Peer Reviewed
An Atlas of Lingual Lesions, Part 3
Lymphangioma
Lymphangioma is a hamartomatous malformation of the lymphatic vessels arising from sequestration of portions of the lymphatic tissue that has lost its communication with the other lymphatic tissue but has retained its embryonic growth potential.1-3
The incidence has been reported to be 1.2 to 2.8 per 1000 births.4 The male to female ratio is 2:1.3 Approximately 50% of cases are noted at birth, and 90% are diagnosed before the age of 2 years.3-5 The condition is more common in patients with trisomies, Noonan syndrome, Turner syndrome, familial pterygium colli, hydrops fetalis, and fetal alcohol syndrome.4
Approximately 50% to 75% of lymphangiomas occur in the head and neck region.3,6,7 However, lymphangiomas are rarely reported in the oral cavity.7-9 When lymphangiomas occur in the oral cavity, the most common location is the tongue, followed by the palate, buccal mucosa, gingiva, floor of the mouth, lips, and alveolar ridge of the mandible.3,8,10 If the tongue is affected, the most common site is the anterior two-thirds of tongue’s dorsal surface, as illustrated in the Figure.3,6,7,10
Clinically, lingual lymphangiomas often present as painless macroglossia.3 Superficial lesions typically present as nodules with a pebbly surface that resembles a cluster of translucent vesicles or “frog egg” or “tapioca pudding” appearance (Figure).4,8,9 At various times, the vesicles can appear pink, blue, purple, or red due to rupture of capillaries into the lymphatic inner spaces.1 Deeper lesions present as soft, diffuse nodules without significant change in surface texture or color.3,7
Lymphangioma-related complications include lingual protrusion, cosmetic disfigurement, bleeding associated with oral trauma, sialorrhea, chewing/swallowing/feeding difficulties, speech impairment, poor oral hygiene, halitosis, mandible deformities (eg, open-bite malocclusion, prognathism), dental cavities, and, if large enough, upper airway obstruction.5,7,9-11
The condition is benign, and the prognosis is usually good for most patients.2,3 Spontaneous regression, however, is rare.8 Surgical excision is the treatment of choice.3,10 Other treatment options include intralesional steroids, sclerotherapy, laser surgery, electrocautery, cryotherapy, embolization, radiofrequency tissue ablation, and ligation.4,10 Recurrence of lymphangiomas is common due to the noncapsulated and infiltrating nature of the lymphangioma.2,10
REFERENCES:
- Devi A, Narwal A, Yadav AB, Singh V, Gupta A. Classical cases of lymphangioma—as multiple vesicular eruptions. J Clin Diagn Res. 2016;10(6):ZD22-ZD23.
- Nagpal T, Shah D, Manjunatha BS, Mahajan A. Macroglossia associated with lymphangioma: surgical management of an interesting case. J Clin Diagn Res. 2015;9(11):ZD04-ZD06.
- Sunil S, Gopakumar D, Sreenivasan BS. Oral lymphangioma—case reports and review of literature. Contemp Clin Dent. 2012;3(1):116-118.
- Bhalla AP, Mehta S, Garg R. Anaesthetic management of a child posted for excision of lymphangioma of the tongue. Singapore Med J. 2012;53(3):e45-e48.
- Kayhan KB, Keskin Y, Kesimli MC, Ulusan M, Ünür M. Lymphangioma of the tongue: report of four cases with dental aspects. Kulak Burun Bogaz Ihtis Derg. 2014;24(3):172-176.
- Akyüz C, Ataş E, Varan A. Treatment of a tongue lymphangioma with sirolimus after failure of surgical resection and propranolol. Pediatr Blood Cancer. 2014;61(5):931-932.
- Usha V, Sivasankari T, Jeelani S, Asokan GS, Parthiban J. Lymphangioma of the tongue—a case report and review of literature. J Clin Diagn Res. 2014;8(9):ZD12-ZD14.
- Babu DB, Kumar BR, Boinepally NH, Gannepalli A. A case of intraoral lymphangioma circumscripta—a diagnostic dilemma. J Clin Diagn Res. 2015;9(10):ZD11-ZD13.
- Catalfamo L, Nava C, Lombardo G, Iudicello V, Siniscalchi EN, Saverio DPF. Tongue lymphangioma in adult. J Craniofac Surg. 2012;23(6):1920-1922.
- Bhayya H, Pavani D, Avinash Tejasvi ML, Geetha P. Oral lymphangioma: a rare case report. Contemp Clin Dent. 2015;6(4):584-587.
- Hong JP, Lee MY, Kim EK, Seo DH. Giant lymphangioma of the tongue. J Craniofac Surg. 2009;20(1):252-254.