Blue laser for the exclusive endoscopic transcanal approach to middle ear paraganglioma.

BACKGROUND: The management of glomus tympanicum tumours can be challenging. Blue laser coagulation may improve bleeding control thus facilitating an endoscopic transcanal excision. The objective of this presentation is to illustrate the authors' experience using this novel tool. METHODS: Case report of a patient that underwent exclusive endoscopic transcanal blue laser surgery of a class A2 glomus tympanicum tumour in a tertiary referral center. CONCLUSION: The present study provides evidence of the safety and efficacy of endoscopic blue laser surgery, for the minimally invasive treatment of early-stage glomus tympanicum tumours.

Transcanal endoscopic ear surgery for glomus tympanicum removal: A case series on the necessity of preoperative embolization.

BACKGROUND: Transcanal endoscopic ear surgery (TEES) has become popular in recent years in the treatment of glomus tympanicum tumors (GTT). The most significant risk for TEES is bleeding. In some cases, preoperative vascular embolization is performed to mitigate bleeding during TEES. However, guidelines regarding the necessity and efficacy of preoperative vascular embolization have not been established yet. CASE PRESENTATION: This report aimed to assess the necessity and usefulness of preoperative vascular embolization in TEES for GTT by comparing the surgical findings of TEES without preoperative vascular embolization (Case 1) and TEES with preoperative vascular embolization (Case 2). Compared to Case 1, Case 2 included less bleeding and a more convenient procedure. However, no significant difference was observed. CONCLUSIONS: For GTT confined to the middle ear cavity (Glasscock-Jackson Grade II or less), when performed by a proficient otolaryngologist, TEES alone is sufficient without preoperative vascular embolization.

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.

A Case for Genetic Testing in Isolated Tympanic Paragangliomas.

OBJECTIVE: The aim of this study is to describe two clinical cases, which we believe highlight the need to consider routine genetic testing of all patients with new diagnosis of a tympanic paraganglioma (PGL). PATIENTS: Two patients seen in the ENT clinic at a tertiary center with a diagnosis of isolated tympanic PGL, without family history. INTERVENTION: Since 2016, all patients with newly diagnosed isolated tympanic PGL (glomus tympanicum) are offered review by the clinical genetic team and genetic testing of a panel of paraganglioma/phaeochromocytoma predisposition genes. Previously only those with multiple PGL or a family history were tested. MAIN OUTCOME MEASURES: We describe the results of genetic testing, the clinical course and discuss the ongoing implications for management. RESULTS: Both cases were identified to have a pathogenic variant in the SDHB gene after initial surgery. The clinical course for both cases was complicated by disease recurrence, as well as metastatic and secretory disease in one case. Knowledge of genetic status has influenced ongoing management, with annual MRI surveillance for other SDH-related tumors. CONCLUSION: These two cases reinforce the importance of offering genetic testing for all cases of isolated tympanic PGL. The discovery of a significant underlying genetic variant may affect management decisions and subsequent follow-up.

Management of jugular tympanic paraganglioma: a case report.

Paragangliomas could be localized from the skull base to the pelvic floor. Tympanic localization represents the most common benign tumor of the middle ear. Diagnosis is based on clinical signs with a great contribution of radiology. A 40-year-old male presented with isolated tinnitus of the right ear evolving for 18 months. Examination revealed a red bulging right-sided tympanic membrane and a conductive hearing loss. Tomodensitometry and Magnetic resonance imagery showed findings in favor of a right jugular tympanic paraganglioma. The tumor was classified type B according to FISCH classification. The patient underwent surgery consisting in tympanotomy using a retro auricular access route. The postoperative course was uneventful. There was no recurrence during the one-year follow-up. Jugular tympanic paraganglioma diagnosis is guided by a combination of epidemiological, clinical and radiological features. Treatment is still not consensual, but surgery still have its indications in localized forms of head and neck paragangliomas (HNP´s).

Nuances in transcanal endoscopic surgical technique for glomus tympanicum tumors.

OBJECTIVE: To describe the utility and nuances of transcanal endoscopic surgery (TCES) on glomus tympanicum tumors from a single surgeon's experience. PATIENTS/INTERVENTION: Twelve patients, eight female and four males, diagnosed pre-operatively with glomus tympanicum tumors. They all underwent endoscopic resection by a single surgeon. MAIN OUTCOME MEASURES: Feasibility of endoscopic resection of glomus tympanicum tumors without conversion to a microscopic approach. Secondary outcomes include tumor stage, pre and post-operative audiometry, vertigo, sensorineural hearing loss (SNHL) and integrity of the facial nerve, ossicles, chorda tympani and tympanic membrane. RESULTS: Twelve patients underwent TCES, eight patient's pathology results were glomus tympanicum, ranging from Glasscock-Jackson grade I-III. Due to loss in follow up, 6/8 patients had complete audiometric data, which were analyzed. Average pre-operative air-bone-gap (ABG) was 5.41 compared to post-operative ABG of 5.08 (p > 0.89). No patients resulted in any, post-operative vertigo, tinnitus, SNHL, facial nerve injury or chorda tympani nerve injury. Two patients had intentional tympanic membrane perforations secondary to tumor adherence to the membrane. They were repaired with tragal perichondrium graft. No patients have had any recurrences. CONCLUSIONS: Endoscopic resection of glomus tympanicum tumors is a feasible and effective, alternative visualization modality for the neurotologist. Surgical pearls are described herein.

Tumor de glomus yugulotimpánico, a propósito de un caso / Jugulotympanic glomus tumor, case presentation

Resumen Los tumores de glomus yugulo-timpánico o paragangliomas son originados de células de la cresta neural y representan un número bajo de los tumores de cabeza y cuello (0,5 %). Su crecimiento lento y síntomas progresivos provocan que se retarde el diagnóstico. Conocer la clínica que frecuentemente cursa con pérdida auditiva unilateral, tinnitus pulsátil y afectación de algún par craneal, puede ayudar a sospechar el diagnóstico de glomus. Se mencionan diferentes tipos de métodos diagnósticos, así como nuevas opciones de tratamiento. Dada la localización y su importante vascularización, hay casos en los que la cirugía abierta es muy riesgosa y se buscan procedimientos terapéuticos que disminuyan las complicaciones y morbilidades en estos pacientes, tanto como procedimientos endovasculares. En el artículo se discute el caso de una femenina de 31 años con clínica de inicio insidioso con hipoacusia izquierda progresiva, tinnitus pulsátil izquierdo y disfonía. En 2013, se realiza resonancia magnética nuclear donde se observa proceso infiltrativo tumoral sugestivo de paraganglioma. Se hace embolización endovascular con onyx de glomus yugulotimpánico en cuatro ocasiones, con seguimiento cada 6 meses por no ser candidata a otro tipo de tratamiento, debido al tamaño y localización de la masa y su condición no resecable.

Abstract Jugulo-tympanic glomus tumors or paragangliomas originate from neural crest cells and represent a low number of head and neck tumors (0.5%). Its slow growth and progressive symptoms cause the diagnosis to be delayed. The knowledge of the clinic that frequently involves unilateral hearing loss, pulsatile tinnitus and cranial nerve involvement can help to suspect the diagnosis. Different types of diagnostic methods are helpfun, as well as new treatment options have been proposed. Given the location and its important vascularization, there are cases in which open surgery is highly risky and alternative therapeutic procedures trying to reduce complications and morbidities in these patients need to be considered, as well as endovascular procedures. This article discusses the case of a 31-year-old female with an insidious onset of progressive left hearing loss, left pulsatile tinnitus and dysphonia. In 2013, nuclear magnetic resonance imaging was performed and a tumor infiltrative process suggesting paraganglioma was observed. Endovascular embolization was performed with onyx of jugulotympanic glomus on four occasions, with follow-up every 6 months, due to the size and location of the mass and its unresectable condition the tumor was considered non surgically resectable.

KTP-laser-assisted endoscopic management of glomus tympanicum tumors: A case series.

Endoscopic technology is widely used in rhinology and anterior skull base surgery, but it has been less quickly incorporated into otologic practice. The design of the instrumentation forces surgeons to work one-handed and limits depth perception. Nevertheless, endoscopy also offers wide fields of view and access to spaces that are typically difficult to visualize. Its advantages have broadened the type and extent of operations that can be performed via the ear canal. We describe a method of endoscopic resection of glomus tympanicum tumors in 5 adults who had undergone endoscopic or endoscopy- assisted resection. A successful resection was achieved in all patients-exclusively via the ear canal in 4 of them. A KTP laser was used to address the tumor's vascular supply. Attachment of a neonatal feeding tube to the endoscope for use as a suction catheter obviated the need to repeatedly switch instruments while using the laser. At a minimum of 12 months of follow-up, all patients were free of recurrence. Postoperative audiometry detected no significant adverse hearing outcomes in any patient. We conclude that the minimally invasive endoscopic transcanal approach is a feasible technique for addressing middle ear tumors. We have also developed a method that allows surgeons constant use of the KTP laser to resect a glomus tympanicum tumor.

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.

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.

Endoscopic Management of Middle Ear Paragangliomas: A Case Series.

OBJECTIVE: To investigate the efficacy and safety of endoscopic middle ear paraganglioma (glomus tympanicum) resection. STUDY DESIGN: Case series with chart review. SETTING: Multi-institutional tertiary university medical centers. PATIENTS: Adult patients with middle ear paragangliomas treated via a transcanal endoscopic approach from 1/2012 to 11/2015. INTERVENTION: All tumors were initially approached via a transcanal endoscopic technique. An operating microscope was used only if the tumor could not be adequately visualized or resected with endoscopic techniques alone. MAIN OUTCOME MEASURES: The main outcome was completeness of tumor resection via the endoscopic technique. Secondary measures were resolution of pulsatile tinnitus, audiometric outcomes, surgical duration, and surgical complications. RESULTS: Endoscopic resection was attempted on 14 middle ear paragangliomas. Thirteen patients (93%) were women with a mean age of 61.6 years. The mean tumor size was 6.2 mm (SD, 3.3). Eleven cases (79%) had complete resection via an exclusive endoscopic approach. The mean surgical duration was 108.1 minutes (SD, 55.6). One case required use of an operating microscope via a transcanal route and two cases required postauricular incisions with mastoidectomy. There were no significant postoperative complications. Two patients (14%) had tympanic membrane perforations repaired intraoperatively without residual perforation on follow-up. All patients had normal postoperative facial nerve function. Pulsatile tinnitus resolved after surgery in all 13 patients who presented with this symptom preoperatively. The mean pure-tone average improved by 5.9 dB (SD, 4.6) after surgery. CONCLUSIONS: Endoscopic management of middle ear paraganglioma is safe, feasible, and effective.

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.

Treatment of glomus tympanicum tumors by preoperative embolization and total surgical resection.

PURPOSE: The purpose of this study was to evaluate the effectiveness on function preservation and tumor control of the treatment of glomus tympanicum tumors with pre-operative embolization followed by total surgical resection. MATERIAL AND METHODS: We describe a series of 6 patients with a glomus tympanicum tumor who were treated in our hospital using the same technique: the day before surgery selective tumor embolization due to denaturation with 96% ethanol. Following parameters were considered: tumor classification, tumor control, clinical and audiological outcome, effectiveness of embolization, percentage of tumor necrosis and treatment complications. RESULTS: There were no severe complications due to embolization or surgery. Tumor blush disappeared completely in 5 patients on DSA post embolization and histologic evaluation of the resected tissue showed a median of 69.2% of tumor necrosis. Pulsatile tinnitus disappeared in all patients and 3 patients had no symptoms at all. Hearing ameliorated in 4 patients, 1 patient without hearing loss pre- treatment still had normal hearing after treatment and 1 patient's hearing was worse after treatment. Average follow-up was 21.3months. CONCLUSIONS: Treatment of glomus tympanicum tumors by pre-operative embolization with ethanol and surgical resection has not been described before. Our results show that it is a safe procedure with a good long term tumor control, good clinical and audiological outcome.

(111)In-octreotide SPECT-CT in head and neck paragangliomas.

Head and neck paragangliomas are rare neuroendocrine tumors expressing somatostatin receptors on their cell surface, particularly subtype 2. Due to this distinctive feature, images can be obtained in Nuclear Medicine using synthetic analogues of somatostatin, mainly octreotide, which allow selective display by planar scintigraphy, SPECT-CT or PET-CT imaging after radiolabeling with (111)In or (68)Ga. Three cases have been selected and presented from a series of patients that illustrate the utility of SPECT-CT studies with (111)In-octreotide in the diagnosis and monitoring of this type of tumor. These are characterization at initial diagnosis, staging, and detection of local recurrence or metastasis, with added value with respect to anatomical images (nuclear magnetic resonance, computed axial tomography, angiography), for example in the differentiation between functional tissue or scar in patients who had previously undergone surgery.

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.

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.