de Araújo Pedro Ivo Machado Pires, Soares Vítor Yamashiro Rocha, Queiroz André Luís, dos Santos Aline Marques, Nascimento Luiz Augusto
Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital of Brasília, Ambulatório II, SGAN, Via L2 Norte, Quadra 604/605, Asa Norte, CEP 70840-050, Brasilia, Federal District, Brazil.
Oral Maxillofac Surg. 2012 Jun;16(2):217-20. doi: 10.1007/s10006-011-0286-5. Epub 2011 Sep 3.
McCune-Albright syndrome is a relatively rare disease characterized by the trio of fibrous dysplasia, café-au-lait pigmentation, and endocrine disturbances. It predominantly affects females and may be associated with sarcomatous degeneration in 0.4% to 4% of the cases.
This article reports on the case of a 24-year-old female patient who had presented a mass in the oral cavity for 30 days, probably originating from the left ramus of the mandible. She had a previous diagnosis of polyostotic fibrous dysplasia, along with café-au-lait spots and endocrine disorders, thus characterizing McCune-Albright syndrome. Histopathological examination of a biopsy specimen revealed osteosarcoma of the mandible. The patient underwent chemotherapy and a surgical procedure for lesion resection. One year has now passed since the patient's operation, and the disease is under control.
The patient presented sarcomatous degeneration in areas of fibrous dysplasia, consisting of mesenchymal cells that produced osteoid. Malignant degeneration is rare when it is not associated with McCune-Albright syndrome. There is no curative treatment for the syndrome. Attending physicians need to bring endocrine disorders under control, with surgical treatment in cases of significant deformities, as well as providing clinical and psychological care.
McCune-Albright综合征是一种相对罕见的疾病，其特征为纤维性发育异常、咖啡牛奶斑色素沉着和内分泌紊乱三联征。该病主要影响女性，在0.4%至4%的病例中可能与肉瘤样变性有关。
本文报道了一名24岁女性患者的病例，该患者口腔内出现肿物30天，可能起源于下颌骨左支。她之前被诊断为多骨型纤维性发育异常，伴有咖啡牛奶斑和内分泌紊乱，从而确诊为McCune-Albright综合征。活检标本的组织病理学检查显示为下颌骨肉瘤。患者接受了化疗和病变切除手术。自患者手术至今已过去一年，病情得到控制。
患者在纤维性发育异常区域出现肉瘤样变性，由产生类骨质的间充质细胞组成。当不伴有McCune-Albright综合征时，恶性变性很少见。该综合征没有治愈性治疗方法。主治医生需要控制内分泌紊乱，对于严重畸形的病例进行手术治疗，并提供临床和心理护理。
