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手部尤因肉瘤:一例罕见病例报告及诊断与治疗考量

Ewing sarcoma of the hand: A rare case report with diagnostic and management considerations.

作者信息

Ali Sheikh Sarfraz, Khurram Mohd Fahud, Singh Nimisha, Anand Shishir

机构信息

Department of Plastic and Reconstructive Surgery, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh 202001, India.

Department of Plastic and Reconstructive Surgery, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh 202001, India.

出版信息

Int J Surg Case Rep. 2025 Jun;131:111379. doi: 10.1016/j.ijscr.2025.111379. Epub 2025 Apr 25.

Abstract

BACKGROUND

Ewing sarcoma (ES) is a rare malignant tumour primarily affecting adolescents, typically involving the axial and appendicular skeleton. Its occurrence in the hand is exceedingly rare, comprising less than 1 % of all cases. Early diagnosis is often challenging due to its nonspecific symptoms and similarities to other conditions. This case report highlights a rare presentation of Ewing sarcoma in the hand.

CASE REPORT

A 20-year-old male presented with a six-month history of swelling and discomfort in the left thumb, without prior injury or inflammatory symptoms. Physical examination revealed a firm, non-tender, fusiform swelling involving the proximal and distal phalanges. Radiographic imaging showed a mass affecting the bone, with signs of periosteal reaction and cortical disruption. MRI revealed a well-demarcated soft tissue mass contacting the flexor tendon, skin, and neurovascular bundle, with bone involvement. Potential differential diagnoses included giant cell tumour, enchondroma, and aneurysmal bone cyst. Following consent, thumb amputation was performed, revealing a grey tumour surrounding the bone and soft tissues. Histopathological analysis revealed monomorphic small round cells with finely split chromatin and CD99 positivity which confirmed Ewing's sarcoma. Postoperative chemotherapy included Vincristine, Ifosfamide, Doxorubicin, Etoposide, and Actinomycin-D. After one year of follow-up, there was no evidence of recurrence or metastasis.

DISCUSSION

Ewing sarcoma of the hand is rare, often misdiagnosed as benign conditions or infection due to its soft tissue presentation and bone involvement. Timely diagnosis and surgical intervention, followed by chemotherapy, are crucial for effective treatment. The prognosis for hand Ewing sarcoma is favourable with early treatment, and survival rates for localized disease are around 70-80 %.

CONCLUSION

Importance of early suspicion in atypical soft tissue swellings of the hand in young adults is crucial. Ewing sarcoma in the hand, although rare, should be considered in young patients presenting with unexplained soft tissue masses. Early detection and a multidisciplinary approach involving surgery and chemotherapy can lead to favourable outcomes.

摘要

背景

尤因肉瘤(ES)是一种罕见的恶性肿瘤,主要影响青少年,通常累及中轴骨和四肢骨骼。其在手部位的发生极为罕见,占所有病例的比例不到1%。由于其症状不具特异性且与其他病症相似,早期诊断往往具有挑战性。本病例报告突出了尤因肉瘤在手部位的罕见表现。

病例报告

一名20岁男性,有左手拇指肿胀和不适6个月的病史,之前无受伤或炎症症状。体格检查发现一个坚硬、无压痛的梭形肿胀,累及近节指骨和远节指骨。影像学检查显示有一个肿块累及骨骼,伴有骨膜反应和皮质破坏迹象。磁共振成像(MRI)显示一个边界清晰的软组织肿块,与屈肌腱、皮肤和神经血管束接触,并累及骨骼。可能的鉴别诊断包括骨巨细胞瘤、内生软骨瘤和动脉瘤样骨囊肿。在获得同意后,进行了拇指截肢手术,发现一个围绕骨骼和软组织的灰色肿瘤。组织病理学分析显示为单形性小圆形细胞,染色质精细分裂且CD99呈阳性,确诊为尤因肉瘤。术后化疗包括长春新碱、异环磷酰胺、多柔比星、依托泊苷和放线菌素-D。经过一年的随访,没有复发或转移的迹象。

讨论

手部尤因肉瘤罕见,由于其软组织表现和骨骼受累,常被误诊为良性病症或感染。及时诊断和手术干预,随后进行化疗,对于有效治疗至关重要。手部尤因肉瘤早期治疗预后良好,局限性疾病的生存率约为70%-80%。

结论

对于年轻成人手部非典型软组织肿胀早期怀疑的重要性至关重要。手部尤因肉瘤虽然罕见,但在出现不明原因软组织肿块的年轻患者中应予以考虑。早期发现以及包括手术和化疗的多学科方法可带来良好的结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8909/12076796/4d5625cb9086/gr1.jpg

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