Angiosarcoma of the pericardium is a rare malignancy, often difficult to diagnose. Currently, fewer than 30 case reports exist in the literature. In many of these cases, the chief complaint was dyspnea resulting from cardiac tamponade. Nearly all patients had at least a mild pericardial effusion, though most were large and hemorrhagic. A case report by Park et al. describes a young adult man who presented with hemorrhagic pericardial effusion of unknown etiology
]. Workup including cytology, CT, and echocardiography failed to identify a malignancy. The effusion recurred 3 months later, at which time CT scan demonstrated an unresectable pericardial angiosarcoma. This highlights the difficulty in early diagnosis of this malignancy and the limitations of imaging modalities during early stage disease. Holtan et al. described a similar case of recurrent pericardial effusions; however, diagnosis of primary pericardial angiosarcoma was made by open surgical exploration and biopsy [3
In young patients with recurrent hemorrhagic pericardial effusions, suspicion of malignancy should be high and the patient should undergo close monitoring. While chest CT scan appropriately identify many types of pericardial pathology, CT is not effective at consistently detecting pericardial malignancy [4
]. Primary pericardial tumors often appear as thickened pericardium with diffuse enhancement, and thus appear similar to other types of constrictive pericarditis. CT may be more accurate in the later stages as the cancer becomes bulky, as seen by Park et al. While studies examining the efficacy of magnetic resonance imaging (MRI) in detecting pericardial malignancy have not been performed, it is an imaging modality that may hold promise. Despite advances in medical technology, the most accurate way to diagnose pericardial angiosarcoma remains surgical pericardiectomy
with wide margins (both diagnostic and therapeutic).
Early diagnosis may provide the best chance for survival since it allows time to treat with chemotherapy
. There are few case reports demonstrating the efficacy of chemotherapy for primary pericardial angiosarcoma. In a case similar to ours, a 31 year-old man was misdiagnosed with viral pericarditis and was managed with anti-inflammatory medications and pericardiocentesis [5
]. A pericardial mass invading the right atrium was later detected on MRI and surgical biopsy
confirmed pericardial angiosarcoma. Transplantation was considered, but the patient was deemed to be a poor surgical candidate and chemotherapy with vincristine, ifosfamide, doxorubicin, and etoposide was initiated. Failure with this regimen prompted the initiation of paclitaxel
, which reduced the tumor size for only a few months. Pazopanib chemotherapy was then initiated and the patient improved clinically and was alive at 10-month follow up when the case was reported. Other studies have shown either clinical improvement or stabilization of the disease process with chemotherapy; others have demonstrated that patients treated with multimodal therapy (combination of resection, chemotherapy, and radiation) had longer survival than those treated with chemotherapy or resection alone [6
]. A 32 year-old woman who underwent surgical resection and combination therapy was disease free at her 13th postoperative month, suggesting that advancements in multimodal therapy may prolong survival for patients and improve their overall quality of life [8
In conclusion, clinicians should have a high index of suspicion for pericardial malignancy in young patients with unexplained recurrent pericardial effusions
. Historically, these patients benefit from surgical exploration for definitive diagnosis and staging. Echocardiography
and CT imaging have limited utility in the diagnosis of pericardial malignancy during the earliest stages of disease. Chemotherapy can be considered to extend survival or for palliation
. Although early diagnosis may result in earlier surgical resection and chemotherapy administration, the prognosis
remains dismal with few patients surviving past a few years.