Radically resected epithelioid angiosarcoma that originated in the mediastinum.
Summary of "Radically resected epithelioid angiosarcoma that originated in the mediastinum."
Angiosarcoma is a rare neoplasm, accounting for only 1%-2% of all sarcomas. It occurs most frequently in the skin and soft tissue and rarely in the thoracic region. To our knowledge, a mediastinal angiosarcoma is extremely rare. We report on the surgical resection of a rare case of giant epithelioid angiosarcoma originating in the anterior mediastinum, followed by six courses of adjuvant chemotherapy (doxorubicin + ifosfamide). The patient is alive and asymptomatic 1 year after surgery. As the prognosis for unresectable cases is generally dismal, surgical resection and adjuvant therapy can be an option for mediastinal angiosarcoma.
Department of Thoracic Surgery, Hyogo Cancer Center, 13-70 Kitaouji-cho, Akashi, 673-8558, Japan.
This article was published in the following journal.
Name: General thoracic and cardiovascular surgery
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21751114
- DOI: http://dx.doi.org/10.1007/s11748-010-0710-z
Medical and Biotech [MESH] Definitions
Characteristic cells of granulomatous hypersensitivity. They appear as large, flattened cells with increased endoplasmic reticulum. They are believed to be activated macrophages that have differentiated as a result of prolonged antigenic stimulation. Further differentiation or fusion of epithelioid cells is thought to produce multinucleated giant cells (GIANT CELLS).
Perivascular Epithelioid Cell Neoplasms
A family of mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. These cells do not have a normal anatomic homolog. (From Fletcher CDM, et. al., World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone, 2002).
A tumor of medium-to-large veins, composed of plump-to-spindled endothelial cells that bulge into vascular spaces in a tombstone-like fashion. These tumors are thought to have "borderline" aggression, where one-third develop local recurrences, but only rarely metastasize. It is unclear whether the epithelioid hemangioendothelioma is truly neoplastic or an exuberant tissue reaction, nor is it clear if this is equivalent to Kimura's disease (see ANGIOLYMPHOID HYPERPLASIA WITH EOSINOPHILIA). (Segen, Dictionary of Modern Medicine, 1992)
Nevus, Epithelioid And Spindle Cell
A benign compound nevus occurring most often in children before puberty, composed of spindle and epithelioid cells located mainly in the dermis, sometimes in association with large atypical cells and multinucleate cells, and having a close histopathological resemblance to malignant melanoma. The tumor presents as a smooth to slightly scaly, round to oval, raised, firm papule or nodule, ranging in color from pink-tan to purplish red, often with surface telangiectasia. (Dorland, 27th ed)
Removal of only enough breast tissue to ensure that the margins of the resected surgical specimen are free of tumor.
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