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Adenoid cystic carcinoma of the tongue presenting as a hypoglossal nerve

Palsy K. C. Silvester, S. Barnes Department of Oral and Maxillofacial Surgery, The Eastman Dental Hospital, London and Department of Oral and Maxillofacial Surgery, University College Hospital, Dental School, London ._

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SUMMARY. A case of adenoid cystic carcinoma of the tongue is reported. It was unusual in that its initial presentation was that of a twelth cranial nerve palsy of unknown origin. A brief review of the literature of this uncommon condition is presented. _.__. _. ..~..

INTRODUCTION The tongue is a relatively uncommon site for salivary gland neoplasms. In one large study of 2410 cases of all salivary gland tumours, those of the tongue represented only 3.6%, however of these 92% were malignant (Eveson & Cawson, 1985). The adenoid cystic carcinoma is generally slow growing and can be present for up to 20 years before presentation by the patient. Thirty per cent of patients will develop metastasis (Ramsden et al., 1973), the commonest site of which is the lung. Often repeated local recurrences, before metastasis occurs, will be noted. Successful management of the lesion depends on awareness of the variable presentation of the lesion by the clinician, and the variety of the primary sites, together with a knowledge of the natural history of the tumour. Points to be noted, are its slow growth, apparent benignity, yet widespread invasion beyond the visible edge of the tumour, plus the tendency to perineural lymphatic permeation. The case discussed is of a lesion, where the true diagnosis was not made for 5 years after commencement of symptoms. The point of interest is the production by the lesion of a twelfth cranial nerve palsy with no additional external signs, leading to investigation of a possible primary neurological cause, thus delaying treatment.

Fig. 1 -The tongue at presentation at University College

Hospital.

He first noticed difficulty moving the right side of his tongue in 1983. It gradually worsened over the next 2 years. He attended a neurological department in 1985, when he began to notice wasting of the same area. Clinical examination at the time confirmed this and also fasciculation was noted. The remainder of the neurological examination was reported as normal. A CT scan of the brain and a CT scan of the posterior fossa following a myelogram was carried out-both were unremarkable. Vertebral and carotid angiography were contemplated to exclude an aneurysm or a glomerus jugularae tumour in the posterior fossa, but in view of the risks involved in these investigations, it was decided to delay them and await developments. A diagnosis of a right, unilateral twelfth cranial nerve palsy of unknown origin was made. The patient was followed up until October 1987, when he was readmitted for further investigations. His speech and swallowing had started to deteriorate. He complained of biting his tongue on the right side, although sensation of the area was normal on clinical testing. He had difficulty in protruding his tongue; it had a marked deviation to the left when this was attempted. There was no other neurological deficit found. An enhanced CT scan and MRI scan of the brain were normal. Muscle sampling showed no evidence of devervation in arms or legs. The diagnosis of motor neurone disease of the amytrophic

Case report A 43-year-old Caucasian male was referred to the Oral and Maxillofacial Surgery department, at University College Hospital, in early August 1988. The referral was at the patients own request. He complained of a 5-year history of progressive difficulty in moving the right side of his tongue, causing him problems with speech and swallowing. He had over the last 3 months developed pain over the right mandible, radiating down the right side of his neck. The pain was sharp in nature and lasted for several minutes at a time. There were no relieving factors. 122

Adenoid cystic carcinoma of the tongue

123

Fig. 2 - Subpleural nodule noted on CT examination of chest (arrowed).

Fib. 3 - Irregularity of posterior third of tongue as seen on transverse CT of oropharynx.

lateral sclerosis type was suggested. Although there were no other features of this condition, it was felt that they may develop in time. At the time of his attendance at University College Hospital, he was awaiting an admission for nerve conduction studies and a rectal biopsy to exclude amyloidosis. Other relevant medical history was that he contracted syphilis at the age of 20, while in the Merchant Navy. This was treated with procaine penicillin in England. Serology showed a positive TPHA and negative VDRL. He also had a number of petit mal type seizures at the age of 18-usually related to bouts of heavy drinking. He has not had any further attacks since this time and was not on any medication. He smoked 20 cigarettes a day. Examination revealed a thin looking man with obvious dysarthria. No cervical lymphadenopathy was apparent. The right side of the tongue was found to be swollen, firm and indurated, but with normal looking mucosa. There was a marked cleft in the midline. The left side was swollen but soft. There was reduced sensation to pin prick and marked fasciculation was present. Mobility of the tongue was reduced. The appearance of the tongue was not felt to be typical of a wasting disease and a neoplastic lesion was suggested. An incisional biopsy was carried out under local anaesthesia. The specimen showed an unencapsulated salivary gland tumour beneath the covering mucosa, the histopathological appearance being consistent with that of an adenoid cystic carcinoma. A chest X-ray was reported as normal. He was admitted to University College Hospital for an examination under anaesthetic and removal of carious and impacted teeth. The tumour appeared clinically to involve the whole of the tongue, extending posteriorly to the vallecular fold. The fauces appeared normal. There were no other abnormal findings. A CT scan of the region showed a marked mass on the left side of the posterior third of the tongue. It was decided to treat this lesion with radiotherapy and he received 60 Gray to the primary site. He tolerated this well, with only minor mouth soreness. The course of radiotherapy finished in November 1988 and he had been regularly followed up. In March 1989 his

condition had improved considerably. Eating was easier, and his tongue felt much softer. A CT scan of his chest showed two small subpleural nodules which were felt to be either fibrotic in origin or metastases. A further scan is to be carried out to observe any change. DISCUSSION

This presentation of adenoid cystic carcinoma of the tongue is rare. A slowly progressive unilateral hypoglossal nerve palsy, resulting in wasting and fasciculation, led to extensive investigations to exclude intracranial, neoplastic and vascular lesions, and disseminated sclerosis. Motor neurone disease can certainly present as a progressive bulbar palsy, as a result of degeneration of the motor nuclei in the medulla. In this form of the disease bilateral wasting and fibrillation of the tongue are often the most important signs. However this condition is usually fatal within a year after making the diagnosis. Dysarthria and dysphagia are also features of an upper motor neurone lesion (pseudobulbar palsy), as well as extrapyramidal and cerebellar lesions and myasthenia gravis. In this patient however, there was no other evidence of these conditions and investigations were negative. It was only when signs of fixation and induration of the right side of the tongue became apparent that a neoplastic lesion was suspected. It is interesting how the midline fibrous septum of the tongue appeared to act as a barrier to the spread of the lesion. In one survey of 55 cases of minor salivary gland tumours of the tongue (Goldblatt & Ellis, 1987), the most common presentation was dysphagia, followed by pain. This was also supported in a study by De Vries et al. (1987) of 22 cases of posterior third lesions. In a review of 30 cases of adenoid cystic carcinoma

124 British Journal of Oral and Maxillofacial Surgery

at all sites, it was felt that aggressive treatment, by either surgery or radiotherapy, enhanced patient comfort and survival (Spiro et al., 1974). However Kessler et al. (1985) concluded that for malignant tumours in the posterior third of the tongue, radiotherapy was not adequate, that whilst optimal treatment for these tumours remained to be established combination therapy was recommended. De Vries et al. (1987) support this conclusion in their series. In the case reported, radiotherapy alone was the treatment modality chosen, as the lesion was felt to be unresectable. Spiro et al. (1974) in their review of 218 cases of adenoid cystic carcinoma at all sites found that 31% were alive without evidence of disease after 5 years. This had fallen to 16% after 10 years. With lesions of the tongue this figure was 29% after 10 years. Significant factors enhancing survival were, age less than 50 years, the absence of pain as a symptom and the size of the primary lesion. The prognosis for this patient is uncertain. The case does however, give weight to the maxim that ‘if in doubt, biopsy!‘. Acknowledgements We would like to thank Professor M. Harris for his permission to report this case and Dr Khee Wee Lee of the Department of Oral Pathology, Eastman Dental Institute, for preparing and interpreting the histological material.

& Mandell-Brown, M. (1987). Base of tongue salivary gland tumours. Journal of Head and Neck Surgery, 9,329. Eveson, J. W. & Cawson, R. A. (1985). Salivary gland tumours. A review of 2410 cases with particular reference to histological types, site, age and sex distribution. Journal of Patholonv. 146. 51. Goldblatt, LTI. & Ellis, G. L. (1987). Salivary gland tumours of the tongue. Analysis of 55 new cases and review of the literature. Cancer, 60,74. Kessler, P., Michel, R. & Calcaterra, T. (1985). Malignant salivary gland tumours of the base of the tongue. Archives of Otolaryngology,

Journal of Surgery, 43, 102.

Spiro, R. H., Huvos, A. G. & Strong, E. W. (1974). Adenoid cystic carcinoma of salivary origin. A clinicopathological study of 242 cases. American Journal of Surgery, 128,512.

The Authors K. C. Silvester BDS, MB, BS, FDS, FRCS --

Department of Oral and Maxillofacial Surgery The Eastman Dental Hosoital 256 Grays Inn Road London WClY 8LD S. Barnes BDS, FDS Department of Oral and Maxillofacial Surgery University College Hospital Dental School Mortimer Market London WClE 6AU Correspondence

References De Vries, E. J., Johnson, J. T. K., Myers, E. N., Barnes, E. L.

111,664.

Ramsden, D., Sheridan, B. F., Newton, N. C. & De Wilde, F. W. (1973). Adenoid cystic carcinoma of the head and neck. A report of 30 cases. Australia and New Zealand

and address for offprints to Mr K. C. Silvester

Manuscript received 10 November 1989 Accepted 18 December 1989

Adenoid cystic carcinoma of the tongue presenting as a hypoglossal nerve palsy.

A case of adenoid cystic carcinoma of the tongue is reported. It was unusual in that its initial presentation was that of a twelfth cranial nerve pals...
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