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一例罕见的 9 月龄婴儿原发性脊柱孤立性纤维肿瘤/血管外皮细胞瘤。

A Rare Case of a Primary Spinal Solitary Fibrous Tumor/Hemangiopericytoma in a 9-Month-Old Patient.

机构信息

Department of Diagnostic Radiology, University of Puerto Rico School of Medicine, San Juan, Puerto Rico.

Department of Medical Science, University of Puerto Rico School of Medicine, San Juan, Puerto Rico.

出版信息

Am J Case Rep. 2020 Nov 7;21:e923176. doi: 10.12659/AJCR.923176.

Abstract

BACKGROUND Solitary fibrous tumors (SFTs)/hemangiopericytomas (HPCs) are mesenchymal tumors commonly found in middle-aged patients, usually localized to thoracic pleurae. Spinal tumor involvement is rarely seen, and its imaging findings are largely inconsistent because of the rarity of these cases. We present a case report of a 9-month-old girl with a rare intraspinal tumor with histologic evidence of SFT/HPC, but no STAT6 nuclear immunoreactivity. CASE REPORT A 9-month-old girl, born at term with good prenatal care, presented to the emergency room with regression of developmental milestones. The patient was in good health until 2 months, when she developed decreased spontaneous leg movements. Physical exam revealed diffuse muscular atrophy, with no deep tendon reflexes, sensation, or spontaneous movements of the lower extremities. Computed tomography and magnetic resonance imaging showed a heterogeneous irregular mass filling the lumbosacral spinal canal, extending through the neural foramina to the prevertebral/perivertebral and presacral regions. The tumor was biopsied and referred to the National Institutes of Health for consultation and the diagnosis of SFT/HPC was confirmed on the basis of its histologic features, despite the fact that the tumor was negative for STAT6 immunoreactivity. CONCLUSIONS Although the tumor histology was consistent with SFT/HPC, it was negative for STAT6 nuclear immunoreactivity, which is unusual and may exclude the diagnosis. To our knowledge, this is the youngest patient to present with a spinal SFT with these features.

摘要

背景

孤立性纤维瘤(SFT)/血管外皮细胞瘤(HPC)是一种常见于中年患者的间叶性肿瘤,通常局限于胸壁。脊柱肿瘤受累很少见,由于这些病例罕见,其影像学表现也大不相同。我们报告了一例 9 个月大女孩罕见的椎管内肿瘤,其组织学证据为 SFT/HPC,但无 STAT6 核免疫反应性。

病例报告

一名 9 个月大的女孩,足月出生,产前检查良好,因发育里程碑倒退而到急诊室就诊。患者在健康状况良好,直到 2 个月大时,她出现了自发性腿部运动减少。体格检查显示弥漫性肌肉萎缩,下肢无深腱反射、感觉或自发运动。计算机断层扫描和磁共振成像显示腰骶椎管内不均匀不规则肿块,通过神经孔延伸至椎前/椎旁和骶前区域。肿瘤进行了活检,并提交给美国国立卫生研究院进行咨询,根据其组织学特征诊断为 SFT/HPC,尽管肿瘤的 STAT6 免疫反应性为阴性。

结论

尽管肿瘤的组织学表现符合 SFT/HPC,但 STAT6 核免疫反应性为阴性,这很不寻常,可能排除了诊断。据我们所知,这是最年轻的出现这些特征的脊髓 SFT 患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3c7a/7656086/8daf462daa53/amjcaserep-21-e923176-g001.jpg

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