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CASE REPORT
Year : 2023 | Volume
: 9
| Issue : 1 | Page : 79-83
An unusual presentation of a benign giant mature intrathoracic mediastinal teratoma with right ventricular failure and cardiac cachexia
Satyapriya Mohanty1, Anindya Banerjee2, Abhinav Kumar2, Pranjit Deb2, Shashikant Singh2, Jaideep Das Gupta2, Debasish Das2
1 Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
Correspondence Address:
Debasish Das
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/jpcs.jpcs_60_22
Mature mediastinal teratomas are infrequent, slow growing, and often asymptomatic. Traditionally, they present in the fourth decade or earlier and are found in the anterior mediastinum. These teratomas are often found incidentally on imaging, and surgical resection can be a challenge due to the size and location of the tumor; however, complete surgical excision is usually curative. Our case represents an infrequent benign giant (25 cm × 18 cm × 12 cm) mature intrathoracic mediastinal teratoma with uncommon clinical presentation of congestive heart failure and cardiac cachexia. The rare location of the tumor in the mid posterior mediastinum, very large teratoma, clinical presentation with right ventricular failure and cardiac cachexia, preoperative diagnostic dilemma, perioperative surgical challenge, and management marks this case unique.
Keywords: Cardiac cachexia, right ventricular failure, teratoma
Mature mediastinal teratomas are rarely encountered in routine clinical practice and they are often slow growing. Most often teratomas are asymptomatic in nature. If atall they become symptomatic, they present with chest pain, dyspnea, cough and pulmonary infection. Most of the teratomas become symptomatic secondary to compression of the adjacent structures. They are most often located in the anterior mediastinum. We here report a rare case of posterior mediastinal teratoma presenting with clinical right ventricular failure with cardiac cachexia which is its uniqueness.
Case ReportA 46-year-old nondiabetic, nonhypertensive female presented with anorexia, weight loss, easy fatigability, dull aching left-sided chest pain, productive frothy cough, breathlessness (New York Heart Association III), and bilateral pitting pedal edema since the last 6 months. She had no history of tuberculosis, hemoptysis, or thromboembolic events in the past. Her pulmonary function test revealed restrictive pathology with decreased forced expiratory volume 1 s, forced vital capacity (VC), total lung capacity, and VC. Video bronchoscopy revealed a distorted right-side bronchial tree with externally compressed bronchus to the left upper and the left lower lobe. On physical examination, she was conscious, oriented, afebrile, and cachectic with mild pallor and bilateral pitting pedal edema. Her pulse rate was 102 beats/min and regular in nature. She had systemic blood pressure of 110/70 mmHg in the right arm supine position. Jugular venous pressure was raised (7 cm) above the clavicle. Left chest mobility was decreased as compared to the right side. Dullness on the left anterior chest wall was starting from the manubrium sternum to the precordium. On auscultation, left-side air entry was decreased substantially with the presence of fine crepitation over the left lung base. Wheeze and rhonchi were present bilaterally but more on the left side. Cardiovascular examination revealed that the apex beat was shifted to the right side. The first and second heart sounds were normal, and there was no murmur. Abdominal examination revealed huge hepatomegaly of about 10 centimeters below the right costal margin. She had no thyromegaly and lymphadenopathy. Her biochemical markers such as lactate dehydrogenase (LDH), serum alpha-fetoprotein (AFP), and beta-human chorionic gonadotropin (hCG) were within normal limits with the presence of iron deficiency anemia and hypoproteinemia. Her antibody against hydatid cyst (serum immunoglobulin E) was also not raised; chest X-ray revealed a left-side bosselated heterogeneous huge mass widening the mediastinum and shifting the cardiac shadow, apex, and the left bronchial tree toward the right side. The left bronchus was also shifted toward the right side. Twelve-lead electrocardiogram showed features of pericardial effusion, and echocardiography revealed the presence of moderate pericardial effusion without cardiac tamponade. She had undergone contrast-enhanced computed tomography (CT) of the thorax which revealed a mediastinal mass suggestive of teratoma. The mass contained solid, cystic, and fatty elements, with the presence of calcification in the wall of the cystic portion. The solid portion revealed heterogeneous enhancement. Ultrasound-guided fine-needle aspiration cytology revealed a benign cystic lesion. CT-guided core-needle biopsies from different regions of the mass yielded minute fragments of amorphous, acellular material which was insufficient for pathological diagnosis. Ultrasonography of the abdomen and pelvis revealed huge hepatomegaly with the presence of moderate ascites. Transthoracic echocardiography in parasternal long-axis view and apical four-chamber view revealed a large rounded regular smooth-walled intrathoracic mass compressing the right atrium and right ventricle with moderate pericardial effusion and mild bilateral pleural effusion. At the time of admission, her body mass index (BMI), Karnofsky Performance Scale Index, and Eastern Cooperative Oncology Group (ECOG) grade were 18, 50–60, and 2–3, respectively. She was put on nutritional protein supplementation and rehabilitation for 4 weeks before planned surgery.
Prior to surgery, the patient was tried for endobronchial intubation, but it failed. Hence, she was intubated with double-lumen tube with single-lung ventilation facility. This helped in resection of a huge intrathoracic mass with ease without deteriorating the already decompressed pulmonary function. The patient was able to maintain saturation throughout the procedure. Surgical excision was accomplished via a median sternotomy to facilitate resection of any possible adherence of intrathoracic mass to the heart, lung, or great vessels such as aorta, superior vena cava (SVC), pulmonary artery, pulmonary vein, right ventricle, right atrium, left arterial appendage, and left ventricle with the option of possible need of cardiopulmonary bypass when needed. The tumoral mass had encroached on the pleura and pericardium but not the myocardium. Interestingly, it was densely compressing the lung, right atrium, and right ventricle. A huge intrathoracic mass of size (25 cm × 18 cm × 12 cm) and weighing 5 kg [Figure 1] was seen to be arising from the mid and posterior mediastinum at the level of the superior border of the manubrium sternum and extending inferiorly and laterally to compress the left lung parenchyma and pushing the heart toward the right side beyond the mid line [Figure 2]. SVC was also compressed. Trachea was displaced posteriorly and toward the right. No mediastinal lymphadenopathy was observed. Thoracic cavity was washed thoroughly. The resected specimen was sent for histopathology study. The mass contained a bunch of hair, cartilage, thick calcified areas, and soft gelatinous substances and was multicystic in nature [Figure 3]. The cystic tumor had a thin, sharply delineated wall filled with sebaceous material and hair [Figure 4]. Microscopically, the cyst wall was lined by stratified squamous epithelium with underlying sebaceous glands and hair follicles or by simple ciliated columnar epithelium. Cartilage, adipose tissue, and smooth muscle were also seen in the cyst wall. A histological diagnosis of a mature cystic teratoma was made as immature epithelial, mesenchymal, or neural elements were not found and there was no morphological evidence of malignancy in the tumor. Atrophic thymus was found in the tissue that surrounded the tumor. After successful resection, the patient was relieved from congestive right heart failure and distressing airway obstruction. Postoperatively the patient's shifted mediastinum reverted back to the normal position and her chronically atelectatic lung also got expanded. The patient had a good postoperative recovery and was discharged home on the 7th postoperative day, and during the second follow-up after 2 months, her BMI, Karnofsky score, and ECOG score improved significantly to normal range.
Figure 2: Chest X-ray revealed a large intracystic mass compressing the left pleura and displacing the heart toward the right and inferiorly
Figure 3: Cut section of the intrathoracic mass revealed multicystic nature and the presence of calcification and cartilages Discussion This case report reflects the accuracy of complementary clinical examination. All the investigations suggested a multicystic mediastinal mass with calcification. It is difficult to diagnose a teratoma solely on radiographic evidence.[1] Histopathological diagnosis of teratoma needs at least two of three germ layers.[2],[3] The ectoderm tissue generally is predominant and is composed of neural tissues, skin, hair, and teeth. Mesodermal tissues such as fat, cartilage, or bone and endodermic tissue are less common in teratoma. The endoderm layer is characterized by respiratory or intestinal epithelia. The probability of diagnostic accuracy increases with the presence of fat and teeth in the tumor mass. Here, we report a large mature teratoma causing compression of the right atrium and right ventricle due to mass effect [Figure 5] with dense adhesion to the pleura and the diaphragm without involving the pericardium. The choice of biopsy technique for preoperative diagnosis depends on the localization of the lesion, clinical factors, and the availability of special techniques and equipment. Biopsy may be obtained by transthoracic puncture under CT or ultrasound guidance, or by a surgical approach (mediastinotomy or thoracoscopy). Mediastinal masses can be resected and evaluated by minimally invasive approach like video-assisted thoracoscopy.[4] However, for possible presence of dense adhesion and chance of cyst rupture with spillage and for clinical suspicion of SVC invasion which would necessitate extracorporeal circulation, median sternotomy approach is better preferred. Surgical treatment remains the treatment of choice for mediastinal cystic mass, when the patient is operable, so as to decrease the risk of complications. The clinical presentation of mediastinal masses is often nonspecific or incidental.[5] Histological examination is required to exclude malignancy.[6] Evaluation of levels of beta-hCG, AFP, and LDH markers should be done preoperatively in all suspected germ cell tumors because the elevation of these markers can indicate malignancy which can alter the operative strategy and the follow-up of the patient. The authors managed here a giant thoracic benign teratoma, which generally has a good prognosis, and no adjuvant chemotherapy or radiotherapy was needed. Finally, follow-up must be considered for the possibility of malignant transformation, relapse, or metastases.[7] We also encourage all patients with mediastinal germ cell tumors to undergo evaluation for a primary gonadal tumor.
This present case needs to be analyzed with respect to the age of the patient, size of the tumor, histopathological characteristics of tumor, location, and uncommon clinical presentation. These tumors are generally frequent in children and young adults, whereas our patient was in her fourth decade of life which is quite uncommon. Histopathologically, they have predominantly ectodermal tissues such as teeth, hair, sebaceous gland, and skin with few mesodermal tissues. These tumors are mostly benign, although they can complicate to intratumoral hemorrhage, infection, and infarction. On morphological gross tissue examination, they are foul-smelling materials containing huge metacystic mass with derivatives of ectodermal and mesodermal embryonic cells at various stages of development. These benign teratomas are calcified with elements of teeth and bone in about 26% of cases. Histologic description was consistent with mature squamous cells and occasional glandular cells. Generally, thymoma is the most frequent neoplasm in the anterior mediastinum. This germ cell tumor (mature cystic teratoma) in this patient was found interestingly in the mid to posterior mediastinum. Even though germ cell teratomas are mainly located in the gonads, they can also be found in extragonadal tissue and location like in intrathoracic mediastinum in about 15% of cases.
Although these benign teratomas behave asymptomatic for a long time, they become symptomatic due to compression of vital structures such as lungs and bronchus, leading to dull aching pain, cough, and dyspnea.[8] Strikingly, this patient presented with congestive heart failure due to tumor compression right atrium and right ventricle apart from symptoms such as cough and dyspnea. The patient also had uncommon symptoms of weight loss and cachexia with very poor Karnofsky score and ECOG score even though the tumor was benign, which may be attributable to cardiac cachexia secondary to right ventricular failure.
Preoperative diagnostic workup of teratoma includes radiographic tests such as chest X-ray, CT scan thorax, and magnetic resonance imaging (MRI). Benign teratoma is mostly well-defined lobulated anterior mediastinal mass with bulging toward any one side of midline with compression and displacement of vital intrathoracic structures such as heart and lung great vessels.[9],[10],[11],[12] Those widen and displace the mediastinum to the opposite site. A Rokitansky nodule, a solid protuberance projecting from a cyst, often containing calcific dental, adipose, and hair tissue can sometimes be identified on imaging. MRI was not done in this patient as the patient had dental orthodontic wires.
Ultrasound-guided and computerized tomography-guided core-needle biopsies tried from various locations of the tumor were insufficient for pathological diagnosis because of the presence of acellular material. Core-needle biopsy has a diagnostic yield of 77% and can also be inadequate for immunohistochemistry and flow cytometry evaluation.
Assays of various tumor markers such as serum beta-HCG, AFP, and LDH are useful for diagnosing the neoplastic nature of teratoma. While they are within the normal range for benign tumors, their values increase manifolds in case of malignant transformation and they are helpful in diagnosis of extragonadal manifestations of teratoma.
The patient was tried for endobronchial intubation, but it failed, hence he was intubated with double-lumen tube with single-lung ventilation facility. This helped in resection of a huge intrathoracic mass with ease without deteriorating the already decompressed pulmonary function and desaturation of the patient while resection. This signifies the relevance of double-lumen tube with single-lung ventilation in dealing with ventilation issues in such patients with deformed trachea, bronchial tree due to tumor compression, and mediastinal shift.
Complete surgical excision by median sternotomy, thoracotomy, or video-assisted thoracoscopic approach is almost curative for many mature mediastinal teratomas. A thoracotomy incision is the standard approach to a middle or posterior mediastinal mass. But here, median sternotomy was preferred over lateral thoracotomy because the mass crossed the midline and shifted the mediastinum with suspicious vital structure involvement. Where the pathology is inoperable or inaccessible, then subtotal excision can produce symptomatic improvement for the patient. Surgical excision is challenging due to location, size, and attachment to vital structures such as heart, lung, blood vessels, pericardium, sternum, and diaphragm. When they invade the vital structures, they are usually malignant, which is rare. Malignant transformation has been reported to occur in 1%–2% of ovarian cystic teratomas and in mediastinal teratomas, especially giant mediastinal teratomas. Five-year survival approaches 100%; however, patients must be followed. Postoperatively, the patient improved significantly with complete relief of congestive heart failure with improvement of patients' general health status as indicated by BMI, Karnofsky score, and ECOG score.
ConclusionOur case is a unique description of a benign giant mature intrathoracic mediastinal teratoma presenting with congestive cardiac failure due to tumor compression of the right atrium and right ventricle with cardiac cachexia in the fourth decade of life. Detailed clinical examination always adds to the imaging and tissue diagnosis. Wide excision of this large tumor with the aid of double-lumen tube with single-lung ventilation strategy was the key to the procedural and clinical outcome of this interesting case.
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