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一名儿童复发性先天性血管外皮细胞瘤

Recurrent congenital haemangiopericytoma in a child.

作者信息

O'Donnell T M, Devitt A T, Kutty S, Fogarty E E

机构信息

Department of Orthopaedic Surgery, Our Lady's Hospital for Sick Children, Dublin, Ireland.

出版信息

J Bone Joint Surg Br. 2001 Mar;83(2):269-72. doi: 10.1302/0301-620x.83b2.10729.

Abstract

A five-day-old boy was referred with a soft-tissue mass in his right upper arm. Plain radiographs and ultrasound demonstrated a lesion extending from the axilla to the elbow on the posterolateral aspect of the humerus. Open biopsy confirmed the diagnosis of congenital haemangiopericytoma. After MRI and selective angiography, excision biopsy was carried out, but no adjuvant therapy was administered. At further examination, four years and ten months later, he was noted to have three small nodules at the site of the original tumour. Excision biopsy confirmed this to be a local recurrence, although the lesion was less cellular with no appreciable mitotic activity. Congenital haemangiopericytoma is a rare cause of a soft-tissue mass in children. Most tumours are benign, and recurrence is uncommon. The treatment is controversial, but most centres recommend the use of adjuvant chemotherapy, combined with complete excision. We recommend treatment with doxorubicin. Orthopaedic surgeons should be familiar with this tumour since 30% to 50% of cases occur in the limbs.

摘要

一名5天大的男婴因右上臂软组织肿块前来就诊。X线平片和超声检查显示,在肱骨后外侧有一个从腋窝延伸至肘部的病变。切开活检确诊为先天性血管外皮细胞瘤。在进行磁共振成像(MRI)和选择性血管造影后,实施了切除活检,但未进行辅助治疗。在4年零10个月后的进一步检查中,发现他在原肿瘤部位有3个小结节。切除活检证实这是局部复发,不过病变的细胞较少,未见明显的有丝分裂活性。先天性血管外皮细胞瘤是儿童软组织肿块的罕见病因。大多数肿瘤是良性的,复发并不常见。治疗方法存在争议,但大多数中心建议采用辅助化疗并结合完整切除。我们推荐使用阿霉素进行治疗。骨科医生应该熟悉这种肿瘤,因为30%至50%的病例发生在四肢。

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