Lymphangioma of the tongue - A Case Report

INTRODUCTION

Hamartomas of the head & neck region comprise a heterogeneous group of lesions exhibiting a varied clinical presentation, course and histopathology. Lymphangioma is the hamartoma of lymphatic vessels accounting for 75% of all cases in the head & neck region¹. 

Lymphangioma was first described in 1854 by Virchow² & is considered to be the congenital malformation of the lymphatic vessels³ and its occurrence in oral cavity is relatively rare. Oral lymphangiomas frequently occur in the anterior 2/3 rd of the tongue, but can involve palate, buccal mucosa, gingiva and lips³ . It may present as a localized or a diffuse growth⁴.

Classification of lymphangiomas is primarily dependent on their size and are classified as Lymphangioma simplex (Capillary lymphangioma), Cavernous lymphangioma & Cystic lymphangioma (Cystic hygroma).³

Diagnosis of lymphangioma by histopathological means is relatively straight forward and carries few differentials histologically. The present case report highlights the varied clinical presentation of lymphangiomas and summarizes its current concepts on pathogenesis.

CASE REPORT

A 25 year old man reported with a chief complaint of an ulcer and burning sensation of the tongue for the past seven years. There was no history of a similar lesion occurring at birth.

There was gradual enlargement of tongue and patient had mild speech problems and difficulty in maintaining good oral hygiene.

Intra - oral examination revealed multiple small vesicles on the tip, involving both the dorsal & ventral surfaces of anterior 1/3 rd of the tongue (Fig 1). Vesicles were translucent and painful. Right submandibular lymphnode was palpable, mobile and soft in consistency. Considering the clinical diagnosis of Lymphangioma, surgical excision of the lesion was done with primary closure.

Patient was evaluated clinically for clinical findings to rule out syndromic association of Turners syndrome, Downs syndrome & Noonan syndrome.

Microscopic examination revealed an unencapsulated lesion which showed multiple variable- sized lymphatic vessels lined by a single layer of flat endothelial cells surrounded by loose fibro- vascular stroma. Few dilated lymphatic vessels containing eosinophilic proteinaceous lymph were seen in the superficial lamina propria (Fig 2, 3& 4). Few aggregates of lymphocytes were seen along with plasma cells in the stroma. Lymphatic channels infiltrating the muscle bundles were seen (Fig 5). Considering the above histopathological features, the diagnosis of Cavernous Lymphangioma was suggested. On follow-up of 3 years, after surgery, there was an improvement in the tongue function.

DISCUSSION

Hamartoma is a focal overgrowth of normal cells that are indigenous to the site of involvement⁵ . One such example in the oral cavity is Lymphangioma. Histogenesis of lymphangioma is closely related to the maturation of the lymphatic system⁶. Various theories have been put-forth to understand the mechanism of formation of lymphangioma.

1. Sequestration of lymph tissue

2. Abnormal budding of lymph vessels

3. Lack of fusion with venous system

4. Obstruction of lymph vessels.⁶

Lymphangiomas are usually diagnosed in newborns or infants and they may become clinically apparent later in adults. In such situations, pathologic conditions associated with an increased lymphatic volume leads to formation of lymphangioma ⁶. Suk et al consider that the origin of sudden occurrence of lymphangiomas in adults is due to a delayed proliferation of cell rests. Trauma or infection can acts as  triggering stimuli to its proliferation.⁶

Lymphangiomas frequently involve the anterior 2/3 rd of dorsum of tongue³. Bilateral symmetric lymphangiomas of gingiva have been reported in literature.⁷ Rarely the parotid gland can also be involved ⁸. Clinically, it may display varied presentations. Superficial lesions show pebbly, translucent vesicle- like, frog egg or tapioca- pudding appearance. Color of the lesion may vary from yellow to purple⁹. Deeper lesions present as submucosal nodules or masses without any significant change in texture or color.^1,3

Oral lymphangioma is considered an important cause of macroglossia in children. It may result in speech disturbances, poor oral hygiene & bleeding from tongue associated with oral trauma^3. It is associated with Turner's syndrome, Noonan's syndrome, Trisomies, cardiac anomalies, fetal hydrops, fetal alcohol syndrome & familial pterygium colli.³

Clinical differentials of lymphangioma includes hemangioma, Downs syndrome & Neurofibromatosis, all of which may result in macroglossia.¹⁰

Microscopically, lymphangiomas are distinctive, being composed of capillary and cavernous lymph sinuses lined by cytologically bland and seldom with prominent endothelium.¹⁰ Poor circumscription is the rule along with lymph channels dissecting the muscle bundles at their periphery. Overlying epithelium is thin or acanthotic, verrucous and hyperkeratinized.¹¹

Lymphangiomas are seen in association with vascular and smooth muscle proliferation and these variants are termed as hemangiolymphangioma & lymphangiomyoma respectively. Recently, a lymphangioma- like variant of Kaposi's sarcoma has been reported.¹²

Lymphangiomas have to be distinguished histopathologically from cavernous hemangioma¹², acquired progressive l y m p h a n g i o m a ( L y m p h a n g i o e n d o t h e l i o m a ) , lymphangiomatosis & lymphangiosarcoma.⁶

Lymphatic vessels differ from blood vessels by having a discontinuous or fenestrated basement membrane. Unlike blood vessels, they are low flow, low pressure system which lack tight interendothelial junctions. They are less coagulable due to lack of platelets & erythrocytes.¹³ Specific markers for lymphatics are CD 31, factor VIII -associated antigen, Ulex europaeus¹², Lyve-1 ( Lymphatic endothelial hyaluron receptor-1), Podoplanin, Prox-1, VEGF- C ( Vascular endothelial growth factor), & VEGFR-3(Vascular endothelial growth factor receptor)⁶

Lymphangiomas are treated by surgical excision, radiation therapy, cryotherapy, sclerotherapy, steroid administration, embolization, ligation, laser therapy & radiofrequency tissue ablation techniques³. Prognosis of lymphangiomas remain good and larger lesions of neck and tongue may cause airway obstruction and death².

A case of lymphangioma of tongue diagnosed in a 25-yearold male patient who presented with multiple vesicles involving anterior dorsum and ventral surface is described. Histopathology showed classic morphological features of lymphangioma of cavernous type. No evidence of relapse was observed with an improvement in tongue function.

CONCLUSION

Lymphangiomas can be encountered in any age group. The complications involved with the management of lymphangiomas can be functional & psychological and may also cause cosmetic disfigurement³. Early detection & intervention will help in reducing these complications. Understanding the biological processes involved with lymphangiogenesis are incentives to the current knowledge and clinical implications of lymphangiomas.