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.

[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.

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.

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.

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.

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.

External auditory canal and middle ear tumors: characterization by morphology and diffusion features on CT and MRI.

PURPOSE: To explore the value of morphology and diffusion features on CT and MRI in the characterization of external auditory canal and middle ear tumors (EAMETs). METHODS: Forty-seven patients with histologically proved EAMETs (23 benign and 24 malignant) who underwent CT and MRI were retrospectively analyzed in this study. CT and MRI characteristics (including size, shape, signal intensity, border, enhancement degree, and bone changes) and apparent diffusion coefficient (ADC) value were analyzed and compared between benign and malignant EAMETs. Logistic regression, receiver operating characteristic (ROC) curve, and Delong test were performed to assess the diagnostic performance. RESULTS: Compared with benign tumors, the malignant EAMETs are characterized by irregular shape, ill-defined border, invasive bone destruction, and intense enhancement (all p < 0.05). There were no significant differences on the size and signal intensity between benign and malignant tumors. The ADC value of malignant tumors were (879.96 ± 201.15) × 10-6 mm2/s, which was significantly lower than benign ones (p < 0.05). Logistic regression demonstrates the presence of ill-defined margin, invasive bone destruction, and low ADC value (≤ 920.33 × 10-6 mm2/s) have significant relationship with malignant EAMETs. The combination of characterization by morphology and diffusion features on CT and MRI can further improve the diagnostic efficiency when compared with morphology and diffusion features alone (both p < 0.05). CONCLUSION: Some CT and MRI characteristics are helpful in identifying malignant EAMETs from benign ones (especially ill-defined margin, invasive bone destruction, and low ADC value), and the combination of morphology and diffusion features on CT and MRI has best diagnostic efficiency for discriminating these two entities.

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.

Quiste epidermoide en conducto auditivo externo: reporte de caso / Epidermoid cysts of the external auditory canal: case report

Resumen El quiste epidérmico es una lesión benigna y común de la piel. Se desarrolla por un bloqueo de la unidad pilosebácea, con la consecuente proliferación de células epiteliales y secuestro de queratina. El 7% se desarrollan en cabeza y cuello, sin embargo, son infrecuentes en canal auditivo externo. Su patrón de crecimiento es lento y progresivo durante años, siendo asintomáticos. Al aumentar de tamaño causan sintomatología variable, acorde a su localización; en el canal auditivo tienen un comportamiento obstructivo que genera síntomas como otalgia e hipoacusia. Se presenta caso de una paciente de 69 años, con acúfeno e hipoacusia progresiva derecha. Durante la otoscopia se observó una neoformación obstructiva del 100% de la luz del conducto. Se realizaron estudios de imagen que reportaron tumoración de características quísticas de conducto auditivo derecho, bien circunscrita, sin erosión ósea. Para el diagnóstico definitivo, se realizó resección quirúrgica y biopsia reportando quiste epidérmico. Durante el seguimiento posoperatorio sin hallazgos de recidiva.

Abstract The epidermal cyst is a common and benign lesion of the skin. It develops due to a blockage of the pilosebaceous unit, with the consequent proliferation of epithelial cells and keratin sequestration. Seven percent develop in the head and neck; however, they are infrequent in the external auditory canal. Its growth pattern is slow and progressive over the years, being asymptomatic. As they increase in size, they cause variable symptoms, according to their location. In the ear canal they have an obstructive behavior that generates symptoms such as earache and hearing loss. A case of a 69-year-old female with tinnitus and progressive right hearing loss is presented. At otoscopy, a 100% obstructive neoformation of the canal lumen was observed. Imaging studies showed a well circumscribed, cystic tumor of the right ear canal, without bone erosion. For the definitive diagnosis, a resection and biopsy were performed, reporting an epidermal cyst. During follow up there was no recurrence of tumor.

Plasmocitoma extramedular de canal auditivo externo: caso clínico / Extramedullary plasmacytoma of external auditory canal: a case report

Resumen El plasmocitoma extramedular es una neoplasia de células plasmáticas poco frecuente, que se ubica en 80% a 90% de los casos en la cabeza o cuello. Esta neoplasia representa menos del 1% de toda la patología maligna de cabeza y cuello. Dada la poca frecuencia de la patología y la escasez de casos publicados, esta entidad presenta una gran dificultad clínica y terapéutica. El diagnóstico se basa en el análisis histológico con inmunohistoquímica de la muestra obtenida, y el tratamiento varía según la ubicación, donde se puede realizar radioterapia o cirugía. En este artículo presentamos el caso de una paciente de 56 años, con antecedentes de otorrea de larga data en oído derecho, al examen físico presentaba un tumor que obstruía todo el conducto auditivo externo. Se realizó exéresis tumoral y la biopsia evidenció un plasmocitoma.

Abstract Extramedullary plasmacytoma is a rare plasma cell neoplasm, affecting 80% to 90% of the head or neck. This neoplasm represents less than 1% of all malignant head and neck pathology. Given the infrequency of the pathology and the scarcity of published cases, this entity presents great clinical and therapeutic difficulty. Diagnosis is based on histology and immunohistochemistry and treatment varies depending on the location, where radiation therapy or surgery can be performed. In this article, we present the case of a 56-year-old patient with a history of long-standing otorrhea in the right ear. Physical examination presented a tumor that obstructed the entire external auditory canal. Tumor excision was performed, and the biopsy revealed a plasmacytoma.

Cytological features of endolymphatic sac tumors: Case studies with review of literature.

Endolymphatic sac tumors are extremely rare, locally aggressive neoplasms that arise from the endolymphatic sac or duct, primarily in the intraosseous portion. These neoplasms show diverse histomorphological architectures and despite a bland cytologic appearance, can locally recur. Although the clinicopathological and radiological features of this entity are well characterized, the literature on cytological features is extremely sparse. Herein, we describe the cytological features of the endolymphatic sac tumors and discuss the relevant differential diagnoses.

A Rare Case of Primary Neuroendocrine Carcinomas in the Ear and Throat and Review of Literatures.

The neuroendocrine carcinomas (NECs) of the head and neck are rare. The purpose of this article is to explore the diagnosis and treatment of NECs in the ear and larynx. We report a case of a patient with NECs found in the ear and throat simultaneously, and the relevant literatures are reviewed. It is difficult to identify which is the original site. There is no specific clinical manifestation of NECs in the ear and throat, and carcinoid syndrome is a rare situation. Surgery is still the preferred treatment for this disease. For patients with metastasis, radiotherapy and/or chemotherapy are required.

Primary external auditory canal meningioma: Case report and review of the literature.

Meningiomas are tumors that arise from arachnoid cells attached to both the pia mater and the inner portion of the arachnoid. They are common intracranial tumors, representing 12-25% of intracranial neoplasms. Intracranial meningiomas can spread extracranially to involve surrounding structures, including the ear and temporal bone. Ectopic meningiomas, described as primary meningiomas with no intracranial involvement, are rare. We describe a case of a primary external auditory canal meningioma with no evidence of intracranial involvement. We present pre-operative imaging findings proving no intracranial involvement prior to surgical intervention. A literature review of this uncommon clinical entity is presented and a discussion regarding its prognosis and treatment is reviewed.

Multiscale Photoacoustic Tomography of a Genetically Encoded Near-Infrared FRET Biosensor.

Photoacoustic tomography (PAT) with genetically encoded near-infrared probes enables visualization of specific cell populations in vivo at high resolution deeply in biological tissues. However, because of a lack of proper probes, PAT of cellular dynamics remains unexplored. Here, the authors report a near-infrared Forster resonance energy transfer (FRET) biosensor based on a miRFP670-iRFP720 pair of the near-infrared fluorescent proteins, which enables dynamic functional imaging of active biological processes in deep tissues. By photoacoustically detecting the changes in the optical absorption of the miRFP670 FRET-donor, they monitored cell apoptosis in deep tissue at high spatiotemporal resolution using PAT. Specifically, they detected apoptosis in single cells at a resolution of ≈3 µm in a mouse ear tumor, and in deep brain tumors (>3 mm beneath the scalp) of living mice at a spatial resolution of ≈150 µm with a 20 Hz frame rate. These results open the way for high-resolution photoacoustic imaging of dynamic biological processes in deep tissues using NIR biosensors and PAT.