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Simple Summary: Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. SDHx mutations currently account for the vast majority of mutations identified in HNPGLs, and SDHB and SDHD are the two most common types. Surgery remains the definitive treatment, while radiation therapy is being increasingly used as an adjuvant or primary treatment for surgically challenging or inoperable cases. Our study provided clinical and outcome data on a large cohort of patients with HNPGLs and the results can help provide guidance on improving the care and outcomes of patients with HNPGL. Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed between 1974 and 2023. The mean age of diagnosis was 48.8 years (range 10 to 82) with 69.0% female and 26.5% patients with multiple PGLs. Among 119 patients undergoing genetic testing, 70 (58.8%) patients had mutations, with SDHB (30) and SDHD (26) being the most common. The rates of metastasis and recurrence were higher among patients with SDHB mutations or SDHD mutations associated with multiple PGLs. Metabolic evaluation showed elevated plasma dopamine levels were the most common derangements in HNPGL. MRI and CT were the most common anatomic imaging modalities and DOTATATE was the most common functional scan used in this cohort. Most patients (81.5%) received surgery as the primary definitive treatment, while 22.5% patients received radiation treatment, mostly as an adjuvant therapy or for surgically challenging or inoperable cases. Systemic treatment was rarely used in our cohort. Our single-center experience highlights the need for referral for genetic testing and metabolic evaluation and for a team-based approach to improve the clinical outcomes of patients with HNPGLs. [ABSTRACT FROM AUTHOR]

Abstract Objective To evaluate an endoscopic approach in the management of glomus tumor, and also to investigate and evaluate its appropriateness and feasibility. Methods Glomus tumors, also known as paragangliomas, are benign primary tumors of the middle ear. The advent of endoscopic ear surgery has provided new dimensions to the management of this highly vascular tumor. Retrospective analysis of six patients of glomus tympanicum, operated between July 2014 and June 2019, with modified Fisch classification Type A and B1, who were managed by a retroauricular transcanal endoscopic approach. Preoperative and postoperative analysis was done for these patients. Results The chief complaint was pulsatile tinnitus, which disappeared in five cases and reduced in severity in one of them. Hearing was improved with reduction in air‐bone gap in all the cases. No major complications or recurrence were observed in any of the patients after 12 months of follow‐up. Conclusion This endoscopic approach serves as a safe and reliable technique for tumor removal. It thus provides postoperative comfort for most of the patients.

Purpose: Surgery is a standard therapy for tympanojugular paragangliomas (TJP). Maintaining the quality of life (QoL) requires functional preservation. The flexible CO2 laser allows contact-free tumor removal. This retrospective study compares the postoperative functional outcomes of TJP surgery with and without the flexible CO2 laser. Methods: Between 2005 and 2019, 51 patients with TJP were surgically treated at a tertiary hospital. Until 2012, 17 patients received conventional surgery. Thereafter, the flexible laser was used in 34 patients. Tumor extend, pre- and postoperative cranial nerve function, and complications were compared between the groups. Results: The cohort consisted of 33 class A and B tumors and 18 class C and D tumors. Preoperative embolization was performed in 17 cases. Class C/D TJP were usually removed via an infratemporal fossa type A approach. Gross total tumor removal was achieved in 14/18 class C/D tumors. 3/51 patients suffered from long-term partial or complete facial palsy. No differences in post-therapeutic cranial nerve function or complications were noted between the conventional and laser group. One recurrence was observed after complete tumor resection. Conclusion: The flexible CO2 laser was shown to be a safe and effective alternative to conventional bipolar cauterization, which is appreciated by the surgeon in these highly vascularized tumors. Both techniques allowed a high tumor control rate and good long-term results also from a functional point of view. [ABSTRACT FROM AUTHOR]

Paragangliomas are rare neuroendocrine tumors that arise in sympathetic and parasympathetic paraganglion system, derived from neural crest cells. Tympanic paraganglioma is a type of head and neck paraganglioma involving the middle ear cleft. Endoscopic transcanal approach is currently in vogue for the excision of tympanic paragangliomas that is limited to middle ear cleft. We present a series of 3 cases who underwent endoscopic excision of tympanic paraganglioma.

Paragangliomas can be diagnosed accurately using magnetic resonance imaging and dynamic four-dimensional magnetic resonance angiography. Four-dimensional imaging uses the highly vascularised structure of these tumours, which results in a homogenous capillary blush and usually, due to the arteriovenous shunting, in an early draining vein. By these features the tumour can be differentiated from other neoplasms. The authors describe a case of a paraganglioma localised in the middle ear of an elderly patient. Magnetic resonance contraindications led to preoperative diagnostics with high resolution computed tomography of the temporal bone and additionally computed tomography perfusion imaging instead of magnetic resonance imaging with four-dimensional magnetic resonance angiography. Using the computed tomography perfusion dataset, regions of interest were placed in the carotid artery, the sigmoid sinus and the tympanic mass. In the computer-assisted analysis the tumour showed late arterial enhancement and delayed wash-out compared to the enhancement curves of the carotid artery and the sigmoid sinus. This corresponded to the highly vascularised nature of a paraganglioma. On postoperative follow-up imaging computed tomography perfusion showed almost no enhancement of a small residual tympanic mass, which was then considered to be granulation tissue. In conclusion, in the case of magnetic resonance contraindications the preoperative diagnosis of tympanic paraganglioma can be made using computed tomography imaging criteria alone. Computed tomography perfusion imaging may be helpful in these cases to detect residual or recurrent tumour. [ABSTRACT FROM AUTHOR]

Thoracic and cervical paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of the neural crest progenitors located outside the adrenal gland. We describe our current protocol as a multidisciplinary team for the management of cervical and thoracic PGLs. Surgery is generally considered the treatment of choice as it offers the best chance for cure. For resection of thoracic PGLs, video-assisted thoracoscopic surgery (VATS) is the main surgical approach, while open thoracotomy is preferred in case of tumors > 6cm, lacking confirmation of a plane of separation with adjacent structures, or with technical difficulties during VATS. In cervical PGLs, the surgical approach should be individualized according to location, mainly based on the Glasscock-Jackson and the Fisch-Mattox classifications. Surgery is the treatment of choice for most cervical and thoracic PGLs, but radiotherapy or observation could be more suitable options in unresectable cervical and thoracic PGLs or when resection has been incomplete., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Objective: To evaluate an endoscopic approach in the management of glomus tumor, and also to investigate and evaluate its appropriateness and feasibility., Methods: Glomus tumors, also known as paragangliomas, are benign primary tumors of the middle ear. The advent of endoscopic ear surgery has provided new dimensions to the management of this highly vascular tumor. Retrospective analysis of six patients of glomus tympanicum, operated between July 2014 and June 2019, with modified Fisch classification Type A and B1, who were managed by a retroauricular transcanal endoscopic approach. Preoperative and postoperative analysis was done for these patients., Results: The chief complaint was pulsatile tinnitus, which disappeared in five cases and reduced in severity in one of them. Hearing was improved with reduction in air-bone gap in all the cases. No major complications or recurrence were observed in any of the patients after 12 months of follow-up., Conclusion: This endoscopic approach serves as a safe and reliable technique for tumor removal. It thus provides postoperative comfort for most of the patients., Competing Interests: The authors declare no conflict of interest., (© 2023 The Authors. World Journal of Otorhinolaryngology ‐ Head and Neck Surgery published by John Wiley & Sons Ltd on behalf of Chinese Medical Association.)

Glomus tympanicum (GT) tumors are benign arising from paraganglion cells of the tympanic plexus in the middle ear. Although surgical resection remains the best option for definitive treatment of these tumors, the diagnostic and management algorithms have evolved considerably with the introduction of high-resolution computed tomography, MRI, and genetic testing., (Copyright © 2015 Elsevier Inc. All rights reserved.)