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从细微到显著:一例罕见的隆突性皮肤纤维瘤及其临床历程。

From subtle to striking: A rare case of dermatofibroma protuberans and its clinical journey.

作者信息

Nimodia Devyansh, Dudhe Sakshi, Parihar Pratapsingh Hanuman, Patil Ravishankar, Kotla Rishitha, Choudhary Abhishek

机构信息

Department of Radiodiagnosis, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India 442001.

Department of Psychiatry, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India 442001.

出版信息

Radiol Case Rep. 2025 Mar 8;20(5):2557-2561. doi: 10.1016/j.radcr.2025.02.045. eCollection 2025 May.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare type of soft tissue sarcoma, which is slow-growing. It arises from the dermal, subcutaneous layer and is locally aggressive. It commonly affects adults between 20 and 50 years old, with a slight male predominance. This case report emphasizes the case of a 35-year-old male patient who presented with a large, firm, nodular mass on his left upper arm that had been progressively increasing in size over 3 years. On inspection, the lesion measured approximately 13 cm in diameter, was large and lobulated with overlying red-brown discolouration. The patient reported movement discomfort, and mild itching was noted. Diagnostic evaluation was advised, including X-ray, Ultrasound, Computed Tomography and Magnetic resonance imaging. A provisional diagnosis of soft tissue sarcoma was made. An excisional biopsy was performed, and histopathological analysis demonstrated a storiform pattern of spindle-shaped cells infiltrating the dermis and subcutis, consistent with dermatofibrosarcoma protuberans. The patient underwent wide local excision with clear margins to prevent recurrence, and no metastasis was detected. Dermatofibrosarcoma protuberans is characterized by a high rate of local recurrence but a low risk of metastasis. Early diagnosis and complete surgical excision are treatment options for good prognosis. This case underscores the importance of diagnosing and treating Dermatofibrosarcoma protuberans without delays.

摘要

隆突性皮肤纤维肉瘤(DFSP)是一种罕见的软组织肉瘤,生长缓慢。它起源于真皮、皮下层,具有局部侵袭性。它通常影响20至50岁的成年人,男性略占优势。本病例报告重点介绍了一名35岁男性患者,其左上臂出现一个大的、质地坚硬的结节状肿块,3年来大小逐渐增大。检查发现,病变直径约13厘米,体积大且呈分叶状,表面有红棕色变色。患者报告有活动不适,并伴有轻度瘙痒。建议进行诊断评估,包括X线、超声、计算机断层扫描和磁共振成像。初步诊断为软组织肉瘤。进行了切除活检,组织病理学分析显示梭形细胞呈席纹状模式浸润真皮和皮下组织,符合隆突性皮肤纤维肉瘤。患者接受了切缘清晰的广泛局部切除以防止复发,未检测到转移。隆突性皮肤纤维肉瘤的特点是局部复发率高但转移风险低。早期诊断和完整的手术切除是预后良好的治疗选择。本病例强调了及时诊断和治疗隆突性皮肤纤维肉瘤的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c46/11930717/b0a2e7d7bd3a/gr1.jpg

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