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累及颅底和眼眶的鼻软骨间叶性错构瘤:病例报告

Nasal Chondromesenchymal Hamartoma with Skull Base and Orbital Involvement: Case Presentation.

作者信息

Golbin Denis A, Ektova Anastasia P, Demin Maxim O, Lasunin Nikolay, Cherekaev Vasily A

机构信息

Skull Base and Craniofacial Surgery, N.N. Burdenko National Medical Research Center for Neurosurgery, Moscow, RUS.

Pathology, Russian Children's Clinical Hospital, Moscow, RUS.

出版信息

Cureus. 2018 Jun 28;10(6):e2892. doi: 10.7759/cureus.2892.

DOI:10.7759/cureus.2892
PMID:30167348
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6112910/
Abstract

Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the sinonasal tract in children with possible orbit and skull base involvement. We present the 57th published observation of this kind of tumor. A 25-month-old female patient presented with recurrent mass lesion of the sinonasal tract. According to her history, she had feeding difficulties and nasal obstruction since birth. She underwent partial resection at eight months of age via transfacial approach in the local hospital. Due to progression of tumor remnants, a second surgery was performed using an endoscopic endonasal approach resulting in subtotal resection. At 12 months of follow-up, a good postoperative result was observed with no signs of tumor progression despite incomplete resection. Histological and immunohistochemical examination of the biopsy specimens is presented. Comparison of specimens obtained from each of the two surgeries showed a difference in histological patterns. Endoscopic endonasal approach is the mainstay of surgical management. In case of incomplete resection, careful follow-up MRI studies should be recommended.

摘要

鼻软骨间叶性错构瘤(NCMH)是儿童鼻窦道的一种罕见良性肿瘤,可能累及眼眶和颅底。我们报告了此类肿瘤的第57例已发表观察病例。一名25个月大的女性患者出现鼻窦道复发性肿块病变。根据她的病史,她自出生以来就有喂养困难和鼻塞症状。她8个月大时在当地医院经面部入路接受了部分切除术。由于肿瘤残余进展,第二次手术采用鼻内镜鼻内入路,实现了次全切除。在随访12个月时,尽管切除不完全,但术后效果良好,未出现肿瘤进展迹象。本文展示了活检标本的组织学和免疫组化检查结果。对两次手术获取的标本进行比较,结果显示组织学模式存在差异。鼻内镜鼻内入路是手术治疗的主要方法。若切除不完全,建议进行仔细的随访磁共振成像(MRI)检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/5f8bd40cc542/cureus-0010-00000002892-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/cdd8009ca590/cureus-0010-00000002892-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/32ae8a0d7d50/cureus-0010-00000002892-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/11671c05e2e8/cureus-0010-00000002892-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/1e7437f013a3/cureus-0010-00000002892-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/98a3214bccfc/cureus-0010-00000002892-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/5f8bd40cc542/cureus-0010-00000002892-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/cdd8009ca590/cureus-0010-00000002892-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/32ae8a0d7d50/cureus-0010-00000002892-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/11671c05e2e8/cureus-0010-00000002892-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/1e7437f013a3/cureus-0010-00000002892-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/98a3214bccfc/cureus-0010-00000002892-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b693/6112910/5f8bd40cc542/cureus-0010-00000002892-i06.jpg

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