Eur J Cardiothorac Surg 2000;17:384-388
© 2000 Elsevier Science NL
Superior vena cava syndrome of malignant origin. Which surgical procedure for which diagnosis?
Henri Porte,
David Metois,
Laetitia Finzi,
Gilles Lebuffe,
Anne Guidat,
Massimo Conti,
Alain Wurtz
Division of Thoracic Surgery, Calmette Hospital, Lille University Hospital, 59037 Lille Cedex, France
Corresponding author. Tel.: +33-3-20-44-45-59; fax: +33-3-20-44-48-90
e-mail: awurtz{at}chru-lille.fr
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Abstract
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Objective: Since some malignancies causing superior vena cava syndrome (SVCS) are only sensitive to a specific treatment regimen, it is crucial to diagnose the underlying pathology in such cases. The aim of the present study was to review the surgical procedures used to establish the aetiology of SVCS of a malignant origin. Methods: This retrospective study was based on a series of 88 patients referred to surgeons for SVCS, for whom biological and/or endoscopic procedures had failed to establish the diagnosis. On the basis of the results of clinical examination, biological tests and CT-scan presentation, we performed 99 sampling procedures to obtain a diagnosis for all 88 patients. These procedures were the following: biopsy of peripheral adenopathy (n=11), CT-guided biopsy (CTGB; n=23), axial mediastinoscopy (MDS; n=23), anterior mediastinotomy (n=26), anterior mediastinoscopy (n=6), biopsy of the suprascapular mass (n=3), pericardioscopy (n=3), thoracoscopy (n=1), thoracotomy (n=2) and sternotomy (n=1). Results: Per-operative morbidity consisted of one case of massive venous bleeding during MDS requiring a salvage sternotomy to achieve hemostasis. The diagnoses finally established for the 88 patients were non-Hodgkin's lymphoma (NHL) for 36, small cell lung cancer for 25, non-small cell lung cancer for 17, Hodgkin's disease for five, thymoma for three, germ cell tumour for one and sarcoma for one. For the diagnosis of lung cancer, the sensitivities of CTGB and MDS were 85 and 100%, respectively. For the diagnosis of NHL, the sensitivity of anterior mediastinotomy was 95%. Conclusion: The surgical diagnostic procedure, chosen on the basis of the clinical presentation and CT-scan, can be performed safely in the case of SVCS, with the same accuracy as in the absence of this syndrome. Among the patients referred to surgeons, NHL is the most frequent aetiology of SVCS, together with small cell lung cancer.
Key Words: Superior vena cava syndrome • Non-Hodgkin’s lymphoma • Small cell lung cancer • Non-small cell lung cancer
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1. Introduction
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The superior vena cava syndrome (SVCS) is a clinical syndrome of superior vena cava obstruction due to compression, invasion or thrombus formation. Intrathoracic malignancy has far surpassed benign disease as the primary cause of SVCS [1–8]. The progress of the vena cava obstruction process determines the severity of the syndrome and the changes associated with the alteration of venous flow [8]. Consequently, the usual clinical presentations (i.e. facial oedema, venous congestion of the neck, tachypnea and collateral thoracic circulation) depend on the evolution of the underlying malignancy. The prognosis of the patients with SVCS is much more affected by the histology of the tumour obstructing the superior vena cava than by the SVCS itself, which rarely leads to life-threatening complications, such as laryngeal or cerebral oedema [1,4,6,7]. The management of SVCS depends on the severity of the patient's symptoms, including symptoms other than those of SVCS, like airway compression or pericardial tamponade, and on the histological type of the tumour obstructing the superior vena cava.
Two notions are frequently associated with SVCS: (1), that small cell lung cancer is the most common cause of malignant SVC obstruction; and (2), that invasive diagnostic procedures cause increased morbidity, due to the collateral veins that theoretically increase the risk of bleeding [1–8].
However, it was our impression that most of our patients had underlying malignancies other than small cell lung cancer, such as lymphomas or non-small cell lung cancer, and that the morbidity of invasive procedures was very similar to that occurring without SVCS. To clarify these controversial impressions, and to define the most appropriate approach to diagnose the etiology of malignant SVCS on the basis of its clinical and radiological presentation, we reviewed our experience of the systematic diagnostic management of patients with this condition over a period of 13 years.
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2. Material and methods
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2.1. Patient population
Between November 1985 and November 1998, 88 patients with SVCS were treated in our institution. A computer search for patients discharged from the hospital with the diagnosis of SVCS was conducted to complete the manual search of the surgical list. The only patients included in the study were those with SVC obstruction due to a malignant process in the mediastinum, who were referred to us by respiratory physicians after biological and endoscopic procedure had failed to allow a precise pathological diagnosis. These patients comprised 53 men and 35 women, whose age ranged from 19 to 77 years (mean, 52 years). None of them were known to have a disease that had eventually led to superior vena cava obstruction, and SVCS was always the presenting symptom of their disease. The mean duration of symptoms before admission was 1 month. The most common presenting symptoms were oedema and venous congestion of the head and neck or of the upper extremities, combined with dyspnea and cough. None of the patients presented with acute neurological symptoms, but 20 (22%) had respiratory distress due to airway compression.
2.2. Methods
All patients underwent the same management procedures after admission, i.e. a complete clinical examination for the detection of subcutaneous lymph nodes or masses, and then chest roentgenogram and CT-scan with contrast enhancement to determine the location and evaluate the extent of the lesion.
When a subcutaneous lymph node was considered to be abnormal, it was biopsied under local anaesthesia. When there was no subcutaneous lymph node but a palpable supraclavicular mass, it was biopsied under general anaesthesia as a first-step surgical procedure.
When a subcutaneous lymph node or mass to be biopsied was not revealed, and when the clinical history and CT-scan were suggestive of a carcinoma, e.g. age over 50 years, heavy smoker, a mass located in the axial mediastinum without an associated pulmonary lesion compressing the superior vena cava anteriorly or of a thymoma, the procedure of first choice was a CT-guided tru-cut biopsy (CTGB) of the mass, if the radiologist considered it accessible to this procedure. If CTGB failed, or if the mass was not accessible to it, axial mediastinoscopy (MDS) was done when a carcinoma was suspected and when the lesion was located in the axial mediastinum. An anterior mediastinotomy was performed when a thymoma was suspected or when the suspected tumour was located in the anterior mediastinum.
When the patient was under 50 years of age and the SVC displayed a quick evolution, and the CT-scan revealed a heterogeneous mass located in the anterior mediastinum compressing the superior vena cava posteriorly, and/or when there was an important elevation of tumour markers, a lymphoma or a germ cell tumour were advocated. In such an instance, anterior mediastinotomy was the procedure of first choice for an anterior mass lateral to the sternum. When the mass was anterior but only retrosternal, anterior mediastinoscopy was the procedure of first choice. When patients presented with an anterior mediastinal mass associated with a pericardial tamponade, the first choice procedure was pericardioscopy with the 24-cm-long rigid pericardioscope derived from the conventional 17-cm mediastinoscope.
Whatever the suspected diagnosis, video-assisted thoracoscopy (VATS) was reserved for cases in which MDS and anterior mediastinotomy were considered inappropriate, for anatomical reasons. Thoracotomy or sternotomy were only considered when all the minimally invasive procedures had failed to establish a definite diagnosis.
During any surgical procedure, at least three biopsies were taken from different sites. Frozen section was only requested for selected cases.
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3. Results
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3.1. Results of biopsies performed on the basis of clinical examination findings
Eleven patients (12.5%) had subcutaneous lymphadenopathy (jugular, n=10; and axillary, n=1) sampled under local anaesthesia without any morbidity. A diagnosis was obtained for all patients. Seven patients had a carcinoma, two had Hodgkin's disease and two had non-Hodgkin's lymphoma (NHL).
Three patients (3.4%) had a palpable suprascapular mass biopsied under general anaesthesia without any morbidity. All were diagnosed, two patients had NHL and one had Hodgkin's disease.
3.2. Results of CTGB
Twenty-three patients were assumed to have either a carcinoma (n=18) or a thymoma (n=5) on the basis of the clinical and radiological presentation of the mediastinal mass, which the radiologist considered accessible to CTGB. They was no morbidity of this procedure. Six patients had false negative results (26% of the procedures); two of them underwent a MDS (final diagnosis: carcinoma) and the other four underwent anterior mediastinotomy (final diagnosis: NHL for three and thymoma for one). The final diagnoses and sensitivities are given in Table 1.
3.3. Results of MDS
In 21 other patients, the most probable diagnosis was also assumed to be a carcinoma, but the mass was not considered accessible to CTGB. Consequently, MDS was the first-step procedure, and two additional patients underwent MDS after the failure of CTGB. There was one case of morbidity (4.3% of the procedures), which consisted of abundant bleeding from a huge mediastinal vein which required partial sternotomy to achieve hemostasis. Thereafter, the patient concerned recovered normally. For two patients who had a final diagnosis of NHL, the diagnosis could not be established by MDS, and one anterior mediastinotomy and one thoracotomy were necessary. The final diagnoses obtained after the 23 MDS procedures and the corresponding sensitivities are given in Table 2.
3.4. Results of anterior mediastinotomy and of anterior mediastinoscopy
Twenty patients who were assumed to have NHL, Hodgkin's disease, or a germ cell tumour with a lateralized anterior mass, and five patients with failed CTGB and MDS, underwent anterior mediastinotomy. Another patient underwent repeat anterior mediastinotomy after previous anterior mediastinotomy had given a false negative result. Per-operative morbidity was nil, and post-operative morbidity consisted of superficial wound suppuration in four patients (15.3% of the procedures). Further therapeutic management, in particular the institution of chemotherapy, was not affected by these suppurations. The final diagnosis and sensitivities of the 26 anterior mediastinotomies performed in 25 patients are given in Table 3. Overall, there was one false negative result (4% of the patients) for the patient whose final diagnosis of NHL was established after repeated anterior mediastinotomy.
Six patients who had a retrosternal anterior mass underwent an anterior mediastinoscopy without any morbidity. Histological diagnosis was obtained in all cases, including four cases of NHL, one of carcinoma and one of sarcoma.
3.5. Results of pericardioscopy and thoracoscopy
Three patients had a pericardial effusion with tamponade associated with an anterior mediastinal mass. As emergency drainage of the effusion was necessary, pericardioscopy was the first-step procedure. A mass invading the pericardial sac was visualized through the pericardioscope and sampled in every case. There was no morbidity, and a diagnosis was obtained in all three cases (NHL, n=2; and germ cell tumour, n=1). In these patients, cytological examination of the pericardial fluid and pathological examination of the pericardial window were both negative.
Two patients underwent thoracoscopy. In one patient, pleural symphysis led to thoracotomy to establish the diagnosis of NHL. In the other, the diagnosis of NHL was established by thoracoscopy.
3.6. Global results
The overall morbidity was five cases out of 99 diagnostic procedures in 88 patients (5% of the procedures). Sternotomy and thoracotomy were necessary in three cases (3.4% of the patients) because of the failure of minimally invasive approaches in two cases, and as a salvage procedure during MDS in one case.
A precise histological diagnosis (see Table 4) was established in the 88 patients after the 99 following sampling procedures: 26 AM, 23 CTGB, 23 MDS, 11 peripheral lymph node biopsies, six anterior mediastinoscopies, three biopsies under general anaesthesia of the suprascapular masses, three pericardioscopies, two thoracotomies, one sternotomy and one thoracoscopy.
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4. Discussion
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Since the work of Ahman, who reviewed 1986 cases of SVCS published in the English literature from 1934 to 1983, small cell lung cancer has been considered to be the most common cause of SVCS of a malignant origin [1]. Furthermore, in this review, non-small cell lung cancer was more frequently encountered than lymphoma. In 1990, Yellin and associates reported a series of 63 patients treated in a single institution for SVCS; 47.6% had a bronchogenic carcinoma, but small cell was not the most frequent subtype of lung cancer, and 20.6% of the patients had a lymphoma [8]. The main particularity of our study, compared to these series, is that lymphoma accounted for half of the cases of SVCS, with a large majority of large B cell subtype (NHLB). Nevertheless, there was probably a selection bias in our series, due to the fact that only surgical patients were referred to us, and that a certain number of non-referred patients must have had their diagnosis of lung cancer established by bronchoscopy. This frequent association of NHLB with SVCS confirms the report by Lazzarino and colleagues, who showed that NHLB was associated with SVCS in 75 out of 106 patients with mediastinal NHLB (70%) treated in a multicenter study [9].We also confirmed that thymoma is not frequently associated with SVCS, because it is frequently a slowly growing tumour that allows collateral circulation to develop [10].
4.1. Morbidity of invasive procedures
Contrary to a well established opinion, the authors of several recent studies assumed that tissues with increased venous pressure can be biopsied in most patients with SVCS without significantly increasing morbidity. Nevertheless, these authors reported some unusual complications. For instance, one of the 14 patients with SVCS for whom Jahangiri et al. performed MDS had an innominate artery injury [11], and Callejas and colleagues reported one case of wound infection and one carotid artery injury among eight patients with SVCS who also underwent MDS [12]. In our series, a partial sternotomy was required for one patient to achieve hemostasis during an MDS performed by a surgeon who had previously done more than 500 MDS without incurring any morbidity [13]. These events show that although some physicians’ fear of performing this procedure is unjustified, it is certainly more difficult and dangerous in cases of SVCS.
4.2. Choice of the best diagnostic approach
In the present series, we again demonstrated that a careful systematic clinical examination by the surgeon is of great importance in revealing subcutaneous lymph nodes to be biopsied under local anaesthesia. When clinical examination fails to reveal any palpable adenopathy or subcutaneous mass, the chest CT-scan with injection of contrast medium is the cornerstone of diagnostic imaging procedure, which, along with the clinical history, determines the choice of the most appropriate surgical approach. CT-scans show the exact location of the mass, its relationship with the surrounding structures, its density, and therefore, the compression or thrombosis of the vena cava.
We confirm that pericardioscopy may be useful for some patients having both a symptomatic pericardial effusion requiring emergent drainage and an anterior mediastinal mass. In the three patients concerned, two of whom had NHL and one a germ cell tumour with normal markers levels, the results of cytological and biochemical analyses of the pericardial fluid were both negative. Diagnosis was only established by pericardial biopsies under visual control, performed with the 24-cm-long pericardioscope [14].
CTGB is useful to diagnose carcinoma and thymoma [15–18], but is not sufficiently accurate for precise individualization of NHL, which requires larger samples for immunohistochemical and cytogenetic studies [10,19,20]. Even when CTGB permits the diagnosis of NHL, a surgical biopsy is required for its characterization, so that further treatment can be planned in as appropriate a fashion as possible. In cases of suspected NHL compressing the trachea or the main bronchi to a point at which general anaesthesia with tracheal intubation would be extremely hazardous, we advocate anterior mediastinotomy under local anaesthesia rather than CTGB [20].
The role of VATS in patients with SVCS is unclear. In some studies it proved sensitive for diagnosing mediastinal tumours, but most of the lesions in these series were located in the middle or posterior mediastinum rather than the anterior mediastinum, and most of the patients did not have SVCS [21–25]. In our series, nearly all the lesions leading to SVCS were located anteriorly or in the axial mediastinum, and were accessible to anterior mediastinotomy or MDS. For these reasons, we believe that VATS is only indicated for the biopsy of masses that cannot be reached by anterior mediastinotomy, MDS, or anterior mediastinoscopy, or when the exploration of the chest cavity is necessary.
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Received August 23, 1999;
received in revised form January 10, 2000;
accepted February 7, 2000.
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Abstract
1. Introduction
