Asian Cardiovasc Thorac Ann 2000;8:280-282
© 2000 Asia Publishing EXchange Pte Ltd
Superior Vena Cava Syndrome Due to Metastatic Transitional Cell Carcinoma
Bishwo Man Singh Shrestha, MS,
Bhagwan Koirala, MS,
Purna Raj Joshi, MD,
Mod Nath Marhatha, MD,
Govind Prasad Sharma, PhD,
Bhola Raj Joshi, MS,
Mahendra Bhatta, ISc,
Gita Sayami, PhD,
Ramesh Koirala, MBBS,
Laxmi Rajbhandari, MN
Department of Surgery, Cardiothoracic Unit
Tribhuvan University Teaching Hospital
Maharajgunj, Kathmandu, Nepal
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For reprint information contact: Bishwo Man Singh Shrestha, MS Tel: 977 1 22 9137 Fax: 977 1 16 1886 email: bmsingh{at}wlink.com.np Department of Surgery, Cardiothoracic Unit, Tribhuvan University Teaching Hospital, P.O. Box 3578, Maharajgunj, Kathmandu, Nepal.
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Abstract
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Superior vena cava syndrome due to infradiaphragmatic tumor metastasis was detected in a 70-year-old man. A tumor attached to the right atrial wall was excised and subsequent transurethral resection of a bladder tumor revealed high-grade transitional cell carcinoma. The disease progressed and the patient died 6 months later.
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Introduction
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Both primary and secondary tumors of the heart are well-known. The most common neoplastic process that involves the heart is metastatic deposits from primary tumors elsewhere in the body. When the heart is affected, metastatic disease is usually found to be widespread. A clinical diagnosis of metastatic heart disease is seldom made because only 10% of patients have symptoms attributable to metastases in the heart. Symptoms that do arise are overshadowed by the primary disease. An infradiaphragmatic tumor usually causes metastases in the inferior vena cava and right atrium (RA). It is very unusual to have a discrete metastatic mass in the superior vena cava (SVC) from an asymptomatic infradiaphrag-matic primary source.
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Case Report
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A 70-year-old man was admitted with swelling of the face, distension of the neck veins, and headache of 5-months duration, which became worse on leaning forward. He had no history of respiratory, gastrointestinal, or urinary problems. He had no significant weight loss and he neither smoked nor drank alcohol. He was afebrile with stable vitals and no palpable lymph nodes. Except for distended veins on the neck, anterior chest, and upper extremity, physical findings were unremarkable. Laboratory in-vestigations revealed slight anemia with a hemoglobin level of 99 g•L–1. Chest radiography showed slight widening of the upper mediastinum. Normal sinus rhythm was found on electrocardiography. Contrast-enhanced computed axial tomography (Figure 1
) and echo-cardiography indicated a mass in the SVC protruding into the RA. A venacavogram (Figure 2) confirmed a mass obstructing the SVC. Abdominal ultrasonography was normal.
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Figure 1. Contrast-enhanced computed tomography of the chest. The tumor mass can be seen distending the superior vena cava and occupying 95% of the lumen. The mass extended up to the level of the right atrium.
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Figure 2. Venacavogram showing a large oval mass obstructing the lumen of the superior vena cava from close to the junction of the innominate veins up to the level of the right atrium.
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With a clinical diagnosis of right atrial myxoma, the patient underwent surgery through a median sternotomy. The SVC which was bulging with a solid mass, was carefully dissected up to the junction of the innominate veins. Both innominate veins were snared tight and the RA was opened longitudinally by extending the incision over the SVC, under normothermic cardiopulmonary bypass and crystalloid cold cardioplegia. The tumor was pedunculated and attached to the right atrial wall laterally, near the junction of the SVC and the RA. The tumor was excised, the RA and SVC were closed, and the patient was weaned off bypass easily. The tumor measured 8 x 5 x 4 cm, the lower part was hard and the upper part was fragile and superimposed with old and fresh thrombi. Histopathology of the specimen revealed metastatic undifferentiated carcinoma with a suspicion of transitional cell carcinoma (Figure 3). Cystoscopy performed retro-spectively, showed a small sessile polypoid growth in the anterior wall of the urinary bladder. Transurethral resection of the bladder tumor was carried out, the specimen revealed high-grade transitional cell carcinoma. Postoperatively, the patient had frequent premature ventricular contractions that were managed with antiarrhythmic drugs. From the 6th postoperative day, he complained of severe backache and gradually developed a tingling sensation with numb-ness and weakness of the lower limbs. A myelogram revealed spinal metastases. He later developed urinary incontinence and complete paraplegia. Radiotherapy was given but the disease progressed and he succumbed 6 months later.
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Figure 3. Histopathology of the cardiac tumor: polygonal tumor cells can be seen in a solid pattern with stratification, suggestive of transitional cell carcinoma (hematoxylin and eosin stain, original magnification x200; inset x400).
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Discussion
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Metastatic tumors of the heart are up to 40 times more frequent than primary cardiac tumors.1,2 The incidence of metastatic cardiac disease in patients with known malignancies is approximately 10%.1 An increase in cardiac metastases noted in later series may be related to prolonged survival after surgical and adjunctive therapy. The parts of the heart affected by metastatic tumors in decreasing order of frequency are pericardium, myo-cardium, and endocardium. Malignant involvement of the heart may occur by hematogenous routes, by direct invasion from adjacent mediastinal structures, or by retrograde invasion from lymphatic channels. As the tumor in this case was located between the SVC and RA near the sinoatrial node, metastasis might have been via the lymphatic route because in all reported primary infradia-phragmatic malignant tumors, metastasis occurred in the inferior vena cava with extension into the RA.3–5 A case of pericardial metastatic mass from a bladder carcinoma has been previously reported.6
The most frequent clinical expression of secondary cardiac malignancy is pericardial effusion and cardiac tamponade. In addition, a solid tumor growth may impinge on cardiac structures and cause various symptoms.7 Rapidly pro-gressing congestive heart failure of recent onset and refractory to medical therapy, is characteristic of a malig-nant cardiac tumor. To our knowledge, this is the first case wherein an asymptomatic bladder cancer gave rise to a large metastatic deposit in the RA with obstruction of the SVC and without involving the inferior vena cava.
Since there were no symptoms suggesting a urological disorder, the diagnosis in this case could be made only after the operation. Although the patient was relieved of the symptoms of SVC obstruction, long-term survival beyond 6 months could not be achieved. In Western literature, survival of patients varied from 1 week to more than 8 years and depended on the progression of malignancy elsewhere in the body.2,8 As metastatic tumors of the heart are becoming more common due to improved survival afforded by advanced treatment of malignancies, their presence should be sought in a cancer patient who develops new primary cardiac symptoms. A small asymptomatic primary growth might give rise to fatal distant metastases.
Presented at the 7th Annual Meeting of the Asian Society for Cardiovascular Surgery, Singapore, May 28–31, 1999.
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Acknowledgments
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Our sincere thanks to Prof. Bhola Raj Joshi, Prof. Mahesh Khakurel, Prof. Roshana Amatya, Department of Surgery, and all involved nurses. Our special gratitude to our executive director Prof. Govinda Prasad Sharma and the hospital management for facilitating us with a platform for regular open heart surgery in the Tribhuvan University Teaching Hospital and for their continuous moral and physical support.
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References
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Abstract
Introduction
