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神经母细胞瘤颅骨转移的非典型影像学特征为多发多房囊性肿块,内见液-血平面:病例报告。

An atypical imaging characteristic of calvarial metastasis of neuroblastoma as multiple multi-loculated cystic masses with internal blood-fluid levels: a case report.

机构信息

Radiology Department, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.

Department of Neurosurgery, AJA University of Medical Sciences, Tehran, Iran.

出版信息

BMC Neurol. 2022 Dec 9;22(1):471. doi: 10.1186/s12883-022-03001-9.

Abstract

BACKGROUND

As the third most common malignancy of childhood, Neuroblastoma has a great propensity to metastasize to multiple organs. The most common site of metastasis is the bone and bone marrow. Concerning the central nervous system, neuroblastoma usually involves the calvarium and the external dural surface. The skull metastases may show different appearances, including: multiple lytic bone lesions, bone thickening, hair-on-end periosteal reaction, irregular suture widening and/or plaque like epidural deposits. Here we present a case of metastatic neuroblastoma, appearing as multiple multi-loculated cystic epidural masses with internal blood-fluid levels as a rare imaging manifestation of calvarial metastasis.

CASE PRESENTATION

An 8-year-old boy with known history of autism, presented to the emergency department with a 3-month history of intermittent fever, malaise and myalgia and headache along with significant weight loss. Laboratory examination revealed elevated ESR and CRP and anemia. On Abdomino-Pelvic imaging a well-defined, 453024 mm, solid-cystic mass was observed, replacing the normal left adrenal gland. On brain MRI, multiple multi-loculated cystic, lentiform masses were observed on the external surface of cerebral hemispheric dura. Multiple fluid-fluid levels were noted in the locules in some of which the dependent fluid was hyperintense on T1w and FLAIR and hypointense on T2w sequences, compatible with blood, representing blood-fluid level. The wall and septa of the masses, enhanced after contrast administration. Associated abnormal marrow signal and aggressive type periosteal reactions were identified in the overlying bone. All of the lesions had increased uptake in MIBG scan. Bone marrow biopsy revealed small round cells, diagnostic for neuroblastoma. The patient underwent chemotherapy treatment. All calvarial/epidural metastatic lesions resolved after chemotherapy and residual adrenal tumor was resected.

CONCLUSION

Cystic epidural lesions, especially when associated with adjacent abnormal bone marrow signal, or periosteal reaction and containing blood-fluid level should raise the suspicion of a calvarial metastasis.

摘要

背景

神经母细胞瘤是儿童期第三大常见恶性肿瘤,具有广泛转移至多个器官的倾向。最常见的转移部位是骨骼和骨髓。就中枢神经系统而言,神经母细胞瘤通常累及颅骨和硬脑膜外表面。颅骨转移瘤可表现出不同的表现,包括:多发性溶骨性骨病变、骨增厚、发状骨膜反应、不规则缝线增宽和/或硬膜外斑块样沉积物。在此,我们报告了一例转移性神经母细胞瘤病例,表现为多个多房囊性硬膜外肿块,内部存在液-血平面,这是颅骨转移的罕见影像学表现。

病例介绍

一名 8 岁男孩患有自闭症病史,因间歇性发热、不适和肌痛以及头痛和明显体重减轻而到急诊科就诊。实验室检查显示 ESR 和 CRP 升高以及贫血。腹部-骨盆影像学检查发现一个边界清楚的 453024mm 的实性-囊性肿块,取代了正常的左肾上腺。脑 MRI 显示大脑半球硬脑膜外表面有多个多房性、半月形肿块。在一些腔室中观察到多个液-液平面,其中依赖液体在 T1w 和 FLAIR 上呈高信号,在 T2w 上呈低信号,与血液一致,代表液-血平面。肿块的壁和间隔在给予造影剂后增强。在上方骨骼中发现了相关的异常骨髓信号和侵袭性骨膜反应。所有病变在 MIBG 扫描中均有摄取增加。骨髓活检显示小圆细胞,诊断为神经母细胞瘤。患者接受了化疗治疗。化疗后所有颅骨/硬膜外转移病灶均消退,残留肾上腺肿瘤被切除。

结论

囊性硬膜外病变,特别是当伴有相邻异常骨髓信号、骨膜反应和包含液-血平面时,应引起对颅骨转移的怀疑。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/757a/9733139/aaaae778824d/12883_2022_3001_Fig1_HTML.jpg

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