Steinbichler Teresa B, Kral Florian, Reinold Susanne, Riechelmann Herbert
Department of Otorhinolaryngology, Medical University of Innsbruck, Anichstrasse 35, A- 6020 Innsbruck, Austria.
World J Surg Oncol. 2014 Dec 17;12:387. doi: 10.1186/1477-7819-12-387.
Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases.
We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary.
Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless.
马富西综合征是一种罕见的先天性非遗传性中胚层发育异常，表现为多发性内生软骨瘤和血管瘤。这些病变的恶性转化在高达40%的病例中可见。
我们报告一例患有马富西综合征并伴有鼻部软骨肉瘤的患者。治疗方法为手术切除。由于肿瘤分级较低，无需进行放疗等额外治疗。
马富西综合征是一种极其罕见的中胚层发育异常。其在头颈部区域的表现更为少见。相关内生软骨瘤的恶性转化很常见，每当临床病程发生变化时都应予以考虑。随机定期进行的X线检查对恶性转化提供的额外信息很少，被认为是无用的。
