Extensive tongue lymphangioma followed for eight years: Case report

Francisco Nunes Junior; Everton J da Silva; Ana L. L. M Paz; Luiz E. R Volpato

doi:10.4103/jomfp.jomfp_511_20



CASE REPORT

Year : 2023 \| Volume : 27 \| Issue : 5 \| Page : 6-9

Extensive tongue lymphangioma followed for eight years: Case report

Francisco Nunes Junior¹, Everton J da Silva², Ana L. L. M Paz², Luiz E. R Volpato²
¹ Department of Dentistry, Mato Grosso Cancer Hospital, Cuiabá, MT, Brazil
² Department of Dentistry, Mato Grosso Cancer Hospital; Cuiabá School of Dentistry, University of Cuiabá, Cuiabá, MT, Brazil

Date of Submission	18-Dec-2020
Date of Decision	15-May-2022
Date of Acceptance	19-May-2022
Date of Web Publication	04-Feb-2023

Correspondence Address:
Luiz E. R Volpato
Av. Manoel José de Arruda, 3100, Cuiabá, MT, CEP 78065-700
Brazil

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jomfp.jomfp_511_20

Abstract

Lymphangiomas are benign hamartomatous tumours similar to lymphatic vessel neoplasms, originating from lymphatic tissue sequestration and may or may not communicate with the rest of the system. There are several treatment options for lymphangioma, such as surgery, sclerotherapy, cryotherapy, lasers, steroids and bleomycin. Although surgery is the most indicated treatment, it can result in severe sequelae and loss of function in patients. The present case shows a 5-year-old patient diagnosed with extensive tongue lymphangioma and, due to the mutilation that would be caused by its complete excision, the option was for more conservative treatments such as laser therapy and cryotherapy. After follow-up for 8 years, the patient presents with preserved functions and controlled lesion.

Keywords: Cryotherapy, lasertherapy, lymphangioma, tongue

How to cite this article:
Junior FN, da Silva EJ, Paz AL, Volpato LE. Extensive tongue lymphangioma followed for eight years: Case report. J Oral Maxillofac Pathol 2023;27, Suppl S1:6-9

How to cite this URL:
Junior FN, da Silva EJ, Paz AL, Volpato LE. Extensive tongue lymphangioma followed for eight years: Case report. J Oral Maxillofac Pathol [serial online] 2023 [cited 2023 Mar 22];27, Suppl S1:6-9. Available from: https://www.jomfp.in/text.asp?2023/27/5/6/369185

Background

Lymphangiomas are benign hamartomatous tumours similar to lymphatic vessel neoplasms, originating from lymphatic tissue sequestration and may or may not communicate with the rest of the system.^[1]

Approximately 75% of lymphangiomas occur in the head and neck region.^[2] Within the oral cavity, the tongue appears to be the most common site of involvement.^[3] Tongue lymphangioma may present as localised or diffuse growth, such as nodular and elevated lesions above the surface of the tongue.^[4],[5]

It is estimated that half of all lesions are noted at birth, and 90% develop at around 2 years of age.^[6] They are characterised by small vesicles grouped in plaques, skin or mucosa, and may contain translucent or slightly haemorrhagic content.^[7]

There are several treatment options for lymphangioma, including surgery, sclerotherapy, cryotherapy, lasers, steroids, bleomycin. Although surgery is the most appropriate treatment, it can result in severe sequelae and loss of function in patients.^[8],[9]

This study presents the case of a child patient diagnosed with extensive lymphangioma on the tongue followed up for 8 years.

Procedure

A 5-year-old Caucasian male patient was diagnosed with lymphangioma on dorsal part of the tongue by means of an incisional biopsy, which evolved with a tongue enlargement on the 3^rd postoperative day, resulting in dysphagia and risk of dyspnea, worsening the case on the 5^th and 6^th days^[10] [Figure 1].

Figure 1: (a) Initial clinical appearance; (b) clinical appearance of the tongue on the fifth postoperative day of the lingual biopsy showing significant oedema; (c) computed tomography showing volumetric increase of the tongue; (d) clinical appearance on the sixth postoperative day after the first laser therapy session^[10]

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Given the apparent failure of drug therapy with corticosteroids and antibiotics, adjunctive treatment with laser therapy was instituted, with regression of the case in the first session and regression of oedema within 5 days.

For 20 months the patient remained in biweekly follow-up with laser therapy, using Laser Control (Kondortech, São Carlos, Brazil) in the following parameters: 655 nm wavelength (visible red), 40 mW of power at a dose of 3.0 J/cm², totaling 36 intra-oral and 36 extra-oral J, until he abandoned treatment.

At the 29^th month, the patient returned and cryotherapy was started in the dorsal and ventral parts of the tongue. On follow-up at 30, 31 and 32 months, a clear regression of the lesion was observed, with a reduction in granulomatous appearance, exophytic projections and vesicles. After this period the patient discontinued treatment.

At the 37^th month the patient returned, and a new cryotherapy session was performed. He did not attend the appointment scheduled for the following month.

The patient then returned only at the 70^th month, and underwent cryotherapy at 71^st, 72^nd, 73^rd and 74^th months, abandoning treatment after that. He returned at the 79^th month maintaining cryotherapy at 80^th, 81^st, 82^nd, 83^rd and 84^th months. At this moment, verifying the stabilisation of the clinical picture, the patient's parents were instructed to return only if they noticed an increase of the lesion.

Patient then returned at the 98^th month. At this time, the presence of new vesicular pinkish lesions on the right lateral border of the tongue was noted. There was also a lesion on the right ventral part of the tongue papule-like, pediculated, pinkish in color with a vesicle at the apex. Incisional lesion biopsy was performed followed by laser therapy and cryotherapy in the immediate postoperative period. The diagnostic hypothesis of lymphangioma was confirmed by the histopathological examination [Figure 2].

Figure 2: Histopathological examination reveals mucosal fragments covered by keratinised stratified squamous epithelium, formed by dense connective tissue, showing an increased number of lymphatic vessels in the just epithelial region. Capillaries and lymphoid aggregate are present

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Currently, the patient is undergoing biweekly cryotherapy treatment at 99^th, 100^th, 101^st, 102^nd, 104^th and 106^th months [Figure 3]. The evolution of the case and its follow-up are summarised in [Table 1].

Figure 3: Clinical aspect after 8 years of follow-up

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Table 1: Case follow-up history by month of return

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Discussion

The present case shows an 8-year follow-up of a patient diagnosed with extensive tongue lymphangioma, who had a life-threatening complication upon incisional biopsy to confirm the diagnosis.^[10] No similar case report was found with this follow-up and cryotherapy showed to be an effective treatment alternative, little reported.

Lymphangiomas are benign hamartomatous tumours similar to lymphatic vessel neoplasms, originating from lymphatic tissue sequestrations, considered developmental malformations and not true neoplasms. They may also be referred to as cystic hygroma, cystic lymphangioma or lymphangiomatosis.^[1] Lymphangioma may be congenital or acquired, usually is detected at birth or early childhood and is rare in adults.^[11] In the present case, lymphangioma was diagnosed only at 5 years of age, rather late compared to the vast majority of cases.

Possibly this was because, although the lesion was extensive, its clinical manifestation was quite mild and the patient had no symptoms. Its diagnosis was almost accidental – when the biopsy was performed on the dorsum of the tongue, the initial indication for the surgical procedure was to biopsy a lesion with diagnostic hypothesis of ranula at the mouth floor.^[10]

Lymphangioma may manifest in the skin or mucosa, occurring more frequently in the head and neck region.^[2],[12] In the case presented, the patient presented lymphangioma only in the tongue, the most affected site when in the oral cavity; it is rarely found in the palate, buccal mucosa, gingiva and lips.^[3],[13] When in the tongue, its anterior two thirds are usually predominant, and can cause macroglossia resulting in dysphagia, dysphonia and lingual bleeding in response to trauma.^[14],[15] This situation was also found in this case, since lymphoma affected the anterior two-thirds of the tongue, as well as lateral borders and, later, also the ventral tongue. Also in this case macroglossia is present, however there are no changes in functions.

Among the various approaches for treating lymphangioma, surgical excision is the most indicated. However, when in the tongue, its excision becomes difficult due to its infiltration in adjacent structures, which can lead to airway obstruction, nerve damage, infection, dysphagia and dysphonia and high recurrence rates.^{[16],[17],[18]} Due to the size of the lesion and the mutilation that would be involved in the surgical resection of lymphangioma,^[19] more conservative management was chosen from the outset, although previous studies have shown that surgical excision has higher success rates, especially in minor lesions.^{[6],[20],[21],[22]}

Other treatment modalities described for the treatment of lymphangiomas include injection of steroids, sclerosing agents, cryotherapy, laser therapy, radiotherapy and chemotherapy.^{[4],[22],[23],[24]} Initially, laser therapy was chosen in this case because it presented a good result on the complication after the first biopsy^[10] and because it is a therapy routinely used in the hospital.

Cryotherapy has been shown to be the treatment of choice for lymphangiomas that reach large proportions in various areas of the body.^[24],[25] In the present case, since lymphangioma had large proportions and the surgical treatment would be mutilating, the cryotherapy sowed to be effective. No similar reports were found in the literature.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Neville BW, Damm DD, Allen CM, Allen CM. Oral and Maxillofacial Pathology. 3^rd ed. Netherlands: Elsevier Inc; 2009. p. 547-9.
2.	Katsoulas N, Tosios KI, Argyris P, Koutlas IG, Sklavounou A. Lymphangioma circumscriptum, angiokeratoma, or superficial vascular ectasia with epithelial hyperplasia? Oral Surg Oral Med Oral Pathol Oral Radiol 2014;118:e53-7.
3.	Devi A, Narwal A, Yadav AB, Singh V, Gupta A. Classical cases of lymphangioma-As multiple vesicular eruptions. J Clin Diagn Res 2016;10:ZD22-3.
4.	Bloom DC, Perkins JA, Manning SC. Management of lymphatic malformations and macroglossia: Results of a national treatment survey. Int J Ped Otorhinolaryngol 2009;73:1114-8.
5.	Ikeda H, Fujita S, Nonaka M, Uehara M, Tobita T, Inokuchi T. Cystic lymphangioma arising in the tip of the tongue in an adult. Int J Oral Maxillofac Surg 2005;35:274-6.
6.	Usha V, Sıvasankari T, Jeelani S, Asokan GS, Parthiban J. Lymphangioma of the tongue-a case report and review of the literature. J Clin Diagn Res 2014;8:ZD12-14.
7.	Regezi JÁ, Sciubba JJ, Pogrel MA. Atlas of Oral and Maxillofacial Pathology. Rio de Janeiro: Guanabara Koogan; 2000.
8.	Fatima S, Uddin N, Idrees R, Minhas K, Ahmad Z, Ahmad R, et al. Lymphangioma circumscriptum: Clinicopathological spectrum of 29 cases. J Coll Physicians Surg Pak 2015;25:658-61.
9.	Hwang J, Lee YK, Burm JS. Treatment of tongue lymphangioma with intralesional combination injection of steroid, bleomycin and bevacizumab. Arch Craniofac Surg 2017;18:54-8.
10.	Marquezini LA, Almeida FL, Carvalhosa AA, Volpato MCPF, Castro PHS, Volpato LER. Postoperative complication with a risk of death after an incisional biopsy of mouth. Rev Bras Cir Cabeça Pescoço 2013;42:164-8.
11.	Patel NJ, Sciubba J. Oral lesions in young children. Pediatr Clin North Am 2003;50:469-86.
12.	Bhayya H, Pavani D, Avinash Tejasvi ML, Geetha P. Oral lymphangioma: A rare case report. Contemp Clin Dent 2015;6:584-7. [PUBMED] [Full text]
13.	Kheur SM, Routray S, Ingale Y, Desai R. Lymphangioma of tongue: A rare entity. Indian J Dent Adv 2011;3:635-7.
14.	Bernnan TD, Miller AS, Chen S. Lymphangiomas of the oral cavity: A clinicopathologic, immunohistochemical and electron microscopic study. J Oral Maxillofac Surg 1997;55:932-5.
15.	Guelmann M, Katz J. Macroglossia combined with lymphangioma: A case report. J Cli Pediatr Dent 2003;27:167-70.
16.	Kataria P, Passey JC, Agarwal AK. Lymphangioma circumscriptum of the tongue: successful treatment using intralesional bleomycin. J Laryngol Otol 2009;123:1390-2.
17.	Hartl DM, Roger G, Denoyelle F, Nicollas R, Triglia JM, Garabedian EN. Extensive lymphangioma presenting with upper airway obstruction. Arch Otolaryngol Head Neck Surg 2000;126:1378-82.
18.	Brock ME, Smith RJ, Parey SE, Mobley DL. Lymphangioma. An otolaryngologic perspective. Int J Pediatr Otorhinolaryngol 1987;14:133-40.
19.	Hage CA, Araújo JS, Silva PF, Gonçalves FL, Pontes HA. Microcystic lymphangioma of the tongue: Case report. Rev Cir Traumatol Buco-Maxilo-Fac 2016;16:49-52.
20.	Mousumi G, Sanjay S, Gokkulakrishnan S, Singh A. Lymphangioma of the tongue. Natl J Maxillofac Surg 2011;2:86-8.
21.	Vignes S, Arrault M, Trévidic P. Surgical resection of vulva lymphoedema circumscriptum. J Plast Reconstr Aesthet Surg 2010;63:1883-5.
22.	Chakravarti A, Bhargava R. Lymphangioma circumscriptum of the tongue in children: Successful treatment using intralesional bleomycin. Int J Pediatr Otorhinolaryngol 2013;77:1367-9.
23.	Bond J, Basheer MH, Gordon D. Lymphangioma circumscriptum: Pitfalls and problems in definitive management. Dermatol Surg 2008;34:271-5.
24.	Yanazume S, Douzono H, Kubo H, Nagata T, Douchi T, Kobayashi H. Cryotherapy for massive vulvar lymphatic leakage complicated with lymphangiomas following gynecological cancer treatment. Jpn J Clin Oncol 2014;44:1116-9.
25.	Kang J, Qiao F, He S, Wang Y, Nie L, Ji F, et al. Treatment of cystic lymphangioma by transcatheter lymphatic sclerotherapy and embolization. J Vasc Interv Radiol 2015;26:455-7.