肺泡软组织肉瘤。一种罕见的小儿科肿瘤。

Y Perel, J Rivel, N Alos, M L Pignol, J M Guillard
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引用次数: 0

摘要

目的:报告一例12岁女孩的手臂肺泡软组织肉瘤合并转移性肺米利亚。结果:9年来,虽然转移灶的大小逐渐增大,化疗没有改善,但患者的总体情况仍然良好。结论:小儿肿瘤学中罕见的间充质肿瘤病程不同寻常;组织学模式具有特征性,但组织发生机制尚不清楚。治疗的选择是肿瘤切除。其他涉及继发性沉积的治疗方法远不能令人满意,并且由于转移率高,预后较差。
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Alveolar soft part sarcoma. A rare tumor of unusual evolution in pediatrics.

Purpose: A case of alveolar soft part sarcoma of the arm with metastatic pulmonary miliaria is reported in a 12-year-old girl.

Results: Although the size of the metastases increased greatly and progressively for 9 years with no improvement under chemotherapy, the patient's general condition remained good.

Conclusions: The course in pediatric oncology of this rare mesenchymatous tumor is very unusual; the histologic pattern is characteristic but the histogenesis remains unclear. The treatment of choice is tumoral excision. Other therapies involving secondary deposits are far from satisfactory, and the prognosis is poor because of the high rate of metastases.

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