揭露颅底磷化间充质肿瘤:肿瘤诱发骨软化症的罕见且可治疗的病因。

IF 0.6 Q4 SURGERY
Kalyana Sundaram Chithambaram, Amit Keshri, Chappity Preetam, Subhash Yadav, Nazrin Hameed, Mohit Sinha, Nidhin Das, Mohd Aqib, M S Arushi Kumar, Awadhesh Kumar Jaiswal, Sanjay Gambhir, Harrish Govindarajan, Ravi Sankar Manogaran
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引用次数: 0

摘要

肿瘤诱导骨软化症(TIO)是一种罕见的由磷质间充质肿瘤引起的综合征,该肿瘤分泌FGF23等物质,导致磷丢失和骨软化。这些肿瘤通常很小,很难定位。手术切除是主要的治疗方法,通常可以改善症状。本报告提出了7例颅底肿瘤起源的病例,以补充现有文献。本回顾性研究回顾了2015年至2023年在三级保健机构接受手术切除的磷化间质肿瘤(PMT)患者,仅关注局限于颅底前部或外侧的肿瘤。分析包括人口统计学、临床特征、生化和放射学发现、肿瘤位置、手术细节和随访。7例患者(男6例,女1例),年龄40 ~ 65岁,平均年龄52岁。常见症状包括腰痛(70%)、骨折(42%)、行走困难(42%)和肌肉无力(28%)。所有患者均表现为低磷血症,7例患者中有5例(70%)FGF23水平升高。大多数肿瘤(86%)位于前颅底,特别是在筛区。一名患者在侧颅底有肿瘤,局限于乳突。所有患者均经内窥镜和乳突后入路行广泛局部切除。磷酸盐间充质肿瘤诱导的骨软化症是一种罕见的疾病,具有广泛性非特异性症状。尽管这种疾病具有挑战性,但手术切除肿瘤可以完全治愈并显著缓解症状。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Unmasking Skull Base Phosphaturic Mesenchymal Tumors: A Rare and Treatable Cause of Tumor-Induced Osteomalacia.

Tumor-induced osteomalacia (TIO) is a rare syndrome caused by phosphaturic mesenchymal tumors that secrete substances, like FGF23, leading to phosphorus loss and osteomalacia. These tumors are often small and challenging to locate. Surgical excision is the primary treatment and usually results in symptomatic improvement. This report presents seven cases of skull base origin of these tumors to add to the existing literature. This retrospective study reviewed patients with phosphaturic mesenchymal tumors (PMT) who underwent surgical excision at a tertiary care institute from 2015 to 2023, focusing solely on tumors confined to the anterior or lateral skull base. The analysis included demographics, clinical features, biochemical and radiologic findings, tumor location, surgical details, and follow-up. The study included 7 patients (6 males, 1 female) with ages ranging from 40 to 65 years (mean age: 52 years). Common symptoms included lower back pain (70%), fractures (42%), difficulty walking (42%), and muscle weakness (28%). All patients showed hypophosphatemia, and elevated FGF23 levels were noted in 5 out of 7 patients (70%). Most tumors (86%) were in the anterior skull base, particularly in the ethmoid region. One patient had a tumor in the lateral skull base, confined to the mastoid. All patients underwent wide local excision via endoscopic and retromastoid approaches. Phosphaturic mesenchymal tumor-induced osteomalacia is a rare condition, with generalized nonspecific symptoms. Despite the challenging nature of the disease, surgical removal of the tumor can lead to a complete cure and significant symptom relief.

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来源期刊
CiteScore
0.80
自引率
0.00%
发文量
226
审稿时长
6-12 weeks
期刊介绍: Indian Journal of Otolaryngology and Head & Neck Surgery was founded as Indian Journal of Otolaryngology in 1949 as a scientific Journal published by the Association of Otolaryngologists of India and was later rechristened as IJOHNS to incorporate the changes and progress. IJOHNS, undoubtedly one of the oldest Journals in India, is the official publication of the Association of Otolaryngologists of India and is about to publish it is 67th Volume in 2015. The Journal published quarterly accepts articles in general Oto-Rhino-Laryngology and various subspecialities such as Otology, Rhinology, Laryngology and Phonosurgery, Neurotology, Head and Neck Surgery etc. The Journal acts as a window to showcase and project the clinical and research work done by Otolaryngologists community in India and around the world. It is a continued source of useful clinical information with peer review by eminent Otolaryngologists of repute in their respective fields. The Journal accepts articles pertaining to clinical reports, Clinical studies, Research articles in basic and applied Otolaryngology, short Communications, Clinical records reporting unusual presentations or lesions and new surgical techniques. The journal acts as a catalyst and mirrors the Indian Otolaryngologist’s active interests and pursuits. The Journal also invites articles from senior and experienced authors on interesting topics in Otolaryngology and allied sciences from all over the world. The print version is distributed free to about 4000 members of Association of Otolaryngologists of India and the e-Journal shortly going to make its appearance on the Springer Board can be accessed by all the members. Association of Otolaryngologists of India and M/s Springer India group have come together to co-publish IJOHNS from January 2007 and this bondage is going to provide an impetus to the Journal in terms of international presence and global exposure.
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