[3 rare malignant neoplasms of the ear: ceruminoma, cylindroma, rhabdomyosarcoma].
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A patient with multiple, synchronous, non-familial head and neck paragangliomas is reported. There were three primary neoplasms, a glomus tympanicum and glomus vagale on the right side and a glomus tumour of the carotid body on the left. Such a combination has never been reported previously. The reports of all the series with paragangliomas in the literature, as well as the reports of single cases with multiple tumours during the last three decades, are reviewed. Specific problems in diagnosis and management of multiple glomus tumours are discussed.
Thirteen patients with 14 glomus tumours have been examined by subtraction gadolinium-enhanced magnetic resonance imaging (MRI), with T1-weighted MR sequences before and after intravenous gadolinium-DTPA. To eliminate movement between subtraction pairs, the patient remains in the tunnel of the imager during administration of the contrast medium, and the venepuncture is made into the dorsum of the foot. The effect of the subtraction process is to remove the NMR signal from the final image so that the photographic densities recorded are dependent on the vascularity of the tissue concerned, normal or abnormal. A particular advantage is the removal of fat signal: the low vascularity of adipose tissue ensures that it is recorded as of minimal density. The extent of skull base glomus tumours has been shown optimally by this technique. Subtraction can also help differentiate glomus tympanicum from glomus jugulare lesions, which may be of crucial importance when deciding the surgical approach. In addition to diagnosis, the technique is also important post-operatively, when imaging is needed to show residual or recurrent tumour and to monitor the effects of radiotherapy.
Although sun exposure is believed to be associated causally with cutaneous melanoma, the high incidence on less sun-exposed areas such as the back, as well as on chronically exposed sites such as the face, suggests that the association with sunlight is less straightforward than for other skin cancers. To explain this enigmatic site distribution, a theory of site-dependent susceptibility of melanocytes to malignant transformation is proposed. As possible evidence, all melanomas diagnosed in the state of Queensland, Australia, over a one-year period were surveyed for histologic evidence of benign melanocytic nevus cells adjacent to the melanoma, and analyzed according to anatomic distribution. Results showed a regional variation in the proportion of melanomas with adjacent nevi not explicable by regional variation in nevus density, which suggests that there is a varying susceptibility of nevi to malignant change. Given that nevus cells are equivalent to melanocytes, this finding would support the hypothesis that melanocytes at-large have a differential response to the mitogenic stimulus of sunlight according to anatomic site.
A 56-year-old man had approximately forty apocrine cystadenomas of the face and ears. Clinical examination and light microscopic findings were typical for apocrine cystadenomas. Electron microscopic findings showed an absence of annulate lamellae, thus differing from the only previously reported study. Review of the literature indicates that a large number of apocrine cystadenomas, such as on our patient, has not been reported previously.
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Rhabdomyosarcoma of the head and neck often presents with vague symptoms which mimic other disease conditions. These factors lead to undue delay in the establishment of the correct diagnosis and the delivery of acceptable therapy, including surgery, radiation therapy, and chemotherapy. There is, however, evidence of improved results of treatment of these tumors since the addition of multiple drug chemotherapy to surgery and radiotherapy.
Complete microscopical control of the excision of cancer of the skin is achieved by removing tissues layer by layer and examining the undersurface of each layer by means of frozen sections. If the cancer is extensive and complicated or if it is of a type readily spread over an excisional surface, the tissues are fixed in situ with zinc chloride prior to excision (chemosurgery, fixed tissue technique). If the cancer is not too extensive or complicated, the tissues are excised in the fresh, unfixed state (chemosurgery, fresh tissue technique). Both techniques are highly reliable; for example, in two consecutive series of basal cell carcinomas treated chemosurgically, the five-year rate of cure was 99.3% for the 9,351 lesions removed by the fixed tissue technique and 97% for the 127 lesions removed by the fresh tissue technique.
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We report a rare case of bilateral carcinoma of the ears in a 41-year-old man. The second tumor developed from the left mastoidectomized middle ear cavity eight years after the complete healing of the first tumor, which probably originated in the glands of the right external auditory canal.
We present a rare case of a transitional papilloma of the middle ear, occurring several years after excision of a similar lesion from the nose. There was no continuity between the two lesions suggesting that these tumours can be multicentric in origin.
We present the case of a 65-year-old woman who was found to have a concurrent ipsilateral acoustic neuroma and glomus tympanicum tumor. Both tumors were removed in one operation. The acoustic neuroma was removed via the translabyrinthine approach because of preoperative deafness while the glomus tumor was found only during the operation to remove the neuroma. A preoperative biopsy was not associated with significant bleeding while microscopic examination of the biopsy tissue indicated only chronic infection.
With aging, a different variety of ear diseases becomes more common. External ear disease is often a local reflection of generalized skin problems aggravated by retained water or local manipulation. Hearing loss may be the result of long-standing middle ear disease rather than aging. In this situation, the inconveniences and possible dangers of a chronically infected ear may be resolved with surgical intervention. Nasopharyngeal or lymphomatous disease must be suspected in any geriatric patient manifesting middle ear disease for the first time. Like presbyopia, presbycusis is a normal consequence of aging. It may be severe, but it does not lead to total deafness and equally involves both ears. Early discovery of the condition and institution of hearing rehabilitation methods will minimize the individual's communicative handicap and tendency to withdraw from society. Unilateral or asymmetric sensorineural hearing loss should be thoroughly evaluated for the purpose of identifying a curable and potentially life-threatening disease.
A case is reviewed of bilateral temporal bone metastases from a carcinoma of the uterine cervix. The pertinent literature is reviewed and the temporal bone histology is described.
Two cases of rhabdomyosarcoma, one arising in the parotid gland and the other within the middle ear cavity, are presented. Both cases developed neurological signs of intracranial extension, which, in one, were non-localizing. The angiographic characteristics of these two cases as well as those reported in the literature are discussed. Angiography delineated the extent of tumor involvement and influenced the treatment ports. Isolated reports of long term survivals and some promising preliminary results from more aggressive therapy warrant delineation of tumor extent. To this end angiography is helpful.