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Myokymia (fasciculation) of the tongue as a unique presentation of mucoepidermoid carcinoma
Int. J. Oral Maxillofac. Surg. 2007; 36: 79–81 doi:10.1016/j.ijom.2006.05.004, available online at http://www.sciencedirect.com
Case Report Clinical Pathology
Myokymia (fasciculation) of the tongue as a unique presentation of mucoepidermoid carcinoma
Karen V. Andrews, John W. Evesona Division of Oral Medicine, Pathology and Microbiology, Bristol Dental Hospital and School, Bristol, UK
Karen V. Andrews, John W. Eveson: Myokymia (fasciculation) of the tongue as a unique presentation of mucoepidermoid carcinoma. Int. J. Oral Maxillofac. Surg. 2007; 36: 79–81. # 2006 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Neural invasion is a relatively common feature of squamous cell carcinomas of the head and neck and malignant salivary gland tumours. The symptoms depend on the location and the particular nerves involved, and include pain, anaesthesia, paraesthesia and cranial nerve palsy. The present case appears to be unique. A mucoepidermoid carcinoma showed evidence of neural involvement and presented with nerve stimulation inducing myokymia, rather than nerve destruction, which is the usual consequence of tumoral nerve invasion.
A 38-year-old man was referred with ‘a lump on the right side of the tongue that appeared to be pulsing involuntarily’. The patient described a swelling in his tongue that had been present for 2 months and was gradually increasing in size. He also reported that the lesion appeared to be moving involuntarily and was causing him progressive difficulties with speech. The patient’s medical history was noncontributory. Extraoral examination showed no abnormalities. No mass could be detected in the tongue although there was striking myokymia (fasciculation) affecting the right side that was severe enough to cause difficulties with speech. Irregular twitching of groups of muscle fibres gave the overlying mucosa a rippling appearance. Pronouncing ‘ing’ and ‘g’ at the end of words was notably impaired. There was no loss of sensory 0901-5027/01079 + 03 $30.00/0
function and no other cranial nerve deficits were found. The results of routine haematological investigations were normal. A magnetic resonance imaging (MRI) scan performed a month later showed no significant intracranial lesions. Apart from minimal mucosal changes in the paranasal sinuses, the images were reported as unremarkable. The patient was referred to a neurologist and was seen 6 months after his initial symptoms. There was evident wasting and conspicuous myokymia of the tongue, largely confined to the right hand side. No other neurological abnormalities were detected and no lymph nodes or masses in the neck could be palpated. At this appointment the patient also mentioned ‘fullness’ of the left ear and a mild reduction in hearing. A working diagnosis of early bulbar palsy was given, despite the
Accepted for publication 26 May 2006 Available online 11 September 2006
patient’s relative youth. He was referred for electromyography studies and to an ENT department regarding the aural symptoms. The neurophysiologist reported that the upper and lower limb sensory and motor responses were all within normal limits with no evidence of a peripheral sensory or motor neuropathy or multifocal motor neuropathy with conduction block. There was isolated denervation of the tongue. The other muscles supplied by the lower cranial nerves did not show any denervation changes and there was no definite denervation in the cervical or sacrolumbar segments. There was, therefore, no evidence of widespread anterior horn cell dysfunction at that time. It was considered that isolated denervation of both sides of a
Tel: +44 117 928 4304.
# 2006 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
80
Andrews and Eveson identified the lesion as a mucoepidermoid carcinoma. The tumour was resected and proved to be a mucoepidermoid carcinoma with a low mitotic rate, but with evidence of perineural and vascular invasion (Fig. 2). Excision was incomplete so adjuvant radiotherapy was undertaken. At his last review, 4 years after treatment, there was no evidence of recurrence.
Discussion
Fig. 1. MRI scan showing bilobed tumour in the base of tongue.
the tongue could be the beginning of bulbar palsy, but there were no other changes allowing for a specific diagnosis at that time and follow-up in 4–6 months was recommended. The patient failed to keep follow-up appointments and was seen by an ENT consultant for his aural symptoms 12 months after he first noticed the lingual myokymia. The patient’s speech had deteriorated further and the first stage of swallowing was now affected. He had some shooting pain and left earache. There was obvious myokymia and some wasting of the right side of the tongue, but it was not markedly deviated to the right, suggesting either some weakness of the left side of the tongue or tethering. Palpation of the tongue posteriorly revealed a hard mass in the central part of the musculature but indirect laryngoscopy was normal. A provisional diagnosis of a nerve-sheath tumour or a sarcoma was made, and fine-needle aspiration (FNA) and MRI scans were undertaken. The MRI scan showed a 24-mm maximum diameter lesion lying in the base of the tongue; it was bilobed and obliterated the normal fat lines of the lingual septum. Inferiorly it abutted the geniohyoid muscles (Fig. 1). Although the scanning appearances were non-specific it was felt that the enhancement, shape and good definition were compatible with a diagnosis of neurofibroma. The FNA scan in fact
Salivary tumours of the base of the tongue are relatively uncommon. In a study of 178 tumours of minor salivary glands12, 13% (22 cases) were located in the base of tongue and all were malignant. Nearly half of these were mucoepidermoid carcinomas. Mucoepidermoid carcinoma is a malignant glandular epithelial neoplasm characterized by mucous, intermediate and epidermoid cells, with columnar, clear cells and oncocytoid features5. In the majority of large surveys, it is the most common malignant salivary gland tumour in both adults and children, accounting for up to 34% of all malignancies10,11.
Fig. 2. (A) Area of solid mucoepidermoid carcinoma consisting of sheets of intermediate cells and mucocytes (H&E 150) (B) Mucoepidermoid carcinoma with foci of neural invasion (H&E 150).
Myokymia of the tongue The mean age of presentation is approximately 45 years, but tongue lesions may present at an older average age4. Tumours typically form slow-growing, painless and fixed masses. An accelerated rate of growth may immediately precede clinical presentation. Symptoms can include pain or tenderness, evidence of nerve involvement including anaesthesia, paraesthesia or palsy, and dysphagia, otorrhea and trismus. The prognosis of mucoepidermoid carcinoma depends on the clinical stage, site, grading and adequacy of surgical excision. The prognosis for low-grade tumours is excellent and survival is typically over 95%, but even for these tumours management can be difficult, as a small proportion unpredictably undergo metastasis, usually to the regional lymph nodes. In the less common high-grade tumours the mortality rate increases to nearly 50%. If these tumours are incompletely excised there is a very high recurrence rate. The present case was graded histologically as intermediate, but due to the location, the incomplete excision and evidence of neural and vascular involvement, it was considered to be clinically high grade. Following surgery and adjuvant radiotherapy there was no recurrence after 4 years. Myokymia is defined as successive and rapid contractions of motor units associated with chronic nerve injury. The discharges arise from the peripheral aspects of regenerating nerves, and clinically impart a nearly continuous undulation of the body surface overlying the muscle1. Fasciculations are irregular, flickering, twitching movements that are also caused by involuntary contractions of muscle bundles. Fasciculations are finer than myokymia and appear more as rippling movements of the muscles. The difference between the two is best detected by electromyography. Myokymia is most commonly seen as a transient involuntary twitching of muscles controlling the
eyelid and as such is rarely associated with significant clinical disease, but persistent facial myokymia has been associated with a wide range of inflammatory demyelinating diseases, Guillain– Barre´ syndrome, and lesions of the brainstem including pontine glioma, pontine metastases, cerebellar glioma and acoustic neuroma9, and has also been reported in a case of squamous cell carcinoma of the middle ear7. Hyperexcitability of the intraaxial part of the facial nerve in pontine lesions and the extra-axial part of the facial nerve in intrinsic lesions has been postulated as a possible mechanism. Myokymia of the tongue is much less common than facial myokymia, and has been reported in cases of progressive bulbar palsy3, brainstem tumours2, and in relation to an extra-cranial hypoglossal schwannoma8. Several cases have been reported following irradiation of the head and neck region13,6. The present case appears to be unique. Apart from the short case report of lingual myokymia due to a schwannoma8, no other reference could be found to any extracranial tumour presenting with nerve stimulation.
References 1. Adams RD, Victor M. Principles of Neurology. 6th edn New York: McGraw Hill 1997: 1491. 2. Adams RW, Skuse NF. Myokymia of the tongue in a case of brainstem tumour that simulated motor-neuron disease. Med J Aust 1988: 149 494, 496–497. 3. Cerero Lapiedra R, Moreno Lopez LA, Esparza Gomez GC. Progressive bulbar palsy: a case report diagnosed by lingual symptoms. J Oral Pathol Med 2002: 31: 277–279. 4. Ellis GL, Auclair PL. Tumors of the salivary glands. In: Ellis GL, Auclair PL, eds: Atlas of Tumor Pathology. 3rd series, fascicle 17. Washington DC: AFIP 1996: 155.
5.
6.
7. 8. 9.
10.
11.
12.
13.
81
RK, EL-NAGGAR AK. Mucoepidermoid carcinoma. In: Barnes, L, Eveson, JW, Reichart, P, Sidransky, D, eds: Pathology and Genetics of Head and Neck Tumours. Kleihues P, Sobin LH, series eds: World Health Organization Classification of Tumours. Lyon, France: IARC Press, 2005: 219–220. Poncelet AN, Auger RG, Silber MH. Myokymic discharges of the tongue after radiation to the head and neck. Neurology 1996: 46: 259–260. Radu¨ EW, Skorpil V, Kaeser HE. Facial myokymia. Eur Neurol 1975: 13: 499–512. Ranta A, Winter WC, Login IS. Extracranial hypoglossal schwannoma. Neurology 2003: 60: E11. Sharma RR, Mathad NV, Joshi DN, Mazarelo TB, Vaidya MM. Persistent facial myokymia: a rare pathognomonic physical sign of intrinsic brain-stem lesions: report of 2 cases and review of literature. J Postgrad Med 1992: 38: 37– 40. Spiro RH, Huvos AG, Berk R, Strong EW. Mucoepidermoid carcinoma of salivary gland origin. A clinicopathologic study of 367 cases. Am J Surg 1978: 136: 461–468. Spitz MR, Batsakis JG. Major salivary gland carcinoma. Descriptive epidemiology and survival of 498 patients. Arch Otolaryngol 1984: 110: 45–49. de Vries EJ, Johnson JT, Myers EN, Barnes Jr EL, Mandell-Brown M. Base of tongue salivary gland tumors. Head Neck Surg 1987: 9: 329–331. Wang V, Liao KK, Ju TH, Lin KP, Wang SJ, Wu ZA. Myokymia and neuromyotonia of the tongue: a case report of complication of irradiation. Zhonghua Yi Xue Za Zhi (Taipei) 1993: 52: 413–415. GOODE
Address: Dr Karen Andrews Division of Restorative Dentistry Bristol Dental Hospital and School Lower Maudlin Street Bristol BS1 2LY UK E-mail: [email protected]
Case Report Clinical Pathology
Myokymia (fasciculation) of the tongue as a unique presentation of mucoepidermoid carcinoma
Karen V. Andrews, John W. Evesona Division of Oral Medicine, Pathology and Microbiology, Bristol Dental Hospital and School, Bristol, UK
Karen V. Andrews, John W. Eveson: Myokymia (fasciculation) of the tongue as a unique presentation of mucoepidermoid carcinoma. Int. J. Oral Maxillofac. Surg. 2007; 36: 79–81. # 2006 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Neural invasion is a relatively common feature of squamous cell carcinomas of the head and neck and malignant salivary gland tumours. The symptoms depend on the location and the particular nerves involved, and include pain, anaesthesia, paraesthesia and cranial nerve palsy. The present case appears to be unique. A mucoepidermoid carcinoma showed evidence of neural involvement and presented with nerve stimulation inducing myokymia, rather than nerve destruction, which is the usual consequence of tumoral nerve invasion.
A 38-year-old man was referred with ‘a lump on the right side of the tongue that appeared to be pulsing involuntarily’. The patient described a swelling in his tongue that had been present for 2 months and was gradually increasing in size. He also reported that the lesion appeared to be moving involuntarily and was causing him progressive difficulties with speech. The patient’s medical history was noncontributory. Extraoral examination showed no abnormalities. No mass could be detected in the tongue although there was striking myokymia (fasciculation) affecting the right side that was severe enough to cause difficulties with speech. Irregular twitching of groups of muscle fibres gave the overlying mucosa a rippling appearance. Pronouncing ‘ing’ and ‘g’ at the end of words was notably impaired. There was no loss of sensory 0901-5027/01079 + 03 $30.00/0
function and no other cranial nerve deficits were found. The results of routine haematological investigations were normal. A magnetic resonance imaging (MRI) scan performed a month later showed no significant intracranial lesions. Apart from minimal mucosal changes in the paranasal sinuses, the images were reported as unremarkable. The patient was referred to a neurologist and was seen 6 months after his initial symptoms. There was evident wasting and conspicuous myokymia of the tongue, largely confined to the right hand side. No other neurological abnormalities were detected and no lymph nodes or masses in the neck could be palpated. At this appointment the patient also mentioned ‘fullness’ of the left ear and a mild reduction in hearing. A working diagnosis of early bulbar palsy was given, despite the
Accepted for publication 26 May 2006 Available online 11 September 2006
patient’s relative youth. He was referred for electromyography studies and to an ENT department regarding the aural symptoms. The neurophysiologist reported that the upper and lower limb sensory and motor responses were all within normal limits with no evidence of a peripheral sensory or motor neuropathy or multifocal motor neuropathy with conduction block. There was isolated denervation of the tongue. The other muscles supplied by the lower cranial nerves did not show any denervation changes and there was no definite denervation in the cervical or sacrolumbar segments. There was, therefore, no evidence of widespread anterior horn cell dysfunction at that time. It was considered that isolated denervation of both sides of a
Tel: +44 117 928 4304.
# 2006 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
80
Andrews and Eveson identified the lesion as a mucoepidermoid carcinoma. The tumour was resected and proved to be a mucoepidermoid carcinoma with a low mitotic rate, but with evidence of perineural and vascular invasion (Fig. 2). Excision was incomplete so adjuvant radiotherapy was undertaken. At his last review, 4 years after treatment, there was no evidence of recurrence.
Discussion
Fig. 1. MRI scan showing bilobed tumour in the base of tongue.
the tongue could be the beginning of bulbar palsy, but there were no other changes allowing for a specific diagnosis at that time and follow-up in 4–6 months was recommended. The patient failed to keep follow-up appointments and was seen by an ENT consultant for his aural symptoms 12 months after he first noticed the lingual myokymia. The patient’s speech had deteriorated further and the first stage of swallowing was now affected. He had some shooting pain and left earache. There was obvious myokymia and some wasting of the right side of the tongue, but it was not markedly deviated to the right, suggesting either some weakness of the left side of the tongue or tethering. Palpation of the tongue posteriorly revealed a hard mass in the central part of the musculature but indirect laryngoscopy was normal. A provisional diagnosis of a nerve-sheath tumour or a sarcoma was made, and fine-needle aspiration (FNA) and MRI scans were undertaken. The MRI scan showed a 24-mm maximum diameter lesion lying in the base of the tongue; it was bilobed and obliterated the normal fat lines of the lingual septum. Inferiorly it abutted the geniohyoid muscles (Fig. 1). Although the scanning appearances were non-specific it was felt that the enhancement, shape and good definition were compatible with a diagnosis of neurofibroma. The FNA scan in fact
Salivary tumours of the base of the tongue are relatively uncommon. In a study of 178 tumours of minor salivary glands12, 13% (22 cases) were located in the base of tongue and all were malignant. Nearly half of these were mucoepidermoid carcinomas. Mucoepidermoid carcinoma is a malignant glandular epithelial neoplasm characterized by mucous, intermediate and epidermoid cells, with columnar, clear cells and oncocytoid features5. In the majority of large surveys, it is the most common malignant salivary gland tumour in both adults and children, accounting for up to 34% of all malignancies10,11.
Fig. 2. (A) Area of solid mucoepidermoid carcinoma consisting of sheets of intermediate cells and mucocytes (H&E 150) (B) Mucoepidermoid carcinoma with foci of neural invasion (H&E 150).
Myokymia of the tongue The mean age of presentation is approximately 45 years, but tongue lesions may present at an older average age4. Tumours typically form slow-growing, painless and fixed masses. An accelerated rate of growth may immediately precede clinical presentation. Symptoms can include pain or tenderness, evidence of nerve involvement including anaesthesia, paraesthesia or palsy, and dysphagia, otorrhea and trismus. The prognosis of mucoepidermoid carcinoma depends on the clinical stage, site, grading and adequacy of surgical excision. The prognosis for low-grade tumours is excellent and survival is typically over 95%, but even for these tumours management can be difficult, as a small proportion unpredictably undergo metastasis, usually to the regional lymph nodes. In the less common high-grade tumours the mortality rate increases to nearly 50%. If these tumours are incompletely excised there is a very high recurrence rate. The present case was graded histologically as intermediate, but due to the location, the incomplete excision and evidence of neural and vascular involvement, it was considered to be clinically high grade. Following surgery and adjuvant radiotherapy there was no recurrence after 4 years. Myokymia is defined as successive and rapid contractions of motor units associated with chronic nerve injury. The discharges arise from the peripheral aspects of regenerating nerves, and clinically impart a nearly continuous undulation of the body surface overlying the muscle1. Fasciculations are irregular, flickering, twitching movements that are also caused by involuntary contractions of muscle bundles. Fasciculations are finer than myokymia and appear more as rippling movements of the muscles. The difference between the two is best detected by electromyography. Myokymia is most commonly seen as a transient involuntary twitching of muscles controlling the
eyelid and as such is rarely associated with significant clinical disease, but persistent facial myokymia has been associated with a wide range of inflammatory demyelinating diseases, Guillain– Barre´ syndrome, and lesions of the brainstem including pontine glioma, pontine metastases, cerebellar glioma and acoustic neuroma9, and has also been reported in a case of squamous cell carcinoma of the middle ear7. Hyperexcitability of the intraaxial part of the facial nerve in pontine lesions and the extra-axial part of the facial nerve in intrinsic lesions has been postulated as a possible mechanism. Myokymia of the tongue is much less common than facial myokymia, and has been reported in cases of progressive bulbar palsy3, brainstem tumours2, and in relation to an extra-cranial hypoglossal schwannoma8. Several cases have been reported following irradiation of the head and neck region13,6. The present case appears to be unique. Apart from the short case report of lingual myokymia due to a schwannoma8, no other reference could be found to any extracranial tumour presenting with nerve stimulation.
References 1. Adams RD, Victor M. Principles of Neurology. 6th edn New York: McGraw Hill 1997: 1491. 2. Adams RW, Skuse NF. Myokymia of the tongue in a case of brainstem tumour that simulated motor-neuron disease. Med J Aust 1988: 149 494, 496–497. 3. Cerero Lapiedra R, Moreno Lopez LA, Esparza Gomez GC. Progressive bulbar palsy: a case report diagnosed by lingual symptoms. J Oral Pathol Med 2002: 31: 277–279. 4. Ellis GL, Auclair PL. Tumors of the salivary glands. In: Ellis GL, Auclair PL, eds: Atlas of Tumor Pathology. 3rd series, fascicle 17. Washington DC: AFIP 1996: 155.
5.
6.
7. 8. 9.
10.
11.
12.
13.
81
RK, EL-NAGGAR AK. Mucoepidermoid carcinoma. In: Barnes, L, Eveson, JW, Reichart, P, Sidransky, D, eds: Pathology and Genetics of Head and Neck Tumours. Kleihues P, Sobin LH, series eds: World Health Organization Classification of Tumours. Lyon, France: IARC Press, 2005: 219–220. Poncelet AN, Auger RG, Silber MH. Myokymic discharges of the tongue after radiation to the head and neck. Neurology 1996: 46: 259–260. Radu¨ EW, Skorpil V, Kaeser HE. Facial myokymia. Eur Neurol 1975: 13: 499–512. Ranta A, Winter WC, Login IS. Extracranial hypoglossal schwannoma. Neurology 2003: 60: E11. Sharma RR, Mathad NV, Joshi DN, Mazarelo TB, Vaidya MM. Persistent facial myokymia: a rare pathognomonic physical sign of intrinsic brain-stem lesions: report of 2 cases and review of literature. J Postgrad Med 1992: 38: 37– 40. Spiro RH, Huvos AG, Berk R, Strong EW. Mucoepidermoid carcinoma of salivary gland origin. A clinicopathologic study of 367 cases. Am J Surg 1978: 136: 461–468. Spitz MR, Batsakis JG. Major salivary gland carcinoma. Descriptive epidemiology and survival of 498 patients. Arch Otolaryngol 1984: 110: 45–49. de Vries EJ, Johnson JT, Myers EN, Barnes Jr EL, Mandell-Brown M. Base of tongue salivary gland tumors. Head Neck Surg 1987: 9: 329–331. Wang V, Liao KK, Ju TH, Lin KP, Wang SJ, Wu ZA. Myokymia and neuromyotonia of the tongue: a case report of complication of irradiation. Zhonghua Yi Xue Za Zhi (Taipei) 1993: 52: 413–415. GOODE
Address: Dr Karen Andrews Division of Restorative Dentistry Bristol Dental Hospital and School Lower Maudlin Street Bristol BS1 2LY UK E-mail: [email protected]