Subject(s)
Dermoid Cyst , Facial Paralysis , Temporal Bone , Humans , Facial Paralysis/etiology , Temporal Bone/diagnostic imaging , Dermoid Cyst/complications , Skull Neoplasms/complications , Skull Neoplasms/diagnosis , Male , Child , Female , Tomography, X-Ray Computed , Magnetic Resonance ImagingABSTRACT
BACKGROUND: Treatments currently available for ceruminous gland tumors include surgery and radiation therapy, which come with high financial costs and frequent complication and recurrence rates. HYPOTHESIS: To evaluate the treatment, response, complication, and recurrence of ceruminous gland tumors confirmed with biopsy and ablated using CO2 laser. METHODS: Retrospective review of medical records from Iowa State University and the University of Illinois between January 1, 2016 and December 31, 2021. Electronic medical records were evaluated for a histopathologically confirmed ceruminous gland tumor via video-otoscopic aided biopsy and subsequent CO2 laser ablation. Signalment, tumor type, size, location within the ear canal, length of time tumor was present before presentation, advanced imaging, and follow-up including recurrence were assessed. RESULTS: Fourteen ceruminous gland adenomas (4 dogs, 10 cats) and 12 ceruminous gland adenocarcinomas (7 dogs, 5 cats) were identified. Bacterial otitis externa was present in 4/26 (15%) animals. Recurrence was identified in 1/14 (7%) ceruminous gland adenomas and 1/12 (8%) ceruminous gland adenocarcinomas. CONCLUSIONS AND CLINICAL IMPORTANCE: Video-otoscopic aided biopsy and CO2 laser ablation of ceruminous gland tumors in dogs and cats is a viable treatment option with low recurrence and complication rates with CO2 laser ablation.
Subject(s)
Adenocarcinoma , Adenoma , Cat Diseases , Dog Diseases , Laser Therapy , Skin Neoplasms , Humans , Cats , Dogs , Animals , Ear Canal/surgery , Carbon Dioxide , Retrospective Studies , Cat Diseases/surgery , Dog Diseases/surgery , Biopsy/veterinary , Adenocarcinoma/veterinary , Skin Neoplasms/veterinary , Laser Therapy/veterinary , Adenoma/veterinaryABSTRACT
Intrinsic facial nerve tumors are rare lesions. Among the different histology types, schwannomas is the most frequently reported in literature. Other histological types of facial nerve tumors are hemangiomas, meningiomas, and neurofibromas. Chorda tympani schwannomas (CTSs) are extremely rare entities and are considered as an independent subgroup of facial nerve schwannomas because of their clinical characteristics. The aim of this report is to present the clinical and radiological features and the management of a CTS in a 27-year-old male presenting with conductive hearing loss.
Subject(s)
Chorda Tympani Nerve , Neurilemmoma , Male , Humans , Adult , Chorda Tympani Nerve/diagnostic imaging , Chorda Tympani Nerve/surgery , Chorda Tympani Nerve/pathology , Neurilemmoma/diagnostic imaging , Neurilemmoma/surgery , Neurilemmoma/pathologyABSTRACT
Glomus tumors are benign hyperplasia of glomus bodies, and they are rarely found in the head and neck. The middle ear is an exceptionally rare site for a true glomus tumor, and there are only three previously reported cases in this location. Glomus tumors are etiologically different than glomus tympanicum, which are paragangliomas of the middle ear that are often mistakenly referred to as "glomus tumors." This is a common misconception due to the "glomus" misnomer. We report a case of a patient diagnosed with a middle ear glomangioma after initially presenting to our clinic with tinnitus and hearing loss. The mass was surgically removed through a transcanal approach with carbon dioxide laser and sharp dissection. Literature review is also reported and revealed similar presentations in patients with middle ear glomangiomas.
ABSTRACT
A 52-year-old man presented to our emergency department with an acute onset of right-sided facial nerve (FN) palsy of House-Brackmann grade V. Electroneurography (ENoG) was conducted with no response at the right FN, as compared with the left FN (0%). We performed a biopsy of the right middle ear mass and histological studies showed the tumor to be neuroendocrine tumors (NET) of the middle ear. We resected the tumor with canal wall down mastoidectomy and reconstructed the posterior meatal wall with soft tissue. Three months after surgery, the FN paralysis had improved with House-Brackmann grade II. We reviewed cases of NET with FN palsy, and nine patients, including our case, have been reported. Our case is the first report of ENoG for the description of FN palsy due to NET. Although the ENoG value was 0%, it was remarkably improved by surgery. The other cases of NET patients with FN palsy also recovered FN function after surgery. These results suggest that it is recommended to perform the total resection of the tumor to improve the FN function.
Subject(s)
Adenoma , Bell Palsy , Ear Neoplasms , Facial Paralysis , Adenoma/complications , Adenoma/diagnostic imaging , Adenoma/surgery , Ear Neoplasms/complications , Ear Neoplasms/surgery , Ear, Middle/surgery , Facial Nerve/surgery , Humans , Male , Middle AgedABSTRACT
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.
Subject(s)
Carcinoma, Neuroendocrine/pathology , Ear Neoplasms/pathology , Laryngeal Neoplasms/pathology , Neoplasms, Multiple Primary/pathology , Aged, 80 and over , Carcinoma, Neuroendocrine/diagnostic imaging , Ear Neoplasms/diagnostic imaging , Humans , Immunohistochemistry , Laryngeal Neoplasms/diagnostic imaging , Laryngoscopy , Male , Neoplasms, Multiple Primary/diagnostic imaging , Otoscopy , Tomography, X-Ray Computed , Treatment RefusalABSTRACT
Middle ear tumors are relatively rare, and among them, the diagnoses of middle ear lesions originating from cartilage-like tissue are even rarer. Use of transcanal endoscopic ear surgery (TEES) has increased in recent years because of its advantages, such as clear visual field and minimally invasive procedure. Here, we report a middle ear mass originating from cartilage-like tissue treated with TEES. A 62-year-old woman presented with progressive right-sided hearing loss. A white mass was revealed through the tympanic membrane, and pure-tone audiometry detected a mean 50.0 dB conductive hearing loss. Computed tomography showed a mass in the tympanic cavity. TEES was performed for diagnosis and treatment. A white translucent tumor was observed intraoperatively, and it was completely resected. Histopathological examination confirmed the diagnosis of a mass originating from degenerated cartilage-like tissue. To the best of our knowledge, this is the first study of a middle ear mass originating from cartilage-like tissue treated with TEES. TEES with its clear visual field and precise techniques was beneficial in treating the middle ear lesions circumscribed in the tympanic cavity.
ABSTRACT
Objective The aim of this study is to present our experience in dealing with middle ear adenomas (MEAs), very rare tumors of the middle ear. Methods The medical notes of individuals with MEAs treated in tertiary referral; academic settings were retrospectively reviewed. We recorded the presenting symptoms, imaging findings, and pathology results. We additionally examined our surgical outcomes, follow-up period, recurrence, and morbidity. Results We identified four patients with MEAs: two males and two females with an average age of 36.25 years (range = 27-51 years). Despite the detailed imaging studies, including computed tomography and magnetic resonance imaging with intravenous contrast administration, a biopsy was essential in setting the diagnosis. Total surgical resection was achieved in all patients without any recurrence over an average of 6 years (range = 3-10 years). Complete ipsilateral deafness was the commonest surgical morbidity due to footplate infiltration by the tumor. Conclusion Total surgical resection is the treatment of choice in MEAs to minimize the risk for recurrence; this can come with morbidity, mostly sensorineural deafness. Given the very limited literature, long-term follow-up is recommended.
ABSTRACT
Chorda tympani schwannomas are rare benign tumors of the middle ear cleft. This is a case of incidental chorda tympani schwannoma identified intraoperatively. The patient was a 50-year-old male with chronic active otitis media complicated by left-sided facial paralysis. During closed mastoidectomy and tympanoplasty approach, a well-demarcated swelling on the left chorda tympani nerve was identified and sectioned for pathologic evaluation. The histopathologic evaluation established the diagnosis of schwannoma. Although the tumor was unlikely the cause of the disease process in this patient, his facial paralysis and middle ear disease were resolved after surgery.
ABSTRACT
Glandular neoplasms of the temporal-mastoid region and endolymphatic sac (ELS) are rare, and it is quite challenging to differentiate between an adenoma and an adenocarcinoma. ELS tumors (ELST) usually present with papillary, follicular, or solid patterns and can be further distinguished histologically and through immunohistochemistry. The microscopic features and clinical course of this neoplasm have been comprehensively explained by Heffner, who considered it "low-grade adenocarcinoma of likely ELS origin." The papillary form more commonly affects females, and it is a more aggressive form of ELST that is destructive and exhibits extensive local spread. The tumor usually has a close association with von Hippel-Lindau (VHL) disease, but 11%-30% of the ELST cases develop in individuals without a VHL mutation. ELSTs manifest with headaches, hearing loss, ear discharge, and cranial nerve palsies. Currently, the only available curative therapeutic intervention consists of wide local excision and long-term follow-up. Because of the sensitive location of this tumor, the adjuvant radiotherapy options are still questionable. In this case report, the author presents a 74-year-old woman with a past medical history of Schneiderian papilloma and was diagnosed with papillary mucinous adenocarcinoma of the ELS not associated with VHL disease.
ABSTRACT
BACKGROUND: Salivary gland choristoma (SGCh) is a rare benign tumor reported in several unusual sites, such as the gastrointestinal tract, the optic nerve, and the internal auditory canal, but never reported in the inner ear. CASE PRESENTATION: An 8-year-old girl with a history of left profound congenital hearing loss presented to us with ipsilateral progressive severe facial nerve palsy (House-Brackmann Grade VI). The left tympanic membrane was swollen with a pulsatile tumor. Radiological investigations revealed a multilocular tumor in the inner ear extending into the middle ear and internal auditory canal (IAC). We performed a partial resection of the tumor by transmastoid approach to preserve the anatomical structure of the facial nerve. The tumor was pathologically diagnosed as SGCh. Two years after surgery, her facial function recovered to House-Brackmann Grade II and the residual tumor did not show regrowth on MRI. CONCLUSIONS: Although the natural course of this rare tumor is unknown, a partial resection is an acceptable treatment procedure when functional recovery of the facial nerve is anticipated.
Subject(s)
Choristoma/complications , Deafness/congenital , Facial Paralysis/etiology , Labyrinth Diseases/complications , Salivary Gland Diseases/complications , Child , Cholesteatoma, Middle Ear/complications , Cholesteatoma, Middle Ear/diagnostic imaging , Cholesteatoma, Middle Ear/surgery , Choristoma/diagnostic imaging , Choristoma/pathology , Choristoma/surgery , Deafness/complications , Ear/diagnostic imaging , Female , Humans , Labyrinth Diseases/diagnostic imaging , Labyrinth Diseases/pathology , Salivary Gland Diseases/diagnostic imaging , Salivary Gland Diseases/pathologyABSTRACT
Most reported cases of middle ear adenoma (MEA) have focused on histopathology because MEA is usually diagnosed postoperatively, which is considered as a major setback. We focused on the surgical aspect of the disease to facilitate a preoperative diagnosis, resulting in prompt and proper treatment, without requiring a second stage of surgical treatment. In this report, we present the differential diagnoses in a 40-year-old man with MEA requiring surgical treatment. Preoperatively, the patient was suspected to have an MEA. An analysis of the surgical procedures in similar misdiagnosed tumors has enabled us to assess surgical procedures in cases wherein the preoperative diagnosis does not coincide with the postoperative histopathological results.
Subject(s)
Adenoma/diagnosis , Ear Neoplasms/diagnosis , Ear, Middle/surgery , Adenoma/surgery , Adult , Ear Neoplasms/surgery , Humans , Male , Medical Illustration , Preoperative PeriodABSTRACT
OBJECTIVE: To review current literature and experience with glomangiomas, or true glomus tumors of the middle ear and mastoid as well asto report on the exceptionally rare case of a glomangiomastemming from the middle ear space with multiple recurrences. METHODS: Review of existing world literature and description of personal experience with rare cases of a glomangioma of the middle ear and mastoid. RESULTS: Review of existing literature revealed two cases of patients presenting with tinnitus and hearing loss refractory to medical management. Both patients were ultimately diagnosed with glomangioma on histopathology. Complete surgical excision is thought to be curative. PATIENT: A 36-year-old woman presented with a rare case of a glomangioma of the middle ear presenting with unilateral hearing loss. She was noted to have a mass behind the tympanic membrane. Imaging revealed a diffuse mass filling the mastoid air cells. Imaging characteristics and histology were consistent with a glomangioma. INTERVENTION: Initial resection via mastoidectomy using a postauricular approach. The tympanic membrane was reconstructed with temporalis tissue. Follow-up revision tympanomastoidectomy was performed upon recurrence of disease. The chorda tympani were sacrificed due to tumor involvement. The incus and head of the malleus were removed to gain better access to the tumor. The ossicular chain was reconstructed with a Goldenberg Total Ossicular Prosthesis. MAIN OUTCOME MEASURE: Recurrence of disease. FOLLOW-UP: In the 67 months since her most recent surgery, there has been no evidence of recurrence by CT or physical exam. CONCLUSION: Glomangioma of the middle ear represents an exceptionally rare entity that can present in a similar fashion to a paraganglioma.
ABSTRACT
Middle ear adenoma is a very rare disease which is benign and originates from the middle ear mucosa. Patients of middle ear adenoma usually come to the clinic for unilateral hearing loss or tinnitus, but rarely for accompanied facial palsy. It is non-gender specific and occurs over a wide range of ages. The recurrence rate is known to be very low, but few authors argue that neuroendocrine adenoma should be considered as a low grade carcinoma due to some cases of recurrence. A 18 years-old male who had a left side facial palsy about 3 years ago but has currently improved as compared with the initial onset, visited our clinic for the left side hearing loss. Pure tone audiogram showed about 30 dB of conductive hearing loss and a pinkish polypoid mass involving the left tympanic membrane. We removed a tumor via transmastoid approach. The final diagnosis was middle ear adenoma with neuroendocrine differentiation. Neither signs of complication nor recurrence were observed after six months of the surgery.
Subject(s)
Humans , Male , Adenoma , Diagnosis , Ear, Middle , Facial Paralysis , Hearing Loss , Hearing Loss, Conductive , Hearing Loss, Unilateral , Hearing , Mucous Membrane , Rare Diseases , Recurrence , Tinnitus , Tympanic MembraneABSTRACT
Intravasation, active entry of cancer cells into the circulation, is often considered to be a relatively late event in tumor development occurring after stromal invasion. Here, we provide evidence that intravasation can be initiated early during tumor development and proceed in parallel to or independent of tumor invasion into surrounding stroma. By applying direct and unbiased intravasation-scoring methods to two histologically distinct human cancer types in live-animal models, we demonstrate that intravasation takes place almost exclusively within the tumor core, involves intratumoral vasculature, and does not involve vasculotropic cancer cells invading tumor-adjacent stroma and migrating along tumor-converging blood vessels. Highlighting an additional role for EGFR in cancer, we find that EGFR is required for the development of an intravasation-sustaining intratumoral vasculature. Intratumoral localization of intravasation supports the notion that overt metastases in cancer patients could be initiated much earlier during cancer progression than appreciated within conventional clinical tumor staging systems.
Subject(s)
Cell Movement , Neoplasms/pathology , Animals , Cell Line, Tumor , Chick Embryo , Disease Models, Animal , Ear/pathology , ErbB Receptors/metabolism , Green Fluorescent Proteins/metabolism , Humans , Hypoxia/pathology , Mice, Inbred C57BL , Mice, Inbred NOD , Mice, SCID , Neoplasm Invasiveness , Neoplasm Metastasis , Neovascularization, Pathologic , Permeability , Stromal Cells/pathologyABSTRACT
Adenomatous tumors in the middle ear and temporal bone are rare but highly morbid because they are difficult to detect prior to the development of audiovestibular dysfunction. Complete resection is often disfiguring and difficult because of location and the late stage at diagnosis, so identification of molecular targets and effective therapies is needed. Here, we describe a new mouse model of aggressive papillary ear tumor that was serendipitously discovered during the generation of a mouse model for mutant EGFR-driven lung cancer. Although these mice did not develop lung tumors, 43% developed head tilt and circling behavior. Magnetic resonance imaging (MRI) scans showed bilateral ear tumors located in the tympanic cavity. These tumors expressed mutant EGFR as well as active downstream targets such as Akt, mTOR and ERK1/2. EGFR-directed therapies were highly effective in eradicating the tumors and correcting the vestibular defects, suggesting these tumors are addicted to EGFR. EGFR activation was also observed in human ear neoplasms, which provides clinical relevance for this mouse model and rationale to test EGFR-targeted therapies in these rare neoplasms.
Subject(s)
Adenoma/metabolism , Ear Neoplasms/metabolism , Ear, Middle/metabolism , ErbB Receptors/metabolism , Neoplasms, Experimental/metabolism , Skull Neoplasms/metabolism , Temporal Bone/metabolism , Adenocarcinoma/metabolism , Adenocarcinoma/pathology , Adenoma/drug therapy , Adenoma/pathology , Animals , Antineoplastic Agents/pharmacology , Behavior, Animal , Drug Design , Ear Neoplasms/drug therapy , Ear Neoplasms/genetics , Ear Neoplasms/pathology , Ear, Middle/drug effects , Ear, Middle/pathology , ErbB Receptors/antagonists & inhibitors , ErbB Receptors/genetics , Female , Genotype , Humans , Magnetic Resonance Imaging , Male , Mice, Transgenic , Molecular Targeted Therapy , Motor Activity , Mutation , Neoplasms, Experimental/drug therapy , Neoplasms, Experimental/genetics , Neoplasms, Experimental/pathology , Phenotype , Promoter Regions, Genetic , Pulmonary Surfactant-Associated Protein C/genetics , Signal Transduction/drug effects , Skull Neoplasms/drug therapy , Skull Neoplasms/pathology , Temporal Bone/drug effects , Temporal Bone/pathology , Uteroglobin/genetics , Uteroglobin/metabolism , X-Ray MicrotomographyABSTRACT
Adenomatous tumors are an uncommon cause of a middle ear mass. Clinical findings may be nonspecific, leading to difficulties in differentiation from other middle ear tumors. Controversy also exists whether to classify middle ear adenoma and carcinoid as separate neoplasms, or alternatively within a spectrum of the same pathologic entity. Most adenomatous middle ear tumors are indolent in behavior, with a benign histologic appearance and slowly progressive growth. The mainstay of treatment is complete surgical resection, which affords the greatest likelihood of cure.
Subject(s)
Adenoma/pathology , Carcinoid Tumor/pathology , Ear Neoplasms/pathology , Ear, Middle/surgery , Adenoma/classification , Adenoma/surgery , Carcinoid Tumor/classification , Carcinoid Tumor/surgery , Ear Neoplasms/classification , Ear Neoplasms/surgery , Ear, Middle/pathology , Humans , Magnetic Resonance Imaging , Prognosis , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
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.
Subject(s)
Ear Neoplasms/pathology , Ear Neoplasms/surgery , Ear, Middle/surgery , Glomus Tympanicum Tumor/pathology , Glomus Tympanicum Tumor/surgery , Genetic Testing , Humans , Magnetic Resonance Imaging , Prognosis , Tomography, X-Ray ComputedABSTRACT
OBJECTIVE: To characterize the clinical presentation, surgical management, and outcomes of a large consecutive cohort of patients with glomus tympanicum (GT) tumors managed at a single tertiary referral group over 4 decades. STUDY DESIGN: Retrospective review. SETTING: Tertiary neurotological referral center. SUBJECTS AND METHODS: All patients underwent surgical treatment of histopathologically confirmed GT between January 1973 and March 2014. Audiometric outcomes were reported according to AAO-HNS guidelines, and tumor stage was described using the Glasscock-Jackson classification system. RESULTS: There were 115 patients (90.4% women; mean age, 55.2 years) who met the inclusion criteria; 38 (33.0%) cases of GT were stage I, 51 (44.3%) stage II, 10 (8.7%) stage III, and 16 (13.9%) stage IV. There were 108 (93.9%) patients who underwent gross total removal, while 7 (6.1%) received less than complete resection for advanced disease that was adherent to the petrous carotid artery, facial nerve, stapes footplate, or round window. Two patients who underwent gross total resection experienced transient facial paresis, and 1 had internal carotid injury with stroke. No patients had been diagnosed with recurrent disease at a mean follow-up of 30.4 months. CONCLUSION: Surgery remains the treatment of choice for GT, providing a high rate of tumor control and resolution of aural symptoms with a low risk of complications. The surgical approach and extent of resection should be tailored to the patient. Gross total resection can be performed in over 90% of patients; however, leaving a limited adherent tumor remnant on the facial nerve or carotid artery should be considered with advanced infiltrative disease to prevent unnecessary morbidity.
Subject(s)
Ear Neoplasms , Glomus Tympanicum , Paraganglioma , Adult , Aged , Ear Neoplasms/diagnosis , Ear Neoplasms/surgery , Female , Humans , Male , Middle Aged , Paraganglioma/diagnosis , Paraganglioma/surgery , Retrospective Studies , Time FactorsABSTRACT
Nasopharyngeal carcinoma (NPC) evolves most commonly from the pharyngeal recess posteromedial to the medial crura of the Eustachian tube opening in the nasopharynx. Therefore unilateral middle ear effusion with hearing loss is a common presenting symptom of NPC, and its presence in adult patients make physicians suspect NPC. Recently, we experienced a 72- year-old female patient with advanced NPC which invaded Eustachian tube and middle ear cavity. She had long standing chronic otitis media with large perforation of tympanic membrane. Because of tympanic membrane perforation, she did not have any Eustachian tube-related symptoms such as newly developing ear fullness or hearing loss and even the physician had overlooked the diagnosis of NPC. Her NPC was diagnosed at a very advanced stage after performing biopsy of middle ear tumor. This case report shows that NPC can present with very unusual findings like middle ear tumor.