Presentation, Radiologic Features, and Treatment Options of Congenital Tongue Tumors: A Comprehensive Review.

AIM: Congenital tumors of the tongue are rare in pediatric patients but encompass a diverse range of entities. Each tumor type exhibits distinct clinical behaviors, necessitating a precise approach to differentiating the tumor types and a tailored, tumor-specific treatment regimen. Advanced imaging techniques, such as diffusion-weighted imaging and perfusion studies, play a vital role in differentiating benign and malignant tongue tumors. This review summarizes current knowledge regarding the presentation, imaging features, and treatment of congenital tongue tumors. METHODS: A literature review was conducted by searching studies on congenital tongue tumors in databases such as PubMed, Embase, Web of Science, and Scopus. Relevant data, such as clinical features, radiologic characteristics, treatment modalities, and outcomes for different tumor types, were extracted from the selected articles. RESULTS: Our literature review reveals the various entities of congenital tongue tumors, which can be categorized in terms of hereditary pattern, phenotype, and rarity. Congenital tongue tumors include a range of vascular malformations, such as hemangiomas, lymphatic malformations, arteriovenous malformations, and venous malformations. Another entity is represented by cystic lesions, including dermoid cysts, epidermoid cysts, ranulas, and mucous retention cysts. Rare malignant neoplasms include teratomas and rhabdomyosarcomas. These tumor types vary in terms of swelling, respiratory distress, or impaired oral function, depending on size and location. The detection of these tumors can be carried out using imaging modalities, such as ultrasound, magnetic resonance imaging, and computed tomography, which are utilized to facilitate diagnosis and differentiation. At present, surgical excision remains the cornerstone of treatment, while other modalities may be adopted, depending on tumor type and extent. The prognosis of congenital tongue tumors can be affected by tumor's site, size, involvement of vital structures, and malignancy. CONCLUSIONS: Given their diversity and complexity, congenital tongue tumors, albeit uncommon, require specialized clinical treatments tailored to each tumor type's characteristics. Understanding the variable presentations and imaging features enables accurate diagnosis, while customized treatment strategies are key to optimizing outcomes and minimizing morbidity in pediatric tongue tumors. This review summarizes current knowledge aimed at enhancing differential diagnosis and management of these diverse entities.

Congenital Oral Masses: An Anatomic Approach to Diagnosis.

Although congenital oral masses are rare, they are readily detectable during fetal US screening. Most congenital oral masses are benign, but some may cause mechanical airway obstruction, resulting in poor outcomes at delivery. The radiologist's ability to describe these abnormalities and their physiologic sequelae accurately can have a substantial effect on perinatal treatment. Furthermore, despite being rare, congenital oral lesions encountered at screening and at follow up fetal MRI provide the opportunity to make a specific diagnosis by following a simple anatomic approach. This article describes an anatomic algorithm as the framework for accurate diagnosis of congenital oral lesions. The imaging appearance of the most common congenital oral cavity neoplasms is outlined, including vascular anomalies, epulides, choristomas, congenital lingual thyroid anomalies, lingual hamartomas, and epignathi, and other conditions that mimic these at US. Also reviewed are perinatal management of masses that affect the fetal airway and the imaging features key to optimizing delivery outcomes. Online supplemental material is available for this article. ©RSNA, 2019.

Simultaneous, congenital, mucocoele of the Blandin-Nühn glands and teratoma of the tongue: early management and follow up at one year.

Mucocoele of the Blandin-Nühn glands and teratomas of the tongue are rare in newborn babies. We present what is to our knowledge the first documented case of both at the same time, and describe management and follow up over 12 months after the birth. Prenatal diagnosis defined the treatment plan during delivery to establish an airway for the baby promptly. A normal delivery is possible if the cyst is anterior, and can be punctured during delivery. Delayed excision reduces the possibility of oedema and obstruction of the airway, ensures complete resection, avoids recurrence, and confirms the diagnosis. Mucocoeles are classified as extravasation or retention types; our observation supports the hypothesis of a "malformative type". A long follow-up is essential to monitor macroglossia, open bite, and the development of the mandible.

Congenital granular cell lesion of the tongue: a report of two cases and review of the literature.

The congenital granular cell lesion most commonly occurs on the maxillary or mandibular alveolus of neonates. Extra-alveolar congenital granular cell lesion is exceptionally rare, with only 10 cases reported. Two additional cases occurring on the tongue are presented with a description of the clinical, histopathologic, and immunohistochemical features. The differential diagnosis is discussed, and the literature reviewed.

Ex-utero Intrapartum Treatment (EXIT) Procedure for Giant Fetal Epignathus.

BACKGROUND: Large fetal oropharyngeal tumors are rare, and have the potential to cause airway obstruction during birth. CASE CHARACTERISTICS: A 35-year-old woman with antenatally diagnosed large heterogenous mass in fetal neck displacing trachea and filling up the orophanygeal space. INTERVENTION: The infant was delivered at 31 weeks of gestation by ex-utero intrapartum therapy procedure to secure the airway. OUTCOME: Tumor was resected successfully on day 8 of life. Histopathology revealed mixed teratoma. MESSAGE: Ex-utero intrapartum therapy for fetuses with severe upper airway compromise may prove life-saving.

Lower lip cleft, bifid tongue and fibrolipoma: a case report of rare congenital anomaly.

A non-syndromic bifid tongue with fibrolipoma, ankyloglossia, and clefts of the lower lip and alveolus is rare. We describe a case in which a bifid anterior tongue was separated by a fibrolipomatous mass. We excised the mass and repaired the lip and the tongue with satisfactory results.

Huge congenital haemangioma of the tongue.

Haemangiomas, the most common type of benign vascular tumours, are rare in the oral cavity. Some of these lesions are congenital and show symptoms in late childhood or early adult life. A 32-years-old woman presented with a huge lesion on her tongue which caused dysphagia and dysphasia. She had first noticed the lesion when she was 6. Her obstructive symptoms started when she was 28 and, despite various medical treatments, the size of the lesion gradually increased. Magnetic resonance imaging revealed a 7 x 5 x 3 cm mass on the right side of the tongue. Because of severe functional and cosmetic problems, the lesion was excised with partial haemiglossectomy. Histopathological examination was consistent with intramuscular haemangioma. Haemangiomas are benign tumours with a benign course and are rarely seen on the tongue. They have clinical importance when localised in the oral cavity. Different treatment modalities exist, but in cases of large tumours, surgery may be the mainstay treatment.

[A newborn with a tumour on the tongue and alveolar ridge]. / Een pasgeborene met een afwijking op de tong en kaak.

In a newborn boy, a tumour was seen on the tongue and mandibula. A diagnosis of congenital epulis was made. This usually pedunculated tumour is present at birth and located on the maxillary or mandibular alveolar ridge (more commonly on the maxilla), but rarely on the tongue. It varies greatly in size from just a few millimetres in diameter to several centimetres. Treatment is only necessary when feeding problems occur.

Intralingual dermoid cyst in an infant presenting swallowing and sleeping difficulties.

Dermoid cysts of the oral cavity are extremely rare. The most common site is the floor of the mouth whereas intralingual location is the most unusual. They may be congenital or acquired and according to their histological appearance they are distinguished in "true" dermoid, epidermoid or teratoid cysts. We present the clinical and radiographic findings of a large congenital intralingual "true" dermoid cyst in a 10-month-old boy. The large size of the lesion and the subsequent enlargement of the tongue caused difficulties in swallowing and sleeping, symptoms which subsided after the surgical treatment. The uncommon location, the large size and the very young age of the patient were the noteworthy parameters.

Congenital angiomyoma of the tongue: case report.

Angiomyomas of the oral cavity are rare benign vascular neoplasms. In particular, the congenital form has not been reported before in the English language literature. We present a congenital angiomyoma of the tongue that was found on the posterior middle of the tongue in an infant. On MRI, the mass showed an isointense signal to muscle on the T1 weighted image and a slightly hyperintense signal on the T2 weighted image. Immunohistochemically, tumour cells were positive to desmin and smooth muscle actin, but negative to vimentin and S100. The treatment was surgical excision and no recurrence was found during the 26 month follow-up period.

Benign teratoma of the tongue in a neonate: a case report and review of the literature.

Teratomas are true neoplasms of unknown origin that arise from pluripotential cells and have an eccentric microscopic appearance. They are composed of diverse tissues from all 3 germ layers with variable levels of maturity. The purpose of this report was to describe the case of a benign teratoma originating from the tongue in a male neonate.

Congenital benign teratoma of the tongue with bifid tip, ankyloglossia and polydactyly: report of a case.

Teratomas of the tongue are rare, and often accompany other anomalies within the head and neck. We describe a combination of anomalies in a 6-week-old infant with teratoma and bifid tip of the tongue, severe tongue tie, and polydactyly. The teratoma was excised and the tongue tie released with no complications.

Management of congenital lingual dermoid cysts.

BACKGROUND: Lingual dermoid cysts are squamous epithelial-lined cavities with variable numbers of skin adnexae in the capsule and are rare entities in the head and neck. We discuss the presentation, possible aetiologies and the surgical management of these lesions and report on the ninth lingual dermoid cyst as an illustrative example, the first to be excised by laser. METHODS: An extensive literature search was undertaken. We present the case of a 4-month-old di-zygotic twin girl with a firm midline anterior tongue dermoid cyst. Its extent was defined by magnetic resonance imaging. The cyst was excised using the CO(2) laser via an extended median sagittal glossotomy approach. RESULTS: Lingual dermoid cysts most commonly present in early childhood or adolescence and are located in the anterior two thirds of the tongue. Eight lingual dermoid cysts have been reported, all of which were surgically excised, using varying techniques, and no recurrences have been reported. Our patient recovered well and was extubated immediately post-operatively. However, feeding was delayed for 48h post-operatively due to pain, requiring opiate analgesia. The tongue healed completely with only a small indent in the tip. CONCLUSIONS: Treatment of these lesions consists of complete surgical excision. We propose that the midline sagittal glossotomy incision using the CO(2) laser offers surgical precision, superior haemostasis and wound healing and minimal post-operative oedema. The involvement of the sensitive tongue tip in this approach may be its one drawback, however this may be compensated for with appropriate post-operative analgesia.