Neurotologic management of intracranial epidermoid tumors

OBJECTIVES: Epidermoid cysts are the most common intracranial embryonal tumor, although they account for only 1% of all intracranial tumors. Epidermoids often spread into several intracranial compartments. Thorough preoperative surgical planning is imperative for safe epidermoid removal. This paper discusses the neurotologic management of intracranial epidermoid cysts STUDY DESIGN: Retrospective chart review. METHODS: A database search revealed 10 patients with diagnosis of intracranial epidermoid cysts between January 1, 1971 and December 31, 2003 at our institution. RESULTS: Six males and four females with ages ranging from 18 to 54 years of age underwent surgery between September 1, 1971 and November 4, 2003. The average tumor size was 3.9 cm; six originated in the cerebellopontine angle and four in the petrous apex. Six patients had a translabyrinthine approach to the tumor, two with additional transcochlear exposure. Two patients had tumors removed by way of the middle fossa approach and one through the suboccipital approach. Multiple cranial nerves were involved by tumor in all patients, including nerves III through XI. The internal carotid artery was involved by tumor in four patients. Multiple cranial nerve deficits were seen preoperatively, and facial weakness was the most common new deficit postoperatively. Eight patients required intradural access for complete tumor removal. Seven had complete tumor removal. Headaches were the most common complication. One patient had seizures postoperatively, and another had a malignant epidermoid, which resulted in death. CONCLUSIONS: Intracranial epidermoid cysts require complex surgical planning. They involve multiple cranial nerves and vascular structures. Complete resection is frequently possible with minimal new cranial nerve deficits.     

Eustachian tube teratoma and its terminological correctness.

Teratomas are germ cell tumours usually found in the young and are characteristically composed of tissue foreign to the place where they arise. Two teratomas of the middle ear were first described in 1866 and since then, and to the best of our knowledge, 19 additional cases have been reported in the literature under different terms such as hairy congenital polyps, epidermoid cysts, dermoid cysts, hamartoma, cutaneous teratoma and teratomas. The difficulty in classifying germ cell tumours may explain the different terminologies encountered in the literature. The authors describe a case of teratoma of the eustachian tube presenting as a mass in the middle and external ear of a 10-week-old girl. Using this case, a review of the literature is performed in light of the new classification of germ cell tumours proposed by the World Health Organization (WHO). It is of note that most of the teratomas that present in the middle ear arise from, or involve, the eustachian tube.     

Primary sub-mandibular gland tumours: experience based on 68 cases

Sub-mandibulary gland tumours are less common than tumours of the parotid and pose many clinical and therapeutic challengers. OBJECTIVE: We report our experience of sub-mandibular tumours and review the literature. PATIENTS AND METHODS: Retrospective studies of sub-mandibular gland tumours presenting to our department between 1986 and 2000. RESULTS: 68 cases were reviewed comprising 37 benign and 31 malignant tumours (15 females and 33 males). Average age of patient was 46 years and all presented with a sub-mandibular swelling. Clinical suspicion of malignancy was associated with symptom of pain, cervical adenopathy, nerve palsy, skin and/or bone invasion. CAT Scans were performedd to assess tumour extent / invasion. Definitive diagnosis was by complete excision and pathological examination. Pleomorphic adenoma (n= 32) were the most frequent benign tumours. For malignant lesions (n= 31) the most frequent were: Adenoid cystic carcinoma (n= 10), epidermoid carcinoma (n= 5), adenocarcinoma (n= 5), mucoepidermoid carcinoma (n= 3), malignant non Hodgkinien lymphoma (n= 5). Treatment was by total surgical excision of the submandibular gland for the begnin tumours. For the malignant lesions it was associated acording to their extension with other anatomical region or in case of adenopathy with neck dissection. Radiotherapy was performed in 24 cases and chemotherapy in 10 cases. CONCLUSION: Malignity in sub-mandibular gland tumours is more frequent than in the parotid gland. Any delay in diagnosis or inappropriate management may result in a poor prognosis for the patient.     

Intracranial facial nerve reconstruction.

Surgery for tumours of the cerebellopontine angle (CPA) or the internal auditory canal (IAC) is sometimes complicated by the severing of the seventh nerve. Many procedures are available for facial reanimation. Among these, primary intracranial VII-VII reanastomosis is considered as the method of choice. This series reviews all the cases of primary intracranial facial nerve reconstruction that we have performed either directly or with the use of a nerve graft interposition. Functional results are analyzed according to the House-Brackmann grading scale. The advantages and benefits of this technique are discussed as compared with other methods of facial reanimation, namely, the hypoglossal-facial anastomosis.     

Malignant neoplasms of the nasal cavity and paranasal sinuses. Analysis of 216 cases of malignant neoplasms of nasal cavity and paranasal sinuses

This study is a retrospective analysis of 216 cases of malignant tumours of the nasal cavity and paranasal sinuses, treated from 1965 to 1980. The mean age at diagnosis was 59 years, 70 per cent were men and 30 per cent women. There were 121 (56%) epidermoid carcinomas, and 31 (14%) adenocarcinomas. Three-year and five-year actuarial survival rate was 40 and 33 per cent respectively for epidermoid carcinomas, 72 and 50 per cent respectively for adenocarcinomas. Recurrences occurred in 46 per cent of epidermoid carcinomas and 42 per cent of adenocarcinomas. Early diagnosis is important to improve the survival rate, and radical primary treatment should be carried out to reduce the rate of recurrence.     

Outcome of using magnetic resonance imaging as an initial screen to exclude vestibular schwannoma in patients presenting with unilateral tinnitus

The improved cost-effectiveness of gadolinium-enhanced magnetic resonance imaging (MRIg) as a diagnostic tool for vestibular schwannoma has resulted in smaller tumours being diagnosed. There has been a change in the clinical presentation of these tumours and up to four per cent may present with unilateral tinnitus. The limitation of auditory brain stem response (ABR) as a screening tool that detects small tumours is recognized and there is a strong argument for using MRIg as the initial investigation. Various screening guidelines have been proposed, some include submitting patients with unilateral tinnitus for MRIg. This report describes the findings in a group of 174 patients presenting with unilateral tinnitus who underwent MRIg as part of a guideline-directed screen to exclude vestibular schwannoma. Five patients had a cause for their tinnitus diagnosed, one a vestibular schwannoma. Two had intracranial aneurysms and another three had pathology revealed that merited onward referral. A further nine patients had incidental findings that neither accounted for their symptoms nor needed further investigation or referral. The rationale for screening these patients with MRIg is discussed.     

Undifferentiated epithelioma of the parotid and cystadenolymphoma. Apropos of a case

A case of undifferentiated epithelioma of the left parotid gland occurred 2 years after the removal of a cystadenolymphoma in the right parotid gland. The relationship of these two tumours was supported by several statements: the immunohistochemical study demonstrated in the epithelial cells of the 2 neoplasms the existence of ACE, usually thought as characteristic of cystadenolymphoma. Besides, it showed a high number of plasma cells secreting IgA in the stroma of the 2 tumours, the ultrastructural study performed on the epithelioma also demonstrated some similarities between epitheliomatous cells and epithelial cells of cystadenolymphomas (outlined epidermoid or glandular differentiation, numerous mitochondria). A peculiar, perhaps immunological mechanism could induce the cancerization, according to the theories used to explain the histogenesis of cystadenolymphoma.     

Kaposi's sarcoma of the trachea

The most common tracheal neoplasms are epidermoid carcinomas, papillomas and cylindromas. Kaposi's sarcoma, confined primarily to the trachea, is one of the rarest tumours. The clinical and histological picture of a case of Kaposi's sarcoma of the trachea in a young, pregnant woman, presenting with severe airway obstruction, is described here. The emergency tracheoscopy dislodged a mass from the trachea, which turned out to be a Kaposi's sarcoma on histology. Although Kaposi's sarcoma is one of the manifestations of AIDS, this patient had neither any underlying immunodeficiency nor any skin, visceral or lymphatic lesions.     

Ethmoidal epidermoid carcinoma showing as cluster headache. Report of a case and literature review

We report the case of a 54 years old male diagnosed as ethmoidal epidermoid carcinoma which, after almost 1 year--asymptomatic period, debuted with facial pain (cluster headache) without another associated clinic. Sino-nasal malignant tumours represent about 5%-6% in all ENT carcinomas and they use to be of later diagnosis, with high probability of local recurrence and invasiveness on structures like orbit and skull base. The main treatment for ethmoidal squamous carcinomas is surgery and depending on staging can require radiotherapy and/or chemotherapy. A bibliographic review is done at this respect.     

Extracranial meningioma: a rare differential diagnosis of an intranasal tumor

Meningiomas are common intracranial neoplasms, making up 19.5 per cent of all intracranial tumours. Meningiomas occurring outside the central nervous system are uncommon. In this report should be presented an extradural meningioma of the left nasal cavity, which was diagnosticated in an 78-year-old female. These slowly growing non-infiltrating tumours have no characteristical clinical symptoms, they cause nasal obstruction and sinusitis. The origin of these tumours is uncertain. Obviously intranasal meningiomas take their origin from the arachnoideal meningocytes which accompany the olfactory filaments through the lamina cribrosa to the extradural site.     

Recurring dermoid cyst of rare localization

Dermoid and epidermoid cysts are benign tumours developing from dystonic elements of the epidermal tissue during embryogenesis. They occur in different maxillofacial regions but are especially common at the bottom of the mouth and in the submandibular region; fewer cysts are located in the paotid region. A case of parotid dermoid cyst is reported.     

Glomus jugulare tumour with metastases to cervical lymph nodes

Glomus jugulare tumours are classically described as benign tumours with a long time course often measured in decades. Although these tumours may be locally invasive, most cases are histologically benign and metastases are rare. The case of a malignant glomus jugulare tumour with a particularly aggressive pattern of spread is presented. At the time of surgery, which was within 12 months of the development of symptoms, intracranial spread and metastasis to cervical lymph nodes had already occurred, demonstrating that glomus jugulare tumours are not always benign.     

Subtemporal-preauricular approach in the management of infratemporal and nasopharyngeal tumours

OBJECTIVES: To evaluate the indication, advantages, and results of the subtemporal-preauricular approach in the resection of tumours arising in the infratemporal fossa and related structures of the lateral cranial base. DESIGN: Retrospective analysis of patients treated by the subtemporal-preauricular approach to resect nasopharyngeal and infratemporal tumours. SETTING: Tertiary hospital. METHODS: Forty patients with lateral skull base lesions were managed with the subtemporal-preauricular approach. There were 19 benign lesions and 21 malignant tumours, the most common being nasopharyngeal carcinomas, juvenile angiofibromas, and adenoid-cystic carcinomas. Most of the tumours were at an advanced stage, with skull base involvement in 13 benign tumours and in 14 malignant ones. RESULTS: Major complications occurred in 6 (14%) patients, and there were 2 (5%) postoperative deaths. All 19 patients with benign tumours are alive, although 5 of them (26%) presented with residual or recurrent disease. The 5-year estimated survival rate of the 21 patients with malignant neoplasms was 39%. CONCLUSIONS: The subtemporal-preauricular approach is a safe and versatile approach to the infratemporal fossa and neighbouring areas of the lateral cranial base, offering satisfactory oncologic results in the absence of intracranial involvement.     

External ear melanoma

Cutaneous melanomas are the tumours that have increased more their incidence in the last fifty years. Melanomas arising from the external auditory canal are extraordinariously unfrequent. These tumours show an aggressive and silent behaviour, and due to this the diagnosis is frequently made in an advanced stage. A male with a malignant melanoma arising from his left external auditory canal was attended in our department, suspecting an epidermoid carcinoma. The clinical findings and the extension of the lesion required a lateral temporal bone resection, parotidectomy and neck dissection to achieve a total resection. We present a review of the literature about this entity and an analysis of the incidence, significance of the lymph node metastases and value of the elective neck dissection.     

Epidermoid carcinoma involving the parotid gland and orbital region: case report of a rare entity

Epidermoid carcinoma (also known as squamous cell carcinoma) of the parotid gland is more prevalent among the elderly, as are other head and neck tumors. In most cases it is observed either as a nodal metastasis of a primary tumor that drains into the parotid area or as a direct extension of a tumor that has originated in the upper dermis. Primary epidermoid carcinomas arising directly from the parotid gland are extremely rare. Similarly, secondary invasion of the orbital region by a neighboring tumor is more common than primary epidermoid carcinoma of the orbit, which is also rare. In this article, we discuss the diagnosis and treatment of a rare case of epidermoid carcinoma simultaneously invading the parotid gland and the orbit.     

Radiotherapy in carcinoma of the middle ear and auditory canal.

In the decade 1961 to 1970, 89 patients with epidermoid carcinoma of the middle ear or auditory canal were seen. Seventy-eight were treated with 5-year survival figures of 22 per cent for middle ear, 42 per cent for auditory canal and 31 per cent for the total group. The majority of failures occurred within 18 months of diagnosis. The author suggests that radiotherapy and surgery must be combined to improve results. Eleven patients in the series developed second primary tumours.     

Histology of submandibular gland tumours, 10 years' experience

Introduction and objectivesThere are few reports focused on neoplasms in the submandibular gland because they are uncommon and are frequently grouped with the other salivary glands tumours. In the classical studies, the proportion of malignancy reported in these cases is around 50%.ObjectivesDetermining the proportion of malignancy, the most frequent histological types, the gender distribution and average age at diagnosis in patients with submandibular gland neoplasms who were treated in our hospital from 2000 year to 2010.MethodsA retrospective review of our department database of the patients who underwent surgery for submandibular gland neoplasm in a ten-year period was performed.ResultsTwenty-two patient records were included, in which 19 (86%) patients presented benign disease and 3 were of a malignant type (adenocarcinoma, well-differentiated epidermoid carcinoma, follicular lymphoma). Two of the 3 cases of malignant neoplasms were in men.ConclusionsA greater proportion of benign neoplasm was found in submandibular tumours. The most frequent benign tumour is the pleomorphic adenoma. Women are more commonly affected (76%). Benign tumours appear in younger patients than do malignant ones.     

Verrucous carcinoma of the oral mucosa]

Verrucous carcinoma is a distinct variant of differentiated squamous cell carcinoma with low-grade malignancy, slow growth and no metastatic potential. Although it has been reported at extraoral sites, the tumor is mostly found in the oral mucosa. Clinically warty tumours with a grey-white, deeply cleaved surface are detected. The special feature of this tumour type is that it appears macroscopically malignant but histologically benign. The diagnosis of verrucous carcinoma may be difficult, since it is often lumped with verrucous hyperplasia or epidermoid carcinoma. In our study the clinical and histological characteristics of 32 cases of verrucous carcinoma of the oral cavity were investigated. The most common site was the mandibular buccal sulcus. The average age amount to 69 years. Adequate surgical excision is considered the treatment of choice. Because of the lack of metastatic spread a systematic treatment of the regional lymph nodes ist not mandatory. The prognosis of verrucous carcinoma is fairly good.     

Melanoma of sphenoid sinus. Case presentation and review of literature

The primary malignant melanomas of sphenoidal sinus are very uncommon tumours. We are reporting a clinical case of a 56 years-old male who was sent from the Neurology Department with ocular pain and headache of several months evolution and without personal history. Exploration revealed a VIth palsy, diplopia and ptosis. The complementary imaging tests (CT and MR) showed and expansive tumour of the sphenoidal sinus involving the right cavenous sinus. A F.E.S.S. with biopsy was performed to confirm the diagnosis. Our patient was treated by Radiotherapy and after a 4 year follow-up period he is still alive. We have carried to a bibliographic review of this pathology whose differential diagnosis must be done with epidermoid carcinomas, adenocarcinomas and lymphomas on that location.