Clinical characteristics and surgical strategy of glomus tympanicum tumors. / 鼓室体瘤的临床特征及手术策略.

OBJECTIVES: To summarize the clinical characteristics of glomus tympanicum tumors, and to explore the surgical methods and the strategy for auditory protection. METHODS: Ten cases (ears) of glomus tympanicum tumors were collected from the Department of Otolaryngology Head and Neck Surgery, Xiangya Hospital, Central South University from August 2014 to February 2022. All patients underwent endoscopic or microscopic surgery to achieve total removal of the tumor, followed up for 3 months to 8 years. We summarized and analyzed its clinical characteristics, compared the preoperative and postoperative hearing levels of patients, and made a retrospective summary of the surgical methods and the strategy for auditory protection. RESULTS: Ten patients were all female at (49.50±8.00) years old. Their medical history ranged from 15 days to 6 years. Seven patients complained of pulsatile tinnitus, and 80% (8/10) of the affected ears suffered different degrees of hearing loss. According to the modified Fisch & Mattox classification of glomus tympanicum tumors, 3 ears (30%) of 10 ears were A1, 2 ears (20%) were A2 and 5 ears (50%) were B1. In all 10 cases (ears), hearing was improved in 3 cases, bone gas conductance was maintained in 6 cases, and hearing was slightly decreased in 1 case. The difference of bone gas conductance was 0-10 dB in 7 cases (ears) after operation, and 10-20 dB in 3 cases (ears). There was no significant difference in the average air conduction hearing threshold, bone conduction hearing threshold and air-bone conduction difference between before and after operation (all P>0.05). All cases had no postoperative complications, and the external auditory canal and the incision behind the ear healed well. There was no recurrence after follow-up. CONCLUSIONS: Glomus tympanicum tumor is easy to bleed, so it is a challenge for total tumor resection and hearing function protection during operation. For type A and type B1 tumors, they can be completely removed under the condition of keeping the tympanic membrane and the ossicular chain. At the same time, the postoperative hearing function can be preserved, and even the hearing can be improved.

Extramedullary Hematopoiesis Presenting as Bilateral Glomus Tympanicum-First Case Report in a Pediatric Patient and With Bilateral Presentation.

To the best of our knowledge, this is the first case report of middle ear extramedullary hematopoiesis (EMH) in a pediatric patient as well as the first bilateral presentation reported in both children and adults. We report a 13-year-old African-American female with sickle cell disease who developed bilateral hearing loss, with magnetic resonance imaging findings consistent with bilateral glomus tympanicum (GT). Upon excisional biopsy, however, EMH was diagnosed histologically. Besides its novelty, this case highlights the importance of considering EMH in the differential diagnosis of GT including cases with bilateral presentation that may be otherwise highly suggestive of the familial form of GT.

Results of a systematic literature review of treatment modalities for jugulotympanic paraganglioma, stratified per Fisch class.

OBJECTIVE: Key for successful jugulotympanic paraganglioma management is a personalised approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control and complication rates for the different treatment modalities stratified by the broadly accepted Fisch classification. DESIGN: A systematic literature review according to the PRISMA statement was performed. A detailed overview of individual treatment outcomes per Fisch class is provided. MAIN OUTCOME MEASURES: Local control, cranial nerve damage, complications, function recovery. RESULTS: Eighteen studies were selected, resembling 83 patients treated with radiotherapy and 299 with surgery. Excellent local control was found post-surgery for class A and B tumours, and risk of cranial nerve damage was <1%. For class C1-4 tumours, local control was 80%-95% post-surgery (84% post-radiotherapy), and cranial nerve damage was found in 71%-76% (none post-radiotherapy; P < .05). There was no difference in treatment outcomes between tumours of different C class. For class C1-4De/Di tumours, local control was 38%-86% (98% post-radiotherapy; P < .05) and cranial nerve damage/complication rates were 67%-100% (3% post-radiotherapy; P < .05). C1-4DeDi tumours showed lesser local control and cranial nerve damage rates when compared to C1-4De tumours. CONCLUSIONS: An individual risk is constituted for surgery and radiotherapy, stratified per Fisch class. For class A and B tumours, surgery is a suitable treatment option. For class C and D tumours, radiotherapy results in lower complication rates and similar or better local control rates when compared to the surgical group.

A blue middle ear mass: Cholesterol granuloma mimicking a glomus tumor and endolymphatic sac tumor.

Cholesterol granuloma (CG) is the most common benign lesion of the petrous apex, however, it can grow significantly large and become destructive causing a diagnostic dilemma. This case presents a 25-year-old female with 2-year history of left-sided progressive and profound hearing loss, a transient left-sided facial paralysis and cranial nerve 10 palsy who presented with a blue middle ear mass. Her diagnosis did not become apparent until direct visualization intraoperatively. The objective of this case study is to highlight the destructive capabilities of CG and the importance to keep it in the differential diagnosis of a large, erosive, expansile skull base lesion in order to avoid overly aggressive resection or other unnecessary treatment.

Glomus tympanicum tumors.

Glomus tympanicum (GT) tumors are benign arising from paraganglion cells of the tympanic plexus in the middle ear. Although surgical resection remains the best option for definitive treatment of these tumors, the diagnostic and management algorithms have evolved considerably with the introduction of high-resolution computed tomography, MRI, and genetic testing.

Long term surgical and hearing outcomes in the management of tympanomastoid paragangliomas.

OBJECTIVE: To analyze the long term outcomes after surgery in tympanomastoid paragangliomas. STUDY DESIGN: Retrospective study. METHODS: The charts of 145 patients with tympanomastoid paragangliomas managed between 1988 and 2013 were reviewed. The clinical features, audiological data, pre- and postoperative notes were noted. The tumors were staged according to the modified Fish and Mattox classification. The surgical approaches for all patients were formulated according to the surgical algorithm developed at our center. RESULTS: 34 (23.5%), 46 (31.7%), 22 (15.2%), 18 (12.4%) and 25 (17.2%) patients were diagnosed to have TMP class A1, A2, B1, B2 and B3 tumors respectively. Gross tumor resection was achieved in 141 (97.2%) patients. The facial nerve was uncovered in four patients and infiltrated in three. The cochlea was found eroded in seven cases. The mean follow-up was 48.4 months. Recurrence was seen in one patient (0.7%). In the cases where the facial nerve was preserved (n=143), the nerve function was graded as HB grade 1 in 138 patients (97%). Postoperatively, the mean AC showed an improvement in all categories except in class B2 and B3, which corresponds to the classes that include patients who underwent subtotal petrosectomy. CONCLUSION: We report the long term surgical outcomes in tympanomastoid paragangliomas in the largest series published till date. It is possible to completely eradicate all types of tympanomastoid paragangliomas with minimum sequelae by choosing the correct surgical approach to achieve adequate exposure for individual tumor classes as described in our classification and algorithm. LEVEL OF EVIDENCE: IIb.

Diagnosis and management of multiple paragangliomas of the head and neck.

Paragangliomas (PGs) are slowly growing, usually benign neoplasms. The aim of the study was to analyze the incidence, diagnostic and therapeutic management of patients with multiple paragangliomas of the head and neck. A retrospective review of the records of 84 patients with head and neck PGs, diagnosed and treated in our institution was performed for the years 1983-2013 to identify patients with multiple tumors. Fourteen (16.6 %) patients developed multiple PGs, synchronous or metachronous, within 4-21 years of follow-up. Clinical data of these patients were reviewed to evaluate the diagnosis, location, stage and management strategy. There was a total number of 37 tumors in 14 patients. There were 20/37 (54.0 %) carotid PGs, 9/37 (24.3 %) jugular PGs and 8/37 (21.7 %) vagal PGs. Carotid PGs were observed in 12/14 (86 %) patients and in 8/14 (57 %) cases bilateral tumors occurred. Vagal PGs developed in 7/14 (50 %) patients and bilateral tumors were found in 1/14 (7 %) case. Jugular PGs occurred in 9/14 (64 %) patients. There were 30 synchronous tumors and seven metachronous PGs diagnosed 2-18 years after removal of the first tumor. Single metachronous mediastinal PG occurred. All patients had at least one tumor removed, with histopathological confirmation of the diagnosis. One patient had positive history of familial PGs. Carotid PGs are most common multiple paragangliomas. Radiological survey of the head and neck is required to detect multicentric tumors. Metachronous mediastinal and abdominal tumors may occur. Regular, prolonged follow-up is essential to identify metachronous PGs and possible postoperative gradual ICA occlusion.

Leksell Gamma Knife radiosurgery of the jugulotympanic glomus tumor: long-term results.

OBJECT: Glomus tumors usually display indolent behavior, and the effectiveness of radiation in stopping their growth can be assessed after long-term follow-up. Currently only midterm results of radiosurgery are available, so the authors included patients treated by Gamma Knife at least 10 years ago in this study to obtain a perspective of long-term results. METHODS: During the period from 1992 to 2003, the Gamma Knife was used to treat 46 patients with glomus tumors. The age of the patients ranged from 21 to 79 years (median 56 years). Gamma Knife radiosurgery was the primary treatment in 17 patients (37%). Open surgery preceded radiosurgery in 46% of cases, embolization in 17%, and fractionated radiotherapy in 4%. The volume of the tumor ranged from 0.2 to 24.3 cm(3) (median 3.6 cm(3)). The minimal dose to the tumor margin ranged between 10 and 30 Gy (median 20 Gy). RESULTS: One patient was lost for follow-up after radiosurgery. Clinical follow-up was available in 45 patients and 44 patients were followed with MRI in a follow-up period that ranged from 12 to 217 months (median 118 months). Neurological deficits improved in 19 (42%) of 45 patients and deteriorated in 2 patients (4%). Tumor size decreased in 34 (77%) of 44 patients with imaging follow-up, while an increase in volume was observed in 1 patient (2%) 182 months after radiosurgery and Gamma Knife treatment was repeated. One patient underwent another Gamma Knife treatment for secondary induced meningioma close to the glomus tumor 98 months after initial radiosurgical treatment. Seven patients died 22-96 months after radiosurgery (median 48 months), all for unrelated reasons. CONCLUSIONS: Radiosurgery has proved to be a safe treatment with a low morbidity rate and a reliable long-term antiproliferative effect.

[Imaging evaluation of the cases with tympanicum and pulsatile tinnitus].

OBJECTIVE: To analyze the CT and MRI features of glomus tympanicum tumors accompanied with tympanitis and evaluate the diagnostic value of CT and MRI in order to improve the cognition for the disease. METHODS: The clinical materials and images of 8 patients with the symptoms of pulsatile tinnitus and hearing loss in whom glomus tympanicum tumors with tympanitis surgically and pathologically confirmed were retrospectively reviewed. The characteristics and diagnostic value of CT and MR imaging were summarized. RESULTS: By CT examination the lesions in middle ear and mastoid were preoperatively diagnosed as tympanitis in five cases and only in three cases the glomus tympanicum tumors were suspected. In six patients underwent MR examination the lesions were all preoperatively diagnosed as glomus tympanicum tumors accompanied with tympanitis. HRCT scanning of the temporal bone in all patients showed the soft tissue lesions in the tympanic cavity and mastoid, and the caritas tympanic were mostly (n = 3) or completely (n = 5) occupied by soft tissue lesions, but the auditory ossicles were all without destruction. Contrast-enhanced axial CT scanning performed in five cases showed less soft tissue mass on the cochlear promontory, and the size of mass was less than that observed in MR imaging. MR T(1)-weighted imaging showed the presence of isointense lesions in middle ear and isointense (n = 3) or hyperintense (n = 3) lesions in mastoid. On T(2)-weighted imaging the lesions with slight hyperintense were viewed in the middle ear and the lesions with hyperintense in mastoid. T(1)-weighted gadolinium-enhanced MRI showed the masses in tympanum were markedly increased enhancement, but the lesions in mastoid without enhancement. MRI and CT imaging revealed the masses in six cases of eight extending to the eustachian tube. CONCLUSION: When the glomus tympanicum tumor was accompanied with tympanitis the tumor could be misdiagnosed or missed only by CT examination. The patients with pulsatile tinnitus should be taken seriously. MRI with contrast-enhancement is superior to CT in the preoperative diagnosis and accurately evaluation for the glomus tympanicum tumors with tympanitis.

[Endocrine surgery for neck paraganglioma: operation, radiation therapy or wait and scan?]. / Endokrine Chirurgie zervikaler Paragangliome: Operation, Strahlentherapie oder "wait and scan"?

BACKGROUND: Head and neck paraganglioma (HNP) represent rare endocrine tumors. Therapy is decided on genetic findings, tumor characteristics (e.g. tumor size, localization and dignity), age of patient and symptoms. In terms of local control radiation therapy is as equally effective as surgery but surgical morbidity rates secondary to cranial nerve injuries remain high. PATIENTS: Based on 6 patients with 11 solitary (4 patients) and multiple (2 patients) HNP (8 carotid body tumors, 1 vagal, 1 jugular and 1 jugulotympanic paraganglioma) the specific characteristics of the need for surgery as well as correct choice of treatment in cases of sporadic succinate dehydrogenase (SDH) negative and hereditary SDH positive HNP will be exemplarily demonstrated. RESULTS: A total of 6 carotid body tumors (four sporadic, two hereditary) were resected in 4 patients, five as primary surgery and one as a revision procedure. In one case a preoperative embolization was performed 24 h before surgery. Malignancy could not be proven in any patient. The 30-day mortality was zero. In the patient with bilateral hereditary carotid body tumors, unilateral local recurrent disease occurred. After resection of the recurrent tumor permanent unilateral paralysis of the laryngeal nerve, glossopharyngeal nerve and hypoglossal nerve occurred. All patients were followed-up postoperatively for a mean of 64 months (range 23-78 months) with a local tumor control rate of 100%. The overall survival rate after 5 years was 100%. CONCLUSIONS: Given a very strict indication with awareness of surgical risks selective surgery has a key position with low postoperative morbidity in the treatment of HNPs. We prefer surgery for small unilateral paraganglioma, malignant or functioning tumors.

En bloc surgical removal of an asymptomatic glomus tympanicum tumor.

Glomus tympanicum (GT) tumors are usually characterized by pulsatile tinnitus and hearing loss. We report on a woman 53 years of age who was diagnosed with a GT tumor within her right ear with no associated tinnitus or hearing loss on presentation. An early GT tumor without the characteristic symptoms is seldom encountered. Although several papers have dealt with GT tumor diagnosis and management, very few have demonstrated such a condition in which the entire GT tumor appeared in the middle ear cavity and was totally removed during the operation. There was no recurrence at 24 months of follow-up. We describe a novel surgical technique in this article and demonstrate an en bloc GT surgical removal that has been seldom published before in the literature.

Management of complex tympanojugular paragangliomas including endovascular intervention.

OBJECTIVES/HYPOTHESIS: To identify complex cases of tympanojugular paraganglioma (TJP) and to analyze their management and surgical outcome. To present strategy and guidelines on how to deal with the perioperative complicating factors. STUDY DESIGN: Retrospective analysis. METHODS: We retrospectively studied 212 TJP patients to identify the complex cases and analyzed their management and surgical outcome. The criteria for complex TJP are: 1) very large size; 2) large intradural extension (IDE); 3) extension to the cavernous sinus, foramen magnum, and clivus; 4) significant involvement of the internal carotid artery (ICA); 5) a solitary ICA on the lesion side; 6) involvement of the vertebral artery; 7) dominant or unilateral sigmoid sinus on the lesion side; 8) bilateral or multiple paragangliomas; and 9) recurrence. RESULTS: Sixty patients were classified as complex. Forty-five patients had tumors with IDE. Twenty patients with tumor involving ICA underwent preoperative endovascular intervention. Two patients had a solitary ICA and two others unilateral sigmoid sinus on the lesion side. Thirteen patients had multicentric tumors, and three patients had bilateral ones. The main procedure was the infratemporal fossa approach type A. Total removal was achieved in 46 patients, and five of them experienced recurrence. CONCLUSIONS: It is essential to carefully investigate the hemodynamics of the brain in planning surgery. A proper preoperative endovascular intervention facilitates gross total tumor removal. In bilateral paragangliomas, lower cranial nerves' function should be preserved at least unilaterally. Staged removal is recommended for a tumor with a large intradural component.

A histological examination in the cases of initial diagnosis as chronic otitis media with a polypoid mass in the external ear canal.

OBJECTIVE: to analyse a large series of patients with initial diagnosis of chronic otitis media (COM) with a polypoid mass in the external ear canal (EAC). MATERIAL AND METHODS: 185 consecutive patients with COM were evaluated; 75 showed a polypoid mass in the EAC. RESULTS: In 65 out of the 75 (86.7%) patients, histological examination after mastoidectomy revealed cholesteatoma. In the remaining 10 (13.3%) cases, the histological diagnosis was: glomus tumor, melanoma, mucosal adenoma, fibrous dysplasia, squamous cell carcinoma, adenoma of the endolymphatic sac, encephalocele, and tuberculosis. One patient (glomus tumor) had undergone biopsy of the polypoid mass twice preoperatively and the initial histological diagnosis was "inflammatory polyp". Therefore, we examined the temporal bones with a glomus tumor diagnosis from the temporal bone collection of the House Ear Institute. In 1560 temporal bones, a glomus tympanicum was found in 6. In four cases, the tumor extended to the EAC mimicking a polyp covered by squamous epithelium. CONCLUSION: A significant percentage of polyps in COM may be the tip of an 'iceberg'. CT-scan and MRI techniques may be helpful but not always accurate, and biopsies have certain risks. Increased clinical suspicion may limit misdiagnosis and inappropriate management.

Clinical, histopathologic, and radiographic indicators of malignancy in head and neck paragangliomas.

OBJECTIVE: The goal of this study is to review our series of head and neck paragangliomas to identify factors that may help in predicting malignancy. STUDY DESIGN: Case series with chart review. SETTING: Academic medical center. SUBJECTS AND METHODS: Subjects with head and neck paragangliomas at our institution from 1976 to current were reviewed. In addition to statistical comparisons of epidemiologic factors, pathologic and radiographic characteristics were reviewed. RESULTS: Of the 84 subjects, there were seven malignant paragangliomas (8%). Age was found to be significantly different between the benign and malignant subgroups, with an average age of 54 ± 16 and 40 ± 12 years, respectively (P = 0.02). Pain was a presenting complaint in five patients with benign disease (6%), and five of the seven malignant patients (71%) presented with pain, showing a significant association between pain and disease type (P < 0.0001). The odds ratio for a patient with pain having a malignant tumor was 36 (95% CI: 5.5-234). Enlarging neck mass was noted in all cases of malignant disease, but only in 31 percent of cases of benign disease (P < 0.0001). In a secondary analysis of carotid body tumors alone, enlarging neck mass was not found to be significant between benign and malignant disease (P = 0.14). However, pain continued to be significantly different, with 67 percent of malignant lesions demonstrating pain, compared with only 11 percent of benign lesions (P = 0.01). CONCLUSION: This study suggests that pain, a rapidly enlarging neck mass, and younger age are predictive factors of underlying malignancy, which should prompt one to consider an aggressive diagnostic and management approach.

Middle ear adenomatous tumor: a not so rare glomus tympanicum-mimicking lesion.

BACKGROUND AND PURPOSE: Middle ear adenomatous tumors (MEAT) are rare tumors which can be begin or malignant and can present a neuroendocrine differentiation. Their radiological aspect is very similar to glomus tympanicum (GT) which are the most common tumoral lesions of the middle ear. We present several radiological and clinical findings that could help radiologists to accurately identify MEAT. MATERIAL AND METHODS: We retrospectively reviewed the radiological and clinical findings of three patients with MEAT and of eight patients with GT. Diagnostic was obtained after surgical resection in all cases. All patients had high resolution CT and MR of the middle ear associated with a subtracted digital carotid angiography. Tumor location, size, extension, signal intensity, and enhancement were analysed. From the medical records of the patients, clinical manifestations (hearing loss, tinnitus), evolution length and recurrences were noted. RESULTS: MEAT and GT appeared as tissular lesion with significant enhancement on CT and MR. A vascular blush was present on angiography in all cases of GT and absent from all cases of MEAT. A close relationship between the tumor and the Jacobson's nerve or its branches was identified in all cases of GT. Pulsatile tinnitus was present in all patients with GT and absent in all patients with MEAT. CONCLUSION: A middle ear tissular lesion clearly separated from the Jacobson nerve or its branches, showing significant enhancement after contrast medium injection but with a normal angiography, should make one suspicious for MEAT.

The crucial role of imaging in determining the approach to glomus tympanicum tumours.

Glomus tumours are rare vascular tumours arising within the lateral skull base, infratemporal fossa and upper neck. When they are confined to the middle ear cleft, they are termed glomus tympanicum tumours. Traditionally tympanicum tumours have been treated surgically and their removal has generally been regarded as quite straightforward. Our experience with these lesions however, is that they fall into two distinct categories, those with and those without hypotympanic involvement. This distinction can be determined by high resolution CT scanning in coronal and axial planes. Mesotympanic tumours are indeed typically straightforward in their removal and can be approached by a transcanal route. Hypotympanic extension requires additional evaluation by MRI, and a more extensive surgical approach. In this paper, we present our management approach based on a series of nine cases. We conclude that determining the tumour extension preoperatively by using modern imaging techniques is essential in planning the surgery.

[Jugulotympanic paraganglioma]. / Paraganglioma giugulo-timpanico.

Jugulo-tympanic paraganglioma is the most commonly recognized neoplasia involving the middle ear, arising from chemopressure receptors. Clinical presentation is variable, with otalgia, dizziness and cranial nerve palsies. This tumor usually occurs in middle aged women and its presentation may be familiar, sporadic, bilateral or multicentric. In the present case the patients presented with otalgia and bradyacusia diagnosed with chronic otitis. A bleeding polypoid lesion in middle ear was seen during surgery. Frozen section of intraoperative specimen showed monomorphic cells organized into nests, without aspects of malignancy, into a highly vascularized stroma. Immunohistochemical analysis revealed two type of cells: chief cells immunostained for chromogranin and synaptophysin and sustentacular cells immunostained for S-100 protein. Clinical presentation of jugulotympanic paraganglioma, when it isn't associated with evidence of hormone secretion, is referred prevalently to bradyacusia, dizziness, and cranial nerve palsies. In event of localized disease, paragangliomas often can be treated by surgical excision, but some cases need radiation therapy.