Mesenchymal tumors of the mediastinum account for 6% of all mediastinal tumors. Lesions of vascular origin may arise anywhere in the mediastinum and are extremely rare. Angiosarcomas may exhibit a variety of histologic appearances including carcinoma, other sarcomas, melanoma, and even benign reactive processes. We describe a case.
A 36-year-old man with medical history of peptic ulcer disease presented to the emergency department with chest pain and dyspnea. Four months before he had developed postprandial epigastric pain, with no adrenergic symptoms associated. The pain was not associated with breathing or physical activity but worsened on prone or right lateral decubitus position. He denied weight loss or fever, and was in general good condition. On admission a chest CT scan was done, revealing a large mass located at the anterior mediastinum and pericardial effusion. Alpha-fetoprotein and beta fraction of chorionic gonadotrophin were normal. Testicular ultrasound was normal and staging studies revealed no evidence of metastases. The tumor was biopsied via a median sternotomy, but it could not be resected because it involved pericardium and great vessels. Transoperatory biopsy reported a myxoid sarcoma. Definitive histopathological report (M.D. Anderson Cancer Center) reported a high-grade angiosarcoma. The patient received neoadjuvant chemotherapy with four courses of etoposide and carboplatinum, achieving a reduction of 80% of the tumoral size after the third chemotherapy course.DISCUSSION: Angiosarcomas may arise anywhere in the mediastinum and are extremely rare. They usually have direct extension of tumor into adjacent structures, and local recurrence is common. Complete resection may be difficult but is usually curative. In this case, direct infiltration of mediastinal vascular structures and pericardium prevented resection.
Clinicians should be aware and include in the differential diagnosis of a mediastinal mass those tumors of vascular origin, even if they are rare. Given the unusual presentation of mediastinal angiosarcomas this case represented a clinical and therapeutic challenge. Four sessions of chemotherapy with etoposide and carboplatinum were given with good clinical response.
E. Fernandez-Rangel, None.