Endolymphatic Sac Tumors Associated With von Hippel-Lindau: A Case Report Highlighting Opportunity for Novel Orphan Drug Therapy.

OBJECTIVE: To discuss the potential benefit of belzutifan therapy in a patient with von Hippel-Lindau (VHL) disease-associated endolymphatic sac tumor (ELST). PATIENTS: Case report. INTERVENTIONS: Clinical details of a patient with residual ELST after hearing preservation surgery who initiated belzutifan therapy postoperatively for concurrent renal cell carcinoma, as well as literature review of belzutifan and ELST. MAIN OUTCOME MEASURES: The patient remained without radiologic evidence of growth of her residual tumor at 17 months post-initiation of belzutifan. It is unknown whether this represents therapeutic drug effect, nonviability of residual tumor, or slow tumor growth not captured radiographically within the duration of follow-up. CONCLUSIONS: Belzutifan could have direct therapeutic benefit in patients with VHL-associated ELST.

Intralabyrinthine Schwannoma Mimicking Posterior Canalithiasis.

Intralabyrinthine schwannomas are a rare subgroup of vestibular schwannomas located within the membranous labyrinth of the inner ear and are known for their variable clinical presentations and symptoms. In the present study, we report on a patient with a persistent history of dizziness and positional vertigo, who was misdiagnosed with posterior canalithiasis. As hearing loss was not developed until late in the disease course, the patient was not properly diagnosed until magnetic resonance imaging revealed an intralabyrinthine schwannoma, which was not discovered on earlier imaging. In addition to the unusual clinical presentation, we describe the audio-vestibular profile of our patient. We suggest that a thorough vestibular evaluation, including caloric testing and a careful examination of the inner ear on imaging, is warranted in cases of treatment of refractory vertigo, even in patients where a diagnosis seems certain.

Middle Ear Neuroendocrine Tumor: Case Report of a Tympanic Adenoma.

Middle ear neuroendocrine tumors (MeNETs) are an exceptionally rare occurrence. These benign tumors stem from the tympanic mucosa and can easily be misinterpreted by the clinician and the pathologist. Clinical characteristics, otoscopic findings and medical imaging in these cases are non-specific. We present a case of a 60-year-old male patient with bilateral hearing loss following recent coronavirus disease 2019 disease. Diagnostic work-up revealed a soft tissue neoplasm of the left middle ear. Surgical resection of the tumor mass with implantation of a partial ossicular replacement prosthesis (PORP) was the main modality of treatment. Middle ear neuroendocrine tumors was confirmed through positive immunohistochemistry for neuroendocrine tumor markers. Follow-up magnetic resonance imaging 12 months after the surgery reported no tumor recurrence or significant residual disease with a stable PORP. Our report highlights challenges in diagnosing and treating these rare tumors, while emphasizing surgical resection pitfalls and resulting improvement of quality of life of the patient. We recommend a through followup of patients with unclear soft tissue masses in the middle ear to obtain a definitive diagnosis.

Mohs micrographic surgery challenges and new technologies to optimize care of cutaneous malignancies of the ear.

Cutaneous malignancies affecting the ear, exacerbated by extensive ultraviolet (UV) exposure, pose intricate challenges owing to the organ's complex anatomy. This article investigates how the anatomy contributes to late-stage diagnoses and ensuing complexities in surgical interventions. Mohs Micrographic Surgery (MMS), acknowledged as the gold standard for treating most cutaneous malignancies of the ear, ensures superior margin control and cure rates. However, the ear's intricacy necessitates careful consideration of tissue availability and aesthetic outcomes. The manuscript explores new technologies like Reflectance Confocal Microscopy (RCM), Optical Coherence Tomography (OCT), High-Frequency, High-Resolution Ultrasound (HFHRUS), and Raman spectroscopy (RS). These technologies hold the promise of enhancing diagnostic accuracy and providing real-time visualization of excised tissue, thereby improving tumor margin assessments. Dermoscopy continues to be a valuable non-invasive tool for identifying malignant lesions. Staining methods in Mohs surgery are discussed, emphasizing hematoxylin and eosin (H&E) as the gold standard for evaluating tumor margins. Toluidine blue is explored for potential applications in assessing basal cell carcinomas (BCC), and immunohistochemical staining is considered for detecting proteins associated with specific malignancies. As MMS and imaging technologies advance, a thorough evaluation of their practicality, cost-effectiveness, and benefits becomes essential for enhancing surgical outcomes and patient care. The potential synergy of artificial intelligence with these innovations holds promise in revolutionizing tumor detection and improving the efficacy of cutaneous malignancy treatments.

Endoscopic transcanal coblation excision of glomus tympanicum: a novel technique.

OBJECTIVE: To evaluate the feasibility of coblation in excision of glomus tympanicum tumors. PATIENTS AND METHODS: A retrospective study carried out over 28 patients with types I and II glomus tympanicum tumors according to GLASSCOCK-JACKSON classification. Preoperative radiological and endocrinal evaluation were performed. All patients underwent endoscopic transcanal excision of their glomus tympanicum tumors using coblation. RESULTS: None of the patients developed recurrence during the 1-year follow up period proved radiologically. None of the patients developed facial palsy postoperatively. Differences between preoperative and postoperative dizziness and taste disturbance were statistically non-significant. Tinnitus disappeared completely in 22 patients postoperatively. A statistically significant reduction in Tinnitus Handicap Inventory (THI) after surgery was found. Statistically significant reductions in postoperative air conduction (AC) threshold and air bone gap (ABG) were recorded while bone conduction (BC) threshold showed statistically non-significant change. CONCLUSION: Coblation is an effective and safe tool in excision of glomus tympanicum tumors. Further studies comparing coblation with laser and piezosurgery are strongly recommended.

Middle ear lipoma as a mimicker of other diseases: a review of an unusual presentation / Lipoma en el oído medio como un imitador de otras enfermedades: una revisión de una presentación inusual

The finding of a lipoma in the middle ear is much rarer than its occurrence in the external auditory canal or even the inner ear, with fewer than seven cases described in the literature and none of them in Spain or South America. Despite its benign nature, the location of the lipoma may compromise structures that play a significant role in auditory preservation or balance control, necessitating surgical removal as a curative treatment. The main objective of this article is to describe the presentation of lipomas in the middle ear as a possible, although rare, etiology to consider in patients presenting with hearing loss, instability, or both symptoms concurrently, seeking otorhinolaryngological evaluation.

El hallazgo de un lipoma en el oído medio es mucho más raro que su ocurrencia en el canal auditivo externo o incluso en el oído interno, con menos de siete casos descritos en la literatura y ninguno de ellos en España o Sudamérica. A pesar de su naturaleza benigna, la ubicación del lipoma puede comprometer estructuras que desempeñan un papel significativo en la preservación auditiva o en el control del equilibrio, lo que hace necesaria la extirpación quirúrgica como tratamiento curativo. El objetivo principal de este artículo es describir la presentación de los lipomas en el oído medio como una posible, aunque rara, etiología a considerar en pacientes que presentan pérdida de audición, inestabilidad o ambos síntomas simultáneamente, buscando evaluación otorrinolaringológica.

[Unusual presentation of a mast cell tumor in the ear canal of a cat]. / Ungewöhnlicher Mastzelltumor im Gehörgang einer Katze.

A 12-year-old male neutered European Shorthair cat was presented for pruritus in the right ear region, bleeding from ear canal and a suspected polyp-like mass in its lumen.After the diagnostic imaging a biopsy of the mass was taken and submitted for histopathological evaluation. Histopathologic examination led to the diagnosis of low grade mast cell tumor. The subsequent staging examinations included ultrasonography of the liver and spleen as well as a complete blood count. Total ear canal ablation was performed on the same day, and the removed ear canal was again submitted for histopathologic evaluation of the surgical margins. The excision incision margins were free from infiltrating tumor cells. The cat was euthanised 14 months after the surgery. It is unknown whether the reasons for this were associated to metastatic spread of the initial mast cell tumor.A mast cell tumor in the ear canal is an unusual and rare finding, however it should be included in the list of differential diagnoses for ear canal tumors.

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.

Tumor progression in tympanojugular paragangliomas: the role of radiotherapy and wait and scan.

INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.

A Rare Case of Angiofibroma Presenting as an Endolymphatic Sac Tumor.

A 30-year-old man presented with minute-long episodes of vertigo and severe autophony. CVEMP showed a decreased threshold when testing the left side, potentially indicating SSCD. A subsequent MRI demonstrated a multi-lobulated, cystic mass in the temporal bone and the radiological diagnosis at that time was ELST. Tumor excision was performed, and microscopic examination of the excised material revealed fibrovascular tissue without signs of papillary or cystic projections. The conclusion of the histological assessment rendered a diagnosis of angiofibroma. We were unable to find a previous report of ENA originating around the endolymphatic sac. Laryngoscope, 134:1897-1900, 2024.

Neoplasms of the Ear Canal.

Primary EAC neoplasms include benign and malignant lesions of bony, glandular or cutaneous origin. Small, benign slow growing bony neoplasms are often asymptomatic, diagnosed incidentally and might not require intervention. Both malignant and benign neoplasms of cutaneous and glandular origin can present with symptoms of chronic otitis externa, leading to delays in diagnosis. Prompt biopsy of soft tissue lesions associated with non-resolving otitis externa are warranted. Local and regional imaging is helpful to understand disease extent and origin, but even early-stage malignant neoplasms require aggressive surgical treatment.

External Auditory Canal Carcinoma With An Unusually Extensive Invasion.

External auditory canal carcinoma, while starting out as a seemingly benign condition, if left untreated can have an aggressive course of disease and involve multiple lower cranial nerves. Squamous cell carcinoma remains the most frequent histological type of malignant neoplasm of the external auditory canal and temporal bone. Here we describe a patient with a history of chronic suppurative otitis media with an extensive spread, the tumour was reaching from the skull base to the oropharynx involving neurovasculature along with soft tissues and bones, as well as the cerebellum. The involvement of the recurrent laryngeal and hypoglossal nerves were the most unusual presentation here. The case differed from all previous reported cases as the spinal accessory nerve was to spared. Surgery and radiotherapy are the treatment options but for the inoperable cases presenting with an already poor prognosis, concomitant radiotherapy is the only choice.

The Diagnosis and Management of Advanced Endolymphatic Sac Tumor.

Endolymphatic sac tumor (ELST) is a group of low-grade malignant tumors originating from the endolymphatic sac of the inner ear. It is rare in the clinic and has the biological characteristics of slow growth and local aggression. Due to the lack of specificity in the clinical manifestations of patients with ELST, many cases have entered the advanced stage at the time of diagnosis. However, there are still great challenges in the treatment of advanced ELSTs. Here, the authors describe a case of advanced ELST, which relapsed after 2 operations. This time, the authors chose the transotic approach for tumor resection, which achieved the goal of complete resection of the tumor, and the patient recovered smoothly after surgery. There were no surgical complications and no tumor recurrence after the follow-up. Through literature review and our own experience, the authors suggest that complete surgical resection is the first choice for both primary and recurrent advanced ELSTs. The choice of a reasonable surgical approach is the key to ensuring complete resection of the tumor, while preoperative angiography and embolization, fine treatment of important structures during surgery, and postoperative long-term follow-up are equally important for patients with advanced ELST to obtain a good prognosis.

Middle Ear Teratoma: Clinical and Imaging Features.

BACKGROUND: Teratoma is a true neoplasm composed of a number of different types of tissue derived from the three germinal layers but rarely occurs in the middle ear (ME). The features of middle ear teratomas (MET) have not been well described. OBJECTIVE: The objective of this study is to explore the clinical and imaging features of MET, and report 2 rare cases of MET with ear malformation that have never been reported. MATERIALS AND METHODS: The clinical, CT and MRI data of 8 patients with a pathological diagnosis of MET were collected and retrospectively mined, and 14 patients with MET reported in previous literature were also reviewed. RESULTS: ① Female, left ear predominance in MET, and the most common symptoms were otorrhea and hearing loss. ② On CT and MRI, the MET presented as an irregular soft tissue mass that was heterogeneous, with fatty tissue and involved multiple sites, and the ET and tympanum were correspondingly expanded and locally destroyed. ③ Mictotia with MET in two patients was presented, which was the first report. CONCLUSION: MET has female sex and left ear predominance. CT and MRI can be used to diagnose MET and display its extent and its relationship to the carotid canal in detail. Complete surgical excision is the definitive treatment.

Surgical management of endolymphatic sac tumor: classification, outcomes and strategy. A single institution's experience.

PURPOSE: To review the resections of endolymphatic sac tumor (ELST) and describe our experience in the surgical management of ELST. METHODS: Retrospective investigation of consecutive patients who underwent resection of ELSTs at our hospital between 1999 and 2019. The symptoms, diagnosis, surgical findings, and outcomes were analyzed to develop a tumor staging system and corresponding surgical strategy. RESULTS: Retrospective review revealed the surgical treatment of 22 ELSTs. Based on intraoperative findings of tumor extent and size, ELSTs were classified into two types. Type-I (n = 6) referred to the small tumors that were locally confined with limited invasion of semicircular canals and dura; type-II (n = 16) referred to the large tumors that presented extensive erosion of at least one anatomic structure apart from the semicircular canals and the dura around endolymphatic sac. In this case series, Type-I ELST is amenable to resection through a transmastoidal approach, and subtotal petrosectomy is appropriate for the resection of type-II ELST. Sensorineural hearing loss (SNHL) is the most commonly preoperative symptom in both two types of cases. Five type-II ELSTs experienced recurrence and underwent reoperation, whereas all type-I ELSTs did not. CONCLUSION: ELST usually results in SNHL (95%) at the time of diagnosis. The surgical strategy and prognosis of ELST resections are different between type-I and type-II: type-I ELST is amenable to transmastoidal approach with the preservation of facial nerve, whereas type-II ELST increase the surgical difficulty and the risk of recurrence, and subtotal petrosectomy is the basic requirement for the resection of type-II ELST.

Middle Ear Adenomatous Neuroendocrine Tumor: A Rare Entity.

Middle ear adenomatous neuroendocrine tumors (MEANT) are rare, mainly benign neoplasms and account for less than 2% of the middle and inner ear neoplasms. There are no specific radiological or clinical findings; therefore, the diagnosis is often difficult. Biopsy prior to surgery is often proposed. The definite treatment is the complete surgical resection. We present an uncommon clinical report of a MEANT, which was treated successfully with surgical excision.