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Neoplasms of the ear are rare.

A large variety of epithelial and mesenchymal tumours arise in the ear (external, medial, internal). 

All tumours are rare except basal and squamous cell carcinomas of the pinna (external ear).

The tumours of the external ear are mainly derived from skin and bone.

Epithelial Neoplasms of the External Ear:

Squamous Cell Carcinoma of the External Ear

Basal Cell Carcinoma of the External Ear

Basaloid squamous cell carcinoma of the external auditory canal: case report.Eur Arch Otorhinolaryngol. 2007 Jun;264(6):697-9.

Basaloid squamous cell carcinoma (BSCC) is a rare malignancy, with features of both basal cell carcinoma and squamous cell carcinoma. The tumor has a predilection for the upper aerodigestive tract, and has been suggested to behave more aggressively than squamous cell carcinoma (SCC). To the author's knowledge, BSCC confined to the external auditory canal (EAC) has not been previously described. BSCC of EAC manifests similar characteristics as the conventional EAC cancer, presenting a mass with chronic otorrhea and itching sense. Excision of the tumor was accomplished by modified lateral temporal bone resection. This report describes the first case of BSCC in this location, and includes reviews of the pathologic and clinical aspects of this disease.

Verrucous Carcinoma of the External Ear

Neoplasm of the Ceruminous Glands:

Tumors arising from the glandular structures of the external auditory canal. Laryngoscope. 1983 Mar;93(3):326-40.

Neoplasms in the external auditory canal (EAC) of ceruminous gland origin have been generally classified under the title of ceruminoma, which is inaccurate and misleading. There have emerged four distinct types of ceruminous gland tumors of the EAC. They are 1. ceruminous adenoma, 2. adenoid-cystic carcinoma, 3. ceruminous adenocarcinoma, and 4. pleomorphic adenoma (mixed tumor). The natural course and clinical approach to these tumors can be determined by accurate histopathologic evaluation. This paper presents 10 cases of tumors of glandular origin in the EAC, 4 cases being ceruminous adenomas, 3 cases being adenoid-cystic carcinomas, 2 cases being ceruminous adenocarcinoma, and 1 case of pleomorphic adenoma (mixed tumor). In reviewing these cases as well as those in the literature, a number of recommendations are suggested: 1. Identifying a tumor of the glandular structures of the EAC solely as a ceruminoma is no longer acceptable without accompanying histologic specificity. 2. Early wide excisional biopsy is imperative for diagnosis. 3. The signs and symptoms of the tumor do not always correlate with the histopathologic diagnosis and subsequent clinical behavior of these tumors. 4. Ceruminous adenoma and pleomorphic adenoma are benign tumors and are best treated only by wide local excision. 5. Adenoid-cystic carcinoma and ceruminous gland adenocarcinoma are pernicious, malignant tumors which are best treated, in general, by an initial aggressive wide en bloc surgical resection or, if there is extension to the middle ear and temporal bone, by resection of the temporal bone and contiguous structures. 6. Postoperative irradiation has an essential role in managing these malignant tumors. 7. Five year survival rates for the malignant tumors do not reflect the biological behavior pattern of "late" local and distant recurrence and metastasis.

Ceruminous glands are modified sweat glands, confined to the skin lining of the cartilaginous part of the external auditory meatus. Tumours arising from these glands are extremely rare.

Controversy still exists about nomenclature, classification, tissue of origin, and accurate diagnosis of these tumours.

The benign eccrine cylindroma should be included in the ceruminous gland tumour classification.

A wide excisional biopsy should be performed in every external auditory canal lesion. The terms 'ceruminoma' and 'cylindroma' should be avoided.

Classifications of ceruminous gland tumours :

Benign:  Ceruminous  Adenoma (ceruminoma) ; Pleomorphic adenoma ; Syringocystadenoma papilliferum ; Cylindroma.

Malignant:   Mucoepidermoid carcinoma ; Adenocarcinoma ; Adenoid cystic carcinoma  

Ceruminous Adenoma of the External Ear

Pleomorphic Adenoma of the External Ear

Syringocystadenoma Papilliferum of the External Ear

Cylindroma of the External Ear

Ceruminous Adenocarcinoma of the External Ear

Adenoid Cystic Carcinoma of the External Ear

Mucoepidermoid Carcinoma of the External Ear:

Mucoepidermoid carcinoma of the external auditory canal (EAC).Acta Otolaryngol. 2007 Mar;127(3):280-4.

We present the third case of mucoepidermoid carcinoma of the external auditory canal (EAC) in the English literature, and discuss the management of this lesion. The patient underwent a wide local resection, superficial parotidectomy, and selective neck dissection. Although intraoperative frozen section margins were negative, permanent histopathologic examination demonstrated tumor in the medial margin, and the tumor was upgraded to a high-grade mucoepidermoid carcinoma. The patient returned to the operating room for a wider local resection, and EAC reconstruction with a temporoparietal pedicled flap and split thickness skin graft. All margins were negative on final histopathologic examination. Radiotherapy was deferred in the event of a recurrence. The patient is currently disease-free 29 months after the final excision. Most authors advocate an aggressive surgical approach, which includes a form of a temporal bone resection, for the treatment of EAC carcinoma. Although this may be warranted in cases of squamous cell carcinoma, mucoepidermoid carcinoma of the EAC may be amenable to a conservative step-by-step approach for local control with less postoperative morbidity. Given the difficulty in detecting mucoepidermoid carcinoma in surgical margins by frozen section analysis, patients should be informed of the possibility of further surgery (re-resection) when a conservative approach is used.

Mucoepidermoid carcinoma of the external auditory canal: case report. Am J Otolaryngol. 2003 Jul-Aug;24(4):274-7.

This study reports a case of mucoepidermoid carcinoma (MEC) of the external auditory canal, which to date has only been described once in literature. Because the lesion is extremely rare, it is particularly difficult to classify it into stages following normal diagnostic parameters. This obviously limits the possibilities of treatment that consequently are either empirical or based on those of squamous cell carcinoma. The problems in the diagnosis and the possible methods of treatment of mucoepidermoid carcinoma are discussed.

Melanocytic Tumours of the External Ear

                  

Primary tumours of the external auditory canal. Our experience in 34 patients.Acta Otorrinolaringol Esp. 2007 Jan;58(1):20-4.

OBJECTIVES: Malignant neoplasms of the external auditory canal (EAC) and middle ear are rare but have a poor prognosis. The aim of this study is to identify the variables associated with worse prognosis. PATIENTS AND METHOD: Thirty-four patients were treated in our department between 1990 and 2006 for EAC and middle ear tumours. The patients were staged according to the 1990 Pittsburgh classification. In most cases, surgery was followed by post-operative radiotherapy. RESULTS: The overall disease-free survival was 49 % after 5 years. It reached 87 % in stages I and II, whereas the survival for stages III and IV was 21 % (P=.001). Pre-operative facial nerve paralysis (P=.03), lymph node metastasis (P=.01) and dural extension (P=.02) were associated with decreased survival rates. CONCLUSIONS: In carcinomas of the EAC and middle ear, lymph node involvement, facial nerve palsy, and dural extension were associated with a poorer outcome. For tumours in advanced stages, new therapeutic protocols should be evaluated.

Carcinoma of the external auditory canal and middle ear: therapeutic strategy and follow up.Laryngorhinootologie. 2004 Dec;83(12):818-23.

BACKGROUND: Carcinomas of the external auditory canal (EAC) and the middle ear are rare and considered to have a poor prognosis. The recommended therapeutic strategy consists of surgical excision and postoperative radiotherapy. However, there are different opinions about the extend of the primary operation. PATIENTS AND METHODS: A series of 21 patients with carcinoma of the EAC and middle ear were treated at the ENT-Department of the Hospital Fulda from 1985 to 2003. Their records and radiologic findings were reviewed retrospectively with particular reference to tumor type and size, its relation to surrounding tissues, surgical procedures and radiation techniques. The tumors were staged according to the modified Pittburgh staging system for temporal bone carcinomas. The average follow-up time was 6.2 years (range 0.2 - 18.75). RESULTS: 17 patients suffered from carcinoma of the EAC, 4 carcinomas were primarily located in the middle ear. There were 15 squamous cell carcinomas, 3 adenoidcystic carcinomas, 2 adenocarcinomas and one mucoepidermoid carcinoma. 12 patients came primarily to our institution and were staged as follows: pT1 (n = 2), pT3 (n = 2), pT4 (n = 8). 8 patients showed up with recurrent or residual tumors (all of T3 or T4 stage). One patient could not be classified. In 5 cases the tumor was inoperable. These patients underwent combined chemoradiation therapy. All other 16 patients were operated and most of them received adjuvant radiation therapy. In the group of patients who were primarily operated overall 5-year survival rate was 100 %. In contrast, patients who's recurrent or residual tumors were resected had a 5-year survival rate of only 33 %. Patients who received combined chemoradiation therapy showed a 2-year survival rate of 75 %. CONCLUSION: Carcinoma of the EAC and middle ear should be treated primarily by a lateral or subtotal temporal bone resection stage dependent combined with a parotidectomy as well as a neck dissection. Local resection of the EAC is not sufficient, not even in T1 tumors. As from stage T2, in cases of recurrent tumor removal and questionable free margins as well as in cases with lymph node metastases an adjuvant radiation therapy should be added. The most important survival factor is removal of the primary tumor with histologically clear margins.

Malignant tumours of the external auditory canal and of the middle ear.Acta Otorrinolaringol Esp. 2004 Dec;55(10):470-4.

OBJECTIVES: To review our experience and results in the treatment of a low incidence pathology such as the malignant ear tumors. METHODS: The study reviewed 36 patients with malignant tumors of the EAC and middle ear treated between 1977 and 2000 in our hospital, excluding cancer of the pinna, metastatic tumours and sarcomas. It was used the staging system proposed by the M.D. Anderson. RESULTS: The most common histological type in our series is the squamous cell carcinoma, and the otorrhea and pain are the primary symptoms in 100% of patients. Surgery combined with radiotherapy obtained a 41% 5-year survival rate. CONCLUSIONS: The staging system is an important prognostic factor and it is important an early diagnosis to achieve a better therapeutical result.

Avoiding misdiagnosis in ceruminous gland tumours. Auris Nasus Larynx. 2003 Aug;30(3) : 287-90.

Ceruminous gland tumours are infrequent lesions of the external auditory canal (EAC). Controversy still exists about nomenclature, classification, tissue of origin, and accurate diagnosis of these tumours. We present three cases of ceruminous gland tumours, including benign eccrine cylindroma, ceruminous adenoma, and adenoid cystic carcinoma. Superficial biopsy led to an initial erroneous diagnosis of adenocarcinoma in the latter. All cases were positive for cytokeratin and S-100 protein, supporting a ceruminous gland origin. The benign eccrine cylindroma should be included in the ceruminous gland tumour classification. A wide excisional biopsy should be performed in every EAC lesion. The terms 'ceruminoma' and 'cylindroma' should be avoided.

Cancer of the external auditory canal.Arch Otolaryngol Head Neck Surg. 2002 Jul;128 (7): 834-7.

OBJECTIVE: To evaluate the outcome of surgery for cancer of the external auditory canal and relate this to the Pittsburgh staging system used both on squamous cell carcinoma and non-squamous cell carcinoma. DESIGN: Retrospective case series of all patients who had surgery between 1979 and 2000. Median follow-up was 47 months (range, 2-148 months). Data on age, sex, symptoms, TNM status, histopathological diagnosis, surgery, adjunctive therapy, sequelae, recurrence, and status at follow-up were obtained. SETTING: An ear, nose, and throat department in an ambulatory and hospitalized care center. PATIENTS: Ten women and 10 men with previously untreated primary cancer. Median age at diagnosis was 67 years (range, 31-87 years). Survival data included 18 patients with at least 2 years of follow-up or recurrence. INTERVENTION: Local canal resection or partial temporal bone resection. MAIN OUTCOME MEASURE: Recurrence rate. RESULTS: Half of the patients had squamous cell carcinoma. Thirteen of the patients had stage I tumor (65%), 2 had stage II (10%), 2 had stage III (10%), and 3 had stage IV tumor (15%). Twelve patients were cured. All patients with stage I or II cancers were cured except 1 with adenoid cystic carcinoma. No patients with stage III or IV cancer were cured. All recurrences developed in patients with incompletely resected tumors. CONCLUSIONS: The outcome was related to the stage of disease, suggesting that the Pittsburgh staging system is useful also in patients with non-squamous cell carcinoma. Patients with early cancer benefited from a less aggressive surgical approach, while survival was poor in patients with advanced cancer with incompletely resected tumors despite adjuvant radiotherapy.

Tumor of ceruminous gland with intracranial invasion: case report.Arq Neuropsiquiatr. 2000 Jun;58(2A):324-9.

Ceruminous glands are modified apocrine glands, confined to the skin lining of the cartilaginous part of the external auditory meatus. Tumors arising from these glands are rare. Controversy exists regarding the term "ceruminoma". Actually this neoplasia should be classified as adenoma, adenocarcinoma, adenoid cystic carcinoma and pleomorphic ceruminous adenoma. We report a 39-year-old woman first seen at Santa Casa of Belo Horizonte, in 1998, presenting with headache, nausea, vertigo, hearing loss and tinnitus on the right for the past two years. CT scan showed a tumor eroding cartilaginous and bony limits with intracranial invasion. She was submitted to multidisciplinary treatment with surgery followed by radiotherapy (6000 cG). Histology showed a ceruminous adenoid cystic carcinoma. The patient manifested a right peripheral facial palsy and had no recovery of the previous deficits. After one year from surgery she is clinically stable.

Prognostic factors in carcinoma of the external auditory canal.Arch Otolaryngol Head Neck Surg. 1997 Jul;123(7):720-4.

BACKGROUND: Carcinomas of the external auditory canal are rare neoplasms (< 1% of all head and neck malignant neoplasms). OBJECTIVE: To evaluate the prognostic factors in 79 patients treated in a single institution. PATIENTS AND METHODS: The disease was staged as follows: 34 patients with stage T1 to T2 tumors; 43 patients with stage T3 to T4 tumors; 2 patients with stage TX tumors; 68 patients with stage N0 tumors; and 11 patients with stage N1 tumors. The initial treatment was surgery in 59 patients and radiotherapy in 9 patients. Eleven patients were not considered candidates for treatment. RESULTS: To date, 29 patients have experienced local recurrences and 2, neck metastases. The 5-year survival rates were 65% for patients who underwent surgery, 29% for patients who underwent radiotherapy, and 63% for patients who underwent a combination of surgery and radiotherapy. Univariate survival analysis showed statistical difference according to tumor type (P = .003), bone involvement (P = .002), and tumor stage (P < .001). CONCLUSION: Every effort must be undertaken to make an early diagnosis and perform radical surgical resection of squamous cell carcinomas in the external auditory canal. This study validates the staging system used for squamous cell carcinoma of the ear treated with surgery.

Twenty-one cases of malignant tumor of the external auditory canal or middle ear.Nippon Jibiinkoka Gakkai Kaiho. 1996 May;99(5):645-52.

Between 1982 and 1994, 21 patients with malignant tumors of the external auditory canal and middle ear were treated at the Department of Otolaryngology, Niigata University. Eleven patients with tumors of the external auditory canal and 10 tumors of the middle ear were registered. There were 9 males and 12 females, and their ages ranged from 10 to 80 years (median: 61). Otalgia, otorrhea or bloody otorrhea were the chief complaints of most patients with external auditory canal of middle ear tumors. Pathological examination revealed squamous cell carcinoma in 16 patients, adenoid cystic carcinoma in 3 patients, and basal cell carcinoma and rhabdomyosarcoma in 1 patient each. External auditory canal tumors were surgically excised, while radical mastoidectomy and subsequent irradiation were performed for the middle ear tumors. The five-year survival rate determined by the Kaplan-Meier method, was 77.8% for patients with external auditory canal tumors and 40% for those with middle ear tumors.

Ceruminous gland tumours: a reappraisal. J Laryngol Otol. 1992 Aug; 106(8):727-32.

Ceruminous glands should no longer be regarded as purely apocrine glands, but as apoeccrine glands with both apocrine and eccrine modes of secretion. We present two cases of pleomorphic adenoma of ceruminous glands, among the rarest of such tumours. The use of such terms as 'ceruminoma' and 'hidradenoma' should finally be abandoned, and 'ceruminous gland tumour' used instead as a generic term. Classification should be based on Wetli's prototype (adenoma, pleomorphic adenoma, adenoid cystic carcinoma and adenocarcinoma), with the addition of benign eccrine cylindroma and syringocystadenoma papilliferum; the inclusion of mucoepidermoid carcinoma should await full validation. Wide local excision is necessary for all tumours, with only follow-up for histologically benign neoplasms. Malignant tumours need early aggressive surgery and radiotherapy. If marginal invasion cannot be assessed histologically, then adenoma and adenocarcinoma cannot be distinguished and we suggest that the tumour be reported as 'of uncertain malignant potential'. Long-term studies are needed to confirm or refute the view that all ceruminous gland tumours are potentially malignant.

Primary tumors of the external auditory canal.Acta Otorrinolaringol Esp. 1992 Nov-Dec;43(6):439-42.

Primary tumors of the external auditory canal (EAC) are rare, their etiology is uncertain and their prognosis, dim. The most effective therapy combines both surgery and radiotherapy. This study reviews the literature and shows the results from 13 cases, treated between 1981-1991. This series proves a clear prevalence for the epidermoid tumors (10 out of 13) and we call the attention on their poor prognosis even in those tumors confined to EAC. The types of treatment and their complications as well, were reviewed.

Malignant tumors of the external ear.Ann Plast Surg. 1988 Dec;21(6):550-4.

Although only a few of the malignant tumors of the skin develop in the external ear, they are more frequent than one would expect. These tumors recur and develop metastases more often than tumors in other sites. Because of this and because of surrounding vital structures, these tumors have a poor prognosis. Surgical therapy, that is, wide excision, is better than radiotherapy. Many methods for reconstruction of the external ear have been published, and sometimes plastic protheses are acceptable. From 1982 to 1986, 17 patients with malignant tumors of the external ear were treated in our center. There were 15 men and 2 women. The mean age was 73 years. There were 4 basal cell and 12 squamous cell carcinomas, and 1 patient had malignant melanoma of the external ear. Nine of these tumors were on the helix. During the follow-up period, 6 patients had local recurrent disease. In 7 patients, reexcision had to be performed several times after incomplete excision. Six patients are alive without any sign of the disease, and 3 patients died.

Malignancies of the external ear canal and temporal bone: surgical techniques and results.Laryngoscope. 1987 Feb;97(2):158-64.

A combined therapy approach to malignancies of the external auditory canal and middle ear has been developed. A technique of external canal resection and gross tumor removal from the middle ear, parotid gland, and superior cervical lymph nodes is followed by postoperative full-therapy irradiation. This combined approach has been used in 30 patients with malignancies involving the external auditory canal and temporal bone. The preoperative evaluation and surgical technique, including the intraoperative decision-making process, is described. Twenty-four patients had squamous cell carcinoma of the external auditory canal, and two patients had basal cell carcinoma. There was one patient each with adenocystic carcinoma, acinic cell carcinoma, high grade mucoepidermoid carcinoma, and a giant cell tumor of bone. This group of patients was broken down into three groups based on the extent of disease as determined at surgery. Overall control of disease, both locally and distant, for the 30 patients was 66%. There were 12 patients with disease limited to the ear canal. These patients had a 91% survival of this disease process. Seven patients were determined to have limited extension beyond the ear canal. These were treated with combined therapy with an overall control of disease of 72%. Eleven patients had extensive disease outside of middle ear into the carotid jugular spine, stylomastoid foramen, and skull base, with a survival rate of 45%. It is concluded that a step-wise removal of all gross tumor, as opposed to an en bloc dissection of the temporal bone and skull base, followed by full-therapy irradiation gives equally as good, or even better, long-term survival for this malignancy of the external auditory canal and middle ear.

 
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Tumors arising from the glandular structures of the external auditory canal.Laryngoscope. 1983 Mar;93(3):326-40.

Neoplasms in the external auditory canal (EAC) of ceruminous gland origin have been generally classified under the title of ceruminoma, which is inaccurate and misleading. There have emerged four distinct types of ceruminous gland tumors of the EAC. They are 1. ceruminous adenoma, 2. adenoid-cystic carcinoma, 3. ceruminous adenocarcinoma, and 4. pleomorphic adenoma (mixed tumor). The natural course and clinical approach to these tumors can be determined by accurate histopathologic evaluation. This paper presents 10 cases of tumors of glandular origin in the EAC, 4 cases being ceruminous adenomas, 3 cases being adenoid-cystic carcinomas, 2 cases being ceruminous adenocarcinoma, and 1 case of pleomorphic adenoma (mixed tumor). In reviewing these cases as well as those in the literature, a number of recommendations are suggested: 1. Identifying a tumor of the glandular structures of the EAC solely as a ceruminoma is no longer acceptable without accompanying histologic specificity. 2. Early wide excisional biopsy is imperative for diagnosis. 3. The signs and symptoms of the tumor do not always correlate with the histopathologic diagnosis and subsequent clinical behavior of these tumors. 4. Ceruminous adenoma and pleomorphic adenoma are benign tumors and are best treated only by wide local excision. 5. Adenoid-cystic carcinoma and ceruminous gland adenocarcinoma are pernicious, malignant tumors which are best treated, in general, by an initial aggressive wide en bloc surgical resection or, if there is extension to the middle ear and temporal bone, by resection of the temporal bone and contiguous structures. 6. Postoperative irradiation has an essential role in managing these malignant tumors. 7. Five year survival rates for the malignant tumors do not reflect the biological behavior pattern of "late" local and distant recurrence and metastasis.

Carcinoma of the external ear.Scand J Plast Reconstr Surg. 1976;10(2):147-51.

An analysis of 246 operated patients with 260 carcinomas of the external ears from the periods 1949-57 and 1962-67 is presented. Carcinoma of the external ear occurs in Denmark with a frequency of 1.2 cases per 100 000 inhabitants and constitutes approximately 6% of all skin cancers. In the present material 67% of the tumours were squamous-cell carcinomas and 30% were basal-cell carcinomas. These forms of tumours were most frequent in elderly men who have had outdoor employment. Half of these tumours were sited on the helix and lobe, while one quarter, respectively, were sited medially and laterally. 15% of the squamous-cell carcinomas recurred, 3% of the these with metastases of the regional lymph nodes. Of the basal-cell carcinomas 18% recurred but with no metastases of the regional lymph nodes. The frequency of recurrence did not depend on the site of the tumour on the ear, but it did increase with increasing tumour size. The 5-year survival rate without recurrence was 56% +/- 4% for the squamous-cell carcinomas, and for the basal-cell carcinomas 59% +/- 6.5%. It is concluded that surgical treatment is best suited for these tumours, and the minimum excision distance for basal-cell carcinomas of less than 3 cm in size should be 8 mm, while for squamous-cell carcinomas of the same size the distance should be 10 mm. For both forms of tumour where the greatest extent is more than 3 cm the excision distance should be at least 15 mm.

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