Because of the rarity of glomus jugulare tumors, a variety of treatment paradigms are currently used. There is no consensus regarding the optimal management to control tumor burden while minimizing treatment-related morbidity. In this study, the authors assessed data collected from 869 patients with glomus jugulare tumors from the published literature to identify treatment variables that impacted clinical outcomes and tumor control rates.
A comprehensive search of the English-language literature identified 109 studies that collectively described outcomes for patients with glomus jugulare tumors. Univariate comparisons of demographic information between treatment cohorts were performed to detect differences in the sex distribution, age, and Fisch class of tumors among various treatment modalities. Meta-analyses were performed on calculated rates of recurrence and cranial neuropathy after subtotal resection (STR), gross-total resection (GTR), STR with adjuvant postoperative radiosurgery (STR+SRS), and stereotactic radiosurgery alone (SRS).
The authors identified 869 patients who met their inclusion criteria. In these studies, the length of follow-up ranged from 6 to 256 months. Patients treated with STR were observed for 72 ± 7.9 months and had a tumor control rate of 69% (95% CI 57%-82%). Those who underwent GTR had a follow-up of 88 ± 5.0 months and a tumor control rate of 86% (95% CI 81%-91%). Those treated with STR+SRS were observed for 96 ± 4.4 months and had a tumor control rate of 71% (95% CI 53%-83%). Patients undergoing SRS alone had a follow-up of 71 ± 4.9 months and a tumor control rate of 95% (95% CI 92%-99%). The authors' analysis found that patients undergoing SRS had the lowest rates of recurrence of these 4 cohorts, and therefore, these patients experienced the most favorable rates of tumor control (p < 0.01). Patients who underwent GTR sustained worse rates of cranial nerve (CN) deficits with regard to CNs IX-XI than those who underwent SRS alone; however, the rates of CN XII deficits were comparable.
The authors' analysis is limited by the quality and accuracy of these studies and may reflect source study biases, as it is impossible to control for the quality of the data reported in the literature. Finally, due to the diverse range of data presentation, the authors found that they were limited in their ability to study and control for certain variables. Some of these limitations should be minimized with their use of meta-analysis methods, which statistically evaluate and adjust for between-study heterogeneity. These results provide the impetus to initiate a prospective study, appropriately controlling for variables that can confound the retrospective analyses that largely comprise the existing literature.
"For most glomus tumors, embolization followed by surgical excision remains the first-line intervention (Schopp et al., 2009). Stereotactic radiosurgery and SBRT have also been used to treat solitary, residual, and recurrent glomus jugulare tumors with good results (Lim et al., 2007; Wegner et al., 2010; Guss et al., 2011; Ivan et al., 2011; Sheehan et al., 2012). An extensive literature search revealed only one documentation of external beam radiation therapy being utilized to treat multiple peripheral glomus tumors (Nishimoto et al., 1990). "
[Show abstract][Hide abstract] ABSTRACT: The vast majority of glomangiomas are small, benign neoplasms that can occur anywhere in the body but typically arise in the subcutaneous tissues of the extremities and are capable of causing extreme pain. Typically, these lesions are managed surgically with excellent rates of tumor control. On occasion, patients present with a variant of the glomangioma tumor consisting of numerous or recurrent nodules, a condition classified as glomangiomatosis. The authors present a case report of a young patient with multiply recurrent painful glomangiomas of the left foot, who was ultimately diagnosed with glomangiomatosis pedis. After multiple surgeries and surgical consultations, no surgery other than amputation was recommended. Therefore, the patient sought consultation with regard to stereotactic body radiation therapy (SBRT). In the absence of other options, and based on its effectiveness in treating glomus tumors of the head and neck which display similar natural history and histologic features, SBRT was offered. The patient underwent SBRT to the largest of his remaining tumors with excellent local control and significant reduction in pain at two and a half years follow-up.
Frontiers in Oncology 03/2013; 3:26. DOI:10.3389/fonc.2013.00026
"Radiation has been found to be helpful in controlling glomus jugulare tumour growth by inducing fibrosis around the supplying vessels. In 2011, a comprehensive search of the English-language literature identified 109 studies that collectively described outcomes for patients with glomus jugulare tumours
. Data collected from 869 patients were assessed. "
[Show abstract][Hide abstract] ABSTRACT: Stereotactic radiosurgery (SRS) is an important treatment option for intracranial lesions. Many studies have shown the effectiveness of photon-SRS for the treatment of skull base (SB) tumours; however, limited data are available for proton-SRS.
Several photon-SRS techniques, including Gamma Knife, modified linear accelerators (Linac) and CyberKnife, have been developed and several studies have compared treatment plan characteristics between protons and photons.
The principles of classical radiobiology are similar for protons and photons even though they differ in terms of physical properties and interaction with matter resulting in different dose distributions.
Protons have special characteristics that allow normal tissues to be spared better than with the use of photons, although their potential clinical superiority remains to be demonstrated.
A critical analysis of the fundamental radiobiological principles, dosimetric characteristics, clinical results, and toxicity of proton- and photon-SRS for SB tumours is provided and discussed with an attempt of defining the advantages and limits of each radiosurgical technique.
"These masses are categorized as Fisch C and D tumors (1),(7),(41). Thanks to development and implementation of microsurgical techniques, it is possible to resect these tumors completely, and local tumor control can be achieved in 80–90% of cases (41),(49),(50). JP (Fisch classification C and D) are usually resected via an infratemporal approach. Tumors classified as Fisch C1, C2 and De, Di1/2 may demand a variant of the juxtacondylar approach (14). "
[Show abstract][Hide abstract] ABSTRACT: Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I-III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.
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