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Ugurlu, Kaya, Akdogan, Olgac, and Sipahi: Oral lymphangioma of the alveolar ridge – a report of a rare case

Introduction

Lymphangiomas are uncommon hamartomatous tumours of the lymphatic vessels with a marked predilection (≈75%) for the head, neck and oral cavity. They are noted at birth in ≈50% of patients and 90% of the time appear before the age of 2 years.1 Lymphangiomas are developmental malformations arising from sequestration of the lymphatic tissue such that it does not communicate with the normal lymphatic channels.2 Lymphangiomas can also occur in association with haemangioma, Turner syndrome, Noonan syndrome, trisomies, cardiac anomalies, fetal hydrops, fetal alcohol syndrome and familial pterygium colli.3

Oral lymphangiomas may develop at various sites, but the anterior two-thirds of the tongue is the most frequent location, often resulting in macroglossia. They can also present on the palate, buccal mucosa, gingiva and lips. The tumour is superficial in location and has a pebbly surface that resembles a cluster of translucent vesicles. Deeper lesions appear as a nodule or mass without significant changes in surface texture or colour.4,5 Oral lymphangiomas may reach sizes large enough to cause difficulties in mastication and speech. In this report, we present the rare case of a patient with an oral lymphangioma involving the alveolar ridge of the maxilla.

Case report

A 51-year-old male patient was referred to our clinic because of a graduated purple mucosa covering the alveolar ridge of the posterior right maxilla. Intraoral examination revealed a diffuse, non-ulcerated, blue–purple lesion of approximately 3 × 2 cm located on the posterior crest of the alveolar ridge and extending to the buccal surface of the right maxillary ridge (Figure 1). The medical history of the patient was unremarkable. No radiological abnormality was diagnosed on panoramic view and dental volumetric tomography. No unusual relationships between vessels were seen on angiography. On the basis of his history and the clinical and radiological features of the lesion, an excisional biopsy was carried out. With the patient under local anaesthesia, the lesion was removed by surgical excision. After controlling bleeding, primary wound closure was achieved with the buccal flap technique. One week later, the sutures were removed. Histopathological examination of the tissue specimen by microscopy showed numerous dilated lymphatic vessels lined with thin endothelial cells in fibrous connective tissue and a stratified squamous epithelium (Figure 2). The lesion was confirmed to be a lymphangioma. No recurrence has occurred, and follow-up of the patient continues (Figure 3).

FIGURE 1

Blue–purple mucosa on the posterior maxillary ridge.

HMJ-9-3-5-fig1.jpg
FIGURE 2

Lymphatic channels lined by thin endothelial cells and a stratified squamous epithelium (×200, haematoxylin–eosin stain).

HMJ-9-3-5-fig2.jpg
FIGURE 3

Ten days post operation.

HMJ-9-3-5-fig3.jpg

Discussion

Lymphangiomas are hamartomatous congenital malformations of the lymphatic system. They are believed to arise from lymph sac sequestration and enlarge because of a lack of communication with the central lymphatic channels, resulting in inadequate drainage or excessive secretions from the lining cells.6 They commonly present as a painless soft mass that gradually enlarges, and, for a long period of time, becomes relatively quiescent, interrupted by occasional periods of enlargement and shrinkage until a residual mass remains.7 These remnants retain the capacity to proliferate. The accumulation of a large volume of fluid accounts for their cystic appearance.8

Histopathologically, three forms of lymphangioma are recognized:9

  • lymphangioma simplex (capillary lymphangioma) consisting of small, capillary-sized vessels;

  • cavernous lymphangioma comprising large, dilated lymphatic vessels;

  • cystic lymphangioma with large macroscopic cystic spaces.

According to their clinical presentation, they are classified into three different types:10

  • macrocystic (cavities > 2 cm3);

  • microcystic (cavities < 2 cm3);

  • mixed (combining these two types).

On the basis of their anatomical involvement, lymphangiomas are classified into six stages/classes:11

  • stage/class I: infrahyoid unilateral lesions;

  • stage/class II: suprahyoid bilateral lesions;

  • stage/class III: suprahyoid or infrahyoid unilateral lesions;

  • stage/class IV: suprahyoid bilateral lesions;

  • stage/class V: suprahyoid or infrahyoid bilateral lesions;

  • stage/class VI: infrahyoid bilateral lesions.

Three-quarters of lymphangiomas occur in the head and neck region. The most common intraoral site is the tongue, but the palate, buccal mucosa, gingiva and lips are additional sites of involvement. Macroglossia is common in patients with lymphangiomas of the anterior two-thirds of the tongue.12 Lip involvement and the attendant deformity are referred to as macrocheilia. In a study by Levin et al.,13 lymphangiomas of the alveolar ridges were detected in 3.7% of 1470 African-American neonates. These lymphangiomas consisted of blue, domed, fluid-filled swellings on the posterior crest of the alveolar ridge and posterior lingual surface of the mandibular ridge.13 The clinical differential diagnosis of lymphangioma includes lipoma, salivary retention phenomena and haemangioma. The diagnostic workup is based on the history, clinical features and biopsy from histopathological examinations. The treatment of lymphangioma depends on the type, size and location of the lesion, the involvement of anatomical structures and the infiltration of adjacent tissues. The various treatment options are surgical excision, radiation therapy, cryotherapy, electrocautery, sclerotherapy, steroid administration, embolization, ligation, laser surgery using neodymium-doped yttrium aluminium garnet, carbon dioxide and radiofrequency tissue ablation.14

Conclusion

  • Intraoral lymphangiomas more frequently occur on the dorsum of tongue, followed by palate, buccal mucosa, gingiva and lips, and the onset of lymphangiomas is either at birth (60–70%) or up to 2 years of age (90%) and rarely in adults.1115 This report presented a unique case of intraoral lymphangioma on the posterior alveolar ridge of the maxilla in 51-year-old patient, which was managed by surgical excision.

References

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Leboulanger N, Roger G, Caze A, Enjolras O, Denoyelle F, Garabedian EN. Utility of radiofrequency ablation of hemorrhagic lingual lymphangioma. Int J Ped Otorhinolaryngol 2008; 72:953–8. http://dx.doi.org/10.1016/j.ijporl.2008.03.013

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de Queiroz AM, Silva RA, Margato LC, Nelson FP. Dental care management of a young patient with extensive lymphangioma of the tongue: a case report. Spec Care Dentist 2006; 26:20–4. http://dx.doi.org/10.1111/j.1754-4505.2006.tb01505.x

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Shafer’s WG, Hine MK, Levy BM, Rajendran R, Sivapathasundharam B. Benign and malign tumors of the oral cavity. In: Shafer’s Textbook of Oral Pathology, 5th edn. Delhi, India: Elsevier India Pvt Ltd; 2006, pp. 207–8.

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Levin LS, Jorgenson RJ, Jarvey BA. Lymphangioma of the alveolar ridges ın neonates. Pediatrics 1976; 58:881–4.

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Neville BW, Damm DD, Allen CM, et al. Salivary gland pathology. In: Neville BW, Damm DD, Allen CM, Bouquot JE (eds) Oral and Maxillofacial Pathology, 3rd edn. The Netherlands: Elsevier Inc.; 2009, pp. 547–9.

15. 

Bhayya H, Pavani D, Avinash Tejasvi ML, Geetha P. Oral lymphangioma: a rare case report. Contemp Clin Dent 2015; 6:584–7. http://dx.doi.org/10.4103/0976-237X.169851


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