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儿童指骨中的奇异骨旁骨软骨瘤样增殖(Nora 病变)。

Bizarre Parosteal Osteochondromatous Proliferation (Nora Lesion) in Pediatric Phalanges.

机构信息

Cirugía Ortopédica y Traumatología, Hospital Universitario Infantil Niño Jesús, Madrid, Spain.

Departamento de Patología, Hospital Universitario Infantil Niño Jesús, Madrid, Spain.

出版信息

J Hand Surg Am. 2021 Apr;46(4):344.e1-344.e9. doi: 10.1016/j.jhsa.2020.05.002. Epub 2020 Jun 28.

Abstract

Bizarre parosteal osteochondromatous proliferation, or Nora tumor, is an uncommon lesion affecting the tubular bones of the hands and feet. Normally arising from the cortical surface and periosteum of these bones, these lesions histologically consist of a hypercellular cartilaginous cap covering a bony stalk that is surrounded by ossified areas and spindle cell stroma. The differential diagnosis includes conditions involving the periosteum such as chondrosarcoma, parosteal osteosarcoma, osteochondroma, turret exostosis, and florid reactive periostitis. The only effective treatment is wide surgical excision; nevertheless, local recurrence rates are extremely high and may necessitate revision surgery. In the present study, we report 3 cases of Nora lesion located in the hand in pediatric patients. The diagnosis in these cases was challenging owing to their presenting symptoms and radiographic findings. The diagnosis was made based on characteristic findings noted on the radiographic images and was confirmed by histological examination following excision.

摘要

奇异骨旁骨软骨瘤性增殖,或 Nora 瘤,是一种罕见的病变,影响手足的管状骨。这些病变通常起源于这些骨骼的皮质表面和骨膜,组织学上由一个富含细胞的软骨帽覆盖在一个骨性茎干上,周围是骨化区和梭形细胞基质。鉴别诊断包括涉及骨膜的疾病,如软骨肉瘤、骨旁骨肉瘤、骨软骨瘤、炮塔性骨外生骨疣和弥漫性反应性骨膜炎。唯一有效的治疗方法是广泛的手术切除;然而,局部复发率极高,可能需要再次手术。在本研究中,我们报告了 3 例位于儿童手部的 Nora 病变。由于这些病例的临床表现和影像学表现,诊断具有挑战性。诊断是基于切除后影像学图像上的特征性表现,并通过组织学检查得到证实。

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