A case of cervical paraganglioma: usefulness of FDG PET imaging and a possibility of rare origination

Cervical paragangliomas are rare tumors derived from neural crest cells. Anatomic imaging techniques rely upon the characteristic spatial relations and the high vascularity of this tumor. Results of computed tomography (CT), magnetic resonance imaging (MRI), and arteriography initially led us to the preoperative diagnosis of cervical paraganglioma. In our case, 2-[18F]-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET), a type of functional imaging, was also carried out and demonstrated abnormally increased tracer uptake; this approach was thus successful for visualizing paraganglioma. FDG PET imaging may be useful in the detection of benign paraganglioma. On surgical exploration of the neck, the tumor was found to arise from the vagus nerve, whereas the hypoglossal nerve was encompassed by the tumor in its upper portion. The intraoperative findings suggested the possibility that the tumor had arisen from the hypoglossal nerve.     

Tongue viability after bilateral lingual artery ligation and surgery for recurrent tongue-base cancer

A 73-year-old man came to us with a second primary tumor of the tongue base. He had undergone a total laryngectomy, neck dissections, and radiation for squamous cell carcinoma of the glottis 25 years earlier. Surgery was performed, including a subtotal tongue-base resection and pectoralis myocutaneous flap reconstruction. Intraoperative findings confirmed tumor involvement of the left lingual artery and hypoglossal nerve, both of which were sacrificed. Dissection revealed an intact but apparently thrombosed right lingual artery, and this was verified by intraoperative Doppler evaluation. Postoperatively, the preserved oral tongue remained well perfused and viable, and tongue mobility was good. In patients who have undergone previous head and neck cancer resections, collateral blood supply to the tongue might develop if the flow of one or both of the lingual arteries is interrupted. This possibility can be fully evaluated by preoperative angiography, which might offer these patients the potential for tongue preservation.     

Primary paraganglioma in the facial nerve canal

Objective

To describe primary paraganglioma in the facial nerve canal and discuss the characteristics of facial nerve paraganglioma in contrast with other tumors.

Case report

A 23-year-old man developed gradually progressive right facial palsy as the initial symptom. One year later, he exhibited hearing loss without tinnitus in his right ear. CT demonstrated an enlarged facial nerve canal with irregular bony erosion of the circumference. MRI showed a well-enhanced heterogeneous mass with hypo-intensity spots inside it. During surgery, a blood-rich tumor was observed along the facial nerve: however, extensive bleeding interfered with tumor removal. The surgical specimen demonstrated paraganglioma. The tumor was completely removed in the second surgery in combination with arterial embolization. Facial nerve function was reconstructed with a free muscle flap more than one year following resection.

Conclusion

Because paraganglioma is a blood-rich tumor, it is important to perform angiography and embolization. If preoperative facial nerve palsy is demonstrated, careful management of facial nerve function is needed. Paraganglioma must be considered in the differential diagnosis of a facial nerve tumor.     

A skull base extradural hypoglossal neurilemmoma resected via an extended posterolateral approach

The case of a 43-year-old man with a right skull base hypoglossal neurilemmoma excised via the extended posterolateral approach is presented. There is only one previous case of hypoglossal neurilemmoma in the literature. The surgical technique described is a new approach to the posterior skull base involving a suboccipital craniectomy, mastoidectomy and the removal of the lateral process of the atlas. It provides an inferior approach to the jugular foramen and hypoglossal canal that allows the lower cranial nerves to be identified as they exit from their skull base foramina. In the discussion we compare this technique to other surgical approaches previously described for access to the region of the jugular foramen.     

Infratemporal fossa approach to the hypoglossal canal: practical landmarks for elusive anatomy

OBJECTIVE: At the conclusion of the article the readers should be able to safely and reliably find the hypoglossal canal using the infratemporal fossa approach. HYPOTHESIS: Very little has been written on the regional anatomy of the hypoglossal canal as seen through a transtemporal approach. This project attempts to further define the anatomy of the hypoglossal canal and provide the surgeon with guidelines for reaching it. Our hypothesis is that the hypoglossal canal can be safely and consistently reached by way of the temporal bone with preservation of hearing and cranial nerves (CN) IX to XI. STUDY DESIGN: Prospective anatomic study. METHODS: The study was performed using cadaver temporal bones. Infratemporal fossa Fisch type-A dissections were performed. The hypoglossal canal was then completely exposed. The distance from the canal to the jugular bulb, carotid artery, round window, lateral canal, and roots of CN IX to XI were recorded. RESULTS: Fifteen temporal bones were dissected and measured. The position of the hypoglossal canal is consistently located anterior, inferior, and medial to the jugular bulb. The distance from midcanal to the jugular bulb and the roots of CN IX to XI at the posterior fossa dura was 5.3 mm +/- 0.82 and 7.1 mm +/- 2.49, respectively. The distance from the carotid artery where it meets the jugular vein to the midcanal was 15.3 mm +/- 2.09. The distance from the round window to the canal was 21.7 mm +/- 3.17. CONCLUSIONS: The hypoglossal canal can be consistently reached using the infratemporal fossa approach. Hearing and CN IX to XI can be preserved. The distance from the jugular bulb and roots of CN IX to XI can be used as guideposts. If a tumor is involving the bulb, then the carotid artery and the round window are the next most reliable indicators of position.     

Rare localization of paraganglioma in head and neck

In this paper, we describe the clinical course of a 61-year-old female patient with paraganglioma in the head and neck region. Computed tomographic scan (CT), magnetic resonance imaging (MRI), ultrasound scan (US) and arteriogram findings initially led us to suspect that this tumor originated in the vagal nerve. In particular, a color Doppler US enabled an easy diagnosis of hypervascular tumor. We removed this surgically, but the tumor was easy to peel from the vagal nerve and carotid bifurcation. The distal side of the tumor was under the digastric muscle and running into the hypoglossal nerve. The intraoperative findings were highly suggestive of localization at the hypoglossal nerve, although Xth and XIIth cranial nerve palsies have remained.     

Resection of a recurrent paraganglioma via an endoscopic transnasal approach to the jugular fossa.

OBJECTIVE: To present a novel endoscopic, transnasal approach to a recurrent paraganglioma of the jugular fossa (glomus jugulare). STUDY DESIGN: Case report and review of the literature. SETTING: The study was carried out at the University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, U.S.A. PATIENT: A 64-year-old woman presented to the University of Pittsburgh Medical Center with a 9-month history of left otalgia, occasional vertigo, facial pain, and recurrent epistaxis. She had undergone two previous procedures over the past 35 years for a glomus tympanicum. Physical examination revealed a mass in the left nasopharynx originating from the eustachian tube. A computed tomographic scan revealed an expansive mass in the left jugular foramen extending into the posterior parapharyngeal space suggestive of a large jugular paraganglioma. INTERVENTION: The patient underwent preoperative embolization of the paraganglioma. An endoscopic, transnasal approach along the eustachian tube, the ascending parapharyngeal carotid artery, and into the jugular fossa was used to remove the tumor. Intraoperative image guidance assisted in the identification of key anatomic landmarks. RESULTS: Postoperative magnetic resonance imaging revealed a thin rim of enhancement at the posterior aspect of the jugular fossa consistent with residual tumor. The patient was discharged to home on postoperative Day 1 in stable condition. There have been no complications with 4 months of follow-up, and the left facial paralysis secondary to preoperative embolization has resolved. CONCLUSION: We report the successful subtotal resection of a recurrent paraganglioma via a novel endoscopic, transnasal, transclival, transpetrous approach with image guidance. This approach allowed the near-total resection of a recurrent glomus jugulare with minimal surgical morbidity. Technological advances and surgical experience with nasal endoscopy and image guidance allow minimally invasive surgical management of select extracranial lesions of the lateral cranial base.     

Junger Patient mit Paragangliomsyndrom Typ?1

A 33-year old patient presented at our outpatient department with pulse-synchronous tinnitus in the left ear of several years' standing. Examination of the left ear showed a neoplasm at the base of the meatus in front of the ear drum. In addition, there was paresis of the right hypoglossal nerve and right vocal cord. CT scan showed multiple paraganglioma in the head and neck region. Molecular genetic screening analysis showed a mutation of the SDHD gene and thus the diagnosis of paraganglioma syndrome type?1 was made. In the treatment concept presented here the main goal was preservation of function of the left cranial nerves due to the functional loss of the right cranial nerves?X and XII. This goal could be achieved by means of a staged procedure in collaboration with radiotherapists.     

舌下神经-面神经侧端吻合术治疗小脑脑桥角肿瘤术后面瘫

摘要：目的探讨舌下神经-面神经侧端吻合术治疗小脑脑桥角肿瘤术后面瘫的效果。方法6例小脑脑桥角肿瘤切除术后面瘫患者均行舌下神经-面神经侧端吻合术。所有患者术后每3个月随访1次,评估House Brackmann(H B)分级和舌下神经功能。结果术后1年H B III级2例,H B Ⅳ级3例, H B V级1例。静态面部张力4例患者在吻合术后6个月改善明显,1例患者在吻合术后 9个月改善,1例患者在吻合术后1年改善。所有患者均未出现术侧舌肌瘫痪萎缩,发音和吞咽功能均正常。结论舌下神经-面神经侧端吻合术可改善小脑脑桥角肿瘤切除术后面瘫患者的面部张力和面肌功能,借助神经监护可尽量减小对舌肌功能的影响。     

Cochlear implantation after surgery for bilateral jugular foramen paragangliomas: a case report

This paper describes a case of cochlear implantation for deafness due to bilateral glomus jugulare tumors (paragangliomas) as well as an unusual complication after cochlear implantation in a 31-year-old male. A 31-year-old male with profound sensorineural hearing loss on the right side and deafness on the left side, caused by bilateral jugular foramen paragangliomas, was implanted with a multi-channel cochlear implant (Combi 40+, Med-El) on the right side during subtotal petrosectomy for removal of the right-sided glomus jugulare tumor. No postoperative medical complications were observed. The patient responded to acoustic stimuli. Postoperative computed tomography (CT) did not show any misplacement of the electrode. Three years after implantation, an acute, rapidly progressive hearing impairment with pain to acoustic stimuli was observed. A CT scan at that time showed cochlear bone resorption. No radiologic evidence of paraganglioma recurrence was observed. The implant was removed and a biopsy of the cochlea was performed which on histologic examination showed fibrosis without any sign of any tumor recurrence. Cochlear implantation can restore hearing in deafness due to bilateral glomus jugulare tumors. Cochlear resorption may occur as a late complication in the implanted side in the patients.     

Diagnostic evaluation of temporal paragangliomas

Paragangliomas are rare tumours deriving from paraganglia--small bodies of neuroendocrine tissue. Approximately 3% of paragangliomas occur in the head and neck area accounting for about 0,6% of all tumours of this region. Head and neck paragangliomas are divided into two groups: neck tumours (carotid body tumor and vagal paraganglioma) and temporal paragangliomas (jugular bulb tumor and tympanic paraganglioma). Temporal paragangliomas arise from paraganglia located in the petrous part of the temporal bone. Radiological evaluation of these lesions is essential for confirming the diagnosis and surgery planning. Most useful methods are magnetic resonance imaging and computed tomography. These modalities deliver important information about tumour location, extension and the state of the surrounding structures. Carotid angiography is a second step diagnostic method, performed in patients with jugular bulb paraganglioma. It has a diagnostic value and allows palliative or preoperative embolization of the tumor. Ultrasonographic evaluation and scintigraphy also provide advantages in diagnostic management.     

Accessory nerve schwannoma extending extracranially to the second cervical level of vertebra

We described a 67-year-old man who had a right jugular foramen tumour expanding extracranially to the level of C2 cervical body. Paralysis of the glossopharyngeal, vagal, accessory, hypoglossal nerves and sensorineural hearing disturbance were found. The tumour originated from the accessory nerve and the histological examination revealed it was schwannoma. Accessory nerve as an origin of the jugular foramen neurinoma and its pre-operative neurological signs are reviewed.     

Partial neurolysis of the hypoglossal nerve for selective lingual atrophy in a porcine model

OBJECTIVES: Obstructive sleep apnea in children is most commonly treated with adenotonsillectomy; however, in cases of significant tongue base prolapse, this may prove inadequate. Surgical procedures used to increase the retroglossal airway have significant morbidities and low patient acceptability. We theorized that effective reduction of the tongue base can be achieved through partial denervation of the hypoglossal nerve, which is easily accessed in the submandibular space with minimal morbidity. METHODS: We performed a prospective, experimental study in which topographic innervation maps of porcine tongue were generated by stimulating the hypoglossal main trunk and peripheral branches. The effects of complete unilateral nerve sectioning on tongue base volume and linear dimensions were measured and compared to the contralateral control side. In the final stage, only the peripheral nerve that was determined as the main supply to the tongue base was sectioned, and the results were compared to those in the matched controls. RESULTS: A medial branch of the hypoglossal nerve was consistently identified as the main motor supply to the tongue base. Complete denervation resulted in a measurable decrease in the volume of the tongue base as compared to that of controls. Partial neurolysis produced inconsistent changes in 2 subjects, with decreases in linear dimensions that were not proportional to the decrease in volume. Histologically, complete denervation was followed by a significantly greater replacement of muscle with fat and connective tissue as compared with partial neurolysis. CONCLUSIONS: There were inconsistent changes in volume and linear dimensions of the tongue base following partial neurolysis of the hypoglossal nerve over the 3-month experimental period.     

Clinical Presentation and Management of Jugular Foramen Paraganglioma

Objectives

Jugular 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.

Methods

Retrospective 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.

Results

Most 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.

Conclusion

Neurologic 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.     

Computed tomography's ability to predict sacrifice of hypoglossal nerve at resection

OBJECTIVE: To assess whether preoperative computed tomography (CT) scan can determine if the hypoglossal nerve (cranial nerve XII) will be sacrificed in floor-of-mouth, oral tongue, and tongue base tumor resections. STUDY DESIGN: Retrospective review. METHODS: Patients who underwent resection of floor-of-mouth, oral tongue, and tongue base tumors from 1990 to 1999 were identified. Preoperative CT scans were reviewed by a neuroradiologist. The postoperative status of cranial nerve XII was predicted to be "saved" or "sacrificed." Hypoglossal nerve "sacrifice" was predicted if the fat planes surrounding the takeoff of the proximal lingual artery were obliterated by tumor. The nerve was determined to be sacrificed or spared during resection by review of the operative report. RESULTS: Of the 45 patients, 14 tumors were predicted radiographically to involve the hypoglossal nerve. Twenty-seven of 31 nerves that were predicted to be saved were saved at the time of surgery. Seven of 14 nerves that were predicted to be sacrificed were sacrificed at the time of surgery. The sensitivity was 0.64 (95% confidence interval [CI], 0.35-0.86) with a specificity of 0.79 (95% CI, 0.70-0.87). The positive predictive value was 0.50 (95% CI, 0.27-0.68) with a negative predictive value of 0.87 (95% CI, 0.77-0.95). CONCLUSIONS: The ability to predict preoperatively whether a tumor can be resected without sacrificing the hypoglossal nerve would be an important factor in determining management of these tumors. The results indicate that CT scan accurately predicts the ability of the surgeon to spare the hypoglossal nerve (negative predictive value, 87%) with a specificity of 0.79.     

Glomus vagale: Paraganglioma of the vagus nerve

Review of the experience of a single institution with a rare tumor may give inadequate and biased information. In an effort to better understand issues related to diagnosis and subsequent management of paraganglioma of the vagus nerve, review of the experience at two university medical centers was undertaken. A review of the records of all patients with a diagnosis of vagal paraganglioma at the University of Pittsburgh and the Eastern Virginia Medical School was undertaken. Presenting signs and symptoms, treatment, and subsequent outcome were assessed. Data on 19 patients with vagal paraganglioma were available. The most common initial finding was a neck mass. Forty-seven percent (9/19) had vocal cord paralysis at presentation. Angiography was beneficial in helping to make the diagnosis and in identifying synchronous tumors. History of familial paraganglioma was present in 47% (9/19). Multiple paragangliomas were diagnosed in 53% (10/19) cases in our study. Of the patients with familial vagal paraganglioma, 89% (8/9) had multiple paraganglioma. All patients treated with surgery had postoperative vocal cord paralysis. Elective management of patients with bilateral vagal paraganglioma is a special dilemma. Radiation therapy of selected “operable” patients may be indicated because of the potential for bilateral vocal cord paralysis in these patients and its attendant sequela.     

Determination of the changes in the hypoglossal nerve function after suspension laryngoscopy with needle electromyography of the tongue

The purpose of this study was to determine changes in the hypoglossal nerve function after suspension laryngoscopy with needle electromyography of the tongue. This study also attempted to determine the possible relationship between the predictive factors of intubation difficulty by using the intubation difficulty scale, which was introduced by Adnet et al., duration of suspension laryngoscopy and changes in hypoglossal nerve function after suspension laryngoscopy. The study was performed on 39 patients who underwent suspension laryngoscopy for benign glottic pathology. Pre-operative airway assessment was evaluated by the intubation difficulty scale and the duration of suspension laryngoscopy was recorded. Needle electromyography of the tongue was performed three or four weeks after the suspension laryngoscopy. After needle electromyography of the tongue, increased polyphasia was found in 13 patients (33 per cent), bilaterally in three of them. The interference pattern was reduced in two of these 13 patients. There was no statistically significant difference in predictive factors of intubation difficulty and the duration of the operation between these 13 patients with increased polyphasia and the remaining 26 patients with completely normal electromyography findings. These findings show that, in spite of normal clinical tongue function, subclinical changes can be detected by needle electromyography of the tongue after suspension laryngoscopy.     

Prognostic Indicators for Squamous Cell Carcinoma of the Oral Cavity: A Clinicopathologic Correlation

Fifty-three patients with T1 squamous cell cancer of the floor of mouth and ventral surface of the tongue with a known clinical outcome were retrospectively analyzed and arbitrarily divided into “aggressive” and “nonaggressive” groups based on their clinical behavior. Various host and tumor factors were then evaluated in an attempt to determine whether the tumor behavior could have been predicted. The paraffin-embedded tumor specimens were evaluated for tumor differentiation, tumor thickness and tumor invasion, microvessel density, and p53 expression. In addition, a composite morphologic grading score was obtained by combining cell differentiation, nuclear polymorphism, mitosis activity, depth of infiltration, type of infiltration, and lymphatic infiltration. No single technique appeared capable of identifying “aggressive” behavior, although possibly an evaluation of composite factors might show promise in the future.     

Thrombosis of the internal jugular vein in the ENT-department — Prevalence,causes and therapy: A retrospective analysis

ObjectiveLess than 5% of deep vein thrombosis is due to thrombosis of the internal jugular vein. Genetic, malignant or inflammatory underlying diseases as well as insertion of venous catheters can be responsible for this pathology. Due to its rare occurrence, it is difficult to find systematic research about thrombosis of the internal jugular vein.MethodsWe performed a systematic analysis of present patient data from our ENT department with the electronic patient record considering the period from 2012-2017. Search terms were “thrombosis” and “jugular internal vein”. We identified 41 patients with the requested diagnosis and performed further analysis of the cases. Internal jugular vein thrombosis was diagnosed in all patients using Duplex sonography and/or CT/MR angiography.ResultsParaneoplastic thrombosis was found in 22/41 patients (54%), in 15 of the 22 (68%), the tumor was located in the ENT region. Two out of seven (29%) of the patients with tumor entities outside the head and neck region had thrombosis of the internal jugular vein as the first symptom of the disease. Another 14/41 patients (34%) had underlying inflammatory diseases – mostly streptococci-associated – for example a cervical abscess. In two patients, insertion of a central-venous catheter was causal, in three patients we could not find any reason for the development of thrombosis.ConclusionTo diagnose the rare and often asymptomatic thrombosis of the internal jugular vein, ultrasound of the cervical region should always include vascular imaging. Thrombosis of the internal jugular vein results mostly paraneoplastic or due to inflammation/abscess. It can be the first symptom of a malignant primary disease and always requires detailed diagnostic clarification.Level of evidence4.     

Vagal paraganglioma. Apropos of 2 cases. Review of the literature

The authors relate the case of two patients of 61 and 64 years old, each one having a high latero-cervical tumor, arching the soft palate, developed from the right vagus nerve and which was a chromaffin paraganglioma. After a recall about the APUD system tumors and the "paraganglioma" concept, they relate their observations at state of the vagal paraganglioma histology, pathology and treatment.