3. Discussion
In this case, the patient did not present with symptoms at the first
visit. The symptoms of PCA are usually nonspecific and include chest
pain, shortness of breath, malaise, and fever1.
Pericardial effusion is also common in patients with PCA; however, in
general, the detection of angiosarcoma cells is difficult even with a
cytological evaluation of the pericardial fluid7, as
seen in our patient. Hence, when one encounters a patient with
unexplained pericardial effusion, the possibility of malignancies
including PCA should not be excluded, even with a “negative” result of
the pericardial effusion cytology.
Given the low frequency of PCA and a relatively higher incidence of
metastatic cardiac tumor, diagnosis of cardiac angiosarcoma as the
primary lesion is sometimes challenging. In the current case, we
encountered this difficulty as the patient had multiple tumor nodules in
the liver, spleen, and skin. Besides the heart, angiosarcomas can arise
in the soft tissue, breast, liver, bone, spleen, or other visceral
organs8. Furthermore, the incidence of angiosarcoma in
the cutaneous soft tissue, liver, or spleen is higher than that in the
heart8. In this regard, Cunha-Silva et. al.suggested the following factors to evaluate whether the cardiac
angiosarcoma is primary or
not9: (1)
patient’s age and lifestyle history, (2) number of lesions (i.e., single
or multiple) in the heart, and (3) location of the lesion in the heart.
In addition, while cardiac metastasis of liver angiosarcoma is rare, the
liver is a common metastatic site of PCA9. In the
current case, the patient had no history of exposure to thorium oxide,
vinyl chloride, arsenic, or anabolic steroids, all of which are risk
factors for hepatic angiosarcoma. Moreover, the cardiac lesion was
single, and the hepatic lesions were multiple. The cardiac lesion
located in the right atrium, the most common site for PCA. These
findings strongly suggest that the cardiac angiosarcoma in this case was
PCA.
Pericardial extension of the tumor was observed in 71% of 17 cases of
PCA7, and cardiac rupture due to PCA has also been
reported10. However, to the best of our knowledge, a
case of PCA with direct lung invasion has not been described previously.
In the current case, the tumor penetrated the pericardium and invaded
the lung. However, neither fatal cardiac tamponade nor rupture occurred
in this case. Although the incidence of lung invasion may be low, it may
be meaningful for the clinical management of PCA to recognize the
possibility of lung invasion.
In conclusion, we presented an autopsy case of PCA that directly invaded
the right lung. Although PCA is an extremely rare disease, it should be
included in the differential diagnosis of a cardiac mass with
pericardial effusion.