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头皮脂肪纤维瘤病——来自巴基斯坦一名两岁儿童综合治疗的病例报告

Scalp Lipofibromatosis - A case report on comprehensive management in a two-year-old child from Pakistan.

作者信息

Ansari Nasruddin, Shahbaz Aleena, Khan Muhammad Tahir, Khan Zubair Ahmad

机构信息

Dr. Nasruddin Ansari, MBBS Postgraduate Resident, Department of Neurosurgery Unit III, Punjab Institute of Neurosciences, Lahore, Pakistan.

Dr. Aleena Shahbaz, MBBS Postgraduate Resident, Department of Neurosurgery Unit III, Punjab Institute of Neurosciences, Lahore, Pakistan.

出版信息

Pak J Med Sci. 2024 Dec;40(12PINS Suppl):S90-S94. doi: 10.12669/pjms.40.12(PINS).11034.

Abstract

Lipofibromatosis is a rare benign soft tissue tumor that primarily affects children. There is limited cytological description and management of this rare condition in the literature which leads to misdiagnosis. The two years old patient first presented with a big, non-tender swelling on the scalp's right temporoparietal area. Despite receiving chemotherapy for misdiagnosis of small round cell carcinoma on first biopsy, the tumor continued to grow, requiring surgical removal. The histopathology report after surgical excision validated the diagnosis of lipofibromatosis. The cosmetic care of the wound was taken by plastic surgery team. The fact that this case was successfully treated shows how crucial it is to treat rare pediatric cancers with a multidisciplinary strategy that incorporates surgical precision, imaging, histopathology and aesthetic considerations. This strategy highlighted the need of preserving the patient's quality of life by cosmetic reconstruction in addition to facilitating efficient tumor removal. Emphasizing thorough diagnosis and interdisciplinary management, this case adds important context to the little existing research on lipofibromatosis.

摘要

脂肪纤维瘤病是一种罕见的良性软组织肿瘤,主要影响儿童。文献中对这种罕见病症的细胞学描述和治疗方法有限,这导致了误诊。该两岁患者首次就诊时,头皮右侧颞顶区出现一个较大的无痛性肿块。尽管首次活检因误诊为小圆细胞癌而接受了化疗,但肿瘤仍继续生长,需要手术切除。手术切除后的组织病理学报告证实了脂肪纤维瘤病的诊断。伤口的美容护理由整形外科团队负责。该病例得到成功治疗这一事实表明,采用包含手术精准性、影像学、组织病理学和美学考量的多学科策略来治疗罕见的儿科癌症至关重要。这一策略强调了除了促进有效切除肿瘤外,还需要通过美容重建来保留患者的生活质量。该病例强调了全面诊断和跨学科管理的重要性,为现有的关于脂肪纤维瘤病的少量研究增添了重要内容。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f1be/11654665/702484d1698a/PJMS-40-S90-g001.jpg

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