Glomus Jugularis Tumors

Steinberg, N. G.; Holz, W. G.

doi:10.1001/archotol.1965.00760010389009

Cited by 15 publications

(5 citation statements)

References 4 publications

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“…17 The other fact is that the functional consequences of a lower cranial nerve injury are far more significant than injury to other cranial nerves. One is that, although malignant paragangliomas do occur, the majority of patients have benign tumors that grow very slowly.…”

Section: Discussionmentioning

confidence: 99%

Stereotactic radiosurgery in patients with glomus jugulare tumors

Pollock¹

2004

FOC

105

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Object Microsurgical removal of glomus jugulare tumors is frequently associated with injury of the lower cranial nerves. To decrease the morbidity associated with tumor management in these patients, gamma knife surgery (GKS) was performed as an alternative to resection. Methods Between 1990 and 2003, 42 patients underwent GKS as the primary management (19 patients) or for recurrent glomus jugulare tumors (23 patients). Facial weakness and deafness were more common in patients with recurrent tumors than in those in whom primary GKS was performed (48% compared with 11%, p = 0.02). The mean tumor volume was 13.2 cm3; the mean tumor margin dose was 14.9 Gy. The mean follow-up period for the 39 patients in whom evaluation was possible was 44 months (range 6–149 months). After GKS, 12 tumors (31%) decreased in size, 26 (67%) were unchanged, and one (2%) grew. The patient whose tumor grew underwent repeated GKS. Progression-free survival after GKS was 100% at 3 and 7 years, and 75% at 10 years. Six patients (15%) experienced new deficits (hearing loss alone in three, facial numbness and hearing loss in one, vocal cord paralysis and hearing loss in one, and temporary imbalance and/or vertigo in one). In 26 patients in whom hearing could be tested before GKS, hearing preservation was achieved in 86 and 81% at 1 and 4 years posttreatment, respectively. No patient suffered a new lower cranial nerve deficit after one GKS session; the patient in whom repeated GKS was performed experienced a new vocal cord paralysis 1 year after his second procedure. Conclusions Gamma knife surgery provided tumor control with a low risk of new cranial nerve injury in early follow-up review. This procedure can be safely used as a primary management tool in patients with glomus jugulare tumors that do not have significant cervical extension, or in patients with recurrent tumors in this location.

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Section: Discussionmentioning

confidence: 99%

Stereotactic radiosurgery in patients with glomus jugulare tumors

Pollock¹

2004

FOC

105

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“…Wie auch bei den zervikalen Paragangliomen ist die mittlere Wachstumsgeschwindigkeit jugulotympanaler Paragangliome in der Regel gering [49,59] . In der Literatur sind Patienten beschrieben, die auch ohne Operation oder Radiatio Ü berlebenszeiten von mehr als 40 Jahren zeigten [117] . Eine " wait and scan " Strategie sollte deshalb insbesondere bei ä lteren Patienten mit multiplen internistischen Vorerkrankungen als alternative Therapieoption ber ü cksichtigt werden [59] .…”

Section: Alternative Therapieans ä Tzeunclassified

Paragangliome und Paragangliomsyndrome

Boedeker

2011

Laryngo-Rhino-Otol

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Paragangliomas are rare tumors of neural crest origin. They are benign in the majority of cases and are characterized by a strong vascularisation. In the head and neck region they most commonly occur as carotid body tumors. Jugulotympanic and especially vagal paragangliomas are seen less frequently. Complete surgical resection represents the only curative treatment option even though resection of locally advanced tumors regularly results in lesions of the lower cranial nerves and major vessels. Approximately 30% of all head and neck paragangliomas (HNPs) are hereditary and associated with different tumor syndromes. The paraganglioma syndromes 1, 3 and 4 (PGL 1, 3 and 4) make up the majority of those familial cases. PGL 1 is associated with mutations of the succinate dehydrogenase subunit D (SDHD) gene, PGL 3 is caused by SDHC and PGL 4 by SDHB gene mutations. Multiple HNPs and the occurrence of HNPs together with pheochromocytomas are seen in SDHD as well as SDHB mutation carriers. In patients with SDHB mutations the risk for the development of malignant paraganglial tumors is significantly higher compared to SDHC and SDHD patients as well as patients with sporadic tumors. SDHC mutation carriers almost exclusively present with benign HNPs that are unifocal in the majority of cases. The role of transmission is autosomal dominant for all 3 symptoms. Interestingly, there is a "parent-of-origin-dependent-inheritance" in subjects with SDHD gene mutations. This means that the disease phenotype may only become present if the mutation is inherited through the paternal line. We recommend screening for mutations of the genes SDHB, SDHC and SDHD in all patients with HNPs. Certain clinical parameters can help to set up the order in which the 3 genes should be tested.

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“…There are reports of patients in the literature that survived without any kind of treatment for more than 40 years [115]. …”

Section: Discussionmentioning

confidence: 99%