Staging System Performance and Clinical Outcomes for Cutaneous Squamous Cell Carcinoma of the Ear: A Single-Center Retrospective Study.

BACKGROUND: Cutaneous squamous cell carcinoma (cSCC) of the ear is associated with poor outcomes. No studies have evaluated current staging system performance in this specific location. OBJECTIVE: Describe clinicopathologic characteristics and outcomes of ear cSCC and evaluate the performance of current staging systems. METHODS: Retrospective study including cases diagnosed and treated at a cancer center from January 2000 to December 2014. Demographic, clinical, and pathologic data were collected from clinical records. Biopsy slides were rereviewed and patients were staged according to the American Joint Committee on Cancer (AJCC) seventh, eighth, and Brigham Women's Hospital (BWH) staging. RESULTS: Of 125 patients, the mean age at diagnosis was 71.9 years (SD 12.5), with most men (89.6%, n = 112). Median follow-up was 22.3 months. Local recurrence and survival risk factors were similar to cSCC outside the ear. The Akaike's Information Criterion (AIC) estimates showed that the BWH system better predicted outcomes than the AJCC seventh, and the AJCC eighth, with AIC values of 189.9, 270.5, and 274.1, respectively. Limitations of the study include retrospective design, single center study, and no control group. CONCLUSION: Current staging systems perform well at stratifying risk in ear cSCC.

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.

Endolymphatic Sac Tumor as a Ménière-Like Vertiginous Syndrome: A Case Report.

Endolymphatic sac tumors are rare benign neoplasms with locally aggressive behavior located in the posterior petrous ridge of the temporal bone. They cause sensorineural hearing loss and may develop vestibular damage. A 24-year-old male patient arrived at our office with a history of acute vertiginous syndrome, left hearing loss, and tinnitus 1-year ago. His chief complaint was an increase in auditory symptoms. A CT scan and MRI showed an endolymphatic sac tumor. Complete resection of the lesion was achieved by a transmastoid and translabyrinthine approach. Low-grade adenocarcinoma was confirmed by histopathology. The patient remained without clinical vestibular symptoms. However, a small residual tumor was addressed by gamma-ray radiosurgery. Postoperative deep left sensorineural hearing loss was identified, without any vestibular sequelae. Radiologic imaging is the most useful tool for this diagnosis. Endolymphatic sac tumors should be in the differential diagnosis of recalcitrant audio-vestibular symptoms. Complete surgical resection is the most appropriate management.

Resonancia magnética con secuencia HASTE de carcinoma epidermoide del hueso temporal / HASTE magnetic resonance imaging of a squamous cell temporal bone carcinoma

El carcinoma epidermoide del hueso temporal es una neoplasia derivada de células epidérmicas del estrato espinoso, y que dado su baja incidencia, y presentación clínica similar a un cuadro de otitis media crónica colesteatomatosa, su diagnóstico es habitualmente tardío. El estudio del carcinoma epidermoide del hueso temporal se realiza con tomografía computarizada, la que evidencia erosión ósea. Sin embargo, existen distintos diagnósticos diferenciales, tanto malignos como benignos, que pueden provocar hallazgos similares en la tomografía computarizada. La resonancia magnética (RM) es un método de exploración de gran valor complementario para el estudio del carcinoma de oído medio. La RM con secuencia de difusión HASTE ha surgido como herramienta de estudio en patología de hueso temporal, tales como colesteatomas y teratomas de oído medio, pese a esto, no existen reportes en la literatura de la presentación imagenológica del carcinoma epidermoide de hueso temporal utilizando este método. En este artículo se presenta un caso clínico de una paciente con carcinoma epidermoide de oído medio, cuyo estudio preoperatorio incluyó RM-HASTE. Aun cuando se requiere un mayor número de casos para establecer el patrón imagenológico, se concluye que esta técnica es de utilidad para diferenciar este tumor de otitis media crónica colesteatomatosa.

Squamous cell carcinoma of the temporal bone (SCC) is a malignancy originated from epidermis spinous cells. Its low incidence and similar presentation to cholesteatomas, contributes to its late diagnosis. The most widely used study for this tumor is computed tomography (CT), which mainly evidences erosion of the temporal bone. There are several other diagnoses, malignant and benign, that could provoke similar findings at the CT. Magnetic resonance imaging (MRI) is a method of great value to study SCC. MRI with HASTE diffusion sequence has recently emerged as an important tool in the study of temporal bone pathologies, such as cholesteatoma and middle ear teratoma, despite this, there are no reports in the literature of the imaging presentation of SCC using this method. This article presents a clinical case of a patient with SCC, in whose preoperative study was performed HASTE-MRI. Although a larger number of cases are required to establish an imaging pattern, it is concluded that this technique is useful to differentiate this tumor from middle ear cholesteatomas.

Meningioma extradural de oído medio: reporte de un caso y revisión de la literatura / Extradural meningioma of middle ear: reporting a case and literature review

Los meningiomas son los tumores no gliales más comunes del sistema nervioso central constituyendo el 24%-30% de los tumores intracraneales y el 25% de los tumores de médula espinal. Se originan de células de la aracnoides y en general presentan un comportamiento benigno. Existe un subtipo llamado meningioma extracraneal primario o extradural que es poco frecuente y afecta principalmente el área de cabeza y cuello. Debido a su baja prevalencia y síntomas poco característicos son a menudo diagnosticados por fortuna, siendo la inmunohistoquímica fundamental. Se presenta el caso de una mujer adulta con una lesión tumoral en oído medio diagnosticada inicialmente mediante una biopsia incisional como un granuloma de colesterol. Luego del tratamiento quirúrgico y estudio de inmunohistoquímica se concluye el diagnóstico definitivo de meningioma extradural de oído medio. Se analiza la literatura al respecto y se discute sobre su epidemiología, clínica, estudio y manejo.

Meningiomas are the most common non-glial tumors of the central nervous system constituting 24-30% of intracranial tumors and 25% of spinal cord tumors. They originate from arachnoid cells and generally exhibit benign behavior. The subtype primary extracranial meningioma or extradural meningioma that is uncommon and affects the head and neck area. Due to their low prevalence and uncharacteristic symptoms are often diagnosed by fortune, being the immunohistochemistry fundamental. We present the case of an adult woman with a tumor lesion in the middle ear initially diagnosed by an incisional biopsy such as a cholesterol granuloma. After the surgical treatment and immunohistochemical study, the definitive diagnosis of extradural meningioma of the middle ear is concluded. The literature on this subject is analyzed and its epidemiology, clinical practice, study and management are discussed.

Endoscopic Management of Middle Ear Paragangliomas: A Case Series.

OBJECTIVE: To investigate the efficacy and safety of endoscopic middle ear paraganglioma (glomus tympanicum) resection. STUDY DESIGN: Case series with chart review. SETTING: Multi-institutional tertiary university medical centers. PATIENTS: Adult patients with middle ear paragangliomas treated via a transcanal endoscopic approach from 1/2012 to 11/2015. INTERVENTION: All tumors were initially approached via a transcanal endoscopic technique. An operating microscope was used only if the tumor could not be adequately visualized or resected with endoscopic techniques alone. MAIN OUTCOME MEASURES: The main outcome was completeness of tumor resection via the endoscopic technique. Secondary measures were resolution of pulsatile tinnitus, audiometric outcomes, surgical duration, and surgical complications. RESULTS: Endoscopic resection was attempted on 14 middle ear paragangliomas. Thirteen patients (93%) were women with a mean age of 61.6 years. The mean tumor size was 6.2 mm (SD, 3.3). Eleven cases (79%) had complete resection via an exclusive endoscopic approach. The mean surgical duration was 108.1 minutes (SD, 55.6). One case required use of an operating microscope via a transcanal route and two cases required postauricular incisions with mastoidectomy. There were no significant postoperative complications. Two patients (14%) had tympanic membrane perforations repaired intraoperatively without residual perforation on follow-up. All patients had normal postoperative facial nerve function. Pulsatile tinnitus resolved after surgery in all 13 patients who presented with this symptom preoperatively. The mean pure-tone average improved by 5.9 dB (SD, 4.6) after surgery. CONCLUSIONS: Endoscopic management of middle ear paraganglioma is safe, feasible, and effective.

Carcinoma escamoso de conducto auditivo externo: reporte de 3 casos / Squamous cell carcinoma of the external auditory canal: report of three cases

El carcinoma escamoso de conducto auditivo externo es poco frecuente. La sintomatología puede semejar una otitis externa u otitis media crónica, lo que puede retrasar el diagnóstico. La falta de un sistema de clasificación y el número limitados de casos hace difícil la elaboración de guías de tratamiento. Se describe una serie de casos de esta neoplasia, asf como una revisión de la literatura actual.

Squamous cell carcinoma of the external auditory canal is rare. The symptoms may resemble an external otitis or chronic otitis media, which can delay diagnosis. The lack of a classification system and the limited number of cases makes it difficult the development of treatment guidelines. We present a case series of this neoplasia, as well as a review of the literature.

[Apocrine hidrocystoma associated with gouty tophi of the pinna]. / Hidrocistoma apocrino asociado con tofo gotoso en pabellón auricular.

BACKGROUND: Apocrine hidrocystomas, also known as apocrine cystadenomas, are bening cystic tumours derived from the secretory portion of apocrine sweat glands. CLINICAL CASE: A 78-year old female was referred to our division for assesment an asymptomatic translucent, well-defined cystic lesion located on the upper helix. The histological features were consistent with apocrine hidrocystoma coexisting with gouty tophi. CONCLUSIONS: We report the second case of apocrine hydrocystoma located in the pinna, outside of ear canal and the first case of its association with gouty tophi described. It is difficult to know which lesion was first established and if the tophi may lead to ductal obstruction and subsequent cystic retention.

Introducción: los hidrocistomas apocrinos, también conocidos como cistoadenomas apocrinos, son lesiones quísticas benignas derivadas de la porción secretora de las glándulas apocrinas. Caso clínico: remiten al servicio de Dermatología a una mujer de 78 años para valorar una lesión asintomática translúcida, bien definida, localizada en la porción superior del hélix. Los hallazgos histológicos fueron compatibles con un hidrocistoma apocrino asociado a tofo gotoso. Conclusiones: reportamos el segundo caso de hidrocistoma apocrino localizado en el pabellón auricular externo y el primer caso asociado a tofo gotoso. Es difícil de conocer qué lesión se estableció primero, y si el tofo gotoso pudo provocar una obstrucción ductal con la subsiguiente retención quística.

Two friends with eroded nodules on the ears: atypical fibroxanthoma case report.

Atypical fibroxanthoma is an uncommon mesenchymal tumor that manifests clinically as a reddish papule or nodule in sun-exposed areas of the body. The clinical presentation is not specific and histology and immunohistochemistry are both necessary for a correct diagnosis. Surgery is the gold standard of therapy. Recurrence and metastasis should be excluded with a follow-up at 6 months, since this tumor should nowadays be considered a medium-grade neoplasm, rather than low-grade as previously believed. We report the case of two friends who came to our hospital during the same period, complaining of very similar lesions. After biopsy and immunohistochemical examination, a diagnosis of atypical fibroxanthoma in both cases was formulated.

Two friends with eroded nodules on the ears: atypical fibroxanthoma case report

AbstractAtypical fibroxanthoma is an uncommon mesenchymal tumor that manifests clinically as a reddish papule or nodule in sun-exposed areas of the body. The clinical presentation is not specific and histology and immunohistochemistry are both necessary for a correct diagnosis. Surgery is the gold standard of therapy. Recurrence and metastasis should be excluded with a follow-up at 6 months, since this tumor should nowadays be considered a medium-grade neoplasm, rather than low-grade as previously believed. We report the case of two friends who came to our hospital during the same period, complaining of very similar lesions. After biopsy and immunohistochemical examination, a diagnosis of atypical fibroxanthoma in both cases was formulated.

[Clinical characteristics of malignant tumours originating in the external ear]. / Características clínicas de los tumores malignos originados en el pabellón auricular.

BACKGROUND: Skin tumours that originate in the external ear are common in individuals with type 1 skin and phenotype 1 and 2. The skin cancer is associated with chronic or intermittent, but intense sunlight. The most common malignant tumour is basal cell carcinoma, followed by squamous cell carcinoma and melanoma. The diagnosis of squamous cell skin cancer in head and neck area is usually made in the advanced stages and has a poor prognosis. MATERIAL AND METHODS: A cross-sectional, retrospective analysis was performed on the database of patients with skin cancer of the external ear treated between 2011 and 2014. Histology type, stage, rate of clinical and occult metastases, and rate of loco-regional recurrence were evaluated. RESULTS: Of the 42 patients included there were, 25 squamous cell carcinomas, 11 basal cell carcinomas, and 6 invasive melanomas. The rate of lymph node metastases in patients with squamous cell carcinoma was 32%, mostly in the parotid and peri-parotid region, 7% of them with capsular rupture, 2/17 were staged as cN0, and 11.7% had occult metastases. All patients with nodal metastasis were classified as T2 with ulceration. None of the patients with basal cell carcinoma had lymph node metastases. All melanomas were superficial extension type with mean level of Breslow of 3 mm. All underwent lymphatic mapping and sentinel node biopsy, with only one having metastases in the sentinel node. CONCLUSION: The most frequent tumour in the external ear in this series was squamous cell carcinoma. The possibility of lymph node metastases is associated with tumour size (T). Node dissection should be systematic in patients with T2 or greater.

Tufted angioma in ear auricle: importance of the differential diagnosis.

Tufted angioma is an acquired vascular proliferation with specific histological characteristics. The most common clinical features are erythematous macules. It occurs predominantly in children and young adults, especially in the chest and neck. We report the case of a male patient, 12 years old, with an increased right ear auricle associated with erythematous macules and increased local temperature, referred with a diagnosis of lepromatous leprosy; however, this diagnosis was questioned. Histopathology was consistent with tufted angioma.

Tufted angioma in ear auricle: importance of the differential diagnosis / Hemangioma em penacho no pavilhao auricular: importancia do diagnostico diferencial

Tufted angioma is an acquired vascular proliferation with specific histological characteristics. The most common clinical features are erythematous macules. It occurs predominantly in children and young adults, especially in the chest and neck. We report the case of a male patient, 12 years old, with an increased right ear auricle associated with erythematous macules and increased local temperature, referred with a diagnosis of lepromatous leprosy; however, this diagnosis was questioned. Histopathology was consistent with tufted angioma.

O Hemangioma em penacho é uma proliferação vascular adquirida de características histológicas peculiares. Sua característica clínica mais comum são máculas eritematosas. Ocorre predominantemente em crianças e adultos jovens, principalmente no tórax e pescoço. Relata-se o caso de paciente do sexo masculino, 12 anos, com aumento do pavilhão auricular direito associado a máculas eritematosas e aumento da temperatura local, encaminhado com diagnóstico de Hanseníase Virchowiana, entretanto, tal diagnóstico foi questionado. Histopatologia compatível com Hemangioma em penacho.

Folliculosebaceous cystic hamartoma of the external auditory canal in an adult.

Folliculosebaceous cystic hamartoma (FSCH) is a cutaneous hamartoma of pilosebaceous origin that usually occurs on the face. We present what we believe is only the second reported case of FSCH of the external auditory canal, and the first in an adult. The patient was a 59-year-old woman who presented with a 4-year history of a firm nodule on the left external auditory canal that had caused hearing loss, pruritus, and pain. The lesion was excised, and histopathologic examination identified infundibular cysts, hyperplasia of the sebaceous lobules, and a large amount of mucin, features that are consistent with FSCH. One year postoperatively, the patient was asymptomatic, and no evidence of recurrence was noted.