Magnetic resonance imaging of syringocystadenoma papilliferum of the external auditory canal

Syringocystadenoma papilliferum (SCAP) usually occurs on the face or the scalp and is very rare in the external auditory canal (EAC). There has been no information on magnetic resonance (MR) imaging of this tumor irrespective of its site. We report here a case of 57-year-old man having this tumor, which was surgically removed and its histopathology was confirmed. MR imaging demonstrated a lobulated 4-cm mass with clearly defined margins in the EAC. Although the tumor was bulky, these MR findings were different from the malignancies. The mass lesion showed intermediate signal intensity both on T1- and T2-weighted MR images and showed slight enhancement on gadolinium-enhanced T1-weighted images. Signal intensities on T2-weighted images of this tumor were low compared to those of pleomorphic adenoma. All ceruminous gland tumors including SCAP are thought to be potentially malignant; therefore, pre-operative biopsy should not be performed. Even though incisional biopsy is sometimes needed as in our case, the current MR features would be helpful for differential diagnosis of this rare condition and assessing the extension of the tumor.     

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.     

Primary pleomorphic adenoma (chondroid syringoma) of the external auditory canal. Case report and literature review

Chondroid syringoma of the external auditory canal is an extremely rare neoplasm, representing the cutaneous counterpart of pleomorphic adenoma of salivary glands. This tumour is thought to derive from the apocrine duct of the folliculo-sebaceous-apocrine unit. We report the case of a 68-year-old male in whom the clinical and radiological examinations showed a well-circumscribed tumour limited to the external auditory canal. The diagnosis was confirmed by histologic examination. We also reviewed the literature.     

Hidradenomata of the external auditory meatus. (Review of the literature and report of a pleomorphic adenoma).

A case of hidradenoma of the external auditory meatus ('ceruminoma') is presented, with a review of the literature. The tumour has the appearances of a pleomorphic adenoma and is devoid of a capsule. It is suggested that the term hidradenoma be used for tumours of the ceruminous glands and that they be classified histologically as (1) adenoma, (2) pleomorphic adenoma, (3) adenoid cystic carcinoma and (4) adenocarcinoma. This classification aids treatment and prognosis. Attention is drawn to the high incidence of malignancy in hidradenomata of the external auditory meatus. The treatment of choice for pleomorphic adenomata is wide surgical excision with an adequate margin of normal skin. If there is doubt regarding complete removal, or a recurrence develops, a wider surgical excision is necessitated.     

Pleomorphic adenoma of the external auditory canal in Japan, with a case report.

A rare case of a 51-year-old female with a pleomorphic adenoma, measuring 0.9 x 0.7 x 0.6 (cm3), originating from the right external auditory canal (EAC), was reported. The authors discussed the 7 reported cases of EAC pleomorphic adenoma in Japan, comparing them with pleomorphic adenomas occurring in the nasal cavity and the parotid gland. It is currently too early to conclude that pleomorphic adenoma in the EAC does not tend to recur or shows a marked tendency toward canceration. The best possible treatment for EAC pleomorphic adenoma at present seems to be, as in cases of tumors in other sites, surgical resection together with removal of a sufficient range of surrounding normal tissue, followed by careful long-term postoperative observation of the clinical course.     

Extra-major salivary gland pleomorphic adenoma of the head and neck: a 10-year experience and review of the literature

Pleomorphic adenomas, or benign mixed tumors, make up 65% of all salivary gland tumors. They also can be found as solid tumors in other parts of the head and neck region, such as the auditory canal, the eyelids, and the orbital area. In this study, we investigated extra-major salivary gland pleomorphic adenomas of the head and neck region retrospectively at a tertiary care center. Between March 1998 and June 2009, 37 patients underwent primary surgery for extra-major salivary gland pleomorphic adenoma of the head and neck. The duration of symptoms, radiographic findings, operative procedures, and pathologic findings were documented. Of the 37 patients enrolled, 22 were male and 15 were female, with a median age of 57?years. Tumors were found in the soft palate, hard palate, nasopharynx, orbital area, trachea, buccal mucosa, cheek, nasal septum, upper lip, lower eyelid, and external auditory canal. Cellular variant of the pleomorphic adenoma was found in four patients, while the remaining patients presented with the classic variant. No myxoid subgroup was noted in our study. Carcinoma ex pleomorphic adenoma was observed only in one patient for whom radical surgery was performed. Twenty-eight patients (76%) had long-term follow-ups, with the average follow-up period being 4.5?years. Local recurrence was observed in three patients, and they underwent revision surgery during the follow-up period. Our results indicate that extra-major salivary gland pleomorphic adenomas are most commonly found in the soft palate. Wide excision was the treatment of choice, although its efficacy might be compromised with cosmetics and functional structures of the head and neck. Therefore, long-term follow-up of patients is necessary.     

Tubular apocrine adenoma with syringocystadenoma papilliferum arising from the external auditory canal

Tubular apocrine adenoma (TAA) is a very rare sweat gland tumour. Comprehensive review of the literature reveals that TAA in the external auditory canal (EAC) has not yet been reported. We report a case of TAA in the EAC, together with characteristic histopathological findings.     

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.     

Ectopic extra-cranial meningioma presenting as an aural polyp

Ectopic meningiomas located within the external auditory canal without any intracranial connection are an extremely rare finding. A report is made of a 38-year-old woman presenting with a conductive hearing loss due to an aural polyp, which was subsequently diagnosed as a meningioma. Computer-aided tomography (CT) and magnetic resonance imaging (MRI) showed the tumour to be entirely extracranial. Complete tumour excision was achieved using a modified radical mastoidectomy approach.     

MRI of intranasal pleomorphic adenoma

A pleomorphic adenoma in the nasal cavity constitutes a rarity. A 48-year-old woman presented with intranasal pleomorphic adenoma revealed by unilateral continuous obstruction and occasional epistaxis. Nasal endoscopy showed a mucosa-covered and opalescent polypoidal mass arising from the left nasal septum. Unlike cases affecting the major salivary gland, magnetic resonance image (MRI) revealed that the mass showed a low signal intensity on T1-weighted image and a heterogeneous, intermediate signal intensity on T2-weighted image. MRI findings can aid in diagnosis and help exclude the possibility of other neoplasms that occur at this site.     

MRI of intranasal pleomorphic adenoma

A pleomorphic adenoma in the nasal cavity constitutes a rarity. A 48-year-old woman presented with intranasal pleomorphic adenoma revealed by unilateral continuous obstruction and occasional epistaxis. Nasal endoscopy showed a mucosa-covered and opalescent polypoidal mass arising from the left nasal septum. Unlike cases affecting the major salivary gland, magnetic resonance image (MRI) revealed that the mass showed a low signal intensity on T1-weighted image and a heterogeneous, intermediate signal intensity on T2-weighted image. MRI findings can aid in diagnosis and help exclude the possibility of other neoplasms that occur at this site.     

Pleomorphic adenoma of tongue base causing dysphagia and dysphasia

A case of an 87-year-old female with pleomorphic adenoma of the tongue base is reported, with a review of the literature. The tumour had enlarged gradually over a period of three years, causing dysphagia and dysphasia. Computed tomography and magnetic resonance imaging showed that the tumour was exophytic and occupy the oropharynx with little extension into the muscle tissue. The tumour was resected by CO2 laser. Histological examination revealed a benign pleomorphic adenoma that originated from the minor salivary gland of the tongue base.     

A case of pleomorphic adenoma of the parotid gland metastasizing to a mediastinal lymph node

OBJECTIVE: Pleomorphic adenomas are the most common salivary gland tumors and are typically cured with complete surgical excision. There are rare reports, however, in which these histologically benign tumors have inexplicably metastasized to distant sites. We present a case of a patient who presented, 27 years after excision of a parotid pleomorphic adenoma, with a recurrence in the parotid bed and a mediastinal metastasis. STUDY DESIGN: Case report. METHODS: A 43-year-old woman presented with a mass in the right parotid bed 27 years after excision of a pleomorphic adenoma of the parotid. The patient's presentation, workup, and final diagnosis of benign metastasizing pleomorphic adenoma will be discussed, along with a pertinent review of the literature. RESULTS: A diagnosis of recurrent pleomorphic adenoma was made from a fine needle aspiration biopsy of the right parotid mass. On subsequent computed tomographic scan, chest images revealed an incidental left mediastinal mass, which also proved to be a pleomorphic adenoma on computed tomography-guided fine needle aspiration biopsy. The patient underwent a completion parotidectomy and sternotomy with excision of the mediastinal mass. Examination of the pathology specimens confirmed a diagnosis of pleomorphic adenoma in both the parotid bed and the mediastinum. No histologic characteristics of malignancy were seen in either specimen; therefore, a diagnosis of benign metastasizing mixed tumor was rendered. CONCLUSION: Benign metastasizing pleomorphic adenoma is a rare and controversial but distinct clinical entity. Although the definition of the term benign precludes metastatic disease, these tumors do not demonstrate any malignant features yet metastasize to distant sites. It remains to be determined whether these benign metastasizing pleomorphic adenomas are really low-grade salivary malignancies.     

Malignant myoepithelioma arising from recurrent pleomorphic adenoma of the soft palate

Malignant myoepithelioma is a rare salivary gland neoplasm that can arise either de novo or within a pre-existing pleomorphic adenoma. We report a case of malignant myoepithelioma arising from a pleomorphic adenoma of the soft palate. A 70-year-old woman presented in our department with a very large tumor of the soft palate. The patient had a history of a pleomorphic adenoma at the same location that was surgically removed 2 years ago. A second operation, with intraoral excision of the tumor was performed. Histological examination of the recurrence revealed a malignant spindle cell neoplasm with an infiltrative growth pattern and a high mitotic rate. Immunohistochemical investigation confirmed the diagnosis of a low-grade carcinoma of myoepithelial origin with free surgical margins. The patient remains free of disease for more than twelve months after the end of treatment.     

Basal cell adenoma and lymphoepithelial cyst as recurrent tumors of pleomorphic adenoma of the parotid gland

Basal cell adenoma of the parotid gland is a rare benign tumor. Lymphoepithelial cyst of the parotid gland is also a rare benign tumor-like lesion. We report an elderly woman, who previously underwent a removal of pleomorphic adenoma, with multiple masses in the left parotid gland. Physical, MR and intra-operative examination suggested the masses as multiple recurrences of the previous pleomorphic adenoma. A total parotidectomy with facial nerve preservation was performed. The histological examination revealed that the masses were two basal cell adenomas and one lymphoepithelial cyst. These rare tumors should be considered in the differential diagnosis of recurrent masses after a removal of pleomorphic adenoma of the parotid gland.     

区域性切除在腮腺多形性腺瘤手术中的临床应用

目的探讨腮腺多形性腺瘤保留腮腺主导管的区域性切除的可行性。方法对32例腮腺多形性腺瘤进行区域性切除,保留腮腺主导管。结果随访3年～6年无复发,暂时性面神经损伤率为3.1%,术侧腮腺导管唾液引流通畅。结论对腮腺多形性腺瘤,特别是一些小的多形性腺瘤,保留腮腺主导管的区域性切除,能较好地保留残余腮腺的功能及保护面神经。     

Surgical treatment of pleomorphic adenoma of parotid gland in children: a systematic review

ObjectivesTo evaluate surgical approaches and outcomes of pleomorphic adenoma of parotid gland in pediatric population.MethodsA systematic search of PubMed and EMBASE was performed to identify articles reporting surgical treatment of pleomorphic adenoma of the parotid gland in children. A qualitative analysis on results was conducted.ResultsOur search included 23 retrospective studies. The number of children with pleomorphic adenoma underwent surgical treatment were 330. Superficial parotidectomy is the most reported type of surgery, total parotidectomy is performed when the tumor occurs in the deep lobe or in revision surgery and limited excisions in selected case of small lesions mainly in the tail of parotid gland. The rate of recurrences after surgery ranged from 0% to 44.4%. Revision surgery was performed in all cases of recurrences. Considering major complications, transient facial paralysis was observed from 0% to 50% of cases after primary and/or revision surgery; Frey's syndrome from 0% to 14.3% of cases regardless of the type of surgeries.ConclusionSurgical treatment of pleomorphic adenoma of parotid gland in children requires a tailored approach based on the location and extent of the tumor with the main aim of complete excision of the neoplasm. Appropriate surgical approach results in a decrease of recurrence rate and morbidity. Surgical skill is needed to reduce complication rates especially in children.     

Carcinoma ex pleomorphic adenoma mimicking multiple facial nerve schwannoma

Carcinoma ex pleomorphic adenoma (CXPA) is a rare, aggressive, poorly understood malignancy that usually occurs in the salivary glands. CXPA has been reported to account for 3.6% of all salivary neoplasms and 11.7% of salivary malignancies. CXPA not only has a low incidence rate but there are few papers or case reports reporting perineural invasion rates. We report a case of CXPA which occurred in the parotid gland, showing retrograde perineural invasion along the facial nerve back to the internal auditory canal (IAC).     

Pleomorphic adenoma of the submandibular gland: an evolving change in practice following review of a personal case series

We reviewed patients with submandibular gland pleomorphic adenoma treated at a tertiary referral centre in the United Kingdom (1988–2004). Thirty-seven patients were identified, 32 newly diagnosed cases and 5 cases of recurrent disease previously treated elsewhere. The exact pre-operative diagnosis was “unknown” in 76% of the new cases. All cases were surgically excised (41% with extracapsular gland excision and 59% by selective level Ib, IIa and III neck dissection). Temporary marginal mandibular nerve neuropraxia was documented in 25% of cases. All 32 new cases remain clinically tumour free at the time of writing. In patients with recurrent disease, complete microscopic tumour clearance was achieved in three of the five cases, however all remain clinically tumour free. Pleomorphic adenomas of the submandibular gland are uncommon, with good prognosis following complete tumour excision. Recurrent tumours, however, are frequently multi-focal and difficult to excise completely. The adequacy of primary surgery is crucial and supports an approach for a more radical excision primarily by a selective level lb, IIa and III neck dissection; ensuring complete disease clearance for pleomorphic adenoma, avoiding the risks of tumour spillage associated with a limited excision and tumour handling; and removing the primary echelon of lymph nodes at risk of metastasis if the pathology turns out to be malignant.     

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.