Clinical Meetings at RH Year 2005

2005 - TKOH: A 32-year-old man with bilateral lung shadow

Dr. SN Chan, Dr. LS Ip, Dr. PY Tse; Department of Medicine, Tseung Kwan O Hospital

Case History
Mr. Lam was 32 years old and was a non-smoker and non-drinker. He had childhood asthma but did not need regular follow up or medication for years. He presented with chronic productive cough for one month, associated with blood stained sputum and dyspnoea the week before admission. He complained of chest pain after severe cough, subjective weight loss and poor appetite. He did not have nasal congestion, night sweat, TB contact and recent travelling.

On admission, he was stable and not in respiratory distress. The blood pressure was 99/56mmHg. Pulse rate was 100 beats per minute. Oxygen saturation was 92% on room air and body temperature was 37.5 ºc.

On examination, there was bilateral basal crepitations on auscultation. There was no cardiac murmur or lower limb oedema. JVP was not elevated. CXR on admission showed multiple nodular shadows on bilateral lung fields (Fig.1).

Investigation results on admission:


He was given amoxycillin/clavulanate potassium, clarithromycin and tranexamic acid intravenously.

Later the sputum culture, AFB smear and cytology were found to be negative.
Six days after admission, he still complained of mild haemoptysis. Fever kicked up to 38.5ºc. He developed type I respiratory failure and required 50% of oxygen supplement. Haemoglobin level dropped to 7.7g/dL. CXR was repeated and showed increasing alveolar and nodular shadows(Fig.2). Computerized tomography for thorax with contrast was arranged which found a 4 x 5.5 cm filling defect in right atrium (Fig 3).



Mr. Lam finally received an operation for the palliative excision of right atrial tumour.
There was a large non-gelatinous tumour, arising from right atrium near the superior vena cava entry (Fig.4). Both lungs were found to have patchy consolidation and collapsed changes. There was blood stained pleural effusion in both pleural cavities and a straw colored pericardial effusion.


Fig 4: Intra-operative photo of the right atrial tumour

After the operation, Mr. Lam was in critical condition and complicated with multi-organ failure. He succumbed two days after operation.

Pathological examination:
The atrial tumour was fleshy with extensive hemorrhage. Heterogeneous pattern was found on microscopic examination. There was abnormal blood space with feature of malignancy (Fig. 5 & 6). The CD31 and CD34 staining were positive while the HHV8 staining was negative. It was compatible with angiosarcoma.



Diagnosis: Cardiac angiosarcoma with pulmonary metastasis, likely complicated with pulmonary hemorrhage

Discussion:
Primary malignant neoplasm of the heart is very rare. Angiosarcoma accounts for about one third of all malignant cardiac tumours. There is no specific predisposing factor identified.

The age of onset ranges from 8 to 80 years old with mean at 40. There is slight male preponderance. About half of the cases have metastasis identified at the time of diagnosis. Right atrium is being the most commonly involved heart chamber.
The initial presenting signs and symptoms are related to the location, size and extent of regional involvement, as well as to the presence or absence of metastasis. The most common site of metastasis is the lung, followed by the liver and the central nervous system. The presenting clinical features include pericardial effusion with cardiac tamponade, multiple lung metastases and diffuse pulmonary hemorrhage which, though is rare, should be considered in patient with metastatic angiosarcoma to the lung.1, 2, 3, 4, 7

The tumour can be dark red, hemorrhagic and lobular or diffusely infiltrate the pericardium and myocardium. Invasion of the superior and inferior vena cava, pulmonary artery and aortic root is not uncommon. Microscopically, the tumour shows vasoforming vascular channels lined by pleomorphic and atypical cells, and formation of irregular anastomosing sinusoidal structures with papillary intraluminal tufting. Immunohistochemically, the tumour has positive staining for factor VIII-related antigen, vimentin, CD34 and CD31.

Concerning the imaging modalities, chest radiograph is useful in identifying the metastatic lung lesions. The features include multiple nodules, linear infiltrations, pleural effusion, diffuse alveolar shadow or pneumothorax.5 Contrast CT scan of thorax can identify the intracardial filling defect which has low attenuation. The metastatic lung lesions can be either nodular or cystic.6 Two dimensional echocardiogram and MRI scan offer no more specific findings. Confirmatory diagnosis still relies on tissue biopsy.

Tissue can be obtained through thoracotomy, transvenous biopsy under echocardiographic guidance.

Surgical resection is the main state of therapy. Partial resection or orthotopic heart transplantation would be considered if complete resection is not possible. The tumour is not very responsive to either chemotherapy or radiotherapy though a combination of surgical resection, chemotherapy and radiotherapy has been tried with survival benefit.

Overall, the prognosis is grave. The mean postoperative survival is about 10 months. Long term survival could be achieved in just few cases reported in literature.

References

1. Glancy DL, Morales JB, Roberts WC. Angiosarcoma of the heart. Am J Cardiol 1968;21:413-9.
2. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report. Cancer 1986;57:852-9.
3. Hermann MA, Shankerman RA, Edwards WD, Shub C, Schaff HV. Primary cardiac angiosarcoma: a clinicopathologic study of six cases. J Thorac Cardiovasc Surg 1992;103:655-64.
4. Butany J, Yu W. Cardiac angiosarcoma: two cases and a review of the literature. Can J Cardiol. 2000 Feb;16(2):197-205.
5. Ashokakumar MP, Jay HR. Angiosarcoma in the Lung. Chest 1993;103:1531-33.
6. Ukihide T et al. Metastatic Angiosarcoma of the Lung: Spectrum of CT Findings. AJR 2003;180:1671-74.
7. Camilo A, Marie-Christine A, Henry DT, Jeffrey LM. Metastatic Angiosarcoma Masquerading as Diffuse Pulmonary Hemorrhage. Arch Pathol Lab Med 2001 Dec;125:1562-65.

Acknowledgement: We’d like to thank Dr. Y.W. Tsang, Department of Pathology, QEH for lending the histological slides and Dr. C.C. Ma, Department of Cardiothoracic Surgery, QEH for providing the intra-operative photos.