Effectiveness of chemoradiotherapy for radiation-induced bilateral external auditory canal cancer: A case report and literature review.

BACKGROUND: Radiation-induced bilateral external auditory canal cancer is an extremely rare disease that has yet to be fully characterized in the clinical literature. METHODS: Herein, we present a case study of a 75-year-old man with radiation-induced bilateral external auditory canal cancer. The patient's medical history included left maxillary cancer that had been treated with chemoradiation 19 years earlier and local recurrence with total maxillectomy 10 years earlier. Intracavitary radiation was delivered to the site of postoperative recurrence 8 years before the current presentation. The patient declined radical surgery for the external auditory canal cancer at this time, and a customized combined modality regimen was thus administered. RESULTS: There was no recurrence of cancer for 22 months, to date, after completing chemoradiotherapy. CONCLUSIONS: Our finding that radiotherapy can be successfully used for radiation-induced cancer indicates that chemoradiotherapy may be a useful strategy for treating this type of malignancy.

Bilateral radiation-induced squamous cell carcinomas of the external auditory canal 30 years after radiotherapy for a pituitary adenoma: a case report.

INTRODUCTION: The most important long-term effect of radiotherapy is induction of secondary cancers. A rare radiation-induced tumor is a squamous cell carcinoma of the external auditory canal. Case reports have been described in Asian patients after radiotherapy for nasopharyngeal cancer. CASE DESCRIPTION: We describe an unusual case report of a 53-year-old Caucasian man who developed squamous cell carcinomas of the right and left external auditory canal, respectively 29 and 30 years after radiotherapy for a pituitary adenoma. CONCLUSION: In young patients with benign tumors, we should always evaluate whether the benefits of radiotherapy outweigh the risks and side effects. After radiotherapy, screening programs should be developed since early detection improves salvage treatment of these secondary and often morbid tumors.

CAIX and pax-8 Commonly Immunoreactive in Endolymphatic Sac Tumors: A Clinicopathologic Study of 26 Cases with Differential Considerations for Metastatic Renal Cell Carcinoma in von Hippel-Lindau Patients.

Endolymphatic sac tumors (ELSTs) are rare, slowly growing temporal bone neoplasms which show a high association with von Hippel-Lindau (VHL) syndrome. The immunohistochemistry evaluation of these papillary-cystic neoplasms frequently raises the differential diagnosis with renal cell carcinoma, among other metastatic neoplasms, whether in VHL patients or not. A cohort of 26 patients with ELSTs were evaluated for histologic features, immunohistochemistry findings, and association with VHL. Standard immunohistochemistry evaluation was performed. Sixteen females and 10 males ranging in age from 10 to 69 years (mean 44; VHL mean: 32) at initial presentation, comprised the cohort of patients. Most (86%) experienced hearing changes or inner ear symptoms (vertigo, dizziness), with an average duration of symptoms for 39 months (range 2-240 months). The tumors were an average of 2.9 cm (range 0.4-8 cm), with 14 left, 11 right sided and one bilateral tumor. Nine patients had documented VHL, with 3 patients having a concurrent or subsequent clear cell renal cell carcinoma. Patients were followed an average of 6.2 years (available in 24 patients): 19 alive without disease, 7.5 years; 2 dead without disease, 1.2 years; and 3 alive with disease, 3.1 years. The neoplastic cells show the following immunohistochemistry findings: AE1/AE3, EMA, CK7, CAIX, GLUT1, VEGF: 100% of cases tested were positive; pax-8: 85% of cases positive; CD10 and RCC: 0% of cases reactive. Based on this cohort of 26 patients with ELST, 9 of whom had VHL, the strong pax-8 and CAIX should be used in conjunction with negative CD10 and RCC to help exclude a metastatic renal cell carcinoma. As CAIX is an enzyme overexpressed in hypoxia and hypoxia inducible factor is what VHL protein regulates, this is an expected, although previously unreported finding. Whether part of VHL or not, VHL mutations may be a somatic rather than germline finding in the tumors, a possible further explanation for the CAIX reaction.

CT and MRI findings of radiation-induced external auditory canal carcinoma in patients with nasopharyngeal carcinoma after radiotherapy.

OBJECTIVE: To summarize the radiological and clinical features of radiation-induced external auditory canal carcinomas (RIEACCs) in patients with nasopharyngeal carcinomas (NPCs) after radiation therapy. METHODS: CT, MRI and clinical features in 16 patients with histologically proven RIEACCs were retrospectively reviewed. There were 2 females and 14 males, with a median age of 52.5 years at the time of diagnosis of RIEACC. Imaging parameters including lesion extent, size, margin, shape, bone destruction, adjacent structure invasion, density/signal intensity, and pattern and degree of enhancement were assessed. Clinical features including clinical staging, histological type, treatment and radiation dose (RD) of primary NPC as well as the histological type, staging of radiation-induced tumour and the latent period between NPC and RIT were recorded. RESULTS: All patients had a single RIEACC. The lesions had a size of 3.5 ± 1.4 cm and were localized (n = 7) or extensive (n = 9). Most of the lesions were partially or ill defined with an irregular shape and had an intermediate density/signal pattern and moderate homogeneous enhancement. The latent period of RIEACCs ranged from 10 to 20 years in nine patients with a RD of 68-70 Gy; from 2 to 10 years in five patients with a RD of 68-74 Gy; and more than 20 years in two patients with a RD of 70 or 72 Gy. CONCLUSION: An external auditory canal (EAC) mass with homogeneous, intermediate CT density or signal intensity in patients with NPC after radiotherapy is highly suggestive of RIEACC, which should be included in the routine surveillance for patients with NPC after radiotherapy. ADVANCES IN KNOWLEDGE: RIEACCs could occur as short as 2 years after radiotherapy in patients with NPC and have distinct features from otitis media and sarcomas. This EAC malignancy should be included in routine surveillance for patients with NPC after radiotherapy.

Squamous cell carcinoma of the ear arising in patients after radiotherapy for nasopharyngeal carcinoma.

Radiation-induced malignancies are a rare but serious complication arising in patients receiving radiotherapy for nasopharyngeal carcinoma (NPC). To characterize patients who develop post-irradiation squamous cell carcinoma (PISCC) of the ear after radiotherapy for NPC and to compare their outcomes with patients who have de novo squamous cell carcinoma (SCC) of the ear. Clinical and pathological characteristics and their outcomes were analysed and compared between post-irradiation and de novo SCC cases. From 2002 to 2011, 25 patients were treated at our institution for SCC of the ear, of which 8 (32%) occurred after prior irradiation. There were no significant differences between the two groups with regards to age, gender, race, smoking status, tumour size, grade, stage and differentiation. Patients in the PISCC group appeared to have inferior overall survival (median survival 71.2 vs. 85.6 months; p = 0.292) and disease-specific survival (mean 59.6 vs. 71.5 months; p = 0.441). PISSC of the ear in long-standing survivors of NPC has a poor prognosis despite advances in medical care. Surgical resection with clear margins seems to offer the best outcomes.

von Hippel-Lindau disease: deafness due to a non-MRI-visible endolymphatic sac tumor despite targeted screening.

OBJECTIVE: Endolymphatic sac tumours (ELSTs) of the inner ear occur in 16% of patients with the hereditary tumor syndrome von Hippel-Lindau disease (vHL). ELSTs of all sizes can cause irreversible hearing loss which can, however, be prevented through early diagnosis and treatment. We aim to emphasize the challenges of prophylactic ELST screening and to explore the role of audiometry in pre-symptomatic ELST screening. DESIGN: For a period of 17 years our patient was screened for ELSTs with inner-ear MRI (magnetic resonance imaging), audiometry, and clinical interviews. STUDY SAMPLE: A male vHL patient who became deaf in one ear due to a radiologically undetectable ELST. RESULTS: Despite annual MRIs, the ELST was not visible until four months after onset of deafness when it appeared as a 1.4 × 1.4 mm tumor mass. Although his hearing was objectively within normal limits for the first 14 years, a distinct pattern of low-frequency hearing loss could retrospectively be seen at all audiometries. CONCLUSIONS: Audiometry is a candidate screening tool for detection of non-symptomatic pre-MRI-visible ELSTs, and we have initiated an international collaborative study to further determine its application. At present, we suggest an ELST screening protocol of yearly audiological assessment and inner ear MRI.

Tumors and tumorlike lesions of dog and cat ears.

Bacterial and fungal otitis constitutes most ear disease in companion animals. However, a wide spectrum of infectious and noninfectious disease processes involve the structures of the ear and are of primary diagnostic consideration in cases of recurrent otitis or those refractive to traditional treatments. This article discusses several common to reasonably rare neoplastic and nonneoplastic space-occupying lesions of the external, middle, and internal ear. Although some conditions present as unique entities, many present similar to or concurrent with otitis, and should be considered in cases of clinically unresponsive otitis.

[Multidisciplinary approach to the endolymphatic sac tumour]. / Abordaje multidisciplinar al tumor de saco endolinfático.

INTRODUCTION: Papilar adenocarcinoma of endolymphatic sac is related with Von Hippel Lindau disease at 15% of cases, has a slow growing with a high local aggressiveness, and doesn't metastasize. It causes symptoms of Meniere's syndrome due to the compression that produces at endolymphatic duct. When it presents with hearing loss is usually sudden and irreversible manner. The diagnostic is made with image tests and analysis of its structure with immunohistochemical tests. The elective treatment is surgical remove, and its main complication the perioperative bleeding it can be avoided with preoperative embolization or stereotactic radiation. CASE REPORT: A case of endolymphatic sac tumour is presented, in a 17-years-old male with unilateral deafness and crisis of rotate vertigo, with family history of Von Hippel-Lindau disease. Perceptive deafness and right vestibular arreflexia are detected at technical exploration. In a petrous bone computer tomography appears a mass at vestibular aqueduct. We performed a petrosectomy with presigmoidal approach and saving of inner ear. Pathological analysis revealed an endolymphatic sac tumour. DISCUSSION: In patients with a family history of Von Hippel Lindau disease and clinical symptoms of vertigo and normal hearing or with slight hearing loss we should suspect the presence of endolymphatic sac tumor. The clinical presentation of hearing loss can be sudden and irreversible even with negative or inconclusive images. Therefore, a quick action is important for the preservation of this function.

An unusual myofibroblastic proliferation of the pinna.

INTRODUCTION: Myofibrosis of the ear is rare. Myofibromas are mesenchymal tumours which usually present in infancy but have been reported sporadically in adults. OBJECTIVE: To present a rare case of trauma as a cause of pinnal myofibrosis. DESIGN AND METHOD: Case report. A 29-year-old soldier suffered repeated trauma from helmet wear and sports, and presented to our clinic with an exquisitely painful lump in the anti-helix of the pinna. RESULTS: The lump was excised uneventfully. Pathology revealed a reactive myofibroblastic proliferation which, given the history of trauma, raised the possibility of a florid cellular repair process. The main differential diagnosis was myofibroma. Immunohistochemistry was used to exclude other possible causes. CONCLUSION: No similar case has previously been reported. The aetiology of myofibroma is unclear, but repeated trauma may be a trigger. Histological and immunohistochemical analysis are recommended when the diagnosis is ambivalent.

Radiation-induced rhabdomyosarcoma of the brainstem in a patient with neurofibromatosis type 2.

Neurofibromatosis Type 2 (NF2) is a rare autosomal dominant disorder characterized by the development of benign tumors of the peripheral nervous system and the CNS, including schwannomas, meningiomas, and ependymomas. The gene responsible for the development of NF2 acts as a tumor suppressor gene. Stereotactic radiotherapy (SRT) or single-fraction stereotactic radiosurgery has been increasingly used in the past decades to treat benign tumors in patients with NF2. These radiotherapy methods are less invasive and can be potentially used to treat multiple tumors in a single session. The risk of inducing malignancy is unclear. Few reports exist of malignant peripheral nerve sheath tumors, meningiomas, or ependymomas occurring after SRT or stereotactic radiosurgery in patients with NF2. The authors present the first documented case of rhabdomyosarcoma following SRT for multiple NF2-associated schwannomas. Compared with patients with sporadic tumors, NF2 patients having a germline tumor suppressor gene defect may be more prone to secondary malignancies after treatment involving radiation therapy.