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系统性硬化症继发腰椎肿瘤性钙质沉着症:成人晚期疾病神经根病的罕见病因。

Tumoral calcinosis in the lumbar spine secondary to systemic sclerosis: a rare cause of radiculopathy in an adult with advanced disease.

作者信息

Liberato Afonso Celso Pedrotti, Amaral Lazaro Luiz Faria Do, Marussi Victor Hugo Rocha

机构信息

Neuroradiology, Beneficencia Portuguesa Hospital, Med Imagem, São Paulo, Brazil.

出版信息

BJR Case Rep. 2016 Jul 28;2(3):20150435. doi: 10.1259/bjrcr.20150435. eCollection 2016.

Abstract

Calcinosis is frequently associated with systemic sclerosis (SSc) and can be located at various sites, although it is most commonly seen in the hands. When it presents around the synovial joints and is associated with a mass-like appearance, it is classically called tumoral calcinosis. Few cases of tumoral calcinosis have been reported in the paraspinal region. They are usually located in the cervical segment and rarely in the lumbar region. Occasionally, they have been associated with nerve root compression and intraspinal extension. We report the case of a 47-year-old female with advanced SSc who presented to our hospital's radiology department with chronic low back pain and right L5 radiculopathy due to tumoral calcinosis. An initial lumbar spine MRI showed multifocal, low signal, mass-like lesions involving the right paraspinal soft tissues. At the L5-S1 level, one lesion compressed the right L5 exiting nerve root. A CT scan of the lumbar spine performed later demonstrated the calcified nature of the lesions depicted by MRI and evidenced signs of pulmonary fibrosis at the base of the lungs. Further clinical work-up also showed that the patient had Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, dyspnoea, facial telangiectasias, generalized weakness and arthralgia. The diagnosis of a subtype of SSc, called limited cutaneous SSc, was made. Our case describes the CT and MRI findings of tumoral calcinosis in an unusual location secondary to limited cutaneous SSc. Knowledge of the imaging features of this uncommon manifestation of SSc could potentially increase its prospective diagnosis and hence improve patient management.

摘要

钙质沉着症常与系统性硬化症(SSc)相关,可位于身体的不同部位,不过最常见于手部。当它出现在滑膜关节周围并伴有肿块样外观时,经典地被称为肿瘤性钙质沉着症。脊柱旁区域报道的肿瘤性钙质沉着症病例很少。它们通常位于颈椎节段,很少见于腰椎区域。偶尔,它们会伴有神经根受压和椎管内延伸。我们报告了一例47岁的晚期SSc女性患者,因肿瘤性钙质沉着症导致慢性腰痛和右侧L5神经根病而到我院放射科就诊。最初的腰椎MRI显示多灶性、低信号、肿块样病变累及右侧脊柱旁软组织。在L5 - S1水平,一个病变压迫了右侧L5出神经根。随后进行的腰椎CT扫描显示了MRI所显示病变的钙化性质,并在肺底部发现了肺纤维化的迹象。进一步的临床检查还显示患者有雷诺现象、食管运动障碍、指端硬化、呼吸困难、面部毛细血管扩张、全身无力和关节痛。诊断为SSc的一种亚型,称为局限性皮肤型SSc。我们的病例描述了局限性皮肤型SSc继发于不寻常部位的肿瘤性钙质沉着症的CT和MRI表现。了解SSc这种不常见表现的影像学特征可能会提高其前瞻性诊断,从而改善患者的管理。

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