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岩尖单灶性朗格汉斯细胞组织细胞增多症(嗜酸性肉芽肿)。

Unifocal Langerhans' cell histiocytosis (eosinophilic granuloma) of the petrous apex.

作者信息

Goldsmith A J, Myssiorek D, Valderrama E, Patel M

机构信息

Department of Otolaryngology and Communictive Disorders, Long Island Jewish Medical Center, New Hyde Park, NY 11042.

出版信息

Arch Otolaryngol Head Neck Surg. 1993 Jan;119(1):113-6. doi: 10.1001/archotol.1993.01880130115018.

DOI:10.1001/archotol.1993.01880130115018
PMID:8417735
Abstract

Eosinophilic granuloma is a localized form of histiocytosis X, or Langerhans' cell histiocytosis, a benign lesion of proliferating Langerhans' cells. It is the least severe of the histiocytosis syndromes, and is characterized by lytic lesions of one or more bones. Temporal bone lesions usually occur in association with multifocal disease; however, isolated lesions may occur in either the mastoid bone alone, or in the entire temporal bone, without disease elsewhere in the body. We present the first case (to our knowledge) of eosinophilic granuloma, or unifocal Langerhans' cell histiocytosis, limited to the petrous apex. The patient, an 8-year-old girl, presented with facial nerve paralysis. Because of delay in definitive diagnosis, the disease extended beyond its original boundaries and resulted in complete destruction of the temporal bone. We will also review Langerhans' cell histiocytosis, with attention to its involvement of the temporal bone.

摘要

嗜酸性肉芽肿是组织细胞增多症X(即朗格汉斯细胞组织细胞增多症)的一种局限性形式,是朗格汉斯细胞增殖形成的良性病变。它是组织细胞增多症综合征中最不严重的一种,其特征为一处或多处骨骼出现溶骨性病变。颞骨病变通常与多灶性疾病相关;然而,孤立性病变可能仅发生于乳突骨,或累及整个颞骨,而身体其他部位并无病变。我们报告了首例(据我们所知)局限于岩尖的嗜酸性肉芽肿,即单灶性朗格汉斯细胞组织细胞增多症。该患者为一名8岁女孩,表现为面神经麻痹。由于确诊延迟,疾病超出了其最初边界,导致颞骨完全破坏。我们还将对朗格汉斯细胞组织细胞增多症进行综述,并重点关注其累及颞骨的情况。

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Arch Otolaryngol Head Neck Surg. 1993 Jan;119(1):113-6. doi: 10.1001/archotol.1993.01880130115018.
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