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一例酷似丛状神经纤维瘤的散发性弥漫性多发性皮肤平滑肌瘤病:手术挑战。

A sporadic diffuse multiple cutaneous leiomyomatosis mimicking plexiform neurofibromatosis: A surgical challenge.

作者信息

Antakanavar Gajanand M, Sahoo Bijaylaxmi, Kaur Aneet, Ghiloria Ishu, Jangid Ajay, Shetty Bhavishya

机构信息

Department of Dermatology Maulana Azad Medical College New Delhi India.

Department of Pathology Maulana Azad Medical College New Delhi India.

出版信息

Skin Health Dis. 2024 Aug 1;4(5):e414. doi: 10.1002/ski2.414. eCollection 2024 Oct.

Abstract

A 32-year-old male presented with diffuse plaques accompanied by multiple large, painful swellings predominantly distributed over the trunk, face and both upper and lower extremities for 11 years. There was no family history of similar complaints. The histopathological examination (HPE), supplemented with special stains confirmed the diagnosis of cutaneous leiomyoma (CL). The immunohistochemical analysis showed a strong expression of smooth muscle actin. The final diagnosis of sporadic diffuse multiple cutaneous leiomyomatosis was made. This presentation of CL is uncommon and resembled plexiform neurofibroma. The clinical diagnosis of this presentation was challenging. Therefore, HPE combined with special stains aided in confirming this surgically challenging rare tumour. In resource-poor settings regular screening is required for early detection of renal cancer and other associated complications.

摘要

一名32岁男性,躯干、面部及上下肢出现弥漫性斑块伴多发大的疼痛性肿胀11年。无类似主诉的家族史。组织病理学检查(HPE)及特殊染色确诊为皮肤平滑肌瘤(CL)。免疫组化分析显示平滑肌肌动蛋白强表达。最终诊断为散发性弥漫性多发性皮肤平滑肌瘤病。这种CL表现不常见,类似丛状神经纤维瘤。该表现的临床诊断具有挑战性。因此,HPE结合特殊染色有助于确诊这种手术难度大的罕见肿瘤。在资源匮乏地区,需要定期筛查以早期发现肾癌及其他相关并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eafd/11442042/d078b3d30690/SKI2-4-e414-g003.jpg

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