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尺骨远端与髓腔连续的奇异骨旁骨软骨瘤性增殖:病例报告。

Bizarre parosteal osteochondromatous proliferation in the distal ulna where the lesion is continuous with the medullary cavity: a case report.

机构信息

Department of Orthopedics, The Second Xiangya Hospital, Central South University, No 139 Renmin Road, Changsha, Hunan, 410011, China.

Department of Orthopedics, The First People's Hospital of Chenzhou, Chenzhou, Hunan, China.

出版信息

BMC Musculoskelet Disord. 2024 Jul 26;25(1):588. doi: 10.1186/s12891-024-07715-4.

Abstract

BACKGROUND

Bizarre parosteal osteochondromatous proliferation (BPOP) is a rare benign bone tumor, it is also called "Nora's lesion". The lesion is characterized by heterotopic ossification of the normal bone cortex or parosteal bone. The etiology of BPOP is unclear and may be related to trauma. In most BPOPs, the lesion is not connected to the medullary cavity. Here we report an atypical case, characterized by reversed features compared to the typical BPOP, which demonstrated continuity of the lesion with the cavity.

CASE PRESENTATION

An 11-year-old female child had a slow-growing mass on her right wrist for 8 months with forearm rotation dysfunction. Plain X-rays showed an irregular calcified mass on the right distal ulna, and computed tomography (CT) showed a pedunculated mass resembling a mushroom protruding into the soft tissue at the distal ulna. The medulla of this lesion is continuous with the medulla of the ulna. A surgical resection of the lesion, together with a portion of the ulnar bone cortex below the tumor was performed, and the final pathology confirmed BPOP. After the surgery, the child's forearm rotation function improved significantly, and there was no sign of a recurrence at 1-year follow-up.

CONCLUSION

It is scarce for BPOP lesions to communicate with the medullary cavity. However, under-recognition of these rare cases may result in misdiagnosis or inappropriate treatment thereby increasing the risk of recurrence. Therefore, special cases where BPOP lesions are continuous with the medulla are even more important to be studied to understand better and master these lesions. Although BPOP is a benign tumor with no evidence of malignant transformation, the recurrence rate of surgical resection is high. We considered the possibility of this particular disease prior to surgery and performed a surgical resection with adequate safety margins. Regular postoperative follow-up is of utmost importance, without a doubt.

摘要

背景

奇异骨旁骨软骨瘤性增殖(BPOP)是一种罕见的良性骨肿瘤,也被称为“Nora 病变”。病变的特征是正常骨皮质或骨旁骨的异位骨化。BPOP 的病因尚不清楚,可能与创伤有关。在大多数 BPOP 中,病变与髓腔无关。我们报告了一个不典型病例,与典型 BPOP 相比具有相反的特征,其病变与腔具有连续性。

病例介绍

一名 11 岁女性儿童,右手腕部有一个缓慢生长的肿块,前臂旋转功能障碍 8 个月。平片显示右尺骨远端不规则钙化肿块,CT 显示一个类似蘑菇状的带蒂肿块突入尺骨远端软组织。该病变的髓腔与尺骨的髓腔连续。进行了病变的切除术,连同肿瘤下方的一部分尺骨骨皮质一起切除,最终病理证实为 BPOP。手术后,患儿前臂旋转功能明显改善,随访 1 年无复发迹象。

结论

BPOP 病变与髓腔相通的情况很少见。然而,对这些罕见病例的认识不足可能导致误诊或不当治疗,从而增加复发的风险。因此,研究与髓腔连续的 BPOP 病变的特殊病例更为重要,以更好地理解和掌握这些病变。尽管 BPOP 是一种良性肿瘤,没有恶性转化的证据,但手术切除的复发率很高。我们在手术前考虑了这种特殊疾病的可能性,并进行了有足够安全边界的手术切除。定期术后随访是非常重要的,这是毫无疑问的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f517/11282834/3f99256986c9/12891_2024_7715_Fig1_HTML.jpg

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