Donovan S T, Thompson J W, Sandlund J T, Adderson E E, Pivnick E K, Harreld J H
Department of Otolaryngology, University of Florida College of Medicine, 1600 SW Archer Road, Gainesville, FL 32608, USA.
Case Rep Otolaryngol. 2013;2013:272314. doi: 10.1155/2013/272314. Epub 2013 Jun 18.
Gorlin Syndrome (GS), also known as nevoid basal cell carcinoma syndrome, is a rare autosomal dominant condition characterized by developmental abnormalities and predisposition to certain neoplasms. Acute invasive fungal rhinosinusitis (AIFRS) is an uncommon clinical entity characterized by high morbidity and mortality. In immunocompromised patients, computed tomography plays a critical role in screening for suspected AIFRS. However, due to the association between exposure to ionizing radiation and subsequent development of malignancies in patients with GS, patients with GS and suspected AIFRS present a unique and challenging clinical scenario. We present a case of a pediatric patient with GS and acute lymphocytic leukemia (ALL) diagnosed with AIFRS; to the best of our knowledge, it is the only case described in the literature.
戈林综合征(GS),又称痣样基底细胞癌综合征,是一种罕见的常染色体显性疾病,其特征为发育异常和易患某些肿瘤。急性侵袭性真菌性鼻-鼻窦炎(AIFRS)是一种临床少见的疾病,具有高发病率和高死亡率。在免疫功能低下的患者中,计算机断层扫描在筛查疑似AIFRS中起着关键作用。然而,由于GS患者暴露于电离辐射与随后发生恶性肿瘤之间存在关联,GS合并疑似AIFRS的患者呈现出独特且具有挑战性的临床情况。我们报告一例患有GS和急性淋巴细胞白血病(ALL)的儿科患者被诊断为AIFRS;据我们所知,这是文献中描述的唯一病例。