Jugulotympanic paragangliomas in southern Finland : a 40-year experience suggests individualized surgical management

Treatment of jugulotympanic paragangliomas (JTPGLs) remains challenging with no clear guidelines for management or follow-up. The aim of this retrospective case-note study was to assess long-term results of operatively and conservatively managed JTPGLs between years 1974-2013. A total of 36 patients with JTPGLs were identified. Clinical characteristics and management outcomes of patients were reviewed. Data were extracted on demographics, symptoms, timing of diagnosis, tumor location and size, embolization, and management, including pre- and post-operative imaging, analysis of operative techniques, and follow-up. Pulsatile tinnitus and hearing loss were the most common presenting symptoms. Thirty-four (94 %) patients were treated with primary surgical therapy and two (6 %) with radiotherapy. The surgical approaches included endaural approach for Fisch Class A tumors and a variety of approaches for Fisch Class B-D tumors with an increasing predilection for function-preserving surgery. Eight (24 %) patients received subtotal resection. Five (15 %) patients had a local recurrence within 10 years after primary surgery. Two (6 %) patients suffered a permanent cranial nerve (CN) deficit after primary surgery. We advocate radical surgery when tumor resection is possible without compromising CNs. Function-preserving surgery with at least a 10-year follow-up for Fisch Class B-D tumors should be considered if CNs are in danger.Peer reviewe

Biomedical image sequence analysis with application to automatic quantitative assessment of facial paralysis

Facial paralysis is a condition causing decreased movement on one side of the face. A quantitative, objective, and reliable assessment system would be an invaluable tool for clinicians treating patients with this condition. This paper presents an approach based on the automatic analysis of patient video data. Facial feature localization and facial movement detection methods are discussed. An algorithm is presented to process the optical flow data to obtain the motion features in the relevant facial regions. Three classification methods are applied to provide quantitative evaluations of regional facial nerve function and the overall facial nerve function based on the House-Brackmann scale. Experiments show the radial basis function (RBF) neural network to have superior performance

Biomedical image sequence analysis with application to automatic quantitative assessment of facial paralysis

Facial paralysis is a condition causing decreased movement on one side of the face. A quantitative, objective, and reliable assessment system would be an invaluable tool for clinicians treating patients with this condition. This paper presents an approach based on the automatic analysis of patient video data. Facial feature localization and facial movement detection methods are discussed. An algorithm is presented to process the optical flow data to obtain the motion features in the relevant facial regions. Three classification methods are applied to provide quantitative evaluations of regional facial nerve function and the overall facial nerve function based on the House-Brackmann scale. Experiments show the radial basis function (RBF) neural network to have superior performance

Head and neck paragangliomas: clinical and molecular genetic classification

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

Clinical Presentation and Management of Jugular Foramen Paraganglioma

ObjectivesJugular foramen paraganglioma is a locally invasive, benign tumor, which grow slowly and causes various symptoms such as pulsatile tinnitus and low cranial nerve palsy. Complete surgical resection is regarded as the ideal management of these tumors. The goal of this study is to identify the clinical characteristics and most effective surgical approach for jugular foramen paraganglioma.MethodsRetrospective analysis of 9 jugular foramen paraganglioma patients who underwent surgical resection between 1986 and 2005 was performed. Clinical records were reviewed for analysis of initial clinical symptoms and signs, audiological examinations, neurological deficits, radiological features, surgical approaches, extent of resection, treatment outcomes and complications.ResultsMost common initial symptom was hoarseness, followed by pulsatile tinnitus. Seven out of 9 patients had at least one low cranial nerve palsy. Seven patients were classified as Fisch Type C tumor and remaining 2 as Fisch Type D tumor on radiologic examination. Total of 11 operations took place in 9 patients. Total resection was achieved in 6 cases, when partial resection was done in 3 cases. Two patients with partial resection received gamma knife radiosurgery (GKS), when remaining 1 case received both GKS and two times of revision operation. No mortality was encountered and there were few postoperative complications.ConclusionNeurologic examination of low cranial nerve palsy is crucial since most patients had at least one low cranial nerve palsy. All tumors were detected in advanced stage due to slow growing nature and lack of symptom. Angiography with embolization is crucial for successful tumor removal without massive bleeding. Infratemporal fossa approach can be considered as a safe, satisfactory approach for removal of jugular foramen paragangliomas. In tumors with intracranial extension, combined approach is recommended in that it provides better surgical view and can maintain the compliance of the patients

Bezpośrednie i odległe wyniki leczenia przyzwojaków otworu szyjnego z wykorzystaniem różnych zakresów dostępu operacyjnego

Background and purpose The applied approach to the jugular foramen is a combination of the juxtacondylar approach with the subtemporal fossa approach type A. The purpose of this study is to present our results of treatment of jugular paragangliomas using the aforementioned approach. Material and methods Twenty-one patients (15 women, 6 men) with jugular paragangliomas were included in the study. The neurological status of the patients was assessed before and after surgery as well as at the conclusion of treatment. The approximate volume of the tumour, its relation to large blood vessels, cranial nerves and brainstem, as well as consistency and vascularity were also assessed. Results The duration of symptoms ranged from 3 to 74 months. In 86% of patients hearing loss was the predominant symptom. The less frequent symptoms included pulsatile tinnitus in the head, dysphagia and dizziness. Approximate volume of the tumours ranged from 2 to 109 cm3. A gross total resection was achieved in 71.5% of patients. The postoperative performance status improved in 38% of patients, did not change in 38% and deteriorated in 24% of patients. Conclusions A proper selection of the range of the approach to jugular foramen paragangliomas based on their topography and volume reduces perioperative injury without negative consequences for the radicality of the resection.Wstęp i cel pracy Stosowany dostęp do otworu szyjnego to połączenie dostępu okołokłykciowego i dostępu podskroniowego typu A. Celem niniejszej pracy jest przedstawienie własnych wyników leczenia przyzwojaków otworu szyjnego z wykorzystaniem powyższego dostępu. Materiał i metody Analizie poddano grupę 21 chorych z przyzwojakami otworu szyjnego (15 kobiet i 6 mężczyzn). U 10 chorych wykorzystano pierwszy, u 7 – drugi, a u 4 – trzeci zakres dostępu do otworu szyjnego. Ocenie podlegały stan neurologiczny chorych przed rozpoczęciem leczenia, po operacji i po zakończeniu leczenia oraz zmiany ich aktywności życiowej. Określano przybliżoną objętość operowanych guzów, ich stosunek do dużych naczyń, nerwów czaszkowych i pnia mózgu. Wyniki Długość wywiadu wahała się od 3 do 74 miesięcy (mediana wyniosła 6 miesięcy). W 86% przypadków wiodącym objawem był niedosłuch. Rzadziej występowały pulsujący szum w głowie oraz zburzenia połykania i równowagi. Przybliżona objętość usuniętych guzów wahała się od 2 do 109 cm3. W 71,5% przypadków przeprowadzone resekcje były doszczętne. Aktywność życiowa u 38% pacjentów poprawiła się, u 38% nie zmieniła się, a u 24% uległa pogorszeniu. Wnioski Dobór zakresu dostępu do otworu szyjnego, podyktowany topografią i rozmiarami przyzwojaka rozwijającego się w tym otworze i jego otoczeniu, pozwala na ograniczenie urazu operacyjnego bez negatywnego wpływu na radykalność resekcji

Clinical Presentation and Management of Jugular Foramen Paraganglioma

ObjectivesJugular foramen paraganglioma is a locally invasive, benign tumor, which grow slowly and causes various symptoms such as pulsatile tinnitus and low cranial nerve palsy. Complete surgical resection is regarded as the ideal management of these tumors. The goal of this study is to identify the clinical characteristics and most effective surgical approach for jugular foramen paraganglioma.MethodsRetrospective analysis of 9 jugular foramen paraganglioma patients who underwent surgical resection between 1986 and 2005 was performed. Clinical records were reviewed for analysis of initial clinical symptoms and signs, audiological examinations, neurological deficits, radiological features, surgical approaches, extent of resection, treatment outcomes and complications.ResultsMost common initial symptom was hoarseness, followed by pulsatile tinnitus. Seven out of 9 patients had at least one low cranial nerve palsy. Seven patients were classified as Fisch Type C tumor and remaining 2 as Fisch Type D tumor on radiologic examination. Total of 11 operations took place in 9 patients. Total resection was achieved in 6 cases, when partial resection was done in 3 cases. Two patients with partial resection received gamma knife radiosurgery (GKS), when remaining 1 case received both GKS and two times of revision operation. No mortality was encountered and there were few postoperative complications.ConclusionNeurologic examination of low cranial nerve palsy is crucial since most patients had at least one low cranial nerve palsy. All tumors were detected in advanced stage due to slow growing nature and lack of symptom. Angiography with embolization is crucial for successful tumor removal without massive bleeding. Infratemporal fossa approach can be considered as a safe, satisfactory approach for removal of jugular foramen paragangliomas. In tumors with intracranial extension, combined approach is recommended in that it provides better surgical view and can maintain the compliance of the patients

Comparison of radiosurgery and conventional surgery for the treatment of glomus jugulare tumors

Journal ArticleObject. The optimal management of glomus jugulare tumors remains controversial. Available treatments were once associated with poor outcomes and significant complication rates. Advances in skull base surgery and the delivery of radiation therapy by stereotactic radiosurgery have improved the results obtained using these treatment options. The authors summarize and compare the contemporary outcomes and complications for these therapies. Methods. Papers published between 1994 and 2004 that detailed the use of radiosurgery or surgery to treat glomus jugulare tumors were reviewed. Eight radiosurgery series including 142 patients and seven surgical studies including 374 patients were evaluated for neurological outcome, change in tumor size (radiosurgery) or percent of total resection (surgery), recurrences, tumor control, need for further treatment, and complications. The mean age at treatment for patients who underwent surgery and radiosurgery was 47.3 and 56.7 years, respectively. The mean follow-up duration was 49.2 and 39.4 months, respectively. The surgical control rate was 92.1%, with 88.2% of tumors totally resected in the initial surgery. A cerebrospinal fluid leak occurred in 8.3% of patients who underwent surgery and recurrences were found in 3.1%; the mortality rate was 1.3%. Among patients who underwent radiosurgery, tumors diminished in 36.5%, whereas 61.3% had no change in tumor size, and subjective or objective improvements occurred in 39%. Despite the presence of residual tumor in 100% of radiosurgically treated patients, recurrences were found in only 2.1%, the morbidity rate was 8.5%, and there were no deaths. Conclusions. Death and recurrences after these treatments are infrequent, and therefore both treatments are considered to be safe and efficacious. Although surgery is associated with higher morbidity rates, it immediately and totally eliminates the tumor. The radiosurgery results are very promising, although the incidence of late recurrence (after 10-20 years) is unknown