| • | AKA malignant hemangioendothelioma |
| • | a malignant vascular tumor, arising from both vascular and lymphatic endothelium |
| • | a rare tumor in any form; usually lethal |
clinical:
| • | invariably a livid or dusky red color |
| • | on the scalp of elderly patients, has a more edematous, erysipeloid, or frankly tumid appearance |
two classic clinical situations:
| • | angiosarcoma of the elderly: typically scalp or face |
| • | angiosarcoma develops in a chronic lymphedematous area (the so-called "Stewart-Treves syndrome" - see below) |
immunohistochemistry:
| • | antibodies to CD31 and Ulex are the most reliable markers |
| • | ddx histologically = Masson's tumor |
Lymphangiosarcoma of Stewart and Treves
| • | an angiosarcoma arising in an area of chronic lymphedema, usually in an arm following radical mastectomy |
clinical:
| • | the first sign may be an area of bruising in the lymphedema, often thought by the patient to be traumatic |
| • | dusky blue or red nodules develop and grow rapidly |
ddx:
| • | although similar in many ways to KS, the sex ratio is reversed, with more females affected (microscopically, the proliferative change is in, rather than around, the endothelial cells) |
| • | melanoma, pyogenic granuloma, and metastatic carcinoma may have to be ruled out at early stages |
| ◆ | the tumor does not arise in the lymphedema of filariasis |
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