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儿童不可切除性多灶性网膜和腹膜炎症性肌纤维母细胞瘤:重新探讨辅助治疗的作用。

Unresectable multifocal omental and peritoneal inflammatory myofibroblastic tumor in a child: revisiting the role of adjuvant therapy.

机构信息

Liver Surgery Unit, Bambino Gesù Children's Hospital, Rome - 00165, Italy.

Hepatobiliary Radiology Unit, Bambino Gesù Children's Hospital, Rome - 00165, Italy.

出版信息

J Pediatr Surg. 2011 Apr;46(4):e17-e21. doi: 10.1016/j.jpedsurg.2011.01.007.

Abstract

Inflammatory myofibroblastic tumor is an uncommon lesion, also called pseudotumor, with a variable natural course from benign with spontaneous regression to mimicking malignant tumors. We report a case of diffuse peritoneal and omental pseudotumor in a 10-year-old boy characterized by aggressive behavior at the onset followed by stability after subtotal resection and chemotherapy. Total excision was not possible because of the tumor dissemination over the whole peritoneal surface. Adjuvant antiinflammatory drug (ketorolac tromethamine) and chemotherapy (methotrexate-vinblastine followed by ifosfamide-adriamycin and ifosfamide alone) were helpful to obtain rapidly complete resolution of clinical symptoms and anatomic stability of the residual lesions. Long-term evolution, in the absence of continued therapy, has been characterized by progressive involution and reduction of the residual masses.

摘要

炎性肌纤维母细胞瘤是一种不常见的病变,也称为假瘤,其自然病程具有多样性,从良性伴自发性消退到类似于恶性肿瘤。我们报告了一例 10 岁男孩弥漫性腹膜和网膜假性肿瘤病例,其特征为起始时表现出侵袭性行为,随后在次全切除和化疗后稳定。由于肿瘤扩散到整个腹膜表面,无法进行完全切除。辅助抗炎药物(酮咯酸氨丁三醇)和化疗(甲氨蝶呤-长春碱,随后是异环磷酰胺-阿霉素和异环磷酰胺单药)有助于迅速完全缓解临床症状和残留病变的解剖稳定性。在没有持续治疗的情况下,长期演变的特征是进行性退化和残留肿块的减少。

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