Type 1 neurofibromatosis and adult extremity sarcoma - A report of two cases

被引：0
|
作者：
Demiralp, Bahtiyar [1 ]
Ozdemir, M. Taner [1 ]
Erler, Kaan [1 ]
Basbozkurt, Mustafa [1 ]
机构：
[1] Ankara & Corlu Mil Hosp, Gulhane Mil Med Acad, Tekirdag, Turkey
来源：
ACTA ORTHOPAEDICA BELGICA | 2007年 / 73卷 / 03期
关键词：
neurofibromatosis type 1; rhabdomyosarcoma; malignant neurofibromatosis fibrous histiocytoma;
D O I：
暂无
中图分类号：
R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学（修复外科学）];
学科分类号：
摘要：
We report two cases of malignant soft-tissue tumours one myxoid malignant fibrous histiocytoma and one pleomorphic rhabdomyosarcoma- which were diagnosed in two young adult patients with type 1 neurofibromatosis (NF 1). The patients were evaluated with criteria for Neurofibromatosis 1 and NF 1 gene analysis was performed. Four of seven criteria were found in both patients. The tumours; were stage II and III respectively. Both patients were treated with radiotherapy or chemotherapy and surgical intervention. Diagnoses of myxoid malignant fibrous histiocytoma and pleomorphic rhabdomyosarcoma in adult NF 1 patients are exceedingly rare. Thus detection of subtypes of rhabdomyosarcoma and malignant fibrous histiocytoma with immunohistochemistry may be helpful for the management of these tumours among other pleomorphic sarcomas that may occur in type 1 Neurofibromatosis.
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收藏
页码：403 / 407
页数：5
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