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CASE REPORT
Year : 2022 | Volume
: 30
| Issue : 4 | Page : 128-130
Tongue schwannoma, an uncommon oral cavity mass: Experience from a case
Einstein Alfredo Viana-Tapia1, Juan Felipe Coronado-Sarmiento2, Osvaldo Camilo Barraza-Leones3
1 Department of Head and Neck Surgery, Imat Oncomedica Clinic, Chía, Colombia
2 Faculty of Medicine, Universidad de la Sabana, Chía, Colombia
3 Department of Surgery, Universidad del Sinú, Cartagena Branch Office, Colombia
Correspondence Address:
Dr. Juan Felipe Coronado-Sarmiento
Campus del Puente del Común, Km. 7, Autopista Norte de Bogotá, Chía, Cundinamarca, Bogotá 53753
Colombia
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/tjps.tjps_35_22
Schwannomas are benign tumors arising from the Schwann cells, located in the peripheral nerve sheath. The incidence of tumor in the oral cavity is <1%, and the tongue is the most prevalent place in that area. A 32-year-old Hispanic female was sent from the otolaryngology department with a diagnosis of an oral mucocele in the oral cavity floor, after 8 months complaining of dysphonia and dysphagia. Physical examination reveals a submucosal mass in the anterior and ventral portion of the tongue. After characterization with magnetic resonance and biopsy guided by tomography, she underwent surgical management, with no complications. Tongue schwannomas are often misdiagnosed based on their very low incidence. A proper approach including a good physical examination, imaging and histological characterization of the lesion, and proper surgical management of this entity, will lead to cure this condition, with a low relapse rate.
Keywords: Mouth neoplasms, oral cavity, Schwann cells, schwannoma
Schwannomas are tumors arising from the peripheral nerve sheath, specifically from the Schwann cells. They can arise from any nerve associated with this kind of cells, including cranial nerves (excepting the optical and olfactory nerve) and peripheral and autonomic nerves.[1] The most frequent location of these tumors are extremities, head and neck, posterior mediastinum, and retroperitoneum. Approximately 25%–45% of schwannomas locate in the head-and-neck region;[2] being the most common place the lateral side of the neck, and the most uncommon are the oral cavity, paranasal sinus, nasal cavity, nasopharynx, eye orbit, and larynx. Schwannomas located in the oral cavity constitute up to 1% of the cases, and they are mainly present in the tongue, however, other locations reported are the palate, oral mucosa, lips, and gums.[3] These lesions have a slow growth rate, and most of them are asymptomatic and painless before they reach 3 cm size, then symptoms can be referred, including dysphonia, dysphagia, sensorial abnormalities, mostly gustatory; pain, discomfort, and in severe cases, these tumors can lead to sleep apnea/hypopnea syndrome.[4],[5]
Clinical CaseA Hispanic 32-year- old female was referred to the head-and-neck surgery department by the otolaryngologist with the diagnosis of an oral mucocele in the mouth floor. Her symptoms include 8 months of dysphonia and dysphagia. Physical examination revealed a submucosal, bulged lesion, of 5 cm approximately, located in the interior and ventral portion of the tongue, causing a deviation of the ventral branch of the tongue to the left, extending to the mouth floor and to the first cervical level bilaterally, in the bimanual tact [Figure 1].
Figure 1: Presurgical picture of the patient showing left deviation of the ventral branch of the tongueA simple and contrasted magnetic resonance of the neck was performed to characterize and delimit the lesion. These images reported a well-defined mass, encapsulated in the thickness of the right tongue, with a slight compromise of the left tongue, moving the half line, with a measurement of 33 mm × 38 mm × 40 mm, moving the genioglossus and geniohyoid muscles, with solid and cystic areas, without adenopathy [Figure 2]. There was performed a biopsy of the lesion guided by tomography. Histopathology report shown a benign tumoral lesion with a mesenchymal lineage, formed by fusiform cells, with a low mitotic rate and uniform chromatin, arranged in hypercellular zones in a palisading pattern (known as Verocay bodies), and hypocellular zones (Antoni B areas). It was reported as a fusocellular tumor with a histological low-grade, suggesting a Schwannoma. With these findings, the patient underwent surgery. The resection of the lesion was performed by intraoral approach, with the incision in the lateral aspect of the right tongue and right mouth floor, with a section of mucosa and lingual muscles. The inferior longitudinal muscle was opened and dissected, identifying an oval-shaped lesion, with regular borders of 4 cm approximately, extending until the tongue base without compromising it, and preserving the cleavage plane of the lingual nerve; the mass was fully resected. Exhaustive lavage with crystalloids, and hemostasis with an hemostatic agent was performed on the surgical bed. After this, the defect was repaired by design and dissection of a muscle/mucosa flap of 5–10 cm, and a suture was made in two planes, muscular and mucosal. The oropharynx was reviewed under direct laryngoscopy, no obstruction was detected, and an additional stitch was placed at the tip of the tongue for achieving traction in case of the oropharynx was obliterated; feeding was achieved by a nasoenteric tube, and placed before the beginning of the surgical act. The postoperative course was uneventful, she did not present any surgical complications, and now she is in her 6th month after surgery, with a notorious improvement, without relapse, with full remission of the initial symptoms [Figure 3].
Figure 2: Box (a) magnetic resonance of neck, coronal, and T2 sequence. (b) Magnetic resonance of neck, sagittal, and T2 sequence. Both boxes show an isointense mass in the right half of the tongue, highlighted with contrast
Figure 3: Postsurgical picture of the patient showing the proper location of the tongue Discussion Oral cavity schwannomas typically present in people between their 20s and their 30s, however, these tumors can present all lifelong and the sex distribution is equal.[6] Most of these cases are sporadic but they can be associated with genetic syndromes as well, including type 2 neurofibromatosis, schwannomatosis, and Carney complex.[7]
Imaging is the most common diagnostic approach for these lesions, along with a biopsy.[1],[5] Schwannomas features on tomographic imaging include soft-tissue appearance, homogeneous, dense, and well-circumscribed masses that are usually highlighted with contrast. Magnetic resonance has a higher performance than CT scan for schwannomas. Most schwannomas look hypo or isointense when compared to muscle in T1, hyperintense in T2 with highlight when contrast is administered,[8] like the image presented in our case.
A definitive diagnosis is made with histopathological study, where hypercellular zones can be seen, named Antoni A zones. These are characterized by fusiform cells in a palisading pattern known as Verocay bodies.[1],[6],[7],[8] Malignant transformation of schwannomas is extremely rare, and differential diagnosis should be made with other fusocellular lesions such as neurofibromas, leiomyomas, and fibrous histiocytoma or even synovial sarcoma.[8],[9]
Lingual schwannomas can represent a significant risk for upper airway obstruction; therefore, requiring early surgical intervention. These tumors are often treated by surgical excision along with the affected nerve, but during the procedure, it is difficult to establish the origin of the tumor, especially in the tongue, given the proximity between the lingual and the glossopharyngeal nerve,[10] creating technical difficulties for achieving full excision. Transoral resection is the most common surgical approach, but recently, other successful approaches have been reported like the submandibular, commonly used for the posterolateral or base located lingual schwannoma. Most recently, laser resection with carbon dioxide has been also used for treating base-located schwannomas. On the other hand, a tumor located in the posterolateral base region of the tongue, which result inaccessible through the mouth or has a size over 4 cm; other surgical techniques such as submandibular or split-lip approach are used, with usually easy elimination due to the capsule of the tumor.[9]
ConclusionsLingual schwannoma is a low frequent pathology; thus, it is usually not considered a differential diagnosis in the evaluation of lingual masses. A proper clinical evaluation includes a complete medical history, a good physical exam, proper imaging, and a biopsy. The current treatment is surgical excision of the lesion, preserving the origin nerve, and this treatment is usually curative, and it is not associated with relapse.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Ethics statement
Informed consent was obtained from the patient for publication of the case report and accompanying images. The present publication was also approved by the local ethics committee.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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