Postauricular Transcanal Posterior Tympanectomy (PT2) Approach - A Modified Surgical Technique for Jugulotympanic Paragangliomas

Abstract Introduction The surgical management of jugulotympanic paragangliomas has remained challenging. They are the second most common type of tumor of the temporal bone after acoustic neuroma. It has been noticed by the authors that the jugulotympanic paragangliomas may have extensions to the epitympanum and aditus in addition to the mesotympanum and hypotympanum. The modified technique could be an alternative to the conventional facial recess technique for complete removal of the tumors. Objective To highlight the modified surgical technique for the surgical treatment of jugulotympanic paragangliomas. Methods This is a retrospective review of 34 cases of jugulotympanic paragangliomas treated in a tertiary center with respect to clinical presentation, diagnosis, and surgical treatment. Tinnitus and hearing loss were predominant symptoms. A modified technique of postauricular transcanal posterior tympanectomy with extended hypo-tympanic access was performed in 29 patients. Only two cases were operated with a classical transcanal approach. A canal wall down the mastoidectomy was required in three patients. Results The patients operated on with the modified technique had complete excision evident by absence of any lesion in computed tomography and the disappearance of tinnitus. However, two patients had recurrence of symptoms and presence of tumor in the follow-up period. These two patients underwent revision surgery. None of the patients required postoperative radiotherapy or gamma knife therapy. Conclusions Jugulotympanic paragangliomas can be effectively managed with the modified technique to ensure complete removal of the lesions. This technique has not been reported earlier in the literature.

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.

Glomus tympanicum

Glomus tympanicum is a tumour classified under the group glomus tumours, and is also knownas paragangliomas. It is thought to commonly occur in women in the fifth to sixth decades of life.Here, we report a case of a 77-year-old lady with multiple co-morbids and a diagnosis of glomustympanicum presenting to us. Her symptoms included pulsatile tinnitus, and reduced hearing,and the management of the case was done with consideration for her underlying multiple comorbidities.This paper also describes the best modality of treatment for this patient with regardto her background history. The treatment goal was to improve her quality of life and control thedisease.

Granulation tissue mimicking a glomus tumor in a patient with chronic middle ear infection

@#<p style="text-align: justify;"><strong>OBJECTIVE:</strong> To present an unusual cause of pulsatile tinnitus, presenting in a young adult suffering from chronic recurrent foul-smelling discharge from the same ear.<br /><strong>METHODS:</strong><br /><strong>Design:</strong> Case Report<br /><strong>Setting:</strong> Tertiary National University Hospital<br /><strong>Patient:</strong> One<br /><strong>RESULTS:</strong> A 24 year-old woman presented with pulsatile tinnitus on a background of chronic recurrent foul-smelling discharge. Clinico-radiologic findings seemed consistent with a glomus tympanicum coexisting with chronic suppurative otitis media with cholesteatoma. She underwent tympanomastoidectomy with excision of the mass. Histopathologic evaluation revealed the mass to be granulation tissue.<br /><strong>CONCLUSION:</strong> Pulsatile tinnitus is rarely associated with chronic middle ear infection. Granulation tissue arising at the promontory may mimic glomus tumors when accompanied with this symptom. Despite this revelation, it would still be prudent to prepare for a possible glomus tumor intraoperatively so that profuse bleeding and complications may be avoided.</p>

A Case of Salivary Gland Choristoma Mimicking Glomus Tympanicum / 대한이비인후과학회지

Salivary gland choristoma is defined as the architecturally normal salivary gland tissues found in abnormal locations. Middle ear salivary gland choristoma usually presents with conductive hearing loss. We present a case of middle ear mass with conductive hearing loss in a 6-year-old boy. A reddish mass was incidentally found behind the normal tympanic membrane. Magnetic resonance image showed the markedly enhancing lesion which was in accordance with glomus tympanicum. Preoperative angiography was performed, but supplying artery and mass was not identified. Surgical exploration was made and the mass was dissected easily without profuse bleeding. Final diagnosis was salivary gland choristoma by histopathology. We discuss the clinical features and management of middle ear salivary choristoma with the review of literature.

Jugulotympanic Paraganglioma, Mimicking a Vascular Tumor: A Brief Case Report

Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.

Jugulotympanic Paraganglioma, Mimicking a Vascular Tumor: A Brief Case Report

Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.

Temporal bone paraganglioma

OBJECTIVE: The aim of the study was to describe the diagnosissurgical management and outcomes of eight patients with bone paraganglioma.PATIENTS: A series of 8 patients with temporal bone paraganglioma were seen over a 10-year period and operated n by the senior author.RESULTS: The majority of patients presented with hearing loss and pulsatile tinnitus. Other symptoms were facial numbness, facial asymmetry, dizziness, hoarseness, and dysphagia. All patients had pre-operative high resolution computed temography (HRCT) scan and/or magnetic resonance imaging. Pre-operative angiography and embolization facilitated the excision of these tumors in all but one case. There was reversal of pre-existent cranial nerve neuropathy in 3 of 8 cases. Unique to this series were the management of a patient who previously underwent gamma knife radiosurgery, one case in whom the glomus tumor grew from the better hearing ear thus posing the surgical management challenges and a complicated case of postoperative necrosis that required latissimus dorsi flap reconstruction. Other complications were described including post-operative facial paresis which eventually resolved in all patients.CONCLUSION: In our setting, it is possible to achieve good result with acceptable morbidity with surgery with or without postoperative radiotherapy.

Tumor de glomus yugular, presentación de un caso clínico y revisión de la literatura / Glomus juglare tumor, case study and bibliographic reference

Los tumores del glomus yugular o timpánicos son poco comunes. Aunque son considerados tumores benignos, de crecimiento lento y tratamiento quirúrgico, pueden llegar a adquirir grandes dimensiones. El pronóstico de estos tumores está relacionado estrechamente con su localización anatómica y el volumen de la lesión. Si la remoción quirúrgica no es posible la radioterapia constituye una alternativa de tratamiento válida con intenciones paliativas.

The glomus juglare tumors are very uncommon. They are considered as benign and slow growing tumors. The treatment of choice is surgery. The prognosis of these tumors is related to the anatomical location and size of the lesion. If surgery is not posible the next step would be radiotherapy.

Imaging of glomus tympanicum tumors / 中华放射学杂志

Objective To evaluate the imaging diagnosis value of glomus tympanicum tumors.Methods Images of 10 patients with surgically and pathologically confirmed glomus tympanicum tumors were reviewed.The image characteristics and diagnostic value were summarized.Results In 8 patients with high resolution CT scan and 2 patients with routine CT scan,soft tissue nodules within the tympanic cavity were identified on axial or coronal images in 7 of them.And in 3 patients with chronic tympanitis,glomus tympanicum tumors were not differentiated on CT images.Eight patients had undergone MR examination,on T2WI the tumors were moderate hyperintense(n=6)and hyperintense(n=2),and the tumors were enhanced obviously after contrast.In 3 patients with chronic tympanitis,moderate high signal of the tumor can be differentiated with the high signal of tympanitis on T2WI.On contrast MRI,the signal of enhanced tumor or unenhanced tympanitis was identified clearly.Conclusion On diagnosis of glomus tympanicum tumors,MRI is prior to CT,especially in patient with glomus tympanicum and tympanitis.

Intraoperative HistoacrylR Injection of Glomus Tympanicum Tumor / 대한이비인후과학회지

The therapeutic options for glomus tumor management are either palliative (ie, radiation, embolization or a combination) or definitive (ie, surgery or combinations of surgery-radiation, radiation-surgery or embolization-surgery). Of these, surgical excision is the standard treatment modality for glomus tympanicum tumors. As glomus tympanicum tumor has highly vascular nature, profuse hemorrhage may be intermittently induced during its removal. Preoperative embolization is used frequently to reduce intraoperative bleeding. We report a case of glomus tympanicum tumor which was removed completely with the help of direct inject of HistoacrylR into the mass during the operation. This method allows almost bloodless ablation of the entire tumor.

A Case of Glomus Tympanicum / 대한이비인후과학회지

Glomus tympanicum has to be considered to differentiate the etiology of middle ear mass with pulsatile tinnitus. MRI helps to differentiate the mass. Angiography helps to identify its feeding vessels and to embolize to reduce bleeding on operation. When the mass is not focally localized, tympanotomy combinded with mastoidectomy approach via posterior auricular incision is helpful to remove the mass completely.