Roth P, Grätz K, Sailer H
Clinique et Policlinique de Chirurgie Maxillo-Faciale, Université de Zurich.
Swiss Dent. 1991;12(5):37-8, 40-1, 43 passim.
We present here a case of a patient with Gorlin-Goltz Syndrome treated and followed up for more than 22 years. During the evolution of the disease, we have noted a severe development of symptoms that necessitated the resection of multiple face and trunk basalioms, as well as numerous maxillary keratocysts. One of the cysts has presented the histologic characteristics of an ameloblastic fibroma. Later on, the growth of a squamous cell carcinoma required a partial mandibular resection, primary reconstruction (with AO-plate) and postoperative radiotherapy. Afterwards, a relapse in the symphysar region necessitated, in spite of the presence of lung metastases, another resection of the same region including part of the lower lip with secondary reconstruction by the means of a myocutaneous flap (PMM). A review of the main features of this syndrome has been carried out. With the help of a literature review, all the similar cases already published are here presented and discussed. Furthermore, our therapeutic concept concerning this syndrome is briefly discussed.
我们在此呈现一例患有戈林-戈尔茨综合征的患者，其接受治疗及随访已超过22年。在疾病进展过程中，我们注意到症状严重发展，需要切除多个面部和躯干基底细胞癌，以及大量上颌角化囊肿。其中一个囊肿具有成釉细胞纤维瘤的组织学特征。后来，鳞状细胞癌的生长需要进行部分下颌骨切除、一期重建（使用AO钢板）及术后放疗。之后，尽管存在肺转移，耻骨联合区域的复发仍需要再次切除同一区域，包括下唇的一部分，并通过肌皮瓣（胸大肌肌皮瓣）进行二期重建。我们对该综合征的主要特征进行了综述。借助文献回顾，在此呈现并讨论了所有已发表的类似病例。此外，我们还简要讨论了针对该综合征的治疗理念。
