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Challenging Problems in Advanced Malignancy

CASE 1. CHYLOPERICARDIUM DURING THE TREATMENT OF NON-HODGKIN’S LYMPHOMA

Ewha Womans University, College of Medicine, Seoul, Korea

A 14-year-old boy presented with blood-tinged sputum and night sweats persisting for 3 months. He didn’t have any weight loss or fever. Physical examination showed facial edema, supraclavicular lymphadenopathy, and absence of hepatosplenomegaly. The complete blood analysis showed a hemoglobin level of 12.8 g/dL, a leukocyte count of 7,700/µL with normal differential count, and a platelet count of 297,000/µL. Other biochemical tests were within normal range, except for lactate dehydrogenase, which was 2,300 U/mL. A chest x-ray film showed a large mediastinal mass. A computed tomography (CT) scan of the thorax confirmed a multilobulated, heterogenous mass extending from the manubrium to the diaphragm, and measuring 8 x 8 x 14 cm (Fig 1A). The superior vena cava was markedly compressed and distorted (Fig 1A, arrow). Biopsy of the mass revealed a diffuse large-cell lymphoma (Fig 2A) of B-cell lineage (Fig 2B, L26-positive). Bone marrow aspiration and biopsy revealed invasion of lymphoma (stage IVB). Combination chemotherapy with cyclophosphamide, vincristine, doxorubicin, and dexamethasone plus intrathecal methotrexate and cytarabine was started. During the first cycle, dyspnea was markedly improved, and the mass size decreased. On day 17, sudden severe dyspnea developed. Tachycardia and decreased cardiac tones without murmurs were noted. Chest x-ray films showed new cardiomegaly, and ECG revealed a sinus tachycardia of 112 beat/min with low voltage. Echocardiography confirmed the presence of a moderate pericardial effusion with swinging heart image, but cardiac tamponade was not present (Fig 1B). A contrast-enhanced CT scan of the thorax also showed pericardial effusion of 2 cm in depth (Fig 1C). Lymphangiography showed a communication between lymphatics of thorax and cardiopericardiac space (Fig 1D). Pericardial drainage revealed 300 mL of a milky fluid (Fig 2C). Fluid analysis showed a triglyceride level of 1,330 mg/dL, a cholesterol level of 98 mg/dL, a protein level of 7.0 mg/dL, a leukocyte count of 170/µL (lymphocytes, 76%), no atypical cells, and negative cultures. Sudan III–stained fat droplets were found in the fluid (Fig 2D).

View larger version (107K): [in this window] [in a new window]   Fig 1. (A) Computed tomography (CT) scan of thorax (superior vena cava was markedly compressed and distorted, arrow); (B) echocardiography confirmed presence of moderate pericardial effusion, but cardiac tamponade was not present; (C) CT scan of thorax showed pericardial effusion of 2 cm in depth; (D) lymphangiography showed communication between lymphatics of thorax and cardiopericardiac space.

View larger version (136K): [in this window] [in a new window]   Fig 2. Biopsy of the mass showing (A) a diffuse large-cell lymphoma of (B) B-cell lineage. Pericardial drainage revealed (C) milky fluid with (D) Sudan III–stained fat.

The incidence of cardiac involvement by neoplasm in autopsy series is approximately 10%.¹ The mechanical obstruction of lymphatic drainage is caused by postoperative or postirradiation fibrosis and superior vena cava syndrome. Chylopericardium is related to thoracic duct obstruction, with failure of adequate collateral drainage and resultant reflux of chylous lymph through lymphatics draining the heart and pericardium.^2–4 When mechanical obstruction increases above 15 cm H₂O pressure in lymphatic vessels, and when there is compression of venous collateral drainage, chylopericardium occurs.⁵ Although 44% of patients are asymptomatic, clinical features include dyspnea (39%), cough (12%), palpitation (3%), and chest pain (3%).² These symptoms are similar to those observed from other pericardial effusions. Only pericardial fluid examination will give the correct diagnosis. Although the levels of fluid composition depend on the patient’s diet, chylous fluid is characterized by a milky appearance and cholesterol levels higher than 100 mg/dL, triglyceride levels over 1,000 mg/dL, and protein levels higher than 3 g/dL.² The combination of lymphangiography with CT scan is an important diagnostic approach.^6,7 Lymphangiography in our patient showed a connection between the thoracic duct and pericardium. In most cases, especially those associated with obstruction, a surgical approach, such as thoracic duct ligation and placement of a pleuropericardial window, may be necessary. Medical therapy should not be continued for longer than 2 weeks.⁶ Therapeutic goals are prevention of cardiac tamponade, constrictive pericarditis, and the loss of lymphatic fluid. Treatment usually begins with a diet rich in medium-chain triglycerides, which are directly absorbed into the portal venous system, to reduce the amount of lymph ascending into the thoracic lymphatics.⁴ Repeated pericardiocentesis or use of a pericardiostomy tube are necessary in less than 50% of patients.⁸ Our patient presented with superior vena cava syndrome caused by primary mediastinal, diffuse, large B-cell lymphoma. Isolated spontaneous chylopericardium during treatment was managed successfully only with pericardiocentesis and tube drainage. If pericardial effusion develops in non-Hodgkin’s lymphoma, chylopericardium should be considered as one of the differential diagnoses.

REFERENCES

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