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左上肢婴儿型纤维肉瘤误诊为血管瘤1例报告

Infantile fibrosarcoma of the left upper limb mimicking a hemangioma: A case report.

作者信息

Daly Hafedh, Boughanmi Faiez, Zayati Mohamed, Limayem Leith, Chaouch Mohamed Ali, Jebali Fethi

机构信息

Department of Cardiovascular Surgery, Monastir University Hospital, Tunisia.

Department of General Surgery, Monastir University Hospital, Tunisia.

出版信息

Int J Surg Case Rep. 2025 Jun;131:111417. doi: 10.1016/j.ijscr.2025.111417. Epub 2025 May 9.

Abstract

INTRODUCTION AND IMPORTANCE

Infantile fibrosarcoma (IFS) is a rare soft tissue malignancy that primarily affects children under one year of age. Its clinical and radiologic features often resemble benign vascular tumors like hemangiomas, making diagnosis challenging. Early and accurate identification is essential for effective management.

CASE PRESENTATION

We report a case of a 3-year-old child with a progressively enlarging mass in the left upper limb. Clinical examination revealed a firm, non-pulsatile mass with prominent venous collateral circulation. Doppler ultrasound and MRI findings suggested an infantile hemangioma. Surgical excision revealed a highly vascularized tumor closely associated with the brachial artery and median nerve. Histopathological analysis confirmed infantile fibrosarcoma, characterized by spindle-shaped cells with mild atypia, high mitotic activity, hemosiderin deposits, and significant vascular proliferation. Immunohistochemistry was negative for myogenin, cytokeratin, desmin, CD68, and TLE1.

CLINICAL DISCUSSION

This case underscores the diagnostic challenge posed by IFS, which can closely mimic infantile hemangioma on imaging. MRI findings suggested but did not confirm the diagnosis. Histopathology remains the definitive method for diagnosis. Multidisciplinary management-including surgical resection and, when indicated, chemotherapy-is essential for optimal outcomes.

CONCLUSION

IFS should be considered in the differential diagnosis of congenital soft tissue masses, particularly when atypical features are present. Definitive diagnosis relies on histopathological and immunohistochemical evaluation. Multidisciplinary management plays a critical role in ensuring favorable clinical outcomes.

摘要

引言与重要性

婴儿纤维肉瘤(IFS)是一种罕见的软组织恶性肿瘤,主要影响一岁以下儿童。其临床和放射学特征常类似于血管瘤等良性血管肿瘤,这使得诊断具有挑战性。早期准确识别对于有效管理至关重要。

病例介绍

我们报告一例3岁儿童,其左上肢有一逐渐增大的肿块。临床检查发现一个质地坚硬、无搏动的肿块,伴有明显的静脉侧支循环。多普勒超声和MRI检查结果提示为婴儿血管瘤。手术切除显示一个血管高度丰富的肿瘤,与肱动脉和正中神经紧密相连。组织病理学分析证实为婴儿纤维肉瘤,其特征为梭形细胞,有轻度异型性、高有丝分裂活性、含铁血黄素沉积和明显的血管增生。免疫组织化学检测肌生成素、细胞角蛋白、结蛋白、CD68和TLE1均为阴性。

临床讨论

该病例强调了IFS带来的诊断挑战,其在影像学上可酷似婴儿血管瘤。MRI检查结果提示但未确诊。组织病理学仍然是确诊的方法。多学科管理,包括手术切除以及必要时的化疗,对于取得最佳治疗效果至关重要。

结论

在先天性软组织肿块的鉴别诊断中应考虑IFS,尤其是存在非典型特征时。确诊依赖于组织病理学和免疫组织化学评估。多学科管理在确保良好临床疗效方面起着关键作用。

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