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Neoplasms of the External Ear
Abstract
The external ear is a specialised appendage of the skin, so that its neoplasms are most frequently those derived from skin. Bony neoplasms also occur, as would be expected from the presence of bone nearby, but the cartilage of the ear seems to have no neoplastic propensity. The following account will deal only with those tumours that have a predilection for that region and those that pose special diagnostic histological and clinical problems.
... Exostosis are multiple, bilateral, appear as wide based and smooth lesions of the EAC and are usually considered to be a reactive condition secondary to multiple cold-water immersions or recurrent otitis externa. 9,10 There are limited histopathological studies done for osteoma and exostosis as these lesions are drilled during surgical removal. Exostosis are concentric, dense layers of subperiosteal bone with abundant osteocytes, lacking fibrovascular channels covered with periosteum and squamous epithelium. 1 Studies by Fenton et al suggested that fibrovascular pathway is a characteristic feature of osteoma, and could also be found in exostosis also and so it could not be differentiated histopathologically. 2 In some cases they might also occur together. ...
p>External auditory canal (EAC) osteomas are rare, solitary benign unilateral lesion. Small lesions are incidentally diagnosed and larger lesions produce symptoms of pain, ear discharge and defective hearing. Diagnosis is made based on a combination of clinical history and examination, radiographic imaging, and histopathology. They are usually confused with exostosis which are multiple, bilateral, smooth-bordered, broad-based lesions without deep extension. We present a case of 21-year-old male patient who came to our outpatient department, with complaints of left ear blocking sensation for past 6 months. On evaluation, it was diagnosed as left EAC osteoma and the same was excised via post-auricular approach. This case is being presented for its rarity</p
... They are also known as cancellous or osteoid osteomas. Osteomas are composed of lamellar bone, although osteomas of the ear canal have been reported to have a thin layer of woven bone on the surfaces of the lamellar bone [2]. Compact osteomas have a wider base and are very slow growing whereas spongy osteomas are more likely to be pedunculated and grow relatively faster. ...
O steomas are benign tumours of the lamellar bones. In routine ENT practice they are commonly seen as dense radio opaque shadows arising from within the paranasal sinuses. Osteoma of the temporal bone occurs infrequently, and when they occur, are seen most common in the external ear canal. Osteoma originating from the middle ear is very rare. That from the mastoid are rarer. We document such a rare presentation of osteoma arising from the mastoid.
Malignant melanoma of the ear, other than the pinna, rarely occurs. This is the first report of a melanoma confined to the external auditory canal. The case presented is of a 75-year-old man with a malignant melanoma confined to the external auditory canal. Excision of the tumor and surrounding lymphatics was accomplished with a lateral temporal bone resection, superficial parotidectomy, and selective neck dissection. The patient initially refused postoperative radiation therapy. Histologic evaluation documented a 17-mm thick malignant melanoma. All margins were clear and none of the nodes was positive. Eight months later, he returned with local, regional, and distant recurrence. Despite attempts at salvage with external radiation, brachytherapy, and chemotherapy, he died 13 months postoperatively with widespread disease. Melanoma confined to the external auditory canal has not been previously reported. A 17-mm thick melanoma carries a dismal prognosis. Despite initial negative surgical margins and lack of regional metastases, this patient developed a rapid, widespread pattern of recurrence.
Verrucous carcinoma is a differentiated variant of squamous cell carcinoma and may present diagnostic difficulties as it may be erroneously diagnosed as squamous papilloma. In this study, the sizes of the intermediate cells in these two conditions were measured by means of morphometric analysis. Biopsies from 28 patients with verrucous carcinoma, 25 patients with squamous papilloma, and ten squamous cell carcinomas were analysed. A significant difference was shown (P less than 0.001) by an uncorrelated t-test between verrucous carcinoma and squamous papilloma. The former had a mean cell area of 373 microns 2 and the latter 184 microns 2. Squamous cell carcinomas differed from the other two neoplasms by their large range of cell areas both within and between cases. Thus, image analysis can be of diagnostic help in cases where no firm initial histopathological diagnosis can be obtained. The diagnosis should be made on morphological grounds, but a mean cell area greater than 300 microns 2 supports a diagnosis of verrucous carcinoma whereas an area less than 250 microns 2 supports a diagnosis of squamous papilloma.
A consecutive series of 111 patients treated between 1964 and 1989 with primary cutaneous malignant melanoma of the head and neck and histologically proven regional metastases was reviewed to determine the patterns of nodal metastases. The primary sites were grouped in the following categories: face (34), anterior scalp (25), anterior neck (16), posterior scalp (15), ear (11), and posterior neck (10). All patients underwent radical neck dissection: 80 of the procedures were therapeutic and 31 elective. A total of 106 specimens were positive for metastases. Thirty-three of the 57 patients undergoing parotidectomy had positive results for metastases (14 of 20 in therapeutic parotidectomies and 19 of 37 in elective parotidectomies). Based on the incidence of involvement of the parotid gland and the patterns of lymph node metastases from levels I through V, three observations are made. Patients undergoing regional lymphadenectomy for primary melanomas on the ear, face, and anterior scalp should be considered for parotidectomy. The use of selective limited neck dissection for elective regional lymphadenectomy appears justified based on the location of the primary site. In patients undergoing therapeutic neck dissection, a complete neck dissection should be performed due to the unpredictable distribution of lymph node metastases to the cervical lymph nodes.
Patients with malignant lymphoma may have cutaneous and subcutaneous involvement that exhibits a striking symmetry about the coronal axis. The symmetry of these lesions may be caused by site-specific migration from the circulation, preferential proliferation by lymphocytes of the neoplastic clones at defined anatomical sites, or both mechanisms. Similar behaviour by benign lymphocytes may explain the symmetry and selective anatomical distribution of lesions in other skin diseases.
Involvement of the temporal bone by eosinophilic granuloma is rare. Two cases are presented and their clinical manifestations, histopathological findings and treatment procedures discussed.
Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood, involves the temporal bone in approximately 7% of reported cases. Until recently, the outcome of this disease was always fatal. The recent Intergroup Rhabdomyosarcoma Study Group (IRS-I) reported on the efficacy of multimodality therapy consisting of multiagent chemotherapy, radiation, and surgical resection when indicated.
Twelve patients with rhabdomyosarcoma involving the temporal bone were treated between 1966 and 1988. Three patients were treated according to the IRS-I protocols and the remaining nine patients received various combinations of treatment modalities. Ten patients succumbed to their disease, most with distant metastases or intracranial extension. Two patients are alive; one at 5½ years and one at 19 years.
It is apparent that, although survival for rhabdomyosarcoma in general has improved with the use of IRS-I protocols, prognosis remains poor for disease involving the temporal bone and other parameningeal sites.
Considerable confusion exists regarding the nature of benign dermal cylindroma of the external auditory canal. In the past, authors have used the term cylindroma to refer to malignant lesions such as adenoid cystic carcinoma. Benign dermal cylindroma is a specific clinical entity consisting of benign tumors arising from the epidermal appendages; however, whether they are apocrine- or eccrine-derived is not conclusively known. Six per cent are reported to be related to the ear. Histologically they are characterized by cords of basophilic cells, hyaline material, and cystic duct-like structures. Rarely, cases of malignant degeneration are reported. However, for the most part, these lesions are benign and should be treated with complete local excision. Radiotherapy may be useful in treating massive lesions not amenable to surgery.
The apparent rarity of verrucous carcinomas of the ear is reflected by the fact that only three well-documented cases have previously appeared in the literature. However, in other instances the diagnosis of verrucous carcinoma may have been missed because of the unfamiliarity of many clinicians and pathologists with the morphologic characteristics of these lesions. We describe the first case of a verrucous carcinoma confined to the external auditory canal in which a positive diagnosis was only made histologically after local recurrence of what was initially diagnosed as a senile keratosis. The difficulties inherent in diagnosing this lesion and the necessity of close cooperation between the surgeon and pathologist if a diagnostic error is to be avoided are emphasized. Careful follow-up of all warty lesions of the external auditory canal and middle ear cleft is recommended.
Light and electron microscopic features of a case of ceruminous adenocarcinoma are presented and compared to normal ceruminous glands. A survey of the literature disclosed 4 types of ceruminoma: adenoma, adenocarcinoma, adenoid-cystic carcinoma, and mixed tumor. The pathogenesis and histologic features of each type are discussed. Because biologic activity varies with the histologic pattern, and because of the greater frequency of malignancy as compared to other cutaneous apocrine tumors, it is recommended that the generic term “ceruminoma” be retained for apocrine tumors arising in the external auditory canal.
Fibrous dysplasia is a fairly common, localized misdifferentiation of the bone-forming mesenchyme affecting a single or many bones, in which skeletal aberrations represent the cardinal feature, but in which certain endocrinopathies, abnormal pigmentation of skin and mucous membrane, and occasionally other abnormalities form part of the entire disease process. The craniofacial skeleton is one of its predilective sites and therefore the temporal bone may become involved. In such instances the disease manifests itself with 1) progressive loss of hearing, 2) increasing obliteration of the external ear canal, and 3) enlargement and distortion of the temporal bone. The first part of this communication is concerned with a review of the clinical and pathological aspects of the disease in general. The second part is concerned with a discussion of the clinical manifestations in the temporal bone, based upon analysis of the entire literature and the authors' personal experience. The indications for surgical intervention include 1) maintenance of a normal outer ear canal, 2) preservation of cochlear and vestibular function, and 3) prevention of secondary complications.
• Cutaneous carcinoma of the auricular and periauricular region can be diagnosed early and often cured; however, many of these tumors become difficult to treat because of local spread and regional metastasis. We have treated 122 patients with 145 cutaneous carcinomas of the auricular and periauricular region with either Mohs' histographic surgery or conventional excision with total microscopic marginal control. Inherent in these treatment modalities is the construction of detailed serial tissue maps for each tumor. From these maps, a three-dimensional composite for each tumor is derived, giving information about the direction of local spread of the tumors. The tumors have been found to extend locally through soft tissue into perichondrial, periosteal, and neurovascular planes, allowing for more distant occult spread. This knowledge of such patterns of tumor spread can be useful in planning treatment.
(Arch Otolaryngol 106:692-696, 1980)
This paper reports on 16 cases of primary malignant melanoma of the external ear and the patients were followed up for two to 116 months. Fourteen patients had surgical excision with wide margins as initial treatment, whilst two had an amputation performed. Eleven patients had no recurrences, two died of other diseases, and four died of malignant melanoma. The five patients who developed recurrences received further surgery and two also radiotherapy.
Seven of the cases were histologically of the nodular type, six were superficial spreading, two were in situ melanoma, and one was a lentigo maligna. The thickness ranged from 0.15 to 11.5 mm. Classification according to Clark et al. (1969) revaled that as many as nine cases were Clark level IV or more. Immunostaining with PCNA yielded strong positivity in all cases, however, statistical analysis did not reveal any differences that could be correlated to the prognosis. Estimation of the vascularization at the base of the tumours by means of immunostaining with CD31 did not reveal any significant differences either.
We concluded that in our material the thickness of the tumour is of greater prognostic value than the estimation of proliferation by PCNA and vascularization by CD31. The value of PCNA and CD31 as possible prognostic parameters needs to be evaluated in a larger series. It is emphasized that malignant melanoma of the external ear is a highly malignant tumour, and that four of our 16 patients died of their disease, three of them within a year after diagnosis. Malignant melanoma of the external ear is readily inspected and thereby an early diagnosis should be possible.
monostotic. Although most patients had a conductive hearing loss, 17% of patients demonstrated profound sensorineural hearing loss ascribable to the lesion, and facial nerve sequelae were noted in nearly 10% of cases. Cholesteatoma complicated almost 40% of cases, usually in the form of a canal cholesteatoma. Ten new cases of temporal bone fibrous dysplasia are described not only to further clarify the spectrum of otologic sequelae but also to help illustrate available treatment options. In addition, this report documents, in three new cases, the previously undescribed progression of conductive hearing loss to profound sensorineural deafness secondary to fibrous dysplasia.
To differentiate a novel type of benign circumscribed bone lesion of the external auditory canal from those described previously, such as exostoses and osteomas.
Information was obtained from computed tomography (CT) images, surgical findings, and pathologic study.
Five patients (26 to 82 years old) who presented a hard, round, unilateral, skin-covered mass occluding the external auditory canal to varying degrees were studied. A CT study carried out before resection of the lesions by curettage disclosed the absence of a bony connection to the underlying structures. All the tissue specimens underwent pathologic study.
CT and surgical findings demonstrated the absence of a connective pedicle. The pathologic findings showed lesions consisting of an osteoma-like bone formation with sparse osteoblastic areas; mature lamellar bone was observed in three cases, bone marrow containing adipose tissue and hematopoietic remnants in two, and a dense, collagenous stroma in another. They all showed irregular trabeculae, bordered by osteoid osteoblasts. In no case was there evidence of a relationship to the cartilaginous tissue or to the bony structures of the external auditory canal.
The data obtained from the clinical, CT, surgical, and pathologic findings suggest the existence of a lesion unlike those previously known, possibly related to ossifying reactions in other parts of the organism.
Pilomatrix carcinoma, the malignant counterpart of pilomatrixoma, is rare, with only 55 cases reported, and only four cases with visceral metastases described in the literature. Here we present a case report and a literature review on this rare tumour. A 74-year-old male with a pilomatrix carcinoma from the left temporal region presented in July 1996 and the tumour was excised. One month after diagnosis, metastases to both lungs and to a regional lymph node were found and histologically verified. The patient also developed metastases in the abdomen, back and thoracic spine. The latter resulted in spinal cord compression and paraplegia. Despite systemic chemotherapy with intravenous cisplatin and 5-fluorouracil and localised radiotherapy to the thoracic spine, progression and deterioration led to death within 3 months from time of diagnosis. Pilomatrix carcinomas are usually indolent. In our patient, however, the malignant disease progressed rapidly and it appeared to be resistant to both chemotherapy and irradiation.
Chapter 80 The ear In: Damjanov I, Linder J. Anderson’s pathology, 10th edn
- L Michaels
Pneumocystis carinii otitis media
- M A Smith
- L S Hirschfield
- G Zahtz
- MA Smith
Tumours of ceruminous glands
- C V Wetli
- V Pardo
- M Millard
- CV Wetli
Fibrous dysplasia of the temporal bone: ten new cases demonstrating the spectrum of otologic sequelae
- C A Megerian
- R A Sofferman
- M J Mckenna
- CA Megerian
Cutaneous carcinoma of the auricular and preauricular region
- P H Bailin
- H L Levine
- B G Wood
- PH Bailin