Aggressive glomus tumor of the tongue: Report of a case

QUESADA R, GONZÁLEZ-LAGUNAS J, RASPALL G. AGGRESSIVE GLOMUS TUMOR OF THE TONGUE: REPORT OF A CASE. MED ORAL 2004;9:350-4.

SUMMARY

A glomus tumor or glomangioma is a benign neoplasm originatingfrom the modified smooth muscle cells of the vascular glomus Glomangiomas of the head and/or neck are extremely rare, with anincidence of 0.6%. We present an observational case report of arecurrent glomic tumor originally located in the mobile tongue ,with an aggressive clinical course that complicated the therapeuticoptions of the case.

Key words: Glomus tumor, tongue.

INTRODUCTION

Glomus tumors or glomangiomas are benign tumors derived from themodified smooth muscle cells of the glomus (1,2). The normal glomusis a neuromyoarterial receptor that is sensitive to variations intemperature and regulates arteriolar blood flow. The glomus possessesan afferent arteriole, arteriovenous anastomoses and efferent veins(3). Although glomus tumors are not frequent, many cases have beenreported in the literature – fundamentally located in the dermisand subcutaneous tissues of the subungual region and fingers (4).Glomangiomas of the head and/or neck are extremely rare, with anincidence of 0.6% (5).

The present study reports a very unusual case of locallyaggressive glomus tumor of the tongue. (see Table 1 Table2)

CASE REPORT

A 61-year-old male was referred to our Service in September 1992with a tongue tumor for a number of weeks. There were no otheraccompanying manifestations. The initial physical examinationrevealed the presence of a tongue tumor without surface ulceration.The lesion was hard though not petrous and occupied the anteriorportion of the right half of the tongue, without crossing themidline. The tumor was not painful to palpation. At the time of theinitial examination the patient suffered no ankyloglossia, and theneck exploration was unremarkable, showing no adenopathies.

Magnetic resonance imaging revealed a submucosal lesion in thefree right portion of the tongue and measuring about 3 cm in diameter (Fig.1) . Fine needle aspiration cytology was performed, suggesting apossible benign glomic tumor.

In October 1992, the patient was subjected to surgery undergeneral anesthesia. The lesion was apparently completely removed enbloc via a midline tongue incision, with important hemostasiameasures due to the vascular nature of the lesion and followed bylayered suturing. No reconstruction of any kind was required, and thepostoperative course was uneventful.

The pathology report on the surgical piece indicated a lesioncomposed of vascular structures with prominent cells distributed inthe form of nodules separated by sclerotic bands. Numerousfine-endothelium vascular elements were identified within thenodules, with no cellular atypias or mitotic figures.

Follow-up in the outpatient clinic (initially every month and thenon a yearly basis) showed no recurrence, and the patient wasasymptomatic.

In December 1999, one year after the last follow-up visit, thepatient presented with a new tongue tumor associated with pain in theanterior mandibular sector. Exploration revealed an important tumorlesion that crossed the lingual midline and affected the floor of themouth, with ankyloglossia. Important mobility was observed in theanterior mandibular sector, with intense pain in response to toothmobilization.

Orthopantomography revealed an important lytic lesion atmandibular symphysis level, encompassing the region from the rightlower first premolar to the left lower second premolar, together witha pathological fracture between the two lower central incisors (Fig..2 ).

Magnetic resonance imaging showed the presence of an importantinfiltrating vascular-type lesion (Fig.3) affecting the entire righthalf of the tongue, crossing the midline and extending to the lefthalf of the organ at the anterior lingual margin, and to the floor ofthe mouth, right submaxillary space, mandibular symphysis,gingivobuccal sulcus and mental and submental soft parts. Theinsertions of the anterior bellies of the two digastric muscles werealso infiltrated. The maximum dimensions of the lesion were 7 cmanteroposteriorly and 4.5 cm craniocaudad and laterolaterally. Theright half of the soft palate in turn showed a tumor of the samecharacteristics, though without evidence of any anatomical connectionbetween the two lesions.

An arterographic study of the lesion was performed before decidingsurgery (Fig.4), showing important hypertrophy of both submental ramiof the two facial arteries, and hypertrophy of both inferior alveolararteries (branches of the internal maxillary artery) - globallyconforming an important expansive tumor lesion of vascularorigin.

Due to the great size and vascular nature of the lesion,emboliza-tion of both external carotids was decided before surgery,to ensure control of the vascular supply and thus reduce the surgicalrisk as far as possible. A tracheostomy was performed beforeembolization to ensure airways patency and avoid possible obstructionsecondary to the important post-embolization facial swelling.

Different selective embolization sessions were performed of thebranches of both external carotid arteries that nevertheless failedto achieve the desired effects upon the lesion. Consequently,vascular control prior to the operation was not possible.

Due to the impossibility of performing effective combinedsurgical-embolization treatment, radiotherapy was decided in the hopeof controlling the expansive process of the lesion and allowingposterior surgical management.

During this period of time the patient suffered multiple thoughminor bleeding episodes that were resolved by the adoption of localmeasures. The pain was controlled with analgesic medication, whilethe size of the lesion was responsible for important speech andeating problems.

DISCUSSION

Until 1990, Tokiba et al. (9) found only 16 cases of oral orfacial glomus tumors published in the literature: 4 gingival lesions,4 palatal tumors, 2 lingual lesions, 2 jugal mucosal lesions, onetumor in the preauricular region, and 2 lip lesions. We added anothercase prior to 1990 and published by Potter et al. in 1984 (10),involving an intranasal lesion; these authors in turn documented 5more cases in this same location.

A review of the recent years has only yielded one further lesionlocated in the tonsillar fossa (11), another in the parotid region(12), one affecting the jugal mucosa (5), and a few more in theregion of the lips (13-16). The review shows most lesions to manifestas palpable soft tumors that are initially asymptomatic butsubsequently upon growing cause repeated bleeding, pain and spatialoccupation with the lysis of neighboring structures under theexpansive pressure.

In 1981, Tajima et al. (17) published the case of an asymptomaticlingual glomus tumor in a 63-year-old woman, located superficially inthe right underside of the tongue. Exeresis under local anesthesiawas performed, without recurrence. Sato has presented another lesionwith similar features (18).

However, not all glomus tumors appear so benign. In 1975, Spectoret al. (19) published a series of 75 glomus tumors located in thehead and neck - including the inner ear and skull base. Clinically,intracranial spread was observed in 14.6% of cases, with a 37%incidence of cranial nerve paralysis.

Surgery was the treatment modality in all published cases ofglomus tumors of the oropharynx. Complete exeresis was performed,followed by resolution of the symptoms. Reconstruction in turndepended on the size and location of the growth. In no case waslesion recurrence or the need for adjuncts such as arterialembolization and/or radiotherapy mentioned (20,21). Asymptomaticpatients not amenable to surgery were subjected to serial imagingevaluations, while radiotherapy was provided in symptomatic cases inwhich surgery was not possible (22,23).

CONCLUSIONS

The great majority of oropharyngeal glomus tumors are benign andcan be adequately treated by local resection. Clinically, the tumorappears as a soft, slow-growing mass (painless or otherwise). Overtime, growth causes local obstruction, repeated bleeding episodes,and structural lysis due to the pressure of expansion.

Local postsurgical recurrence is infrequent. The present caseinvolves a very rare lingual location. Only two similar cases havebeen reported to date (17,18) - in both instances corresponding tosoft and asymptomatic tumors.

The benign nature of the lesion led us to attempt to preserve thetongue in the first surgical intervention and avoid more aggressiveresection for ensuring safe surgical margins. This conservativeapproach was the likely cause of the recurrence observed yearslater.

We have presented the first and only case documented to date ofglomus tumor recurrence in this location, with a course thatcomplicated surgery as the only therapeutic option. In this context,large lesions with the involvement of multiple spaces may requirecombined radiotherapy and surgery

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