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一例罕见的小儿鼻咽横纹肌肉瘤伴脑膜旁扩展,表现为托恩瓦尔德特囊肿。

A Rare Case of Pediatric Nasopharyngeal Rhabdomyosarcoma With Parameningeal Extension Presenting as a Thornwaldt Cyst.

作者信息

Adeel Rao, Fatima Kaneez, Shahid Muneeb, Memon Haris, Haque Abul

机构信息

Emergency Department, Broomfield Hospital, Mid and South Essex NHS Foundation Trust, Chelmsford, GBR.

Internal Medicine, Nishtar Medical University, Multan, PAK.

出版信息

Cureus. 2023 Nov 22;15(11):e49224. doi: 10.7759/cureus.49224. eCollection 2023 Nov.

DOI:10.7759/cureus.49224
PMID:38143702
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10739347/
Abstract

Rhabdomyosarcoma (RMS) is a common soft tissue malignancy of the pediatric age group, frequently involving the head and neck region; however, nasopharyngeal RMS is a rare entity. By virtue of its parameningeal involvement and nonspecific presentations, nasopharyngeal RMS has become a clinical challenge to diagnose. We present a case of an eight-year-old boy presenting with signs and symptoms of nasal obstruction who was initially being treated for tonsillitis. Recurrent episodes led to detailed examination and radiologic imaging, and a diagnosis of Thornwaldt cyst was made. For the relief of symptoms, a debulking surgery was performed followed by a biopsy which revealed the mass to be a nasopharyngeal RMS. Our case highlights the importance of including nasopharyngeal RMS into the differentials of midline nasal masses along with the role of biopsy for confirming its diagnosis as treatment modalities for it are strikingly different than the other more common group of conditions, i.e., benign nasal masses. While surgery is usually delayed in the latter group, it can be of prime importance while treating nasal RMS, along with chemotherapy and radiotherapy.

摘要

横纹肌肉瘤(RMS)是儿童期常见的软组织恶性肿瘤,常累及头颈部区域;然而,鼻咽部RMS是一种罕见的疾病。由于其累及颅底旁组织且临床表现不具特异性,鼻咽部RMS已成为临床诊断的一项挑战。我们报告一例8岁男孩,最初因扁桃体炎接受治疗,表现为鼻塞的症状和体征。反复发作促使进行详细检查和影像学检查,最初诊断为桑沃尔德特囊肿。为缓解症状,先行减瘤手术,随后活检显示肿块为鼻咽部RMS。我们的病例强调了将鼻咽部RMS纳入中线鼻腔肿块鉴别诊断的重要性,以及活检在确诊中的作用,因为其治疗方式与其他更常见的疾病(即良性鼻腔肿块)截然不同。对于良性鼻腔肿块,手术通常会延迟进行,而在治疗鼻腔RMS时,手术连同化疗和放疗可能至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/b228e6809f50/cureus-0015-00000049224-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/92996b11732f/cureus-0015-00000049224-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/fd65123dec91/cureus-0015-00000049224-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/e355b3f45030/cureus-0015-00000049224-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/a13cd2ddcb3f/cureus-0015-00000049224-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/05dbefd6f4f1/cureus-0015-00000049224-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/82f693e398b2/cureus-0015-00000049224-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/ebaa4eb1e74f/cureus-0015-00000049224-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/b228e6809f50/cureus-0015-00000049224-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/92996b11732f/cureus-0015-00000049224-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/fd65123dec91/cureus-0015-00000049224-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/e355b3f45030/cureus-0015-00000049224-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/a13cd2ddcb3f/cureus-0015-00000049224-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/05dbefd6f4f1/cureus-0015-00000049224-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/82f693e398b2/cureus-0015-00000049224-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/ebaa4eb1e74f/cureus-0015-00000049224-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf0b/10739347/b228e6809f50/cureus-0015-00000049224-i08.jpg

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