Introduction


Hemangioendothelioma is a tumor that rarely involves the heart. We present the case of a 44-year-old woman who developed cardiac tamponade due to spontaneous bleeding from cardiac hemangioendothelioma. This may be the first case report of such a complication of this rare tumor.


Case presentation


A 44-year-old female patient was admitted to our hospital because of recurrent chest pain. The pain was intermittent and progressive in severity on the right side, associated with palpitation, orthopnea and paroxysmal nocturnal dyspnea. She also had productive cough, with blood-tinged sputum.

The patient gave a history of weight loss (about 8kg in 2 weeks). Six months previously, the patient was admitted because of shortness of breath due to large pericardial effusion and she was treated by echo-guided pericardiocentesis, draining about 800 ml of thin bloody fluid. The patient was discharged in stable condition and remained well until her current admission.

Transthoracic echocardiography revealed a large localized anterior pericardial effusion measuring 3.4 x 8.0cm. CT scan of the chest showed right pericardial mass, measuring 7.0 x 9.0cm, arising from the right atrium with active extravasations of the contrast from the right atrium into the pericardial cavity (Figure 1). Multiple pulmonary nodules were present and were diagnosed as possible small multiple pulmonary emboli. MRI showed pericardial blood-filled collection measuring 5.0 x 10.0 x 6.0cm, communicating with the right atrium through a 2cm defect at the appendage with clot formation in the pseudo-aneurismal mass. The other cardiac chambers were normal; the pulmonary nodules were highly suggestive of multiple pulmonary emboli.

While being worked-up during her current hospitalization, she suddenly developed increased tachypnea and hypotension. Rupture of the pseudo-aneurysm and cardiac tamponade was suspected and an emergency operation was performed. A large pseudo-aneurysm was found in the pericardium attached to the right atrium. The right atrial wall was friable and had to be reconstructed with autogenous pericardium.

Histological examination of the resected tissue revealed a vascular tumor characterized by solid nests and cords of atypical spindled endothelial cells, some of which have distinct intracellular lumina with moderate atypia and mitotic figures (Figure 2a). The mass was a mesenchymal tumor which stained positively for vascular markers CD31, CD34, and factor VIII confirming the diagnosis of hemangioendothelioma (Figure 2b). Bone scan and CT scan of the head, abdomen and pelvic did not show any metastasis. The patient did well and was discharge home 12 days postoperatively.

However, 25 days later she started to complain again of chest pain, productive cough and increasing dyspnea. She was re-admitted, and CT scan of the chest showed a large antero-lateral pericardial mass measuring 8.2 x 3.7 x 6.8 cm, attached to the right atrium with signs of leaking communication between the right atrium and the mass. Transesophageal echocardiogram confirmed the same findings. The patient underwent urgent re-exploration. A similar mass with a false aneurismal cavity formation was found. The mass was more completely excised and the right atrial wall defect was repaired with autogenous pericardium.

The patient was discharged home 4 days after the operation. She received 2 courses of radiation therapy with mild improvement in the size of the residual mass. Three courses of chemotherapy were also given but she did not respond. She deteriorated progressively and eventually expired, eight months after diagnosis.

 

 

Fig.1: CT scan of the chest showing the mass to the right aspect of the right atrium. The red arrow points to the area of possible communication of the false aneurysm with the right atrium.

Discussion


Primary tumors of the heart are rare, representing less than 5% of all cardiac tumors, with an incidence between 0.0017 and 0.19 percent in unselected patients at autopsy1-3. Three quarters of the tumors are benign. Myxomas comprise nearly half of the benign heart tumors and the majority of the rest are lipomas, papillary fibroelastomas, and rhabdomyomas. Whereas rhabdomyomas are the most common primary tumors of the heart in children, myxomas clearly predominate in adults1.

Epithelioid hemangioendothelioma (EHE) is in the middle of the spectrum where epithelioid hemangioma and epithelioid angiosarcoma represent the benign and malignant variants, respectively. EHE was initially considered a low grade vascular neoplasm, but the occurrence of systemic metastases in 21%, and the fact that 17% of 20 patients died of the tumor led to the conclusion that EHE should be regarded as fully malignant, rather than a borderline, vascular neoplasm4.

EHE is a rare vascular tumor, more commonly found in the liver, less commonly in the intestine, and very rarely in the heart. The tumor is histologically characterized by capillary-size vessels lined up by proliferating, often multilayered, epithelial-like endothelial cells with cytoplasmic vacuoles. Clinically, primary cardiac EHE may be found incidentally or after causing congestive heart failure, pericardial effusion, outflow tract obstruction, or thromboembolic event5-7.

EHE is classified as an intermediate malignancy that sometimes metastasizes and may recur5-7. Allaire8 reported that radiation or chemotherapy resulted in palliation, whereas Hongquan9 reported that the prognosis of EHE was relatively good if it was completely resected. Due to the malignant potential of EHE, radical resection is recommended to prevent metastasis2,4-9. However, prognosis after surgical resection is usually good.

MEDLINE search identified only 9 cases of primary cardiac EHE reported in the English literature (Table 1). We did not find any previously published report of a patient with primary cardiac EHE presenting with spontaneous rupture and cardiac tamponade prior to this case report.
 

 

Fig.2a: Microscopic examination shows the atypical endothelial cells. Notice occasional intra-cytoplasmic lumina. (Hematoxylin & Eosin x400).

 

 

Fig.2b: Immunoperoxidase antibody against CD31 antigen (endothelial marker) highlights the tumor cells (Immunohistochemistry x400).

 

Table 1: Reported EHE cases in the English literature:

Conclusion


Primary EHE is a vascular tumor that rarely involves the heart. Only nine such case reports were identified in the English literature. Echocardiography, CT scan and MRI are very helpful in making the diagnosis, but it can only be confirmed histologically. It acts as a low-grade malignant tumor, but the prognosis is usually good after surgical resection.¨



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