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小儿颞骨朗格汉斯细胞组织细胞增多症:影像学表现及随访

Langerhans' cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up.

作者信息

Fernández-Latorre F, Menor-Serrano F, Alonso-Charterina S, Arenas-Jiménez J

机构信息

Department of Radiology, Hospital General Universitario de Alicante, Spain.

出版信息

AJR Am J Roentgenol. 2000 Jan;174(1):217-21. doi: 10.2214/ajr.174.1.1740217.

DOI:10.2214/ajr.174.1.1740217
PMID:10628482
Abstract

OBJECTIVE

The purpose of this study was to review the initial clinical and radiologic manifestations and the follow-up of pediatric patients with Langerhans' cell histiocytosis affecting the temporal bone.

MATERIALS AND METHODS

We retrospectively studied 14 patients with Langerhans' cell histiocytosis affecting the temporal bone. All patients were examined initially and sequentially with CT. In six patients, MR imaging was also done.

RESULTS

Temporal bone involvement was the initial form of presentation in 12 patients. In eight patients, temporal bone involvement presented as an isolated manifestation, and in four it was associated with multisystemic involvement. In the remaining two patients, temporal bone involvement appeared during the course of the Langerhans' cell histiocytosis. Bilateral involvement was seen in four patients. In two patients, the temporal bone was affected only at the petrous apex. CT showed destruction of bone in all 14 patients and an associated soft-tissue homogeneous mass after injection of i.v. contrast material in 12 patients. CT showed a heterogeneous appearance of the soft-tissue mass in two patients. The average period of follow-up was 5 years. In seven of the 14 patients, the disease had a satisfactory evolution in which the bony lesions of the temporal bone reossified and remodeled over the course of a year.

CONCLUSION

In Langerhans' cell histiocytosis, involvement of the temporal bone is usually seen on radiographs as extensive lytic lesions associated with soft-tissue masses. The lesions that remit show early disappearance of the soft-tissue mass, followed by reossification and remodeling of the involved bone. Patients with limited initial involvement of the temporal bone have a better prognosis on long-term follow-up than do patients with the multisystemic form.

摘要

目的

本研究旨在回顾朗格汉斯细胞组织细胞增多症累及颞骨的儿科患者的初始临床和影像学表现及随访情况。

材料与方法

我们回顾性研究了14例朗格汉斯细胞组织细胞增多症累及颞骨的患者。所有患者均首先接受CT检查,并按顺序进行。6例患者还进行了磁共振成像检查。

结果

12例患者颞骨受累为初始表现形式。8例患者颞骨受累表现为孤立性表现,4例与多系统受累相关。其余2例患者颞骨受累出现在朗格汉斯细胞组织细胞增多症病程中。4例患者为双侧受累。2例患者仅岩尖部颞骨受累。CT显示所有14例患者均有骨质破坏,12例患者静脉注射对比剂后可见相关软组织均匀肿块。2例患者CT显示软组织肿块呈不均匀表现。平均随访时间为5年。14例患者中有7例病情进展良好,颞骨骨质病变在1年内重新骨化并重塑。

结论

在朗格汉斯细胞组织细胞增多症中,颞骨受累在X线片上通常表现为广泛的溶骨性病变伴软组织肿块。病情缓解的病变表现为软组织肿块早期消失,随后受累骨质重新骨化并重塑。颞骨初始受累局限的患者长期随访预后优于多系统受累形式的患者。

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