Ettl Tobias, Kleinheinz Johannes, Mehrotra Ravi, Schwarz Stephan, Reichert Torsten E, Driemel Oliver
Department of Oral and Maxillofacial Surgery, Muenster University, Germany.
Head Face Med. 2008 Aug 11;4:18. doi: 10.1186/1746-160X-4-18.
A cutaneous angiosarcoma is a rare malignant tumour of vascular endothelial cells with aggressive clinical behaviour and poor prognosis. Diagnosis is often delayed due to its variable and often benign clinical appearance.
This case presents a 64-year-old man with a six-month-history of a recurrent diffuse and erythematous painless swelling below the left eye. Several resections with intraoperatively negative resection margins followed, but positive margins were repeatedly detected later on permanent sections. Histopathologic examination of the specimen diagnosed a cutaneous angiosarcoma. Neither, finally achieved negative margins on permanent sections, nor a following chemotherapy could prevent the recurrence of the disease after five months and the patient's dead 21 months after the first diagnosis.
The case elucidates the current diagnostic and therapeutic dilemma of this entity, which shows an unfavourable clinical course in spite of multimodal therapy.
皮肤血管肉瘤是一种罕见的血管内皮细胞恶性肿瘤，具有侵袭性的临床行为和较差的预后。由于其临床表现多变且常呈良性，诊断往往延迟。
本病例为一名64岁男性，左眼下方反复出现弥漫性、无痛性红斑肿胀6个月。随后进行了多次手术切除，术中切缘阴性，但在永久切片上后来多次检测到切缘阳性。对标本进行组织病理学检查诊断为皮肤血管肉瘤。最终，永久切片既未达到切缘阴性，后续化疗也未能防止疾病在5个月后复发，患者在首次诊断后21个月死亡。
该病例阐明了这种疾病目前的诊断和治疗困境，尽管采用了多模式治疗，其临床病程仍不容乐观。
