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腮腺未分化多形性肉瘤:一例罕见的儿科病例。

Undifferentiated pleomorphic sarcoma of the parotid gland: a rare pediatric case.

作者信息

Chang Ray C, Dave Sandeep P, Robinson Philip G

机构信息

Department of Otolaryngology-Head and Neck Surgery, University of Miami Miller School of Medicine, Miami, Florida, USA.

出版信息

Head Neck. 2008 Jul;30(7):970-3. doi: 10.1002/hed.20752.

Abstract

BACKGROUND

Undifferentiated pleomorphic sarcoma, or malignant fibrous histiocytoma (MFH) of the head and neck is an uncommon malignancy that is exceedingly rare in the pediatric population. Although MFH was once considered the most common soft tissue sarcoma in adults, recently authors have questioned its existence as a distinct pathologic entity.

METHODS

In light of this debate, we present a case of MFH with giant cells involving the parotid gland, and we review its pathology.

RESULTS

We describe a 6-year-old male with parotid gland MFH. Diagnosis was fraught with difficulty, typical of this disease. He was treated with a combination of chemotherapy and radiation therapy with a good initial response.

CONCLUSION

The classification of MFH has been recently debated. Nevertheless, our case is the second report of pediatric MFH involving the parotid gland. Surgical resection is the preferred treatment, but combined chemoradiation may be necessary in the head and neck region.

摘要

背景

头颈部未分化多形性肉瘤,即恶性纤维组织细胞瘤(MFH)是一种罕见的恶性肿瘤,在儿科人群中极为罕见。尽管MFH曾被认为是成人中最常见的软组织肉瘤,但最近有作者质疑其作为一种独特病理实体的存在。

方法

鉴于这一争论,我们报告一例累及腮腺的伴有巨细胞的MFH病例,并回顾其病理学特征。

结果

我们描述了一名患有腮腺MFH的6岁男性。诊断充满困难,这是该病的典型情况。他接受了化疗和放疗相结合的治疗,初期反应良好。

结论

最近对MFH的分类存在争议。尽管如此,我们的病例是第二例关于累及腮腺的儿科MFH的报告。手术切除是首选治疗方法,但在头颈部区域可能需要联合放化疗。

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