scholarly journals Head and Neck Paragangliomas in the Czech Republic: Management at the Otorhinolaryngology Department

Diagnostics ◽  
2021 ◽  
Vol 12 (1) ◽  
pp. 28
Author(s):  
Anasuya Guha ◽  
Martin Chovanec
Keyword(s):  
Head And Neck ◽  
Cranial Nerve ◽  
Case Series ◽  
Clinical Aspects ◽  
Benign Tumors ◽  
Tumor Control ◽  
Cranial Nerve Dysfunction ◽  
Critical Issues ◽  
Head And Neck Paragangliomas ◽  
Jugular Paragangliomas

Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors, comprising only 3% of all head and neck tumors. Early diagnosis forms an integral part of the management of these tumors. The two main aims of any treatment approach are long-term tumor control and minimal cranial nerve morbidity. The scope of this article is to present our case series of HNPGLs to stress most important clinical aspects of their presentation as well as critical issues of their complex management. Thirty patients with suspected HNPGLs were referred to our otorhinolaryngology clinic for surgical consultation between 2016–2020. We assessed the demographical pattern, clinicoradiological correlation, as well as type and outcome of treatment. A total of 42 non-secretory tumors were diagnosed—16.7% were incidental findings and 97% patients had benign tumors. Six patients had multiple tumors. Jugular paragangliomas were the most commonly treated tumors. Tumor control was achieved in nearly 96% of operated patients with minimal cranial nerve morbidity. Surgery is curative in most cases and should be considered as frontline treatment modality in experienced hands for younger patients, hereditary and secretory tumors. Cranial nerve dysfunction associated with tumor encasement is a negative prognostic factor for both surgery and radiotherapy. Multifocal tumors and metastasis are difficult to treat, even with early detection using genetic analysis. Detecting malignancy in HNPGLs is challenging due to the lack of histomorphological criteria; therefore, limited lymph node dissection should be considered, even in the absence of clinical and radiological signs of metastasis in carotid body, vagal, and jugular paragangliomas.

Active surveillance management of head and neck paragangliomas: case series and review of the literature

2017 ◽  
Vol 131 (7) ◽  
pp. 580-584 ◽  
Author(s):  
L Harrison ◽  
R Corbridge
Keyword(s):  
Head And Neck ◽  
Cranial Nerve ◽  
Relevant Literature ◽  
Case Series ◽  
Surgical Excision ◽  
Major Surgery ◽  
Review Of The Literature ◽  
Lower Cranial Nerve ◽  
Cranial Nerve Palsies ◽  
Head And Neck Paragangliomas

AbstractBackground:Head and neck paragangliomas are rare. They are usually slow-growing, benign, non-catecholamine secreting tumours, traditionally treated with surgical excision. Complications of surgical excision include lower cranial nerve palsies, stroke and death.Method:A retrospective case note analysis was conducted of patients with head and neck paragangliomas treated with a watch-and-scan policy from March 2003 to September 2015, and the relevant literature was reviewed.Results:Fifteen head and neck paragangliomas were identified. None of the patients developed a new lower cranial nerve palsy or progression of their presenting hearing loss during the follow-up period. Five patients displayed an increase in maximum linear dimension of 4 mm over an average of 57.4 months. A review of the literature showed that a watch-and-surveillance scan policy is evolving as a treatment option for head and neck paragangliomas without malignant risk factors.Conclusion:Readily available surveillance scanning in head and neck paragangliomas enables the monitoring of head and neck paragangliomas, which may allow for avoidance of major surgery.

scholarly journals SDHB mutated paraganglioma imitating thyroid tumor: A case report and review of literature

2018 ◽  
Vol 5 (2) ◽  
pp. 5
Author(s):  
Vygante Maskoliunaite ◽  
Gabija Makunaite ◽  
Ausra Garnelyte ◽  
Agne Abraitiene ◽  
Virgilijus Beisa
Keyword(s):  
Head And Neck ◽  
Screening Method ◽  
Thyroid Tumor ◽  
Benign Tumors ◽  
Review Of Literature ◽  
Palpable Mass ◽  
Parasympathetic Ganglia ◽  
Sdh Mutation ◽  
Head And Neck Paragangliomas ◽  
Immunohistochemical Testing

Paragangliomas of the head and neck are uncommon tumors arising from parasympathetic ganglia. Paragangliomas are mostly asymptomatic and may manifest as palpable mass of neck. The morphologic features are non-specific and comparable to the other neuroendocrine tumors. Most of hereditary cases are associated with alterations in genes of succinate dehydrogenase (SDH). SDHA and SDHB immunohistochemistry is considered as reliable screening method to detect tumors with genetical alterations. Of note, SDHB mutated paragangliomas have the highest risk of local recurrence, distant metastasis and the development of other tumor phenotypes, which are associated with mutation. Therefore, active surveillance of patients and early surgical treatment are essential. In contrast, SDHB mutated head and neck paragangliomas was considered as completely benign tumors, although, the latest literature describes more controversial cases, which may increase awareness. Here, we present a rare case of 21 years old female with asymptomatic neck paraganglioma, which was unexpectedly diagnosed after pathological and immunohistochemical testing of removed thyroid gland and showed unusual immunohistochemical variation for SDH mutation.

Effects of octreotide therapy in progressive head and neck paragangliomas: Case series

Head & Neck ◽  
2013 ◽  
Vol 35 (12) ◽  
pp. E391-E396 ◽  
Author(s):  
Leonie T. van Hulsteijn ◽  
Nicolette van Duinen ◽  
Berit M. Verbist ◽  
Jeroen C. Jansen ◽  
Agatha A. van der Klaauw ◽  
...  
Keyword(s):  
Head And Neck ◽  
Case Series ◽  
Octreotide Therapy ◽  
Head And Neck Paragangliomas

TRANSSPHENOIDAL DECOMPRESSION OF THE SELLAR FLOOR FOR CAVERNOUS SINUS MENINGIOMAS

Neurosurgery ◽  
2009 ◽  
Vol 65 (1) ◽  
pp. 54-62 ◽  
Author(s):  
Hiroyoshi Akutsu ◽  
Jürgen Kreutzer ◽  
Rudolf Fahlbusch ◽  
Michael Buchfelder
Keyword(s):  
Cavernous Sinus ◽  
Cranial Nerve ◽  
Adjuvant Radiotherapy ◽  
Complete Recovery ◽  
Endocrine Function ◽  
Hormone Deficiency ◽  
Tumor Control ◽  
Cranial Nerve Dysfunction ◽  
Cranial Nerve Function

ABSTRACT OBJECTIVE The aim of this study was to assess the outcome of cranial nerve and endocrine function after transsphenoidal decompression for symptomatic cavernous sinus (CS) meningioma. METHODS Between 1991 and 2007, 21 patients (19 women and 2 men; mean age, 51.1 ± 10.6 years) harboring symptomatic CS meningiomas underwent transsphenoidal decompression. Sufficient bone removal, opening of the inferomedial wall of the CS, and tumor debulking were performed. RESULTS Notably, the grading of preoperative optomotoric paresis improved in 15 of the 17 patients who presented with that symptom. Complete recovery could be achieved in 8 patients. Complete recovery rates in patients with preoperative grading of “good,” “fair,” and “poor” were 77.7%, 20%, and 0%, respectively (P = 0.0088). Improvement of cranial nerve dysfunction was found in 32 of 34 deficits. No worsening of cranial nerve function occurred. Endocrinologically, the prolactin level was normalized in 13 of the 17 patients with preoperative hyperprolactinemia. Recovery of growth hormone deficiency and hypogonadism were found in 3 patients (37.5%) and 1 patient (33.3%), respectively. Seventeen patients were followed for more than 3 years. Of these 17 patients, 12 patients received initial postoperative adjuvant radiotherapy. The overall tumor control rate after surgery with initial adjuvant radiotherapy was 100% (median follow-up, 65 months; range, 36–126 months). CONCLUSION Transsphenoidal decompression is a safe and effective treatment to improve cranial nerve and endocrine dysfunction in patients with symptomatic CS meningiomas. The less severe optomotoric nerve palsy before surgery, the better the chance of complete recovery of its function. Combined with adjuvant radiotherapy, this minimally invasive management also provided excellent long-term tumor control.

Preoperative Acetazolamide Challenge in Pediatric Chiari I Malformation

2021 ◽  
pp. 1-6
Author(s):  
Vincent Rossi ◽  
Scott Wait ◽  
Sarah Jernigan ◽  
Mark Van Poppel ◽  
Deborah Pfortmiller ◽  
...  
Keyword(s):  
Pain Score ◽  
Cranial Nerve ◽  
Challenge Test ◽  
Case Series ◽  
Chiari I Malformation ◽  
Inclusion Criteria ◽  
Diagnostic Dilemma ◽  
Chiari 1 Malformation ◽  
Cranial Nerve Dysfunction ◽  
Acetazolamide Challenge

<b><i>Purpose:</i></b> In patients who present with headaches and Chiari 1 malformation without cranial nerve or brainstem dysfunction or syrinx formation, the decision to decompress can be difficult to make. We present a case series examining the use of acetazolamide as a diagnostic aid to determine candidacy for decompression. <b><i>Methods:</i></b> A single pediatric neurosurgeon’s (M.M.H.) experience from 2003 to 2018 was retrospectively reviewed. All cases evaluated in the clinic for CPT code of Chiari 1 malformation were analyzed. Inclusion criteria were patients with diagnosis of Chiari 1 malformation and headache-predominant symptoms who underwent an acetazolamide challenge test. Exclusion criteria included age over 18, syrinx, or significant clinical evidence of brainstem compression or cranial nerve dysfunction. Data were recorded with respect to response to acetazolamide. Surgical outcomes were assessed using the Chicago Chiari Outcome Scale (CCOS) pain category and the improve/same/worse (IWS) scale. Overall satisfaction from surgery was also recorded. <b><i>Results:</i></b> Forty-five patients met inclusion criteria and underwent acetazolamide challenge. Thirty-three (73.3%) patients showed improvement of their symptoms with acetazolamide challenge (responders). Of the 33 responders, 25 (75.8%) underwent Chiari decompression. The remaining 8 (24.2%) responders experienced persistent improvement of their symptoms after the acetazolamide trial and did not require intervention. Twelve (26.7%) patients did not improve with acetazolamide (nonresponders) of which 11 (91.7% of nonreponders) never required intervention. One nonresponder eventually underwent Chiari decompression due to progressively worsening and debilitating headaches. All twenty-five (100%) responders improved after surgery and 24 (96%) were satisfied with the outcome of surgery. The average responder CCOS pain score was 3.52 out of 4. The nonresponder who did not improve with acetazolamide had persistent severe headaches after decompression. The CCOS pain score for this nonresponder was 2 out of 4. <b><i>Conclusion:</i></b> Pediatric headache-predominant Chiari 1 malformation presents a difficult diagnostic dilemma. The authors describe a trial of acetazolamide as a preoperative diagnostic tool to aid the decision to decompress. Further studies will need to be performed to determine the effectiveness of preoperative acetazolamide in identifying which patients may benefit from surgical decompression.

scholarly journals Repeat stereotactic radiosurgery for progressive vestibular schwannomas after previous radiosurgery: a systematic review and meta-analysis

2021 ◽  
Author(s):  
Anne Balossier ◽  
Jean Régis ◽  
Nicolas Reyns ◽  
Pierre-Hugues Roche ◽  
Roy Thomas Daniel ◽  
...  
Keyword(s):  
Systematic Review ◽  
Hearing Loss ◽  
Facial Nerve ◽  
Stereotactic Radiosurgery ◽  
Cranial Nerve ◽  
Meta Analysis ◽  
Cranial Nerves ◽  
Vestibular Schwannomas ◽  
Benign Tumors ◽  
Tumor Control

AbstractVestibular schwannomas (VS) are slow-growing intracranial extraaxial benign tumors, developing from the vestibular part of the eight cranial nerves. Stereotactic radiosurgery (SRS) has now a long-term scientific track record as first intention treatment for small- to medium-sized VS. Though its success rate is very high, SRS for VS might fail to control tumor growth in some cases. However, the literature on repeat SRS after previously failed SRS remains scarce and reported in a low number of series with a limited number of cases. Here, we aimed at performing a systematic review and meta-analysis of the literature on repeat SRS for VS. Using PRISMA guidelines, we reviewed manuscripts published between January 1990 and October 2020 and referenced in PubMed. Tumor control and cranial nerve outcomes were evaluated with separate meta-analyses. Eight studies comprising 194 patients were included. The overall rate of patients treated in repeat SRS series as per overall series with first SRS was 2.2% (range 1.2–3.2%, p < 0.001). The mean time between first and second SRS was 50.7 months (median 51, range 44–64). The median marginal dose prescribed at first SRS was 12 Gy (range 8–24) and at second SRS was 12 Gy (range 9.8–19). After repeat SRS, tumor stability was reported in 61/194 patients, i.e., a rate of 29.6% (range 20.2–39%, I2 = 49.1%, p < 0.001). Tumor decrease was reported in 83/194 patients, i.e., a rate of 54.4% (range 33.7–75.1%, I2 = 89.1%, p < 0.001). Tumor progression was reported in 50/188 patients, i.e., a rate of 16.1% (range 2.5–29.7%, I2 = 87.1%, p = 0.02), rarely managed surgically. New trigeminal numbness was reported in 27/170 patients, i.e., a rate of 9.9% (range 1.4–18.3%, p < 0.02). New facial nerve palsy of worsened of previous was reported in 8/183 patients, i.e., a rate of 4.3% (range 1.4–7.2%, p = 0.004). Hearing loss was reported in 12/22 patients, i.e., a rate of 54.3% (range 24.8–83.8%, I2 = 70.7%, p < 0.001). Repeat SRS after previously failed SRS for VS is associated with high tumor control rates. Cranial nerve outcomes remain favorable, particularly for facial nerve. The rate of hearing loss appears similar to the one related to first SRS.

scholarly journals Clinical aspects of SDHx-related pheochromocytoma and paraganglioma

2009 ◽  
Vol 16 (2) ◽  
pp. 391-400 ◽  
Author(s):  
Henri J L M Timmers ◽  
Anne-Paule Gimenez-Roqueplo ◽  
Massimo Mannelli ◽  
Karel Pacak
Keyword(s):  
Head And Neck ◽  
Susceptibility Gene ◽  
Clinical Aspects ◽  
Apoptosis Resistance ◽  
Neuronal Precursor Cells ◽  
Positron Emission ◽  
Genes Encoding ◽  
Head And Neck Paragangliomas ◽  
Chromaffin Tissue

Paragangliomas (PGLs) derive from either sympathetic chromaffin tissue in adrenal and extra-adrenal abdominal or thoracic locations, or from parasympathetic tissue of the head and neck. Mutations of nuclear genes encoding subunits B, C, and D of the mitochondrial enzyme succinate dehydrogenase (SDHB 1p35-p36.1, SDHC 1q21, SDHD 11q23) give rise to hereditary PGL syndromes PGL4, PGL3, and PGL1 respectively. The susceptibility gene for PGL2 on 11q13.1 remains unidentified. Mitochondrial dysfunction due to SDHx mutations have been linked to tumorigenesis by upregulation of hypoxic and angiogenesis pathways, apoptosis resistance and developmental culling of neuronal precursor cells. SDHB-, SDHC-, and SDHD-associated PGLs give rise to more or less distinct clinical phenotypes. SDHB mutations mainly predispose to extra-adrenal, and to a lesser extent, adrenal PGLs, with a high malignant potential, but also head and neck paragangliomas (HNPGL). SDHD mutations are typically associated with multifocal HNPGL and usually benign adrenal and extra-adrenal PGLs. SDHC mutations are a rare cause of mainly HNPGL. Most abdominal and thoracic SDHB-PGLs hypersecrete either norepinephrine or norepinephrine and dopamine. However, only some hypersecrete dopamine, are biochemically silent. The biochemical phenotype of SDHD-PGL has not been systematically studied. For the localization of PGL, several positron emission tomography (PET) tracers are available. Metastatic SDHB-PGL is the best localized by [18F]-fluorodeoxyglucose PET. The identification of SDHx mutations in patients with PGL is warranted for a tailor-made approach to the biochemical diagnosis, imaging, treatment, follow-up, and family screening.

scholarly journals Effectiveness and Toxicity of Fractionated Proton Beam Radiotherapy for Cranial Nerve Schwannoma Unsuitable for Stereotactic Radiosurgery

2021 ◽  
Vol 11 ◽  
Author(s):  
Tanja Eichkorn ◽  
Sebastian Regnery ◽  
Thomas Held ◽  
Dorothea Kronsteiner ◽  
Juliane Hörner-Rieber ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Proton Beam ◽  
Cranial Nerve ◽  
Tumor Control ◽  
Special Importance ◽  
Nerve Function ◽  
Proton Beam Radiotherapy ◽  
Cranial Nerve Dysfunction ◽  
Cranial Nerve Function

PurposeIn this benign tumor entity, preservation of cranial nerve function is of special importance. Due to its advantageous physical properties, proton beam radiotherapy (PRT) is a promising approach that spares healthy tissue. Could PRT go along with satisfactory preservation rates for cranial nerve function without compromising tumor control in patients with cranial nerve schwannoma unsuitable for stereotactic radiosurgery?MethodsWe analyzed 45 patients with cranial nerve schwannomas who underwent PRT between 2012 and 2020 at our institution. Response assessment was performed by MRI according to RECIST 1.1, and toxicity was graded following CTCAE 5.0.ResultsThe most common schwannoma origin was the vestibulocochlear nerve with 82.2%, followed by the trigeminal nerve with 8.9% and the glossopharyngeal nerve as well as the vagal nerve, both with each 4.4%. At radiotherapy start, 58% of cranial nerve schwannomas were progressive and 95.6% were symptomatic. Patients were treated with a median total dose of 54 Gy RBE in 1.8 Gy RBE per fraction. MRI during the median follow-up period of 42 months (IQR 26–61) revealed stable disease in 93.3% of the patients and partial regression in 6.7%. There was no case of progressive disease. New or worsening cranial nerve dysfunction was found in 20.0% of all patients, but always graded as CTCAE °I-II. In seven cases (16%), radiation-induced contrast enhancements (RICE) were detected after a median time of 14 months (range 2–26 months). RICE were asymptomatic (71%) or transient symptomatic (CTCAE °II; 29%). No CTCAE °III/IV toxicities were observed. Lesions regressed during the follow-up period in three of the seven cases, and no lesion progressed during the follow-up period.ConclusionThese data demonstrate excellent effectiveness with 100% local control in a median follow-up period of 3.6 years with a promising cranial nerve functional protection rate of 80%. RICE occurred in 16% of the patients after PRT and were not or only mildly symptomatic.

Proposed Treatment Strategy for Cavernous Sinus Meningiomas: A Prospective Study

Neurosurgery ◽  
2004 ◽  
Vol 55 (5) ◽  
pp. 1068-1075 ◽  
Author(s):  
Keisuke Maruyama ◽  
Masahiro Shin ◽  
Hiroki Kurita ◽  
Nobutaka Kawahara ◽  
Akio Morita ◽  
...  
Keyword(s):  
Cavernous Sinus ◽  
Treatment Strategy ◽  
Cranial Nerve ◽  
Treatment Protocol ◽  
Growth Control ◽  
Tumor Control ◽  
Karnofsky Performance Scale ◽  
Cranial Nerve Dysfunction ◽  
Median Volume ◽  
A Prospective Study

Abstract OBJECTIVE: To establish a safe and effective treatment strategy for cavernous sinus (CS) meningiomas, we prospectively analyzed the outcome of a treatment protocol combining surgery and radiosurgery during the past 7 years. METHODS: Tumors confined to the CS and distant from the optic apparatus and the brainstem were treated with radiosurgery alone. Tumors attached to or compressing the optic apparatus and brainstem and that were larger than 3 cm in mean diameter, extended into the multiple cranial fossae, and were suspected of being malignant were treated with combined nonradical microsurgery and radiosurgery. RESULTS: In accordance with this treatment protocol, 40 patients aged 26 to 72 years (median, 51 yr) with primary (n = 27) or recurrent (n = 13) CS meningiomas (volume range, 0.9–39.3 cm3; median volume, 5.4 cm3) were treated with combined surgery and radiosurgery (n = 23) or radiosurgery alone (n = 17). During radiosurgery, 12 to 18 Gy (median, 16 Gy) was delivered to the tumor margin. The follow-up period ranged from 14 to 79 months (median, 47 mo). The actuarial tumor control rate was 94.1% at 5 years. The improvement of cranial nerve function was significantly frequent in patients with primary CS meningiomas (P&lt; 0.05). Permanent cranial nerve dysfunction was significantly frequent in patients with tumors compressing the brainstem or smaller than 10 cm3 (P&lt; 0.05). All 36 patients with a pretreatment Karnofsky Performance Scale score of 90 or more maintained the same range after treatment. CONCLUSION: Proper combination of microsurgery and radiosurgery for CS meningiomas provides excellent growth control with favorable functional state. Outcomes were better when this protocol was adopted at the initial diagnosis for patients with smaller tumors that did not compress the brainstem.

scholarly journals Gamma Knife radiosurgery for hemangioma of the cavernous sinus

2017 ◽  
Vol 126 (5) ◽  
pp. 1498-1505 ◽  
Author(s):  
Cheng-Chia Lee ◽  
Jason P. Sheehan ◽  
Hideyuki Kano ◽  
Berkcan Akpinar ◽  
Roberto Martinez-Alvarez ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Gamma Knife ◽  
Cavernous Sinus ◽  
Cranial Nerve ◽  
Tumor Resection ◽  
Gamma Knife Radiosurgery ◽  
Vascular Tumors ◽  
Tumor Control ◽  
Gradual Improvement ◽  
Cranial Nerve Dysfunction

OBJECTIVECavernous sinus hemangiomas (CSHs) are rare vascular tumors. A direct microsurgical approach usually results in massive hemorrhage and incomplete tumor resection. Although stereotactic radiosurgery (SRS) has emerged as a therapeutic alternative to microsurgery, outcome studies are few. Authors of the present study evaluated the role of SRS for CSH.METHODSAn international multicenter study was conducted to review outcome data in 31 patients with CSH. Eleven patients had initial microsurgery before SRS, and the other 20 patients (64.5%) underwent Gamma Knife SRS as the primary management for their CSH. Median age at the time of radiosurgery was 47 years, and 77.4% of patients had cranial nerve dysfunction before SRS. Patients received a median tumor margin dose of 12.6 Gy (range 12–19 Gy) at a median isodose of 55%.RESULTSTumor regression was confirmed by imaging in all 31 patients, and all patients had greater than 50% reduction in tumor volume at 6 months post-SRS. No patient had delayed tumor growth, new cranial neuropathy, visual function deterioration, adverse radiation effects, or hypopituitarism after SRS. Twenty-four patients had presented with cranial nerve disorders before SRS, and 6 (25%) of them had gradual improvement. Four (66.7%) of the 6 patients with orbital symptoms had symptomatic relief at the last follow-up.CONCLUSIONSStereotactic radiosurgery was effective in reducing the volume of CSH and attaining long-term tumor control in all patients at a median of 40 months. The authors' experience suggests that SRS is a reasonable primary and adjuvant treatment modality for patients in whom a CSH is diagnosed.

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