A rare diagnostic challenge in a female patient with a rapid recurrent pleural effusion: Autopsy revealed cardiac angiosarcoma with bilateral pleural and pulmonary metastases. A case report

Abstract

Introduction: We present a very rare case with diffuse cardiac angiosarcoma. Because all symptoms are often non-specific, this diagnosis is difficult to establish. To our knowledge this is the first clinical description of this rare disease.

Presentation of case: A 47-year-old female presented with bilateral pulmonary infiltrates and non-specific symptoms as fever, chest pain and dyspnoea on exertion. She was treated with antibiotics for suspected lung infection but deteriorated developing rapid recurrent pleural effusion. Her transthoracic- and transoesophageal-echocardiography as well as the thoracentesis and endobronchial ultrasound findings were normal. A minimally invasive pulmonary wedge resection, partial pleurectomy and pericardial fenestration was performed. The pathologic interpretation of these specimen was very difficult and a correct diagnosis could be made only by the second reference pathologist. While awaiting reference histology report she was administered high-flow oxygen therapy for hypoxia, antibiotics, catecholamines and corticosteroids. The patient deteriorated very rapidly and died in the ICU.

Discussion: As in earlier studies, misdiagnosis delayed the actual diagnosis, especially because there was no clinical suspicion for angiosarcoma. Pathologic evaluation may be difficult because different growth patterns may be present in the same tumour and pleural or lung specimen may show only very tiny tumour formations within a fibrosing tissue changes.

Conclusion: This case report highlights the difficulties to establish a diagnosis of diffuse angiosarcoma in time. An early diagnosis, to initiate oncologic treatment, require a high level of clinical suspicion and a histological proof from pericardial or myocardial biopsy.

Keywords: Autopsy; Cardiac angiosarcoma; Haemoptysis; Pulmonary infiltrates; Transoesophageal echocardiography.

Fig. 1

Chest CT scans. Chest CT scan with contrast medium (a,b) and follow-up native CT-scan showing a certain progression of pleural effusion and pulmonary infiltrates, non-specific for lung metastases. 1a & b: chest CT scan 11/04/2019 with small infiltrates, atelectasis and a subpleural nodule. Mild cardiomegaly. 1c & d: chest CT scan 15/05/2019 with bilateral pleural effusion and blurry infiltrates on the right side.

Fig. 2

Chest X-rays. Chest X-rays with mild cardiomegaly at initial presentation and massive progression of bilateral pleural effusion during second admission. 2a: Chest X-ray at the time of first admission. 2b: Chest X-ray, 5 weeks thereafter.

Fig. 3

Histology from autopsy specimen. Histology from lung wedge resection specimen. a H&E-stained alveolar lung tissue, 100×. Visceral pleura covered with a vascular tumor and a fibrin coat on the right side. b H&E stained 400× pleural specimen with intratumor vessels. c Positive stain with the panvascular marker CD31, 400×. d Immunohistochemistry stained with MIB-1 showing 60% proliferative cells with brown nuclear staining, 200×. e Calretinine stained lung tissue negative inside the tumor but one positive mesothelial layer, 400×. f CD34 stained tumor: negative within the tumor vessels but positive within the internal control of non-neoplastic vessels, 200×. g H&E-stained heart muscle with diffuse angiosarcoma infiltration of the myocardiumh H&E-stained specimen showing pulmonary vein infiltration, 25×.