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Keywords
Superior vena cava syndrome, splenic metastasis, small cell carcinoma, endoscopic ultrasonography.
Imaging Information
Video’s were created by digitizing SVHS tapes using a Canopus ADVC-500 capture board and Adobe Premiere Pro 1.5 software.
Introduction
Superior Vena Cava (SVC) syndrome is a serious consequence of mediastinal tumors, which can require urgent intervention. A tissue diagnosis of the tumor type is important to guide treatment after the initial alleviation of SVC syndrome. Staging is also important in that it allows tailored therapy with the longer course in mind.
We present a case of a man who presented with SVC syndrome who also had a mass in his spleen. EUS was used to biopsy both the mediastinum and spleen to rapidly establish a tissue diagnosis and stage of his disease.
Case/Body
HISTORY OF PRESENT ILLNESS: A 58-year-old man was seen in thoracic surgery clinic because of mediastinal adenopathy.
He had had two episodes of lower lobe pneumonia during the prior month. Despite antibiotic therapy, he continued to have difficulty breathing and remained hypoxic.
His chest radiograph. displayed lower lobe infiltrates and mediastinal adenopathy. A CAT scan confirmed the presence of massive mediastinal adenopathy but no primary lung mass. There was compression of the superior vena cava and trachea just above the carina.
He reported night sweats, progressive fatigue and shortness of breath. He had lost 20-30 lbs. over the prior 3-4 months.
The patient drank 3-6 beers a day and had smoked more than a pack of cigarettes a day for over 20 yrs.
On physical exam he displayed mild dyspnea at rest. His vital signs were normal.
He had some facial swelling, bulging of the external jugular veins, and enlarged veins on the chest and forehead consistent with an SVC syndrome. There was no palpable cervical or supraclavicular adenopathy. His lung exam revealed prolonged expirations, but no rales. A small amount of stridor was noted, and he was unable to lie flat due to dyspnea. His abdominal exam was unremarkable.
As noted above, a CT scan showed massive mediastinal adenopathy and compression of the superior vena cava and the trachea (Figure 1). No primary lung mass was seen, but there was atelecstasis in the lungs. His liver was normal, and a lesion was seen in the spleen (Figure 2).
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Figure 1 |
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Figure 2 |
His blood count was normal, and he had normal liver chemistries, electrolytes, calcium, LDH and uric acid.
The patient was diagnosed clinically with SVC syndrome requiring urgent intervention due to his clinical condition. A primary lung cancer and lymphoma were the leading diagnostic considerations.
The thoracic surgeon consulted briefly with the endoscopist and medical oncologist. The patient was sent directly to the endoscopy suite where he immediately received 20 mg of intravenous dexamethasone. An EGD/EUS with FNA was performed with the radiation oncologist in attendance. After the procedure the patient was examined by the radiation oncologist and the medical oncologist.
The EGD was significant for proximal esophageal varicies as shown (Video 1). EUS revealed massive mediastinal adenopathy and a splenic mass, but no other adenopathy or lesions. The splenic lesion was located and biopsied under EUS guidance using a 25 g needle (Video 2). Visualization of the relevant anatomy regarding this biopsy can be seen in the visible human image (Figure 3) which is linked to the anatomy data base. The mediastinum was also biopsied with a 25 g needle avoiding the varicies (Video 3), to provide both a tissue diagnosis and pathologic staging. The cytology was reviewed immediately by a pathologist who suggested a diagnosis of extensive stage small cell lung carcinoma, since both the mediastinal nodes (Figure 4) and spleen (Figure 5) showed the same pathologic features.
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Figure 3: Click here to launch Interactive Atlas |
Figure 4 |
Figure 5 |
The patient was taken directly from endoscopy to the radiation oncology department for palliative therapy. Since the disease was shown to be extensive stage small cell lung cancer rather than limited stage, he was started on limited field treatment with accelerated doses (30 Gy over 10 fractions) without concurrent chemotherapy rather than lower dose with conventional fields. He was admitted to the hospital from the radiation therapy suite.
After radiation therapy was completed he began chemotherapy with etopiside and carboplatinum. The combination of radiation therapy and steroids provided substantial relief of the patient’s SVC syndrome.
Discussion
Small cell carcinoma is an aggressive tumor that often presents with mediastinal lymphadenopathy. SVC obstruction can be found in up to 10% of patients with small cell carcinoma at diagnosis. In many cases, SVC syndrome can be treated electively, but in some cases problems can occur due to venous or airway obstruction1-4.
Diagnosis of the tumor type is important in patients with SVC syndrome since the type of therapy varies significantly depending on tumor histology. The differential diagnosis in a patient such as this would include a primary lung malignancy, particularly small cell lung cancer, or an aggressive lymphoma5,6.
Not only is a diagnosis critical, but surgical staging is also important in determining the extent of therapy. Small cell cancer that is limited to the chest has a potential for cure, and radiation to the primary tumor is often employed as part of the curative intent. Disease outside of the chest is probably incurable, and different radiation therapy objectives are employed, particularly in limiting potential damage to other chest structures7,8. Likewise, lymphoma that crosses the diaphragm is more extensive, and aggressive systemic, rather than aggressive local therapy is employed.
This patient had a splenic metastasis which is uncommon in the absence of widespread metastatic disease. Isolated splenic metastases have been reported in a variety of tumors, including gynecologic malignancies, colon cancer, melanoma, hepatoma, esophageal gastric cancer and non-small cell lung cancer9-16. Reports of splenic metastases from small cell lung cancer are rare.
EUS was able to rapidly diagnose and stage this patient, allowing definitive treatment to begin within a few hours after presentation to the thoracic surgery clinic. The confirmation of a splenic metastasis provided essential staging information that guided therapeutic decisions. EUS splenic biopsy appears to be safe and can provide vital staging information17.
In summary, a man presented with SVC syndrome and a splenic mass. EUS biopsy demonstrated small cell lung cancer in both the mediastinum and spleen, allowing definitive, stage-specific therapy to proceed. EUS is an important tool in the multidisciplinary management of patients with complex malignancies.
References
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