Treatment decision and estimation of growth of head and neck paragangliomas

PurposeHead and neck paragangliomas are slow growing tumors where observation has become more widely accepted. Tumor growth rate as well as predictors of increased tumor growth were analyzed with the goal to identify factors to better predict disease progression and counsel patients.Materials and methodsMulti-institutional retrospective cohort study from 2011 to 2020.Results130 head and neck paragangliomas in 125 patients were analyzed. 38 were observed (30.4%), 16 radiated (12.8%), and 71 underwent surgery (56.8%). Surgical patients were significantly younger (p = 0.038) and with more genetically mediated paragangliomas (p = 0.026). Significantly more patients were asymptomatic in the observation group (p = 0.005). Of the 39 observed tumors, 43.6% (n = 17) grew with a tumor doubling time of 5.67 years. More than half of the observed paragangliomas had no growth. When examining symptoms postoperatively and at follow-up, the surgical cohort had significantly more worsening symptoms (p = 0.007) and new cranial neuropathies (p = 0.031).ConclusionsHead and neck paragangliomas have slow growth rates if they grow at all. Patients in the surgical cohort had more clinical symptoms at presentation and worsening postoperative symptoms.     

Multiple paragangliomas of the head and neck

Multiple paragangliomas of the head and neck are rare conditions. The incidence of multiple paragangliomas is reported to the approximately 10% of the total patients, but in familial cases it increases up to 35-50%. In the head and neck region, the most common association is represented by bilateral carotid body tumors or by carotid body tumor associated with tympanic-jugular glomus. The presence of three synchronous glomus tumors is really rare, as well as association with vagal glomus and carotid body. In this paper the authors present a patient affected ipsilaterally by a carotid body tumor and vagal paraganglioma, focusing on the diagnostic options offered by imaging techniques (CT and MRI).     

头颈部副神经节瘤致病基因研究进展

副神经节瘤(paraganglioma,PGL)是一组起源于外胚层神经嵴细胞,分布于从颅底至盆腔和骶椎的神经源性肿瘤,临床表现因肿瘤部位不同而各有特点.头颈部副神经节瘤多来自副交感神经节细胞,无内分泌功能,曾称为化学感受器瘤或非嗜铬副神经节瘤.     

What we call them: the nomenclature of head and neck paragangliomas

? Historically, paragangliomas have been referred to by a number of different terms (‘glomus’ tumours of different sorts, ‘carotid body tumour’). ? WHO classification (2005) refers to these tumours as paragangliomas, with further anatomical detail (‘vagal paraganglioma’, ‘carotid body paraganglioma’). ? This system recognises the commonality between these tumours and aids understanding of their pathogenesis and clinical management.     

Cellular aspects of SDHD mutations in head and neck paragangliomas

Introduction. Mutations in the SDHD gene, a nuclear gene encoding for a mitochondrial protein involved in oxidative phosphorylation, are responsible for the majority of hereditary head and neck paragangliomas. The aim was characterization of cellular manifestations of SDHD mutations in paragangliomas. Methods. Tumours of patients with and without inherited mutations were examined using electron microscopy, enzyme histochemistry and immunohistochemistry. Results. Ultrastructurally, large numbers of mitochondria with disturbed morphology were detected. In almost all cases, the SDH enzyme activity was virtually absent. The expression of two catalytic proteins in the SDH complex displayed two general patterns: the flavo protein was strongly upregulated, whereas the iron protein was reduced in the tumour cells. However, in some cases deviations from these patterns in enzyme activity and protein expression were observed. Conclusion. Cellular aspects of SDHD mutations are composed of increased numbers of mitochondria with disturbed morphology, reduced SDH enzyme activity and aberrant expression of catalytic components of the SDH complex. In some cases, deviations from these patterns are observed. Analysis of histopathological changes will lead to further understanding of the biology of paragangliomas and contribute to clinical treatment strategies in the future.     

Current trends in the diagnosis and management of head and neck paragangliomas

PURPOSE OF REVIEW: The purpose of this review is to provide an overview of the diagnosis and management of head and neck paragangliomas. RECENT FINDINGS: The review will focus on three main areas of interest: genetics, newer imaging modalities, and management with radiation therapy. SUMMARY: The succinate dehydrogenase enzyme complex is important in the pathogenesis of paragangliomas. Magnetic resonance angiography and functional imaging have become very helpful in diagnosing these tumors. Surgery is still the mainstay of therapy, but conventional or stereotactic radiotherapy seems to be a very safe option in very large or inoperable tumors.     

Malignant schwannoma in the head and neck

The authors' experience in dealing with three cases of malignant Schwannoma in the head and neck is outlined. The symptomatology of this tumor is discussed with its investigation and management. A correct diagnosis with a CT scan to delineate tumor extent is imperative. A wide surgical resection is the preferred method of treatment with postoperative irradiation of value for residual microscopic disease. The pernicious nature of this tumor often leads to a dismal outcome despite aggressive therapy.     

Malignant melanoma of the head and neck

The authors present 29 cases of malignant melanoma of the head and neck, treated at the ENT Department of S. Zeromski's Hospital in Kraków. The localisation, symptoms, diagnostic methods and treatment are discussed.     

Experiences in the treatment of patients with multiple head and neck paragangliomas

Purpose

To analyze treatment results in the multidisciplinary management of patients with multiple head and neck paragangliomas (HNPs).

Methods

Retrospective analysis including all patients with multiple HNPs (VP, vagal paraganglioma; JTP, jugulotympanic paraganglioma; CBT, carotid body tumor) treated between 2000 and 2013 at a tertiary referral center.

Results

Ten patients (three men, seven women) had 25 HNPs (two VPs, eight JTPs, and 15 CBTs). The age range at diagnosis was 31–71 years (mean 40.9 years, median 37 years). Nine tumors (four CBTs, three JTPs, two VPs) were treated only with stereotactic radiotherapy (SRT; 50.4–56 Gy, mean 55.3 Gy) or in one case intensity-modulated radiotherapy (60 Gy). Nine tumors were treated with surgery alone (eight CBTs, one JTP) and three JTPs with subtotal surgery combined with adjuvant SRT. A “wait and scan” strategy was used in three cases (two CBTs, one JTP). The mean follow-up period was 4.3 years (range 0.1–13 years, median 4 years). The rate of tumor control with surgery and/or SRT was 100% (21/21). One patient with a wait-and-scan strategy for CBT had slow asymptomatic progression during a 13-year follow-up.

Conclusions

The treatment results in this series of patients with multiple HNPs show that a very high rate of long-term tumor control with low morbidity can be achieved using tailored and individualized approaches. All of the different treatment strategies available should be discussed with the patient. In particular, the treatment should involve a multidisciplinary team of experts in the fields of nuclear medicine, genetics, pathology, radiology, radio-oncology, and surgery.