散发性与VHL相关性颅内血管母细胞瘤的MRI与病理相关性研究

MR features in sporadic and VHL -associated intracranial hemangioblastomas： correlation with pathologic findings

目的 探讨散发性与Von Hippel-Lindau（VHL）病相关性颅内血管母细胞瘤的MRI表现类型及其病理基础.方法 2002年至2009年经手术病理证实的颅内血管母细胞瘤患者29例,将肿瘤MRI影像表现特点与手术病理结果做对照分析.结果 29例颅内血管母细胞瘤患者,散发性17例（59%）,VHL病12例（41%）,共计66个血管母细胞瘤,MRI表现为实性小结节型（28个）、实性肿块型（18个）及囊结节型（20个）,病理光镜下表现为网状型、细胞型及混合型.结论 血管母细胞瘤的MRI表现类型与其病理基础密切相关.对VHL病患者颅内无症状的实性小结节型血管母细胞瘤应长期追踪观察.

Objective To evaluate the MR imaging features of intracranial hemangioblastomas accompanying sporadic and yon Hippel - Lindau （VHL） disease with special attention to tumor types,correlation with surgical and pathologic findings,and any differences between patients with sporadic and VHL disease.Method The surgical pathology database of our institution was searched to identify all patients with histologically verified intracranial hemangioblastomas treated from 2002 to 2009.The medical,MR imaging,surgical,and pathological records of these patients were reviewed retrospectively and statistically analyzed.Serial MRI was obtained in von Hippel-Lindau disease patients.Results Twenty-nine patients （14 males and 15 females） presented with intracranial hemangioblastomas.Seventeen patients （59%）harbored sporadic hemangioblastomas.Twelve patients （41%） had VHL syndrome.These 29 patients presented with 66 hemangioblastomas（11 patients had multiple lesions）.Patients with VHL disease tended to present with neurological symptoms and signs from younger age than patients with sporadic disease （P=0.000）.The MRI findings of hemangioblastomas were classified as three types including small（10 mm or less） solid tumor,larger solid tumor,and cystic tumor with a mural nodule.Twenty-eight small solid hemangioblastomas occurred in 11 VHL disease patients,larger solid hemangioblastomas mostly in VHL disease patients,and cystic tumor with a mural nodule mostly in sporadic patients.Larger hemangioblastomas were mostly accompanied by vascular flow voids.Histologically,larger solid hemangioblastomas showed a large number of capillaries and a moderate number of stromal cells;the reverse was true in cystic-nodule tumors where capillaries were seen in moderate numbers and stromal cells were in abundance.Serial MRI in 7 VHL disease patients clearly showed 10 small solid hemangioblastomas that developed and evolved into 7 larger solid tumors and 3 cystic-nodule tumors.Conclusions The MRI features of intracranial hemangioblastomas were correlated with pathologic findings.In patients with VHL disease,MR imaging allows identification of lesions before they become symptomatic.Because patients with VHL disease are at risk of development of new lesions,they require life-long follow-up.