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 originating from the modified smooth muscle cells of the vascular glomus Glomangiomas of the head and/or neck are extremely rare, with an incidence of 0.6%. We present an observational case report of a recurrent glomic tumor originally located in the mobile tongue, with an aggressive clinical course that complicated the therapeutic options of the case.

Key words: Glomus tumor, tongue.

INTRODUCTION

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

The present study reports a very unusual case of locally aggressive glomus tumor of the tongue. (see Table 1 Table 2)

 

CASE REPORT

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

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

In October 1992, the patient was subjected to surgery under general anesthesia. The lesion was apparently completely removed en bloc via a midline tongue incision, with important hemostasia measures due to the vascular nature of the lesion and followed by layered suturing. No reconstruction of any kind was required, and the postoperative course was uneventful.

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

Follow-up in the outpatient clinic (initially every month and then on a yearly basis) showed no recurrence, and the patient was asymptomatic.

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

Orthopantomography revealed an important lytic lesion at mandibular symphysis level, encompassing the region from the right lower first premolar to the left lower second premolar, together with a pathological fracture between the two lower central incisors (Fig.2).

Magnetic resonance imaging showed the presence of an important infiltrating vascular-type lesion (Fig.3) affecting the entire right half of the tongue, crossing the midline and extending to the left half of the organ at the anterior lingual margin, and to the floor of the mouth, right submaxillary space, mandibular symphysis, gingivobuccal sulcus and mental and submental soft parts. The insertions of the anterior bellies of the two digastric muscles were also infiltrated. The maximum dimensions of the lesion were 7 cm anteroposteriorly and 4.5 cm craniocaudad and laterolaterally. The right half of the soft palate in turn showed a tumor of the same characteristics, though without evidence of any anatomical connection between the two lesions.

An arterographic study of the lesion was performed before deciding surgery (Fig.4), showing important hypertrophy of both submental rami of the two facial arteries, and hypertrophy of both inferior alveolar arteries (branches of the internal maxillary artery) - globally conforming an important expansive tumor lesion of vascular origin.

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 surgical risk as far as possible. A tracheostomy was performed before embolization to ensure airways patency and avoid possible obstruction secondary to the important post-embolization facial swelling.

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

Due to the impossibility of performing effective combined surgical-embolization treatment, radiotherapy was decided in the hope of controlling the expansive process of the lesion and allowing posterior surgical management.

During this period of time the patient suffered multiple though minor bleeding episodes that were resolved by the adoption of local measures. The pain was controlled with analgesic medication, while the size of the lesion was responsible for important speech and eating problems.

DISCUSSION

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

A review of the recent years has only yielded one further lesion located in the tonsillar fossa (11), another in the parotid region (12), one affecting the jugal mucosa (5), and a few more in the region of the lips (13-16). The review shows most lesions to manifest as palpable soft tumors that are initially asymptomatic but subsequently upon growing cause repeated bleeding, pain and spatial occupation with the lysis of neighboring structures under the expansive pressure.

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

However, not all glomus tumors appear so benign. In 1975, Spector et al. (19) published a series of 75 glomus tumors located in the head 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 of glomus tumors of the oropharynx. Complete exeresis was performed, followed by resolution of the symptoms. Reconstruction in turn depended on the size and location of the growth. In no case was lesion recurrence or the need for adjuncts such as arterial embolization and/or radiotherapy mentioned (20,21). Asymptomatic patients not amenable to surgery were subjected to serial imaging evaluations, while radiotherapy was provided in symptomatic cases in which surgery was not possible (22,23).

CONCLUSIONS

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

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

The benign nature of the lesion led us to attempt to preserve the tongue in the first surgical intervention and avoid more aggressive resection for ensuring safe surgical margins. This conservative approach was the likely cause of the recurrence observed years later.

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

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