(Chest. 1999;116:835-837.)
© 1999
American College of Chest Physicians
Multimodality Treatment of Malignant Superior Vena Caval Syndrome*
John R. Roberts, MD, FCCP;
Raphael Bueno, MD, FCCP and
David J. Sugarbaker, MD, FCCP
*
From the Department of Cardiac and Thoracic Surgery (Dr. Roberts), Vanderbilt University Hospital, Nashville, TN; and the Division of Thoracic Surgery (Drs. Bueno and Sugarbaker), Brigham and Women's Hospital, Boston, MA.
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Abstract
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Malignant superior vena caval (SVC) syndrome due to non-small cell
lung cancer is invariably fatal, with most therapy directed toward
palliating the manifestations of the disease. A cure, by means of any
modality, is unusual. We report a patient with SVC syndrome secondary
to documented ipsilateral peritracheal nodal involvement (stage IIIB
disease) who underwent neoadjuvant chemoradiotherapy and resection. At
surgery, his superior vena cava was not involved and his tumor had been
downstaged to stage I (T1 nanoseconds). He remains alive and free of
disease 60 months after surgery. Neoadjuvant chemoradiotherapy may be
used to downstage malignant SVC syndrome to resectable lesions in good
functional candidates.
Key Words: chemoradiotherapy • multimodality treatment • neoadjuvant • non-small cell lung cancer • superior vena cava • superior vena caval syndrome • surgery
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Introduction
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Malignant
superior vena caval (SVC) syndrome from non-small cell lung cancer is
invariably fatal. Reported 5-year survival rates have ranged from 0 to
5%. The primary therapy is radiotherapy, generally with palliative
intent. When added to radiotherapy, chemotherapy with cisplatin-based
regimens may improve survival, though even the combination is seldom
successful. Surgical bypass of the SVC obstruction is effective
palliation but yields poor long-term survival (mean survival, 10.7
months).1
2
The venous obstruction may result from direct
tumor invasion or from malignant involvement of peritracheal lymph
nodes with compression of the superior venal cava.
Despite these dismal statistics, recent information indicates improved
response rates when patients with non-small cell lung cancer are
treated with modern chemotherapies and radiotherapy. Better response
rates with neoadjuvant therapies may allow for complete resections,
even in lesions that cannot be sterilized by chemotherapy or
radiotherapy. We report a case of a patient with ipsilateral nodal
involvement (documented by mediastinoscopy) and SVC syndrome who
received neoadjuvant chemoradiotherapy, who was resected, and who is
alive at 60 months. At pathologic analysis, he was found to have a
residual 2-cm tumor with negative parenchymal, hilar, and mediastinal
nodes. This case argues for the extension of the application of
neoadjuvant chemotherapy to lesions generally not considered
resectable.
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Case Report
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The patient is a 47-year-old white man with a 62 pack-year
smoking history who developed a persistent cough. His chest radiograph
(Fig 1 ) and CT scan demonstrated a right upper lobe mass invading the
mediastinum (Fig 2
), with no contrast filling the superior vena cava. Shortly before
mediastinoscopy, the patient developed upper extremity and facial
swelling consistent with SVC syndrome. SVC obstruction was confirmed
with bilateral upper arm extremity phlebography (Fig 3
).
At mediastinoscopy, 4R and 2R nodes demonstrated an extracapsular
extension of a metastatic non-small cell tumor. Contralateral nodes
were negative.
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Figure 3. Top: a left arm venogram
demonstrating some filling of the hemiazygous system, but no
contrast filling the innominate vein or the superior vena cava.
Bottom: a right arm venogram demonstrating that there is
no filling of the subclavian or superior veins.
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The patient then received 55 Gy over 6 weeks and cisplatin-based
chemotherapy as definitive therapy. He demonstrated a good partial
response (> 50% reduction in the greatest tumor diameter), and the
results of the head and bone scans were negative. Because he was
anxious to pursue all possible options, he was explored. At surgery, a
2-cm mass in the right upper lobe was associated with significant
mediastinal scarring. Mediastinal lymphadenectomy and right upper
lobectomy were straightforward, and no surgery on the superior vena
cava was necessary. The patient's postoperative course was complicated
only by a transient change in mental status. He was discharged home on
postoperative day 7, and he remains alive and without evidence of
disease 60 months later. He has had no evidence of significant
treatment-related side effects, and he has been able to function
entirely normally.
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Discussion
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Malignant causes of SVC syndrome include advanced lung cancer
(67%), mediastinal tumors (20%), and metastatic solid malignancy
(5%).3
Most cases of SVC syndrome from advanced lung
cancer result from small cell lung tumors. The treatment of this entity
is strikingly different and never involves surgery.
SVC obstruction may either be caused by direct invasion and compression
of the superior vena cava by the tumor or by nodal compression of the
superior vena cava. The difference is important because resection of
the superior vena cava directly invaded by non-small cell bronchogenic
carcinoma can result in cures,4
5
whereas resection
of the superior vena cava with peritracheal nodal metastases results in
no 5-year survivors.5
Thus, the patient presented would
have been unlikely to benefit from immediate lung and SVC resection
because of his nodal involvement.
Neoadjuvant cisplatin-based chemotherapy and subsequent surgery
have been demonstrated to prolong median survival and to increase the
proportion of 5-year survivors among patients with stage IIIA non-small
cell cancer in three separate prospective, randomized
studies.6
7
8
Although it is not universal, this therapy
has become the standard of care in many communities. A Southwestern
Oncology Group study found that neoadjuvant chemoradiotherapy and
subsequent surgery gave 24% 3-year survival in patients with
contralateral nodal involvement or stage IIIB disease.9
This is not standard therapy for malignant SVC syndrome, which is
usually treated with radiotherapy, with or without chemotherapy.
Malignant SVC syndrome is generally considered a contraindication to
curative resection, although palliative bypasses are done for symptoms
that do not respond to medical therapy.1
2
10
The outcome for this patient indicates that malignant SVC
syndrome can be cured with the use of cisplatin-based chemotherapy,
radiotherapy, and subsequent resection. This combined modality
therapy should be considered for patients with malignant SVC syndrome.
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Figure 4. A postoperative chest radiograph done 48 months
after surgery demonstrating no evidence of a recurrent tumor. Scarring
at the apex of the chest is unchanged from its condition immediately
after surgery.
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Footnotes
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Abbreviation: SVC = superior
vena caval
Corrrespondence to: John R. Roberts, MD, FCCP, Department of
Cardiac and Thoracic Surgery, Vanderbilt University Hospital, 2986 The
Vanderbilt Clinic, Nashville, TN 37027; e-mail:
Bob.Roberts@mcmail.vanderbilt.edu
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References
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Doty, DB, Doty, JR, Jones, KW (1990) Bypass of superior vena cava: fifteen years' experience with spiral vein graft for obstruction of superior vena cava caused by benign disease. J Thorac Cardiovasc Surg 99,889-895[Abstract]
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Inoue, H, Shohtse, A, Koide, S, et al (1990) Resection of the superior vena cava for primary lung cancer: 5 years' survival. Ann Thorac Surg 50,661-662[Abstract]
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Dartevelle, PG, Chapelier, AR, Pastorino, U, et al (1991) Long-term follow-up after prosthetic replacement of the superior vena cava combined with resections of mediastinal-pulmonary malignant tumors. J Thorac Cardiovasc Surg 102,29-65[Abstract]
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Pass, HI, Pogrebniak, HW, Steinberg, SM, et al (1992) Randomized trial of neoadjuvant therapy for lung cancer: interim analysis. Ann Thorac Surg 53,992-996[Abstract]
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Rosell, R, Gomez-Codina, J, Camps, C, et al (1994) A randomized trial comparing preoperative chemotherapy plus surgery with surgery alone in patients with non-small-cell lung cancer. N Engl J Med 330,153-158[Abstract/Free Full Text]
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Roth, JA, Fossella, F, Komaki, R, et al (1994) A randomized trial comparing perioperative chemotherapy and surgery with surgery alone in resectable stage IIIA non-small-cell lung cancer. J Natl Cancer Inst 86,673-680[Abstract/Free Full Text]
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Albain, KS, Rusch, VW, Crowley, JJ, et al (1995) Concurrent cisplatin/etoposide plus chest radiotherapy followed by surgery for stages IIIA (N2) and IIIB non-small-cell lung cancer: mature results of southwest oncology group phase II study 8805. J Clin Oncol 13,1880-1892[Abstract/Free Full Text]
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Abstract
Introduction
