Primary pleomorphic adenoma of the external ear canal. Report of a case and literature review

INTRODUCTION: Primary neoplasms of the external ear canal are rare, and 5% of these tumors are of glandular origin. Ceruminal glands are modified sweat glands of the skin of the external auditory meatus that may give rise to (a) benign tumors such as ceruminous adenoma, pleomorphic adenoma, and syringocystadenoma papilliferum, and (b) malignant tumors such as ceruminous adenocarcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma. An alternative theory holds that pleomorphic adenomas may well arise from ectopic salivary tissue present in the external ear canal. These tumors are very rare and usually have a benign course. In this report, we describe an unusual case of pleomorphic adenoma of the external auditory canal associated with chronic infection of the middle ear. CASE REPORT: A 60-year-old woman presented with exacerbation of left otalgia over a 6-month period. She had been affected with chronic otitis media and aural polyps for the last 13 years, for which she had received medical treatment only. A canal-filling aural polyp was noted on clinical examination. Subsequent biopsy and histologic examination revealed pleomorphic adenoma of the external ear canal, possibly with malignant elements. Magnetic resonance imaging showed no intracranial extension or any association with the adjacent parotid gland. The patient underwent modified radical mastoidectomy and complete resection of the tumor and the entire skin of the external auditory canal. Final histology and immunohistochemistry confirmed the absence of malignancy, and no recurrence has been reported 1 year postoperatively. CONCLUSIONS: Pleomorphic adenoma is an extremely rare tumor arising from the ceruminal glands of the external ear canal. Nonspecific presentation and difficult histologic diagnosis characterize this benign neoplasm. Wide local excision is the mainstay of treatment.     

外耳道耵聍腺源性恶性肿瘤(附13例报告)

目的 探讨外耳道耵聍腺源性恶性肿瘤的临床特征和治疗效果。方法 对13例耵聍腺源性恶性肿瘤手术病例进行随访和回顾性分析。结果 随访5年以上11例，随访3年以上2例，其中失访4例，无瘤生存7例，复发死亡2例。结论 对耵聍腺源性的恶性肿瘤，应早期行局部扩大或根治手术。放疗可作为手术切除后的辅助治疗。对所有病例应进行长期随访。对腺样囊性癌病例，尤其应注意肺部随访。     

Tumors arising from the glandular structures of the external auditory canal

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.     

Ultrastructural morphology of a middle ear ceruminoma

The ultrastructural morphology of a ceruminous gland adenoma in the middle ear was examined electron microscopically. The epithelial tumor cells displayed apocrine caps, microvilli, cell junctions, secretory granules, vacuoles, lipid droplets and siderosomes, which are the characteristic ultrastructural features of apocrine glands. Concentric membranous bodies of the endoplasmic reticulum, phagocytic activity of the tumor cells, intracytoplasmic lumina, ciliated cells and also spiny collagen in the tumor stroma could be seen. The myoepithelial cells are an important tumor marker in the differential diagnosis between ceruminomas and adenomas of the middle ear. The ectopic origin in the modified apocrine ceruminous glands, the specific localization, the clinical features and the extremely rare occurrence of the ceruminoma makes this tumor a unique neoplastic entity.     

Ceruminoma revisited

Ceruminoma is a catch-all term that has caused much confusion both in the literature and in clinical practice in regard to the specific histologic diagnosis and proper treatment for tumors arising from the ceruminous glands of the external ear canal. The term ceruminoma has been used in the past to refer to both benign and malignant lesions. To clarify the terminology and better determine appropriate treatment, two cases of benign adenoma of the ceruminous glands along with their histopathologic findings will be presented. The specific characterization of the individual types of ceruminous gland neoplasms, their clinical manifestations, histopathology, and recommended treatment will then be discussed. Finally, suggestions for the appropriate nomenclature for these rare tumors will be reviewed.     

Ceruminous gland adenoma of the external auditory canal: a case report

Ceruminous adenoma (ceruminoma) of the external auditory meatus is a rare neoplasm with benign clinical behavior. This tumor is mainly composed of the ceruminous gland cells (modified apocrine sweat glands). These glands are localized deep in the skin mostly in the cartilaginous part of the external auditory meatus. The most often symptom of the ceruminous adenoma of external auditory meatus is the unilateral conductive hearing loss. Occasionally symptoms of this tumor (pain, otorrhoea) can result from an otitis externa secondary to meatus obstruction. Ceruminous adenoma should be radical excised with adequate margins of the normal tissues. The prognosis of these tumors is good. The difficulties of nomenclature, histological structure of the auditory external meatus ceruminoma, as well as symptomatology, treatment and clinical behavior are discussed on the basis of the literature. A case of histologically confirmed ceruminoma (ceruminous adenoma) of the external auditory meatus in a surgically treated 53 years old men is presented.     

Ceruminous gland tumours: a reappraisal.

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.     

Ceruminous adenoma mimicking furunculosis in the external auditory canal

Tumors arising from the ceruminous glands are very rare. Herein, we report a case of ceruminous adenoma mimicking furunculosis in the external auditory canal (EAC), along with a discussion of the various pathological types and a review of their management.     

Middle-ear ceruminous adenoma as a rare cause of hearing loss and vertigo: case reports

Middle-ear ceruminous adenomas are rare benign neoplasms arising from the epithelium of the middle ear. Progressive hearing loss, ear fullness and tinnitus are common symptoms of this tumour; facial nerve paresis and vestibular disturbances occur very infrequently. We present two cases of middle-ear ceruminous adenomas, one showed rapid unilateral hearing loss with aural fullness, followed by purulent aural discharge and vertigo. In the second case, the disease affected an already deaf ear and the only symptom of the disease was increasing vertigo. The clinical features, intraoperative findings, and histological and radiological findings are presented. The cases are compared to those described in the literature.     

Ceruminoma

A unique case of adenoid cystic carcinoma arising in a ceruminous gland, with widespread pulmonary metastases, is presented. This is the first case in the English literature in which the diagnosis was made from material obtained by needle aspiration from a pulmonary metastasis. The literature is reviewed and the histological features and clinical course are discussed in the paper. Adenoid cystic carcinoma is the most common tumor of ceruminous gland origin, and is the only one which produces widespread metastases in the lungs. Tumors arising from ceruminous glands are slightly more prevalent in females, and pulmonary metastases occur almost three times as often in females as in males. Suggested treatment is wide local excision of the primary tumor and of local recurrences, and excision of distant metastases when possible. Irradiation is useful only for palliation. All patients, but especially females, should be followed carefully for the detection of possible pulmonary metastases.     

Human ceruminous gland innervation

Human ceruminous glands have been examined for the presence of adrenergic nerves using the histofluorescence method according to Falck and Hillarp, and immunohistochemistry for the presence of peptidergic nerves. The effect of adrenaline, alpha- and beta-blockers have been tested in vivo. No adrenergic or peptidergic nerves could be seen innervating the ceruminous glands. In spite of that, the glands reacted to local administration of adrenaline. This adrenaline effect could be blocked by alpha-blockers but not by beta-blockers. The cerumnious glands seem to possess alpha-adrenergic receptors, possibly capable of reacting with circulating catecholamines.     

Pleomorphic adenoma of minor salivary glands

Pleomorphic adenoma, a benign tumor often seen in ENT practice, arises either from minor as well from major salivary glands. 5-14% of tumors occur in minor glands. Sixteen cases of minor salivary gland pleomorphic adenomas are studied. Eight of them originate from the oral cavity: 6 from the palate, one from the lower lip and one from the cheek. Two oral adenomas were malignant. In 4 patients tumors occur in the nasal cavity and in two other patients--in the neck. The clinical and pathological features of these patients are presented. Special attention is given to malignant transformation and the rate of recurrence of the tumors.     

An immunohistochemical study of adenoid cystic carcinoma of the external auditory canal

Summary Specimens from five cases of adenoid cystic carcinoma of the external auditory canal were studied by immunohistochemical staining, and findings were compared with those from adjacent non-neoplastic tissues containing ceruminous glands. In the ceruminous gland, cytokeratin showed diffuse positive staining, while myoepithelial cells were stained for smooth muscle actin, desmin, S-100 protein and vimentin. The epithelial markers used were cytokeratin, carcinoembryonic antigen and secretory component and stained at various densities the inner cells of the tubular component and ductlining cells in the cibriform component of tumor tissues. In contrast, the muscular markers, smooth muscle actin and desmin, and the mesenchymal marker, vimentin, were positive in the outer cells of the tubular component and in the cyst-lining cells of the cribriform component. S-100 protein immunoreactivity showed pradoxical results; positive findings occurred in the myoepithelial cells of the ceruminous glands and in the inner cells of the tubular component and duct-lining cells of the cribriform component of the tumor. Present findings demonstrate that adenoid cystic carcinoma of the external auditory canal had dual epithelial and myoepithelial differentiation and can mimic the ceruminous glands of the auditory canal.     

Salivary tumours--experience over thirty years.

A clinicopathological survey of tumours of salivary glands seen in a special surgical clinic at the Christie Hospital and Holt Radium Institute is reported. Nine hundred and seventy-seven benign and malignant tumours have been seen within the past 30 years. The results of treatment are included with particular attention to those of the parotid glands which present the greatest therapeutic problem. The approach to surgical treatment of pleomorphic adenomas is described in detail and guidelines offered as to the procedure that should be used based on full exploration and assessment of each tumour. The need for adjuvant irradiation depends on the standard of surgery that is done. The relationship between carcinomas and benign adenomas is discussed and the need for surgery in all cases is stressed in order to make a definite histological diagnosis in view of the wide range of pathological abnormality encountered.     

Salivary tumours—experience over thirty years

A clinicopathological survey of tumours of salivary glands seen in a special surgical clinic at the Christie Hospital and Holt Radium Institute is reported. Nine hundred and seventy-seven benign and malignant tumours have been seen within the past 30 years. The results of treatment are included with particular attention to those of the parotid glands which present the greatest therapeutic problem. The approach to surgical treatment of pleomorphic adenomas is described in detail and guidelines offered as to the procedure that should be used based on full exploration and assessment of each tumour. The need for adjuvant irradiation depends on the standard of surgery that is done. The relationship between carcinomas and benign adenomas is discussed and the need for surgery in all cases is stressed in order to make a definite histological diagnosis in view of the wide range of pathological abnormality encountered.     

A case of ceruminous adenocarcinoma of the external auditory canal presenting as an aural polyp

Ceruminous adenocarcinoma is a rare malignant tumor arising from the ceruminous gland of the external auditory canal ceruminous gland. Usually it displays no specific clinical symptoms or signs, so most reported cases have involved an advanced tumor and the treatment results have been disappointing. It is important to make an early diagnosis to improve the treatment's outcome. We present the case of a 54-year-old female patient with a 1-year history of feeling blockage in her left ear. Otoscopic examination revealed an aural polyp arising from the cartilaginous ear canal's anterosuperior wall. A wide local excisional biopsy was performed, and the pathologic result was a ceruminous adenocarcinoma. We were able to detect the tumor early, and en bloc resection was possible in the form of a partial temporal bone resection.     

Flow cytometric analysis of DNA content and Ki-67-positive fractions in the diagnosis of salivary gland tumors

To explore the utility of flow cytometry (FCM) for the diagnosis of histopathology of salivary gland tumors, fresh materials taken at surgery from 23 Warthin’s tumors, 57 pleomorphic adenomas, and 14 malignant tumors were analyzed for DNA ploidy and proliferative cell activities, including S-phase fraction (SPF), G2- plus M-phase fraction (G2M), and Ki-67-positive fraction. To facilitate this study, glands were taken from all major salivary sites and minor glands in the head and neck. DNA aneuploidy was not detected in the benign tumors. Nine of 14 malignant tumors showed DNA aneuploidy. The percentage of SPF or G2M of the malignant tumors was significantly higher than those of the benign tumors. The percentage of Ki-67-positive fraction of pleomorphic adenomas was comparable to that of malignant tumors and was significantly higher than that of Warthin’s tumors. Ki-67 of 20% as a cut-off had a sensitivity of 88%, specificity of 100%, and accuracy of 91% for differentiating pleomorphic adenomas from Warthin’s tumors. In analyzing DNA content and proliferative activities by FCM, we could distinguish among the three major histopathologies of salivary gland tumors. Warthin’s tumors showed low SPF+G2M with low Ki-67, pleomorphic adenomas had low SPF+G2M with high Ki-67, and malignant tumor showed high SPF+G2M with high Ki-67. The high percentage of the Ki-67-positive fraction seen in pleomorphic adenomas may reflect their potential biological aggressiveness manifested as tumor recurrence or malignant transformation. Received: 26 June 1997 / Accepted: 5 November 1997     

Primary tumors of the external and middle ear. Benign and malignant glandular neoplasms

Glandular neoplasms represented 14% of primary tumors of the external and middle ear at the University of Minnesota. Although the collective term "ceruminoma" has been used in the past, four distinctive histopathologic patterns were recognized among our 12 cases: adenoma (four cases), pleomorphic adenoma (one case), adenoid cystic carcinoma (two cases) and adenocarcinoma (five cases). The prognosis correlated with these subgroups. A thorough review of the English literature also tended to support the rationale for the subclassification of so-called ceruminomas. The prevailing histogenetic view is that these tumors are derived from the modified apocrine glands of the auditory canal, the ceruminous glands. In some instances, these tumors may originate from ectopic salivary gland tissue.     

The ceruminous glands in chronic suppurative otitis media. A histological and histochemical study.

The ceruminous glands have been studied histologically and histochemically in twenty patients suffering from chronic suppurative otitis media with discharging ears, and in ten patients with dry central perforations, in comparison with normal ears. The present study revealed hyperactivity of the glands initially; later on, atrophy and fibrosis took place. Thus the diminution of wax may be due to: (1) The washing effect of the discharge. (2) Atrophy of the glandular structures. (3) Periglandular fibrosis. As the discharge stops, regeneration of the glands occurs.     

Basaloid monomorphic adenomas

Monomorphic adenomas can be divided into basaloid and nonbasaloid type. With the exception of the canalicular adenoma, the basaloid monomorphic adenomas are preponderantly tumors of the major salivary glands, most often the parotid gland. The dermal analogue monomorphic adenomas, so named because of their histologic similarity to eccrine tumors of the skin, are a unique subset of the basaloid adenomas. They are often multifocal, recur frequently, may undergo malignant change, and can be part of a salivary-cutaneous tumor diathesis.