Gujrati M, Thomas C, Zelby A, Jensen E, Lee J M
Department of Pathology, Loyola University Medical Center, Maywood, Illinois, USA.
Surg Neurol. 1998 Aug;50(2):164-8. doi: 10.1016/s0090-3019(98)00039-1.
Bannayan-Zonana syndrome is a rare hamartomatous disorder, characterized by macrocephaly, multiple lipomas, and hemangiomas. Inheritance is by autosomal dominant transmission with few reported sporadic cases. Male predominance is also reported.
We describe a patient who presented with multiple subcutaneous lipomas, mild macrocephaly, and an extradural spinal hemangioma. Other affected family members and 24 other previously reported cases are discussed.
Spinal hemangiomas have not been described previously with this syndrome. The patient also had a "malignant bone tumor" removed from his humerus 20 years ago. Two of the patient's siblings also had lymphoma, which is an unusual accompaniment not reported previously. Only the male members in the family showed multiple subcutaneous lipomas.
Some patients with Bannayan-Zonana syndrome may have hamartomatous lesions producing cord compression or intracerebral hemorrhage, or they may rarely have other malignant tumors; therefore it is important that neurosurgeons are aware of the entity. The early diagnosis of BZS is also important for genetic counseling.
班纳扬 - 佐纳纳综合征是一种罕见的错构瘤性疾病，其特征为巨头畸形、多发性脂肪瘤和血管瘤。遗传方式为常染色体显性遗传，散发病例报道较少。也有男性发病居多的报道。
我们描述了一名出现多发性皮下脂肪瘤、轻度巨头畸形和硬膜外脊柱血管瘤的患者。讨论了其他受影响的家庭成员以及另外24例先前报道的病例。
此前尚未有该综合征合并脊柱血管瘤的描述。该患者20年前还曾从肱骨切除过一个“恶性骨肿瘤”。患者的两个兄弟姐妹也患有淋巴瘤，这是此前未报道过的不寻常伴随情况。家族中只有男性成员出现多发性皮下脂肪瘤。
一些班纳扬 - 佐纳纳综合征患者可能有错构瘤性病变导致脊髓受压或脑出血，或者很少会有其他恶性肿瘤；因此神经外科医生了解这种疾病很重要。班纳扬 - 佐纳纳综合征的早期诊断对遗传咨询也很重要。
