Cardiac epithelioid angiosarcoma presenting as cutaneous metastases.
BACKGROUND
Cardiac angiosarcoma is a rare tumor that has a predilection for middle-aged males and a marked predominance in the right atrium. The tumor may present abruptly with a fulminant clinical course. Initial presentation with metastatic disease is rare. Only one case has been reported of a patient who presented with cutaneous metastases.


RESULTS
We here report the case of a 51-year-old man who initially presented with cutaneous metastases in the absence of cardiac symptoms. The skin biopsy was diagnosed as metastatic undifferentiated sarcoma. The patient died 26 days later with widely disseminated disease. At autopsy a tumor arising in the wall of the left atrium and in the interatrial septum was found. After an immunohistochemical study including CD31 and CD34 stains the diagnosis of cardiac pure epithelioid angiosarcoma was made. To the best of our knowledge this tumor variant has not been documented in the heart until now.


CONCLUSIONS
Although cardiac angiosarcoma is a rare neoplasm, its presence should be suspected in patients with cutaneous metastatic angiosarcoma without an evident source of the tumor, even in absence of cardiac symptoms.
Val-Bernal
J F
JF
Department of Anatomical Pathology, and Service of Oncology, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain. apavbj@humv.es
Figols
J
J
Arce
F P
FP
Sanz-Ortiz
J
J
D018952:Antigens, CD34 / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D003937:Diagnosis, Differential; D015622:Epithelioid Cells / Q000473:pathology*; D017809:Fatal Outcome; D006325:Heart Atria / Q000473:pathology; D006338:Heart Neoplasms / Q000737:chemistry / Q000175:diagnosis*; D006394:Hemangiosarcoma / Q000737:chemistry / Q000175:diagnosis* / Q000556:secondary; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009363:Neoplasm Proteins / Q000032:analysis; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000032:analysis; D012878:Skin Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000556:secondary
D018952:Antigens, CD34; D014408:Biomarkers, Tumor; D009363:Neoplasm Proteins; D019408:Platelet Endothelial Cell Adhesion Molecule-1