Value of systematic analysis of the olfactory cleft in case of cerebrospinal rhinorrhea: incidence of olfactory arachnoid dilatation.

To report on the presence of olfactory arachnoid dilatations (OAD), a previously undescribed radiologic feature of spontaneous cerebrospinal fluid (CSF) rhinorrhea originating from the cribriform plate of the ethmoid bone. The medical records of all patients treated between 2001 and 2011 at a tertiary care center for a spontaneous CSF rhinorrhea originating from the cribriform plate were retrospectively reviewed. The radiological work-up included high-resolution computed tomography and magnetic resonance imaging with at least the following sequences: T1, T2, and T2 with fast imaging employing steady state acquisition (FIESTA). Thirty cases were identified. The mean age at diagnosis was 49. Fourteen patients (47 %) had a body mass index (BMI) of 30 or more and 3 patients (10 %) had a BMI between 25 and 29.9. Five patients had a history of meningitis. The imaging work-up revealed a bone defect of the cribriform plate in 6 cases (20 %), associated to a typical meningocele in 14 cases (47 %). In ten patients (33 %), there was no defect of the cribriform plate, but ultrathin coronal T2-FIESTA sequences revealed an OAD, i.e. a dilatation of the arachnoid sheath of the olfactory fibers, in nine cases (30 %), or a "pseudo-polyp" outlined by a thin layer of arachnoid (1 patient, 3 %). Preoperative imaging should be carefully analyzed for the presence of OAD or "pseudo-polyp" in patients presenting with a CSF rhinorrhea without bony defect of the cribriform plate.

Minimally invasive video-assisted thyroidectomy: tips and pearls for the surgical technique.

BACKGROUND: The objectives of this study were first to show principles of the minimally invasive video-assisted thyroidectomy (MIVAT), based on a video highlighting critical steps, and second to discuss tips and pearls to assist surgical teams that would like to start using this technique. METHODS: Based on a video, we described tips and pearls of a MIVAT. RESULTS: MIVAT includes 5 main steps: (1) skin incision and identification of the common carotid artery, (2) dissection and ligation of the upper pedicle, (3) identification of the inferior laryngeal nerve and parathyroid glands, (4) isthmectomy and lobe extraction, and (5) closure. DISCUSSION: Coordination between the surgeon and the 2 assistants is of paramount importance for the performance of MIVAT. Appropriate material is also required. The magnification and tissue contrast emphasizes the identification of the vessels, the superior and inferior laryngeal nerves, and parathyroid glands, on a large-view screen.

Critical analysis of recurrences of esthesioneuroblastomas: can we prevent them?

Esthesioneuroblastoma (ENB) involving the anterior skull base is a rare malignant tumour derived from the olfactory epithelium. The gold standard of surgical treatment is currently craniofacial resection (CFR), which allows efficient removal of the tumour but entails significant morbidity. To reduce morbidity combined with good visualization of tumour limits removal, endonasal endoscopy resection (EER) has developed. The objective of this work was (1) to describe the EER surgical procedure, the morbidity, and the limitations of this endoscopic approach as compared with CFR, (2) analyse recurrences to define risk factors of recurrences and (3) to discuss a therapeutic decision algorithm. Retrospective series of 18 patients with ENB endoscopically treated in a university tertiary referral centre over 13 years. Fifteen of those underwent radiotherapy. Epidemiological data, clinical and imaging findings, histology, treatment modalities and outcome of patients were studied. Mean follow-up was 31 months. Morbidity was mainly related to radiotherapy. Three recurrences were detected: one bone and one sylvian metastasis, and a local recurrence in a patient not irradiated. One recurrence spread through leptomeningeal propagation. Dural extension and frontal invasion were significantly associated with recurrences (p = 0.001 and p = 0.019, respectively). Patients with dural extension or frontal invasion should receive aggressive treatment. With a low rate of perioperative morbidity and efficient local control, EER seems to be a promising approach for selected cases of ENB.

Curative high-dose reirradiation for patients with recurrent head and neck adenoid cystic carcinomas: outcomes and analysis of patterns of failure.

BACKGROUND: To investigate the outcomes of patients who underwent curative reirradiation (reRT), with intensity-modulated radiation therapy (IMRT) or proton therapy (PT) for unresectable recurrent or second primary head and neck adenoid cystic carcinoma (HNACC). METHODS: Ten patients, mostly KPS 90%, were reirradiated (3/10 with IMRT and 7/10 with PT) at a median maximum dose to the CTV of 64.2 Gy from July 2011 to November 2021. Locations at the time of reRT were mainly the sinus (4/10) and the salivary glands (including the parotid and submandibular gland, 3/10). CTCAEv5 was used to assess acute and late toxicities. Follow-up was the time between the end of reRT and the date of last news. RESULTS: The median time between the two irradiations was 53.5 months (IQR: 18-84). After a median follow-up of 26 months (range, 12.5-51.8 months), six patients had developed a locoregional recurrence (LR), of which four occurred within the previously irradiated volume. Two and three-year locoregional failure-free survival (LFFS) and overall survival (OS) were 55.6% [95%CI: 31-99.7%], and 41% [18.5-94%] and 66.7% [42-100%] and 44.4% [21.4-92.3%], respectively. LFFS and OS were significantly better in the subgroup of sinus tumors (p = .013) and the subgroup of patients re-irradiated more than two years after the first course of irradiation (p = .01). Seven patients had impairments before the start of reRT, including hearing impairment (3/10) and facial nerve impairment (3/10). The most severe late toxicities were brain necrosis (2/10), osteoradionecrosis (1/10) and vision decreased (1/10). CONCLUSION: Curative reRT for HNACC is possible for selected cases, but the LR rate in the irradiated field and the risk of severe toxicity remain high. Improved selection criteria and more carefully defined target volumes may improve outcome in these patients. A further study including larger cohort of patients would be useful to confirm these results.

Geriatric assessment and quality of life for 2 years in older patients with head and neck cancer.

Due to the location and toxicity of treatments, head and neck cancer (HNC) has a major impact on quality of life (QoL). Objective: to assess the effects of geriatric-assessment (GA)-driven interventions on QoL over 2 years in older adults with HNC.EGeSOR was a randomized study of HNC patients aged ≥65, receiving a pretreatment GA, a geriatric intervention and follow-up (intervention) or standard of care (control). The primary endpoint was QoL score using the European Organisation for Research and Treatment of Cancer's (EORTC QLQ-C30) and HNC (QLQ-HN35) QoL questionnaires over 24 months.In total, 475 patients were included (median age: 75.3; women: 31%; oral cancer: 44%). QoL scores improved over 24 months with various trajectories, without significant differences between the groups. A total of 74% of patients (interventional group) did not receive the complete intervention. Cancer characteristics, functional status, and risk of frailty were associated with change in the Global Health Status QoL score.There is a need to develop an alternative model of implementation such as patient-centered health-care pathways. TRIAL REGISTRATION: NCT02025062.

[Rhino-orbito-cerebral mucormycosis, surgical treatment, state of the art]. / Mucormycoses rhino-orbito-cérébrales - Traitement chirurgical, état de l'art.

Rhino-orbito-cerebral mucormycoses constitute a severe fungal infection. These infections mostly arise in immunosuppressed patients. The surgery aiming at resecting necrosed hurts showed its interest in term of survival for lung and cutaneous mucormycosis. However, treatment of rhino-orbito-cerebral location of mucormycosis is not well defined. Transnasal endoscopic surgery allows local control of the disease, better post-operative outcomes than transfacial approaches and less sequelae. However, transfacial approaches are sometimes necessary to allow cutaneous resection or exenteration, the indications of which still remain controversial. The retrospective study of 22 patients with mucormycosis allowed to show that radical surgical treatment allowed local control of the disease with an improved survival. Further prospective studies (PHRC MICCA, current) are required to standardize the management of this rare but potentially lethal pathology.

Proton therapy and/or helical tomotherapy for locally advanced sinonasal skull base adenoid cystic carcinoma: Focus on experience of the Institut Curie and review of literature.

BACKGROUND: Sinonasal adenoid cystic carcinomas (SNACC) have high propensity for skull base (SB) infiltration. Unresectability or incomplete surgical resection in such cases make radiotherapy treatment paramount. Curative dose escalation is challenging because of adjacent organs at risk, especially in locally advanced cases. METHODS: Eighteen patients that had locally advanced SB SNACC with unresectable or incomplete surgical resection treated by proton therapy and/or helical tomotherapy at Institut Curie between 3/2010 and 8/2020 were retrospectively included. RESULTS: After median follow-up of 52 months, 5-year OS, LRRFS, DMFS, DFS rates were, respectively, 47% (95%CI: 26-83), 50% (95%CI: 36-88), 39% (95%CI: 26-81), 33% (95%CI: 22-73). One patient had grade 4 late optic nerve disorder. Eight patients had grade 3 late toxicity including mainly hearing impairments. CONCLUSION: Proton therapy and helical tomotherapy are effective and safe methods for curative dose escalation of locally advanced SB SNACC, which are a poor prognosis subgroup. Available literature suggests carbon-ion therapy could be an efficient alternative.

Effectiveness of Geriatric Assessment-Driven Interventions on Survival and Functional and Nutritional Status in Older Patients with Head and Neck Cancer: A Randomized Controlled Trial (EGeSOR).

This study assesses the efficacy of Geriatric Assessment (GA)-driven interventions and follow-up on six-month mortality, functional, and nutritional status in older patients with head and neck cancer (HNC). HNC patients aged 65 years or over were included between November 2013 and September 2018 by 15 Ear, Nose, and Throat (ENT) and maxillofacial surgery departments at 13 centers in France. The study was of an open-label, multicenter, randomized, controlled, and parallel-group design, with independent outcome assessments. The patients were randomized 1:1 to benefit from GA-driven interventions and follow-up versus standard of care. The interventions consisted in a pre-therapeutic GA, a standardized geriatric intervention, and follow-up, tailored to the cancer-treatment plan for 24 months. The primary outcome was a composite criterion including six-month mortality, functional impairment (fall in the Activities of Daily Living (ADL) score ≥2), and weight loss ≥10%. Among the patients included (n = 499), 475 were randomized to the experimental (n = 238) or control arm (n = 237). The median age was 75.3 years [70.4-81.9]; 69.5% were men, and the principal tumor site was oral cavity (43.9%). There were no statistically significant differences regarding the primary endpoint (n = 98 events; 41.0% in the experimental arm versus 90 (38.0%); p = 0.53), or for each criterion (i.e., death (31 (13%) versus 27 (11.4%); p = 0.48), weight loss of ≥10% (69 (29%) versus 65 (27.4%); p = 0.73) and fall in ADL score ≥2 (9 (3.8%) versus 13 (5.5%); p = 0.35)). In older patients with HNC, GA-driven interventions and follow-up failed to improve six-month overall survival, functional, and nutritional status.

Endoscopy skull-base resection for ethmoid adenocarcinoma and olfactory neuroblastoma.

STATEMENT OF PROBLEM: Olfactory neuroblastoma (ON) and ethmoid adenocarcinoma (AC) are rare sinonasal malignancies that often involve the skull base. Standard surgical treatment is craniofacial resection (CFR), which allows for efficient removal but entails significant morbidity and mortality. Because expanded endoscopy nasal approaches are newly developed, we aimed to describe the procedure in patients with ON and AC and compare it with CFR in terms of efficiency and morbidity. METHODS: This work reports on a retrospective series of 16 patients with AC and ON treated endoscopically with anterior skull-base resection in a single institution over 9 years. Invasion of the frontal sinus, massive extension to the cerebral parenchyma, spread of the tumour above the orbits or lysis of anterior facial skeleton were contraindications for endoscopy resection. RESULTS: Of the 16 patients, 11 had AC and 5 ON. In total, 37.5% (6) exhibited skull-base invasion. All patients had postoperative radiotherapy. In the early postoperative period, one patient experienced delayed seizure due to a minor subdural hematoma. Two delayed complications were observed: one encephalocele related to inappropriate postoperative care, which required revision surgery, and one extended radionecrosis. Five-year disease-free survival was 83% and 5-year recurrence-free survival 58%. Local control rate was 91% for AC and 100% for ON. CONCLUSIONS: With low perioperative morbidity and efficient local control, ethmoidectomy combined with anterior skull-base resection is a promising approach for managing selected cases of AC and ON. These findings need further investigation with prolonged follow-up.

Arterial wall uptake of fluorodeoxyglucose on PET imaging in stable cancer disease patients indicates higher risk for cardiovascular events.

BACKGROUND: We aimed to evaluate the additional information of 18 fluorodeoxyglucose (FDG) arterial uptake with respect to other conventional cardiovascular risk factors and arterial calcifications in patients with stable cancer. METHODS AND RESULTS: We compared the rate of cardiovascular events in 2 groups of patients with (n = 45) and without (n = 56) enhanced arterial 18FDG uptake, matched for the main clinical parameters. The extent and intensity of 18FDG uptake were quantified. A calcification index was also determined. About one third of the selected patients had a history of cardiovascular events and thus could be defined as "vulnerable patients." Old cardiovascular events (>6 months before or after positron emission tomography [PET]) and recent cardiovascular events (<6 months before or after PET) were significantly more frequent in the high-FDG uptake group than in the low-FDG uptake group (48% vs 15%, respectively [P = .0006], and 30% vs 1.8%, respectively [P = .0002]). The extent of 18FDG arterial uptake was the unique factor significantly related to the occurrence of a recent event by either logistic regression or discriminant analysis (P = .004 for all). Conversely, calcium index was the single factor related to old events (P = .004 and P = .002, respectively). CONCLUSIONS: Extensive arterial 18FDG uptake might be an indicator of an evolving atherosclerotic process and should be mentioned in PET/computed tomography reports.

Aberrant internal carotid artery in the temporal bone: imaging findings and management.

OBJECTIVES: To describe the clinical and radiological features of the vascular anomaly aberrant internal carotid artery (ICA) in the temporal bone and to discuss management strategies. DESIGN: Retrospective study. PATIENTS: Sixteen cases of aberrant ICA were diagnosed between 1982 and 2003. RESULTS: Of 16 cases, 11 were recognized by imaging assessment, 4 were recognized during middle ear surgery, and 1 was recognized clinically. Among the 11 cases, 8 malformations were diagnosed because of otologic symptoms related to the abnormal ICA or chronic otitis, while the other 3 were identified incidentally because of an underlying accompanying disease. In 4 cases, the diagnosis was made during surgery related to chronic otitis media (n = 2) or conductive hearing loss (n = 2). In these 4 cases, massive bleeding resulted from surgical injury to the vessel. Packing the external auditory canal and the middle ear first controlled the bleeding. Endovascular procedure was required in 2 cases to exclude an aneurysm or to control bleeding but was followed by anterior cerebral stroke in 1 case. The aberrant ICA could be identified on computed tomographic scan by the following features: intratympanic mass, enlarged inferior tympanic canaliculus, absence of the vertical segment of the ICA canal, and absence of bone covering the tympanic portion of the ICA. Conventional angiography was mandatory when intervention was planned to control bleeding or aneurysm. CONCLUSIONS: This study highlights that aberrant ICA has to be identified before any middle ear surgery because misdiagnosis may lead to dramatic surgical complications, whereas diagnosis with computed tomographic scan is easy. Bleeding is a minor complication compared with the putative neurologic deficit due to endovascular occlusion.

Diode endovascular laser treatment in venous malformations of the upper aerodigestive tract.

PURPOSE: Venous malformations of the upper aerodigestive tract can cause pain, dysphagia, obstructive sleep apnea, and rarely bleeding. We studied 980-nm diode endovenous laser therapy. MATERIAL AND METHODS: This is a 2007-2014 retrospective study in our vascular anomalies center. Data on patients' clinical history, polysomnography, magnetic resonance imaging, and treatment were collected. Patients were contacted for Epworth Sleepiness Scale and Eating Assessment Tool (EAT-10) scores to evaluate sleepiness and dysphagia before and after laser therapy. RESULTS: We included 32 patients (mean age 41 years) presenting with obstructive sleep apnea (n = 18) and dysphagia (n = 13). With a mean follow-up of 39 months, average Epworth Sleepiness Scale score fell from 17.3 to 10.4 (p = 0.015), EAT-10 score from 8.2 to 3.5 (p = 0.002) and apnea-hypopnea index from 47.5 to 24.7 (p = 0.01). Of the sleep apnea patients, 89% required continuous positive airway pressure before and 50% afterward (p = 0.016). CONCLUSIONS: Diode endovascular laser treatment seems to be a safe and effective treatment option in venous malformations of the upper airways.

Early postoperative CT scanning for juvenile nasopharyngeal angiofibroma: detection of residual disease.

BACKGROUND AND PURPOSE: High recurrence rates and early recurrence have been reported for juvenile nasopharyngeal angiofibroma (JNA) involving the skull base. The purpose of this study was to evaluate the diagnostic accuracy of contrast-enhanced CT scanning for the detection of residual disease (RD) in the early postoperative course after surgical excision of JNA. METHODS: We retrospectively reviewed data in 20 male patients (mean age +/- SD, 15.4 +/- 5 years; range, 10-32 years) who underwent enhanced helical CT in the days after apparent complete surgical excision of JNA with initial expansion in the skull base. Four independent, blinded readers evaluated the occurrence of RD. Final diagnoses were rendered on the basis of histologic examination of excised specimens of RD or clinical and radiologic follow-up. The Cohen kappa test was performed to examine interreader agreement. RESULTS: Postoperative contrast-enhanced CT had a sensitivity of 75%, a specificity of 83%, a positive predictive value of 75% and a negative predictive value of 83% for the detection of RD. The prevalence of RD was 40%. The base of pterygoids was the most frequent location of RD. Interreader agreement was high for the detection of putative RD (kappa=0.83). Variabilities in readers' interpretations were encountered for false-positive results and for disease in the foramen lacerum. False-negative results involved the base of pterygoids. Early postoperative CT scanning was well tolerated by all patients. CONCLUSION: Contrast-enhanced helical CT is an accurate tool to evaluate excision of JNA in the days after surgery.

In vivo demonstration of the absorptive function of the middle ear epithelium.

The present study investigated in vivo fluid and ion transport across the middle ear epithelium. The tympanic membrane of rats was punctured under general anesthesia. A capillary tube was fitted to the external auditory canal and the bulla filled with various solutions. Middle ear (ME) fluid volume variations were then measured at constant pressure. When saline was used, a linear decrease of fluid volume was apparent. Replacement of sodium with a non-permeable cation (N-methyl-D-glucamin) reduced the absorption rate from 0.065+/-0.008 to 0.019+/-0.003 microl/min (P<0.05, n=6). Similarly, amiloride (10(-3)M), a sodium channel antagonist, reduced the absorption rate to 0.027+/-0.006 microl/min (P<0.05, n=6). Net absorption was abolished when chloride was substituted with gluconate: -0.008+/-0.004 microl/min (P<0.02, n=6), which might have been related (i) to the role of chloride as a diffusible anion through the paracellular pathway, or (ii) to the secretion of chloride through apical channels. However in this condition, 4,4'-diisothiocyanostilbene-2,2'-disulfonic acid, a chloride channel blocker, did not affect the rate of fluid exchange -0.008+/-0.007 microl/min (P=0.75, n=6). This model provides the first in vivo evidence for the absorptive function of the ME. Fluid introduced into the ME cavity disappears due to active transport through the mucosa. This process is sodium-dependent and can be hindered by high concentration of amiloride. The rate of absorption is high enough to allow total clearance of fluid from the cavity of the middle ear within 13 h. This process might play a role in the maintaining a fluid-free and gas-filled middle ear cavity.

Idiopathic sudden sensorineural hearing loss is not an otologic emergency.

OBJECTIVE: To investigate whether delay in treatment has any influence on the audiometric outcome at Day 30 in idiopathic sudden sensorineural hearing loss. STUDY DESIGN: Prospective study. SETTING: Otorhinolaryngologic emergency center in Paris, France. PATIENTS: Three hundred forty-seven consecutive cases of sudden sensorineural hearing loss were examined. A neurologic or retrocochlear cause was revealed in 17 cases. Four additional cases were lost for follow-up. Three hundred twenty-six cases of "idiopathic" sensorineural hearing loss seen within 7 days of onset were enrolled and classified by type according to five audiogram shapes: low tone (Type A), flat (Type B), high tone (Type C), cup-shaped (Type D) or total or subtotal (Type E). Because of loss for follow-up, the hearing outcome at 1 month could be evaluated in only 249 cases. INTERVENTION: All 326 patients were given 1 mg/kg per day corticosteroids intravenously for 6 days and 500 ml mannitol 10% in the subgroup presenting ascending audiometric shape. MAIN OUTCOME MEASURES: The following parameters were used. The first parameter was hearing recovery (initial PTA-PTA at Day 6 or Day 30). It was considered as complete if final PTA was below 25 dB. The second parameter was incidence of hearing recovery based on the following formula: (initial PTA-PTA on a given test day)/(initial PTA) x 100%. Using regression analysis and ANOVA, the audiometric outcome was analyzed at Day 6 and Day 30 as a function of the day of onset of treatment and of the audiometric type. RESULTS: Whatever the audiometric type, there was no significant difference in final outcome whether the treatment was started within the first 24 hours or within the first week. CONCLUSIONS: Delay in initiating treatment does not appear to influence the final degree of hearing loss. Idiopathic sudden sensorineural hearing loss cannot be considered as an otologic emergency.

Sudden sensorineural hearing loss as a revealing symptom of vestibular schwannoma.

CONCLUSIONS: Sudden sensorineural hearing loss (SSHL) is a frequent symptom of vestibular schwannoma (VS), often reveals small VSs and does not exhibit specific features. Therefore, every case of SSHL should be evaluated using systematic MRI to rule out VS in order to improve hearing and preservation facial nerve function. OBJECTIVE: SSHL leads to the discovery of a VS in a small proportion of cases (2%). However, SSHL appears to be a more frequent occurrence in the history of patients with VS (3-23% in the literature), suggesting a large disparity in the evaluation of SSHL. MATERIAL AND METHODS: A total of 139 consecutive unilateral VSs operated on between 2000 and 2002 were reviewed and analyzed regarding the prevalence, clinical and audiological features of SSHL and their relation to the size of the tumor. RESULTS: SSHL was observed in 20% of cases at some point in their VS history. The characteristics of SSHL were: (i) lack of a specific audiometric pattern, except that low-tone loss was rare; and (ii) a high rate of hearing recovery (50%). Tumor size was significantly smaller in SSHL-associated VSs compared to other VSs. In the former cases, 96% involved the internal auditory canal.

Petrous and sphenoid arachnoid cysts: diagnosis and management.

BACKGROUND: Intraosseous arachnoid cysts are rare and difficult to diagnose. The purpose of this study was to describe the clinical and radiological semiology of petrous and sphenoid arachnoid cysts and to propose a specific management strategy. METHODS: This was a retrospective, descriptive study of patients with arachnoid cysts, which utilized CT, MRI, and the patients' medical histories. RESULTS: Ten patients were included in this study. On CT, the lesions were lytic with bony delineation. On MRI, the lesions exhibited the same signals as cerebrospinal fluid and were not enhanced after contrast. On fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted sequences, the arachnoid cysts' signal disappeared, which is a key feature for eliminating the diagnosis of cholesteatoma. Two patients underwent surgery because of misdiagnosis, either with a meningocele or a cholesteatoma. CONCLUSION: MRI FLAIR and diffusion-weighted sequences, together with osseous CT scans, help to distinguish arachnoid cysts from meningoceles and avoid unnecessary surgeries with potential complications.

Clinical impact of somatostatin receptor scintigraphy in the management of paragangliomas of the head and neck.

UNLABELLED: Paragangliomas are neuroendocrine tumors expressing somatostatinergic receptors and, thus, may be imaged by somatostatin receptor scintigraphy (SRS). The purpose of the study was to assess the contribution of SRS in the management of paraganglioma of the head and neck. METHODS: (111)In-Pentetreotide (148 MBq) was intravenously administered to 3 groups of patients. Group A consisted of 9 patients with tumors of the head and neck that did not exhibit typical radiologic features of paragangliomas and required further diagnostic evaluation. Group B consisted of 28 patients with known paragangliomas or paraganglioma metastatic lymph node who required staging and assessment of multicentricity. Group C consisted of 5 asymptomatic relatives of affected individuals who required screening. All patients underwent clinical, laboratory, radiologic, and audiovestibular evaluation. RESULTS: In group A (n = 9), SRS was positive in 6 cases, 4 paragangliomas and 2 meningiomas, and negative in 3 patients in whom the initial diagnosis of paraganglioma was excluded. In group B (n = 28), SRS was strongly positive in all of the 34 head and neck masses diagnosed on conventional imaging to be paragangliomas; it localized 1 primary malignant paraganglioma and revealed 9 unexpected foci. In group C (n = 5), SRS was positive in 3 of the 5 relatives, CT or MR scan confirming the diagnosis of paraganglioma in all cases. CONCLUSION: Because of very high sensitivity in detecting paraganglioma, SRS should be included in a multiple-step strategy for patients' management. It could be useful in ruling out the diagnosis of schwannoma, but its major advantage may involve patients' staging. Once biologic genetic testing of affected patients' relatives evidenced the predisposing mutation, SRS could be proposed to identify individuals who express the disease.