Original Article
Surgery for huge mediastinal tumors Igor Ya Motus1, Alexander V Bazhenov1 and Gilbert Massard2
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(7) 846–850 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315592072 aan.sagepub.com
Abstract Background: Mediastinal tumors can reach a huge size and give rise to serious surgical and oncological problems. Methods: A review of 18 patients with huge mediastinal tumors was performed. Ages ranged from 16 to 61 years, and 10 were male. In 4 cases, exploratory thoracotomies had been undertaken in other hospitals; in one patient, the tumor recurred after incomplete resection. We performed a median sternotomy in 17 patients and a lateral thoracotomy in one. Radiotherapy and platinum-based chemotherapy was administered in cases found to be malignant. Results: In 15 patients, the tumor was removed completely. In one patient, the operation was palliative because of vena caval and atrial invasion, and another had exploration only; biopsies revealed malignant transformation in these 2 patients. The blood loss was 425–2530 mL (average 690 mL); bleeding was fatal in one patient. Tissue diagnosis showed mature teratomas in 10 patients. Foci of malignant transformation were found in 5 cases. Chemodectoma was found in one patient, and thymoma in two. Patients with mature teratomas are free of disease. Two patients with malignant teratomas died from tumor recurrence despite irradiation and chemotherapy. Conclusions: Clinical and oncological problems lie in the fact that malignant transformation of the tumor is diagnosed only after surgery. Extensive blood loss is a crucial surgical problem. Median sternotomy is the optimal operative approach. Surgical treatment of mature mediastinal tumors results in good outcomes unless malignancy is detected.
Keywords Diagnosis, differential, Mediastinal cyst, Mediastinal neoplasms, Sternotomy, Teratoma
Introduction Huge mediastinal tumors (MT) are not very common, and the majority of studies are case reports.1–4 A slowly growing MT may be asymptomatic until considerable tumor growth has occurred. It can reach a giant size and sometimes presents with complications requiring emergency treatment.5–8 Most MT are teratomas, and because of their location in the mediastinum close to the heart, great vessels, and lungs, they may cause severe clinical symptoms. The aims of this study were to present our experience and to draw attention to the main problems in this field.
Patients and methods This was a retrospective study on 18 patients operated on between 2001 and 2013 in the Clinic of Thoracic Surgery in Ekaterinburg (Table 1). The patients’ ages ranged from 16 to 61 years, and the male-to-female ratio was 10/8. Inclusion criteria were MT occupying more than two compartments of the mediastinum and
encroaching on the pleural spaces. According to these criteria, the tumors concerned were considered huge, and they represented 6.0% of all 299 MT operated on in our clinic during this period. Clinical and anamnestic data were initially nonspecific in the vast majority of patients: fatigue and weight loss in 7 patients, dyspnea and slight dysphagia in 2, fever and chest pain in one; 7 patients were asymptomatic. One patient was admitted to the emergency room after blunt chest trauma, and the disease was initially interpreted as clotted hemothorax. In 6 cases, there had been attempts to remove the tumor in other hospitals, thus the tumor 1 Department of Thoracic Surgery, Urals Research Institute for Phthisiopulmonology, Ekaterinburg, Russia 2 University Hospital, Strasbourg, France
Corresponding author: Igor Ya Motus, Department of Thoracic Surgery Urals Research Institute for Phthisiopulmonology, 50 XXII Parts’ezda str., 620039 Ekaterinburg, Russia. Email: [email protected]
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Table 1. Characteristics of 18 patients with huge mediastinal tumors. Case no.
Sex
Age (years)
Previous surgery
Our intervention
Pathology
Further treatment
Outcome
FMS, removal
Mature teratoma
No
Good
FMS, removal FMS, removal
Mature teratoma Chemodectoma
No No
Good Good
Partial sternotomy, removal FMS, removal FMS, removal FMS, removal
Mature teratoma
No
Good
Mature teratoma Mature teratoma Thymoma A
No No CT þ RT
FMS, removal FMS, removal
Mature teratoma Malignant teratoma
No CT þ RT
Good Good Free of disease (11 months) Good Died (13 months)
Thoracotomy, removal FMS, removal þ lobectomy FMS, incomplete resection FMS, removal þ lobectomy FMS, exploration FMS, removal FMS, removal
Mature teratoma
No
Good
Matured teratoma
No
Good
Malignant teratoma
CT þ RT
Died (6 months)
Thymoma B1
CT þ RT.
Free of disease (23 months)
Malignant teratoma Malignant teratoma Mature teratoma
CT þ RT No
Died (7 months) Good
FMS, removal FMS, removal
Mature teratoma Malignant teratoma
No CT þ RT
Good Died (11 months)
1.
F
17
2. 3.
M F
34 25
4.
F
16
Partial sternotomy, incomplete resection No Carotid chemodectoma removal No
5. 6. 7.
F M M
29 53 57
No No No
8. 9.
F M
25 35
10.
F
57
No Exploratory thoracotomy No
11.
M
20
12.
M
17
13.
F
61
14. 15. 16.
M M M
17 24 39
17. 18.
F M
16 43
Exploratory thoracotomy No Exploratory thoracotomy No No Exploratory thoracotomy No No
CT: chemotherapy; FMS: full median sternotomy; RT: radiotherapy.
morphology had been confirmed at the first surgical intervention. In the other patients, biopsy under computed tomography or radiography guidance was carried out. The main reasons for inability to resect were firm adhesions to the chest wall, simulating tumor invasion, and severe bleeding during tumor dissection. In one patient, a partial sternotomy and incomplete resection of the tumor had been performed 4 years earlier. In another, a chemodectoma of the carotid region had been removed 5 years earlier, and a mediastinal mass without clinical symptoms was detected on follow-up chest radiography. There were no special considerations regarding anesthesia. Conventional double-lung ventilation and intravenous anesthesia were maintained. We performed a full median sternotomy in 16 patients and a partial sternotomy in one; one patient whose tumor recurred after previous incomplete resection, had a repeat sternotomy. In one patient, the tumor was removed via a
lateral thoracotomy. To estimate blood loss in different stages of the operation, blood loss was measured during 3 periods in 8 patients: on performing the approach, on mobilization of the tumor in the mediastinum, and during dissection of the tumor from adhesions in the chest wall and lung. In cases of 530% loss of circulating blood volume and hemoglobin level 480 g L1, transfusion of red cell concentrate and lyophilized plasma was indicated. No further treatment was administered when a benign mature teratoma was confirmed by pathologic examination of the excised tumor. Radiation therapy with 50–65 Gy and intensive platinum-based chemotherapy were administered in patients found to have malignant teratomas. In 2 cases of thymoma, radiation therapy with 45–54 Gy and 4 courses of Adriamycin, cisplatin, vincristine, and cyclophosphamide chemotherapy were carried out. All patients were followed up for 2 to 7 years after the operation.
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Figure 1. Computed tomography showing a giant mediastinal tumor displacing the heart and right lung and fully compressing the left lung. Bones, calcified foci, and cysts are visible in the tumor.
Results Computed tomography in all cases revealed a huge mediastinal mass that displaced the heart and great vessels, and squeezed the lungs to full-compression atelectasis in 3 patients (Figure 1). The appearance of mature teratomas was heterogenous in most cases, with foci of fat and calcification as well as cystic areas. Malignant MT were mostly solid and homogeneous, but in 3 patients, computed tomography showed a heterogenous structure. Tracheobronchoscopy revealed moderate compression of the trachea in 2 patients. Operative time ranged from 135 to 280 min (average 166 min). In 15 patients, the tumor was removed completely. Resection of the pericardium was necessary in 9 cases. Only in one patient (case no. 11) was subtotal resection of the left wall of the pericardium performed (Table 1, Figure 2, Figure 3), and we closed the defect with a wide flap of parietal pleura to prevent cardiac herniation. In the other 8 patients subjected to resection of the pericardium, the defects were small and we did not cover the pericardial window. A left upper lobectomy was undertaken in one patient with malignant thymoma because of tumor invasion of the lung. Wedge lung resections were performed in 6 cases where the tumor adhered firmly to the lungs. In one patient, the operation was palliative because of vena caval and atrial invasion, and in another, the operation was limited to exploration only for the same reason; pathologic examination revealed malignant transformation in these 2 patients. Blood loss ranged from 425 to 2530 mL (average 690 mL) and originated mainly from enormously vascularized adhesions between the tumor and the chest wall. Dissection of the tumor from adhesions to the chest wall was the most difficult stage of the surgical procedure. Blood loss measurements showed that 83% of the total blood loss took place during dissection of
Figure 2. After a median sternotomy, the tumor was mobilized and partially elevated in the operative wound.
Figure 3. The excised tumor.
the tumor from adhesions. According to the above criteria, transfusions were carried out in 6 patients. Tissue diagnosis of the excised specimens demonstrated mature teratoma in 10 patients. The histologic picture of benign MT was typical of a benign cystic mature teratoma. Malignant transformation of a teratoma was found in 5 patients, including foci of malignant
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tissue in 3 patients in whom mature tissue alternated with areas of immature teratoma and areas of undifferentiated malignant tissue. In 2 cases of malignancy, the tumor was composed of moderately and poorly differentiated tumor tissue that was obvious in the entire tumor mass removed (cases 9 and 12 in Table 1). Thymoma was found in 2 patients (A and B1 types, both stage III according to the Masaoka classification) and a chemodectoma in one patient (Table 1). The results of previous biopsies under computed tomography or radiography guidance agreed with the final morphologic diagnosis in all cases of mature teratoma, in 2 cases of total malignant transformation, and in patients with thymomas, but in cases of MT with a heterogenous structure, the biopsy results were equivocal. Postoperative hospital length of stay was 7 to 21 days. There was one postoperative death due to intractable bleeding in a patient with a malignant teratoma, in whom exploration only was performed. In 4 patients (all older than 50 years), circulatory disorders with marked hypotension occurred after removal of bulky mediastinal masses, and medical support with vasopressive drugs was necessary for 1–3 days after the operation. Compression atelectases in various degrees was found at operation in all patients, which resolved after tumor removal and the lungs gradually reexpanded (Figure 1, Figure 4). Ten patients operated on for mature teratoma were free of disease at the latest follow-up. Two patients operated on for invasive thymoma and subjected to adjuvant irradiation and chemotherapy were still free of disease 23 and 11 months after the operation. The patient operated on for chemodectoma was free of disease for 23 months, and was lost to follow-up after she moved to another region. Three patients in whom malignant transformation of a teratoma was found, died at 7, 11, and 13 months from tumor progression despite irradiation and chemotherapy. The same outcome was observed at 6 months in the patient with incomplete resection of a malignant teratoma.
Discussion Fifteen of 18 (83.3%) patients in our study had teratomas. Teratomas account for 1% to 5% of mediastinal (anterior) tumors, and vary widely in maturity.6,9,10 The largest and heaviest mediastinal teratoma reported in the literature was 14 kg.8 The main clinical issue is that MT are initially asymptomatic, and even at a huge size, symptoms are often nonspecific.1–3,6 Sometimes giant tumors are detected because of complications: dysphagia, rupture, hemothorax, or cardiac problems.5–8,11 In our study, one patient had blunt thoracic trauma, and the giant shadow on a radiograph was initially interpreted as clotted hemothorax. Clinical manifestations
Figure 4. Computed tomography 7 years after the operation.
of mature teratomas are less marked in adults than in pediatric patients.9,12 The radiographic picture of mature teratomas is typically heterogeneous with dense particles of cartilage, bones, and calcification as well as a cystic component.10,13,14 This combination was identified in 39% of teratomas.13 Malignant teratomas are homogeneous and solid with a thick capsule.13,14 In some of the tumors we removed, we found areas of malignant transformation in teratomas that initially presented as mature. Tissue diagnosis obtained by biopsy is often elusive, and it is achieved on tumor excision.10 Attempts at biopsy with the help of needle aspiration often fail to obtain a definite tissue diagnosis. This can be partly explained by the fact that the tumor has a heterogeneous structure. According to the available literature, a median sternotomy is the optimal operative approach for resection of huge tumors in the mediastinum.3,5,15–17 A lateral sternal split with an anterior thoracotomy can be added when necessary.9 A lateral thoracotomy on the side of the tumor can also be used.5 The most difficult aspect of the operation is dividing the tumor from adhesions, as stressed in many reports.1–3,8,17 Severe bleeding that is not easy to control occurs at this stage of the operation.4,15 We also faced such problems as evidenced by our finding that more than 80% of overall blood loss occurred at this stage. The development of vascularized adhesions can be explained by the prolonged slow growth of the tumor in close contact with the parietal pleura. It should be emphasized that we found only adhesions with no tumor invasion into the chest wall, as highlighted by other authors.1–4,17 Neoplastic invasion of the lung tissue was found only in the case of a malignant thymoma where we had to perform a left upper lobectomy. In the other cases, there were firm adhesions. A linear stapling device is the best and simplest mode of separating the tumor from the lung.4 From our experience, we can confirm that a full median sternotomy is preferable for
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visualizing tumor contact with the heart and great vessels, whereas other manipulations including separating adhesions are equally convenient to perform via any approach. For oncological management of huge MT, we have a lack of reliable methods to stop neoplastic progression after radical tumor removal, and in some cases, malignant transformation was revealed in small areas within the tumor. The data available in the literature,15 and the results obtained in our study show that prognosis in such cases is usually unfavorable despite subsequent chemotherapy and irradiation. Platinum-based chemotherapy for immature teratomas provides hope for a favorable prognosis.6,9,10 Without a doubt, the threat of malignant transformation is contained in all of these slow-growing tumors. However, timely detection of the tumor at an early stage is a crucial prognostic factor. Hence the path to the correct diagnosis, radical surgery, and a satisfactory prognosis lies in careful radiological examinations. Acknowledgements The authors would like to thank all the staff of the thoracic surgery, intensive care, and oncology units who took part in the treatment and follow-up of the patients involved. Presented at the 23rd Congress of the European Respiratory Society, Barcelona, Spain, September 16–20, 2013.
Conflict of interest statement The author report no conflict of interest.
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
References 1. Cesario A, Galetta D, Margaritora S, Porziella V and Granone P. Mediastinal cystic teratoma. Ann Thorac Surg 2004; 78: e34. 2. Chen C, Zheng H and Jiang S. An unusual case of giant mediastinal teratoma with malignant transformation. Ann Thorac Surg 2008; 86: 302–304. 3. Takagishi T, Osaki T, Kodate M, Ebi N, Oya M and Yamamoto H. Huge mediastinal cystic tumor penetrating the sternum. Ann Thorac Surg 2010; 90: 664–666. 4. Vrtik M, Larbalestier RI, Cameron D, Gupta A and Sinniah R. Giant superior mediastinal angioleiomyoma. J Thorac Cardiovasc Surg 2004; 128: 786–788.
5. Asteriou C, Barbetakis N, Kleontas A and Konstantinou D. Giant mediastinal teratoma presenting with paroxysmal atrial fibrillation. Interactive Cardiovasc Thorac Surg 2011; 12: 308–310. 6. McLeod NP, Vallely MP and Mathur MN. Massive immature mediastinal teratoma extending into the left pleural cavity. Heart Lung Circ 2005; 14: 45–47. 7. Miyazawa M, Yoshida K, Komatsu K, Kobayashi N and Haba Y. Mediastinal mature teratoma with rupture into pleural cavity due to blunt trauma. Ann Thorac Surg 2012; 93: 990–992. 8. Golash V. A giant anterior mediastinal teratoma presenting as orthopnea and dysphagia in an adult. J Thorac Cardiovasc Surg 2005; 130: 612–613. 9. Takeda S, Miyoshi S, Ohta M, Minami M, Masaoka A and Matsuda H. Primary germ cell tumors in the mediastinum: a 50-year experience at a single Japanese institution. Cancer 2003; 97: 367–376. 10. Vohra LS, Talwar R, Mathur M, Mohan CV, Chawla N and Bharathi RS. Giant mediastinal teratoma—bull in a china shop: management strategies. J Thorac Cardiovasc Surg 2007; 133: 1382–1383. 11. Ruan Z, Wang S, Wang Z and Jing Y. A rare case of bilateral massive hemothorax from spontaneous rupture of a primary mediastinal mixed germ cell tumor. Ann Thorac Surg 2012; 93: 664–666. 12. Yu CW, Hsieh MJ, Hwang KP and Huang CC. Mediastinal mature teratoma with complex rupture into the pleura, lung, and bronchus complicated with mycoplasma pneumonia. J Thorac Cardiovasc Surg 2007; 133: 1114–1115. 13. Gaerte SC, Meyer CA, Winer-Muram HT, Tarver RD and Conces DJ. Fat-containing lesions of the chest. Radiographics 2002; 22: S61–78. 14. Jung JI, Park SH, Park JG, Lee SH, Lee KY and Hahn ST. Teratoma with malignant transformation in the anterior mediastinum: a case report. Korean J Radiol 2000; 1: 162–164. 15. Bergh NP, Gatzinsky P, Larsson S, Lundin P and Ridell B. Tumors of the thymus and thymic region: III. Clinicopathological studies on teratomas and tumors of germ cell type. Ann Thorac Surg 1978; 25: 107–111. 16. Brown KM, Banerjee S, Kane PA and Marrinan MT. Intrapericardial teratoma presenting with circulatory compromise. Ann Thorac Surg 2006; 81: 374. 17. Koga H, Yamataka A, Kobayashi H, Miyamoto H, Lane GJ and Miyano T. Median sternotomy provides excellent exposure for excising anterior mediastinal tumors in children. Pediatr Surg Int 2005; 21: 864–867.
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Surgery for huge mediastinal tumors Igor Ya Motus1, Alexander V Bazhenov1 and Gilbert Massard2
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(7) 846–850 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315592072 aan.sagepub.com
Abstract Background: Mediastinal tumors can reach a huge size and give rise to serious surgical and oncological problems. Methods: A review of 18 patients with huge mediastinal tumors was performed. Ages ranged from 16 to 61 years, and 10 were male. In 4 cases, exploratory thoracotomies had been undertaken in other hospitals; in one patient, the tumor recurred after incomplete resection. We performed a median sternotomy in 17 patients and a lateral thoracotomy in one. Radiotherapy and platinum-based chemotherapy was administered in cases found to be malignant. Results: In 15 patients, the tumor was removed completely. In one patient, the operation was palliative because of vena caval and atrial invasion, and another had exploration only; biopsies revealed malignant transformation in these 2 patients. The blood loss was 425–2530 mL (average 690 mL); bleeding was fatal in one patient. Tissue diagnosis showed mature teratomas in 10 patients. Foci of malignant transformation were found in 5 cases. Chemodectoma was found in one patient, and thymoma in two. Patients with mature teratomas are free of disease. Two patients with malignant teratomas died from tumor recurrence despite irradiation and chemotherapy. Conclusions: Clinical and oncological problems lie in the fact that malignant transformation of the tumor is diagnosed only after surgery. Extensive blood loss is a crucial surgical problem. Median sternotomy is the optimal operative approach. Surgical treatment of mature mediastinal tumors results in good outcomes unless malignancy is detected.
Keywords Diagnosis, differential, Mediastinal cyst, Mediastinal neoplasms, Sternotomy, Teratoma
Introduction Huge mediastinal tumors (MT) are not very common, and the majority of studies are case reports.1–4 A slowly growing MT may be asymptomatic until considerable tumor growth has occurred. It can reach a giant size and sometimes presents with complications requiring emergency treatment.5–8 Most MT are teratomas, and because of their location in the mediastinum close to the heart, great vessels, and lungs, they may cause severe clinical symptoms. The aims of this study were to present our experience and to draw attention to the main problems in this field.
Patients and methods This was a retrospective study on 18 patients operated on between 2001 and 2013 in the Clinic of Thoracic Surgery in Ekaterinburg (Table 1). The patients’ ages ranged from 16 to 61 years, and the male-to-female ratio was 10/8. Inclusion criteria were MT occupying more than two compartments of the mediastinum and
encroaching on the pleural spaces. According to these criteria, the tumors concerned were considered huge, and they represented 6.0% of all 299 MT operated on in our clinic during this period. Clinical and anamnestic data were initially nonspecific in the vast majority of patients: fatigue and weight loss in 7 patients, dyspnea and slight dysphagia in 2, fever and chest pain in one; 7 patients were asymptomatic. One patient was admitted to the emergency room after blunt chest trauma, and the disease was initially interpreted as clotted hemothorax. In 6 cases, there had been attempts to remove the tumor in other hospitals, thus the tumor 1 Department of Thoracic Surgery, Urals Research Institute for Phthisiopulmonology, Ekaterinburg, Russia 2 University Hospital, Strasbourg, France
Corresponding author: Igor Ya Motus, Department of Thoracic Surgery Urals Research Institute for Phthisiopulmonology, 50 XXII Parts’ezda str., 620039 Ekaterinburg, Russia. Email: [email protected]
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Table 1. Characteristics of 18 patients with huge mediastinal tumors. Case no.
Sex
Age (years)
Previous surgery
Our intervention
Pathology
Further treatment
Outcome
FMS, removal
Mature teratoma
No
Good
FMS, removal FMS, removal
Mature teratoma Chemodectoma
No No
Good Good
Partial sternotomy, removal FMS, removal FMS, removal FMS, removal
Mature teratoma
No
Good
Mature teratoma Mature teratoma Thymoma A
No No CT þ RT
FMS, removal FMS, removal
Mature teratoma Malignant teratoma
No CT þ RT
Good Good Free of disease (11 months) Good Died (13 months)
Thoracotomy, removal FMS, removal þ lobectomy FMS, incomplete resection FMS, removal þ lobectomy FMS, exploration FMS, removal FMS, removal
Mature teratoma
No
Good
Matured teratoma
No
Good
Malignant teratoma
CT þ RT
Died (6 months)
Thymoma B1
CT þ RT.
Free of disease (23 months)
Malignant teratoma Malignant teratoma Mature teratoma
CT þ RT No
Died (7 months) Good
FMS, removal FMS, removal
Mature teratoma Malignant teratoma
No CT þ RT
Good Died (11 months)
1.
F
17
2. 3.
M F
34 25
4.
F
16
Partial sternotomy, incomplete resection No Carotid chemodectoma removal No
5. 6. 7.
F M M
29 53 57
No No No
8. 9.
F M
25 35
10.
F
57
No Exploratory thoracotomy No
11.
M
20
12.
M
17
13.
F
61
14. 15. 16.
M M M
17 24 39
17. 18.
F M
16 43
Exploratory thoracotomy No Exploratory thoracotomy No No Exploratory thoracotomy No No
CT: chemotherapy; FMS: full median sternotomy; RT: radiotherapy.
morphology had been confirmed at the first surgical intervention. In the other patients, biopsy under computed tomography or radiography guidance was carried out. The main reasons for inability to resect were firm adhesions to the chest wall, simulating tumor invasion, and severe bleeding during tumor dissection. In one patient, a partial sternotomy and incomplete resection of the tumor had been performed 4 years earlier. In another, a chemodectoma of the carotid region had been removed 5 years earlier, and a mediastinal mass without clinical symptoms was detected on follow-up chest radiography. There were no special considerations regarding anesthesia. Conventional double-lung ventilation and intravenous anesthesia were maintained. We performed a full median sternotomy in 16 patients and a partial sternotomy in one; one patient whose tumor recurred after previous incomplete resection, had a repeat sternotomy. In one patient, the tumor was removed via a
lateral thoracotomy. To estimate blood loss in different stages of the operation, blood loss was measured during 3 periods in 8 patients: on performing the approach, on mobilization of the tumor in the mediastinum, and during dissection of the tumor from adhesions in the chest wall and lung. In cases of 530% loss of circulating blood volume and hemoglobin level 480 g L1, transfusion of red cell concentrate and lyophilized plasma was indicated. No further treatment was administered when a benign mature teratoma was confirmed by pathologic examination of the excised tumor. Radiation therapy with 50–65 Gy and intensive platinum-based chemotherapy were administered in patients found to have malignant teratomas. In 2 cases of thymoma, radiation therapy with 45–54 Gy and 4 courses of Adriamycin, cisplatin, vincristine, and cyclophosphamide chemotherapy were carried out. All patients were followed up for 2 to 7 years after the operation.
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Figure 1. Computed tomography showing a giant mediastinal tumor displacing the heart and right lung and fully compressing the left lung. Bones, calcified foci, and cysts are visible in the tumor.
Results Computed tomography in all cases revealed a huge mediastinal mass that displaced the heart and great vessels, and squeezed the lungs to full-compression atelectasis in 3 patients (Figure 1). The appearance of mature teratomas was heterogenous in most cases, with foci of fat and calcification as well as cystic areas. Malignant MT were mostly solid and homogeneous, but in 3 patients, computed tomography showed a heterogenous structure. Tracheobronchoscopy revealed moderate compression of the trachea in 2 patients. Operative time ranged from 135 to 280 min (average 166 min). In 15 patients, the tumor was removed completely. Resection of the pericardium was necessary in 9 cases. Only in one patient (case no. 11) was subtotal resection of the left wall of the pericardium performed (Table 1, Figure 2, Figure 3), and we closed the defect with a wide flap of parietal pleura to prevent cardiac herniation. In the other 8 patients subjected to resection of the pericardium, the defects were small and we did not cover the pericardial window. A left upper lobectomy was undertaken in one patient with malignant thymoma because of tumor invasion of the lung. Wedge lung resections were performed in 6 cases where the tumor adhered firmly to the lungs. In one patient, the operation was palliative because of vena caval and atrial invasion, and in another, the operation was limited to exploration only for the same reason; pathologic examination revealed malignant transformation in these 2 patients. Blood loss ranged from 425 to 2530 mL (average 690 mL) and originated mainly from enormously vascularized adhesions between the tumor and the chest wall. Dissection of the tumor from adhesions to the chest wall was the most difficult stage of the surgical procedure. Blood loss measurements showed that 83% of the total blood loss took place during dissection of
Figure 2. After a median sternotomy, the tumor was mobilized and partially elevated in the operative wound.
Figure 3. The excised tumor.
the tumor from adhesions. According to the above criteria, transfusions were carried out in 6 patients. Tissue diagnosis of the excised specimens demonstrated mature teratoma in 10 patients. The histologic picture of benign MT was typical of a benign cystic mature teratoma. Malignant transformation of a teratoma was found in 5 patients, including foci of malignant
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tissue in 3 patients in whom mature tissue alternated with areas of immature teratoma and areas of undifferentiated malignant tissue. In 2 cases of malignancy, the tumor was composed of moderately and poorly differentiated tumor tissue that was obvious in the entire tumor mass removed (cases 9 and 12 in Table 1). Thymoma was found in 2 patients (A and B1 types, both stage III according to the Masaoka classification) and a chemodectoma in one patient (Table 1). The results of previous biopsies under computed tomography or radiography guidance agreed with the final morphologic diagnosis in all cases of mature teratoma, in 2 cases of total malignant transformation, and in patients with thymomas, but in cases of MT with a heterogenous structure, the biopsy results were equivocal. Postoperative hospital length of stay was 7 to 21 days. There was one postoperative death due to intractable bleeding in a patient with a malignant teratoma, in whom exploration only was performed. In 4 patients (all older than 50 years), circulatory disorders with marked hypotension occurred after removal of bulky mediastinal masses, and medical support with vasopressive drugs was necessary for 1–3 days after the operation. Compression atelectases in various degrees was found at operation in all patients, which resolved after tumor removal and the lungs gradually reexpanded (Figure 1, Figure 4). Ten patients operated on for mature teratoma were free of disease at the latest follow-up. Two patients operated on for invasive thymoma and subjected to adjuvant irradiation and chemotherapy were still free of disease 23 and 11 months after the operation. The patient operated on for chemodectoma was free of disease for 23 months, and was lost to follow-up after she moved to another region. Three patients in whom malignant transformation of a teratoma was found, died at 7, 11, and 13 months from tumor progression despite irradiation and chemotherapy. The same outcome was observed at 6 months in the patient with incomplete resection of a malignant teratoma.
Discussion Fifteen of 18 (83.3%) patients in our study had teratomas. Teratomas account for 1% to 5% of mediastinal (anterior) tumors, and vary widely in maturity.6,9,10 The largest and heaviest mediastinal teratoma reported in the literature was 14 kg.8 The main clinical issue is that MT are initially asymptomatic, and even at a huge size, symptoms are often nonspecific.1–3,6 Sometimes giant tumors are detected because of complications: dysphagia, rupture, hemothorax, or cardiac problems.5–8,11 In our study, one patient had blunt thoracic trauma, and the giant shadow on a radiograph was initially interpreted as clotted hemothorax. Clinical manifestations
Figure 4. Computed tomography 7 years after the operation.
of mature teratomas are less marked in adults than in pediatric patients.9,12 The radiographic picture of mature teratomas is typically heterogeneous with dense particles of cartilage, bones, and calcification as well as a cystic component.10,13,14 This combination was identified in 39% of teratomas.13 Malignant teratomas are homogeneous and solid with a thick capsule.13,14 In some of the tumors we removed, we found areas of malignant transformation in teratomas that initially presented as mature. Tissue diagnosis obtained by biopsy is often elusive, and it is achieved on tumor excision.10 Attempts at biopsy with the help of needle aspiration often fail to obtain a definite tissue diagnosis. This can be partly explained by the fact that the tumor has a heterogeneous structure. According to the available literature, a median sternotomy is the optimal operative approach for resection of huge tumors in the mediastinum.3,5,15–17 A lateral sternal split with an anterior thoracotomy can be added when necessary.9 A lateral thoracotomy on the side of the tumor can also be used.5 The most difficult aspect of the operation is dividing the tumor from adhesions, as stressed in many reports.1–3,8,17 Severe bleeding that is not easy to control occurs at this stage of the operation.4,15 We also faced such problems as evidenced by our finding that more than 80% of overall blood loss occurred at this stage. The development of vascularized adhesions can be explained by the prolonged slow growth of the tumor in close contact with the parietal pleura. It should be emphasized that we found only adhesions with no tumor invasion into the chest wall, as highlighted by other authors.1–4,17 Neoplastic invasion of the lung tissue was found only in the case of a malignant thymoma where we had to perform a left upper lobectomy. In the other cases, there were firm adhesions. A linear stapling device is the best and simplest mode of separating the tumor from the lung.4 From our experience, we can confirm that a full median sternotomy is preferable for
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visualizing tumor contact with the heart and great vessels, whereas other manipulations including separating adhesions are equally convenient to perform via any approach. For oncological management of huge MT, we have a lack of reliable methods to stop neoplastic progression after radical tumor removal, and in some cases, malignant transformation was revealed in small areas within the tumor. The data available in the literature,15 and the results obtained in our study show that prognosis in such cases is usually unfavorable despite subsequent chemotherapy and irradiation. Platinum-based chemotherapy for immature teratomas provides hope for a favorable prognosis.6,9,10 Without a doubt, the threat of malignant transformation is contained in all of these slow-growing tumors. However, timely detection of the tumor at an early stage is a crucial prognostic factor. Hence the path to the correct diagnosis, radical surgery, and a satisfactory prognosis lies in careful radiological examinations. Acknowledgements The authors would like to thank all the staff of the thoracic surgery, intensive care, and oncology units who took part in the treatment and follow-up of the patients involved. Presented at the 23rd Congress of the European Respiratory Society, Barcelona, Spain, September 16–20, 2013.
Conflict of interest statement The author report no conflict of interest.
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
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