Pleural angiosarcoma mimicking pleural haematoma

Abstract

Pleural angiosarcoma is an extremely rare disease. Although the clinical course could be indolent, the prognosis is very poor once the tumour spreads. Herein, a 69-year old male with a history of thyroid goitre was noted for 5 years before the symptoms of right chest pain and body weight loss developed. His serial chest roenterogram revealed loculated pleural effusion which rapidly progressed to be multiple pleural haematomas. After several sono-guided aspiration/biopsies with undiagnosed pleural haematomas, an exploratory thoracotomy confirmed the diagnosis of pleural angiosarcoma. Whole body image studies did not find other suspicious primary sites except for a thyroid tumour with eccentric calcification extending into the thoracic cage. Aspiration cytology of the thyroid tumour was shown to be morphologically consistent with angiosarcoma. This case reminds clinicians that pleural metastatic angiosarcomas presenting as haematomas have a high risk of massive and refractory haemothorax.

INTRODUCTION

Angiosarcomas are malignant vascular tumours with a very poor prognosis. Although it can arise anywhere in the body, angiosarcoma of the thyroid is a rare condition and occurs mainly in patients from mountainous regions, especially European Alpine regions where iodine-deficient endemic goitre is more prevalent [1]. Generally, the prognosis is very poor once extraglandular tumour spreading occurs. We hereby present a case of unusual pleural metastatic angiosarcoma mimicking multiple pleural haematomas and describe the importance of surgical diagnosis, interesting images of roentgenogram and cytopathology.

CASE PRESENTATION

A 69-year old Chinese male living in a non-mountainous region who had been a worker in military arsenals complained of intermittent right chest pain and decreased in weight by 8 kg within 4 months. He had a past history of asymptomatic thyroid goitre for more than 5 years and coronary artery disease, twice undergoing percutaneous transluminal coronary angioplasties and aspirin treatment for a number of years. Because his chest pain was suspected to be unstable angina, he was referred to the Taipei Veterans General Hospital for further evaluation. However, his echocardiogram showed neither significant myocardial hypokinesia nor valvular dysfunction, and electrocardiography exam had negative findings.

Upon presentation, the patient appeared chronically ill-looking. The chest radiography revealed an intrathoracic goitre with trachea deviation and several small opacities over the right lower hemithorax (Fig. 1A). The initial computed tomography (CT) scans of the chest revealed several loculated pleural effusion at the right lower hemithorax (Fig. 1C). The amount of effusion was small, so sono-guided aspiration could not be performed safely. After that, he complained of shortness of breath and gradual aggravated chest pain. One month later, rapid progression of massive pleural effusion developed and thoracentesis revealed bloody effusion, and therefore, tubal drainage was applied (Fig. 1B). The patient denied any trauma or medical intervention on the chest wall within 1 month period. The coagulation profiles were all within normal limits; however, the repeated sono-guided aspiration at a different location with cytology analyses only directed the diagnosis towards haemorrhage. Brain magnetic resonance imaging, bronchoscopy, whole abdominal ultrasonography and whole body bone scan all revealed no other abnormalities. Even with the tube functioning properly and with the intact coagulation profile of the patient, the drained effusion was persistently bloody. The secondary CT scans of the chest discovered that the original loculated pleural effusion had progressed to be multiple pleural haematomas on the right lower hemithorax (Fig. 1D). Additionally, progressively enlarged intrapleural haematomas impaired the lung function and induced acute respiratory failure requiring mechanical ventilation support.

Figure 1:

Initial chest radiography and CT section compared with the images taken 1 month later. The chest radiography initially revealed several small opacities over right lower hemithorax (A). Above lesions progressively enlarged with massive bloody effusion s/p pig-tail drainage 1 month later (B). Initial chest CT showed several small loculated pleural effusions at right lower hemithorax (C); follow-up chest CT 1 month later depicted multiple intrapleural haematomas and pleural effusion (D).

After performing fine-needle sono-guided aspiration with undiagnosed pleural haematomas several times, an exploratory thoracotomy was performed, which showed severe adhesion over the right lower pleural cavity. Multiple egg-sized intrapleural haematomas over the surface of the right lung were identified. A partial resection of the pleura and lung tissue showed neoplastic endothelial cell proliferation in pleura and subpleural lung parenchyma with marked necrosis and haemorrhage. Angiosarcoma was diagnosed according to the vasoformative features with immunoreactivity of vascular markers, including CD31 and factor VIII-related antigen, focally positive for cytokeratin and negative for calretinin. Whole body image studies and physical inspection did not find other suspicious primary sites, but ultrasonography of the thyroid goitre showed heterogeneous hypoechoic mass. Another sono-guided aspiration of the thyroid lesion showed bloody fluid and malignant cells which were morphologically consistent with angiosarcoma (Fig. 2). Thereafter, the patient died of multiple organ failure 1 month after definite diagnosis despite aggressive supportive care.

Figure 2:

Pathological findings of surgical biopsy for pleural haematoma-like lesion. The tumour cells are large and epithelioid, with prominent nucleoli (arrow) (A) with anastomosing vascular spaces or papillary tufts formation (B). They are immunoreactive with CD31 (C) and factor VIII-related antigen (D). Another fine-needle aspiration cytology of thyroid tumour also shows morphological consistency with angiosarcoma (E).

DISCUSSION

The differential diagnoses for multiple intrapleural haematomas include tuberculosis, nocardiosis, actinomycosis, traumatic tapping and pleural tumours. The diagnosis of pleural angiosarcoma relies on having adequate surgical specimens and on performing a histopathological examination. Additionally, early exploratory thoracotomy may improve the diagnostic process instead of repeated sono-guided aspiration for unknown pleural haematomas. The vasoformative feature is characteristic of angiosarcoma, and the immunoreactivity with vascular markers, including CD31 and factor VIII-related antigen, can confirm the diagnosis. Morphologically, angiosarcoma may be confused with other pleural tumours, such as metastatic adenocarcinoma or mesothelioma, but these two tumours are negative for vascular markers. Scattering cytokeratin expressed by angiosarcoma, as seen in the present case, is distinguishable from adenocarcinoma and mesothelioma with strongly positive cytokeratin [2].

Angiosarcomas of the thyroid are invariably large, solid and haemorrhagic, and moreover, nearly half of them are encapsulated with obvious calcifications inside. Adequate tissue samples for cytopathology and immunohistochemistry stains are essential for the diagnosis of angiosarcoma, although cytology with vascular markers in fine-needle aspiration tissue has been reported [3]. Regarding the management of advanced-stage angiosarcoma, systemic chemotherapy is considered a treatment option, but no standard regimen been established yet. Wide mutilating surgery is not recommended, because there were more local recurrences after wide excision followed by radiation than after marginal local excision with chemotherapy for cutaneous angiosarcoma. Taxane-based chemotherapy without wide excision is effective on both local control and metastasis prevention [4]. Median survival of thyroid angiosarcoma is 9 months; however, anecdotal case reports revealed that patients survived longer if diagnosed at a stage in which extraglandular tumour spread is absent [5].

Pleural angiosarcoma is rare and may be mistaken as a massive haemothorax. The sequential CT scans depicted the communication of some intrapleural haematomas and pleural space causing massive haemothorax. Early tubal drainage prevented cardiovascular compression complications. However, pleural or subpleural lung parenchymal tumour invasion would induce continuous haemorrhage. The possible pathogenesis for this case is assumed to be thyroid angiosarcoma invading beyond its capsule and downward into the pleural cavity initially; therefore, disseminated angiosarcoma rapidly grew as intrapleural haematoma and induced haemothorax.

In conclusion, the diagnosis of pleural angiosarcoma depends on surgical procedure, and we cannot rely on repeated sono-guided aspiration/biopsy. Pleural angiosarcoma should be considered when unknown multiple intrapleural haematomas appear.

Funding

This work was supported by the National Science Council [100-2314-B-075-047-MY3 to K.-Y.Y., 100-2314-B-010-001-MY2 and 100-2314-B-010-044-MY3 to C.-Y.C.]; Taipei Veterans General Hospital [V100C-159 and V101C-065 to K.-Y.Y.] and National Yang-Ming University Hospital [RD2012-006 to C.-Y.C.].

Conflict of interest: none declared.