malignant teratoma is more common in men. The treatment of choice is complete surgical resection. In the case of malignant teratoma, chemotherapy, followed by complete resection provide better long-term survival.[8],[9]
ConclusionsPulmonary stenosis can be caused by compression of extracardiac masses. Echocardiography is an excellent noninvasive tool to quickly disclose the pathology and hemodynamic compromise. CECT thorax and MRI can confirm the extracardiac masses. Biopsy of the mass can confirm the nature of the mass with potential complications and treatment response.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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