Function‐preserving therapy for jugulotympanic paragangliomas: A retrospective analysis from 2000 to 2010

Künzel, Julian; Iro, Heinrich; Hornung, Joachim; Koch, Michael; Brase, Christoph; Klautke, Günther; Zenk, Johannes

doi:10.1002/lary.23268

Cited by 33 publications

(35 citation statements)

References 44 publications

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“…Immediate postoperative facial weakness is frequent, and long-term dysfunction is present in 14-33% of the cases (Briner et al 1999, Huy et al 2009). Hearing function either remains stable or deteriorates; it is subjectively improved in only 6-16% of the cases, while 39% may experience a deterioration (Glassock et al 1979, Gjuric et al 1996, Briner et al 1999, Kunzel et al 2012. Overall, up to 45.5% of the cases have some degree of hearing loss after surgery (Suarez et al 2013a).…”

Section: Jtpglsmentioning

confidence: 99%

The management of head-and-neck paragangliomas

Căpățînă

Ntali

Karavitaki

et al. 2013

Endocrine-Related Cancer

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Paragangliomas (PGLs) are tumours originating from neural crest-derived cells situated in the region of the autonomic nervous system ganglia. Head-and-neck PGLs (HNPGLs) originate from the sympathetic and parasympathetic paraganglia, most frequently from the carotid bodies and jugular, tympanic and vagal paraganglia, and are usually non-catecholamine secreting. Familial PGLs are considered to be rare, but recently genetic syndromes including multiple PGLs and/or phaeochromocytomas have been more thoroughly characterised. Nowadays, genetic screening for the genes frequently implicated in both familial and sporadic cases is routinely being recommended. HNPGLs are mostly benign, generally slow-growing tumours. Continuous growth leads to the involvement of adjacent neurovascular structures with increased morbidity rates and treatment-related complications. Optimal management mostly depends on tumour location, local involvement of neurovascular structures, estimated malignancy risk, patient age and general health. Surgery is the only treatment option offering the chance of cure but with significant morbidity rates, so a more conservative approach is usually considered, especially in the more difficult cases. Radiotherapy (fractionated or stereotactic radiosurgery) leads to tumour growth arrest and symptomatic improvement in the short term in many cases, but the longterm consequences are unclear. Early detection is essential in order to increase the chance of cure with a lower morbidity rate. The constant improvement in diagnostic imaging, surgical and radiation techniques has led to a safer management of these tumours, but there are still many therapeutic challenges, and no treatment algorithm has been agreed upon until now. The management of HNPGLs requires a multidisciplinary effort addressing the genetic, surgical, radiotherapeutic, oncological, neurological and endocrinological implications. Further progress in the understanding of their pathogenesis will lead to more effective screening and earlier diagnosis, both critical to successful treatment.

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Section: Jtpglsmentioning

confidence: 99%

The management of head-and-neck paragangliomas

Căpățînă

Ntali

Karavitaki

et al. 2013

Endocrine-Related Cancer

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“…27,58,93 Whether SRS is used as a primary or salvage treatment modality is dictated by tumor location, the patient's preoperative status, and protocols at respective medical centers. Nonetheless, as a primary treatment modality, radiosurgery achieves local control rates comparable to those achieved with surgical approaches, while In normoxic conditions, the proline residues of hypoxia-inducible factor 1-a (HIF-1-a) are hydroxylated by prolyl hydroxylases, which use oxygen as a cofactor.…”

Section: Stereotactic Radiosurgerymentioning

confidence: 99%

“…1A and C), 28,40,65,66 radiosurgery has become increasingly popular for residual tumors and cases not amenable to surgery. 27,58,93 Due to their highly vascular nature (Fig. 1B) and tendency to bleed intraoperatively, preoperative coil embolization has been used for SHN-PGs.…”

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confidence: 99%

Molecular genetics of paragangliomas of the skull base and head and neck region: implications for medical and surgical management

Hussain¹,

Husain²,

Baredes

et al. 2014

JNS

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Paragangliomas are rare neuroendocrine tumors derived from neural crest cells of the autonomic nervous system. Tumors occurring in the thorax, abdomen, and pelvis are usually derived from sympathetic paraganglia, which are associated with catecholaminemia, headaches, and resistant hypertension. Pheochromocytomas, arising from chromaffin cells of the adrenal medulla, are histologically similar to sympathetic paragangliomas and can have identical clinical presentations. In contrast to these tumors, skull base and head and neck region paragangliomas (SHN-PGs) are usually derived from parasympathetic paraganglia and rarely secrete catecholamines. These tumors can present in a variety of locations, most commonly arising from glomus Type I (chief) cells of the carotid body. Less commonly they arise in regions of the temporal bone including the middle ear (glomus tympanicum) and jugular fossa (glomus jugulare) (Fig. 1A), as well as along the length of the vagus nerve (glomus vagale) and in the nose and paranasal sinuses (sinonasal paraganglioma). 60,69,75,77,93,99 These tumors Paragangliomas are rare, slow-growing tumors that frequently arise in the head and neck, with the carotid bodies and temporal bone of the skull base being the most common sites. These neoplasms are histologically similar to pheochromocytomas that form in the adrenal medulla and are divided into sympathetic and parasympathetic subtypes based on functionality. Skull base and head and neck region paragangliomas (SHN-PGs) are almost always derived from parasympathetic tissue and rarely secrete catecholamines. However, they can cause significant morbidity by mass effect on various cranial nerves and major blood vessels. While surgery for SHN-PG can be curative, postoperative deficits and recurrences make these lesions challenging to manage. Multiple familial syndromes predisposing individuals to development of paragangliomas have been identified, all involving mutations in the succinate dehydrogenase complex of mitochondria. Mutations in this enzyme lead to a state of "pseudohypoxia" that upregulates various angiogenic, survival, and proliferation factors. Moreover, familial paraganglioma syndromes are among the rare inherited diseases in which genomic imprinting occurs. Recent advances in gene arrays and transcriptome/exome sequencing have identified an alternate mutation in sporadic SHN-PG, which regulates proto-oncogenic pathways independent of pseudohypoxia-induced factors. Collectively these findings demonstrate that paragangliomas of the skull base and head and neck region have a distinct genetic signature from sympathetic-based paragangliomas occurring below the neck, such as pheochromocytomas. Paragangliomas serve as a unique model of primarily surgically treated neoplasms whose future will be altered by the elucidation of their genomic complexities. In this review, the authors present an analysis of the molecular genetics of SHN-PG and provide future directions in patient care and the development of novel therapies. 321Abbreviations used in ...

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“…In general, patients either maintain function of their cranial nerves or have improvement in their dysfunction after treatment but many of these studies included patients treated with multiple types of surgical and/or radiation treatment types. 6,12,13,22–26 Our study demonstrated overall stability of cranial nerve function using single modality GKRS. Those with lower cranial neuropathies were rehabilitated well (i.e.…”

Section: Discussionmentioning

confidence: 53%

Treatment Outcomes for Single Modality Management of Glomus Jugulare Tumors With Stereotactic Radiosurgery

Dobberpuhl

Maxwell²,

Feddock³

et al. 2016

Otology &Amp; Neurotology

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Objectives The objectives were to evaluate the audiological outcomes, response of symptoms, and response of tumor volume in patients with glomus jugulare tumors treated solely with single fraction gamma knife radiosurgery. Study Design Single institution retrospective review. Setting Academic, tertiary referral center. Patients The diagnosis code for glomus jugulare was used to identify patients. Only those who underwent gamma knife radiosurgery were included. Those previously treated with any modality were excluded. A total of 12 patients were included for the tumor response and symptom response data and 7 of those were included in the audiometric analysis. Main Outcomes Measures Audiometric data at most recent follow up compared to presentation, subjective improvement in pulsatile tinnitus, and change in tumor volume at most recent follow up compared to pretreatment. Results The average time to most recent follow up was 27.6 months. There was no significant change in pure tone average or word recognition. Pulsatile tinnitus completely resolved or improved in 80% of patients. Cranial neuropathies were stable or improved. A single patient experienced facial nerve paresis 2 years after treatment, which resolved with steroid treatment. Tumor control was 100% and the average change in tumor volume was a decrease of 37%. Conclusion Single modality Gamma Knife radiosurgery treatment of glomus jugulare tumors appears to be safe. Treatment results in decreased tumor volume and improved pulsatile tinnitus in most patients. There was no significant progression of hearing loss after treatment. Lower cranial nerve function remains stable in all patients.

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Function‐preserving therapy for jugulotympanic paragangliomas: A retrospective analysis from 2000 to 2010

Abstract: With these high tumor control rates, consistent with the literature, function-preserving therapy for JTPs currently appears to offer the greatest benefit for patients.

Cited by 33 publications

References 44 publications

The management of head-and-neck paragangliomas

The management of head-and-neck paragangliomas

Molecular genetics of paragangliomas of the skull base and head and neck region: implications for medical and surgical management

Treatment Outcomes for Single Modality Management of Glomus Jugulare Tumors With Stereotactic Radiosurgery

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