Management of recurrent lymphangioma of the cheek in an adult patient: An interdisciplinary approach

Abstract

Lymphangiomas are congenital malformations. Swellings in the cheek may arise from infection, cyst and tumors.Pathological lesions are not always confined to its common site and age of occurrence. Occurrence of lymphangioma in buccal mucosa especially in adults are very rare. Exact knowledge about anatomy and nature of lesion is mandatory for its complete removal as incomplete removal results in recurrence. Herewith,a rare case of recurrent lymphangioma involving left buccal mucosa in an adult is presented with its management. Emphasis is laid on the necessity of combined approach through multidisciplinary team while treating such lesions.

1. Introduction

Lymphangiomas are benign tumors which are congenital in nature affecting lymphatic system.¹ Nearly 50% of the lesions are present during birth and the rest develop during growth period.² Development of lymphangioma in adults are comparatively rare and these lesions have no gender predilection.Complete removal of very large lesions is not always possible through surgery, because of the proximity of the lesion to vital structures and due to the diffusion of lesion between the muscle fibers.³ Therefore management with sclerosants has been considered as an effective alternative.⁴ Lymphangioma rarely occur in adults and in the cheek region and therefore in this article, management of a recurrent lymphangioma of an adult patient through combined modality by an interdisciplinary team has been described.

2. Case report

A 52-year-old female reported with chief complaint of recurrent swelling on the left side of the cheek of four months duration with intermittent intraoral bleeding which was treated by surgery a year before presentation. On extra oral examination, a diffuse swelling measuring about 3 × 4 cm was present superoinferiorly from left malar region to inferior border of the mandible, mediolaterally from the left modiolus of mouth till 1.5cm anterior to the left tragus(Fig. 1). Palpation revealed non tender, soft, fluctuant swelling with no warmth or thrill or lymphnadenopathy. Intraorally left buccal mucosa was red with intermittent blood discharge.

Fig. 1.

Preop to two years follow up.

Ultrasound revealed well-defined mixed echogenic lesion in subcutaneous left maxillary region with minimal vascularity in the periphery. Contrast CT of Head and Neck showed the presence of a well-defined lobulated cystic lesion in the left buccal space. There was neither erosion of maxilla or mandible nor encroachment of retromolar trigone, maxillary sinus, neighboring anatomical spaces, muscles and glands(Fig. 1).

Based on clinical, radiological and FNAC findings, the lesion was provisionally diagnosed as lymphangioma involving left buccal mucosa. Since the lesion was diffuse, complete surgical excision was not a possibility. Hence it was decided to do the management in stages (Fig. 2) with the help of an interdisciplinary team through intralesional injection of bleomycin followed by surgery. (Combined modality).

Fig. 2.

Management in stages.

3. Surgical procedure

An Oral & maxillofacial surgeon, interventional radiologist and anesthetist were involved as a team and the procedure was done in three stages.

3.1. Stage −1

Under IV sedation with Midazolam, the interventional radiologist did ultrasonagram to enable the surgeon to visualize the cystic nature of the lesion. Aspiration of cystic contents from many micro and macrocystic spaces as guided by USG was done with 23 gauge spinal needle. Aspirated fluid was reddish in color which was sent for microbiologic evaluation.

15mg Bleomycin was diluted in 15ml of Normal Saline (NS) and 3 ml Local anesthesia. (1mg/1 ml) Bleomycin was injected into the lesion at multiple sites with the guidance of USG. Care was taken to insert the needle for aspiration of cystic contents and detaching the syringe from the hub without disturbing the needle and then injecting the prepared bleomycin into the emptied cystic spaces under ultrasound guidance. This protocol was followed to prevent spill of the medication on the skin to prevent skin ulcers and reactions. After confirming that, the entire lesion was examined ultrasonographically and all the cystic spaces were aspirated of its cystic contents and injected with Bleomycin. An intraoral bolus and extra oral dressing pressure was placed. After 1st intralesional injection of bleomycin, the patient developed mild fever, pain and swelling in the immediate post operative period (Fig. 2).

3.2. Stage-2

After 1 month, she was posted again for the 2nd dose of intersional bleomycin and the whole procedure as stated above was repeated. Patient developed darkening of skin over left cheek on second post op day which resolved in two weeks (Fig. 2).

3.3. Stage-3

After one month of the second stage procedure, surgical excision of the lesion under general anesthesia was done. Vestibular incision was given from 21 to 27 region. The lesion was well defined due to shrinkage fibrosis. Hence it was dissected out from periosteal layer, left zygomatic buttress region infratemporal and buccal fat pad. The excised lesion was sent for biopsy. The superficial defect was primarily closed with a pressure dressing and patient was followed for two years with no recurrence (Fig. 2).

Histopathologically, the lesion was described to contain loose fibrillar connective tissue with numerous lymphatic vessels of varying sizes filled with lymph. Lymphatic channels lined by single layer of endothelial cells in some areas and multilayered in other areas. Final diagnosis was given as Lymphangioma involving left buccal mucosa. (Figue 2).

4. Discussion

Lymphangioma was first described by Redenbacher in 1828 and the term was coined by Mulliken and Glowacki in 1982.⁵ Lymphangiomas, though common in head and neck region can also occur in proximal extremities, buttocks and trunk.In oral cavity lymphangiomas are commonly found in anterior two-thirds of the tongue, palate, buccal mucosa, gingiva, lips and rarely found in soft palate and retromolar region.6, 7, 8

Whimster in 1976 described the basic pathological process of a lymphangioma as the collection of lymphatic cisterns, in the deep subcutaneous vein. It is presumed that they develop from congenital obstruction of sequestered primitive lymphatic enlargement.⁶ Depending on the anatomical location, it may appear as a swelling on the face as seen in our case.Obstruction of the upper airway, tongue extrusion, deformity of the jaw,difficulties in mastication, speech can be other symptoms. Superficial lesions will have “frog egg” or “tapioca-pudding appearance” while deeper lesions are ill-defined masses.⁹

Complete removal of lesion and restoration of function and cosmesis remains the primary goal while treating such lesions. To achieve this, various treatment modalities such as radiation therapy, cryotherapy, electrocautery, steroid administration, laser have been mentioned in literature.^6,10 Among which, surgical excision and sclerotherapy remains most popular. However, no clear treatment algorithm is still available. Although surgical excision is a definitive treatment for lymphangiomas, it may be difficult due to infiltrative nature and thin-wall of these lesions.They often do not respect tissue planes which can lead to high incidence of complications. To avoid the recurrence associated with surgical excision, serial injections of sclerosing agents have been preferred as it gives satisfactory results as done in our patient.¹¹

Intralesional sclerosants cause irritation of endothelial lining which leads to fibrosis. Several studies have used bleomycin with promising results.¹² Bleomycin was first used for treatment of lymphatic malformation in 1977 due to its sclerosing property which leads to fibrosis of the lesion.¹³ Bleomycin is directly cytotoxic to keratinocytes and ecrine epithelium.Most of the side effects such as pain, erythema, swelling, ulceration, hyperpigmentation, hypopigmentation, pulmonary fibrosis are seen with systemic therapy of Bleomycin.¹⁴ Our patient developed mild pain, swelling and hyperpigmentation of skin post injection which subsided within ten days.

Though sclerotheraphy reduces the size of the lesion and makes the ill-defined margins more defined, combination therapy (sclerotherapy followed by surgery) is effective in complete removal of the lesion as seen in our case.Spontaneous regression of lympangiomas are rare. In our patient, we had a follow up for 24 months with no recurrence (Fig. 1).

5. Conclusion

Comprehensive management of this lesion involving specialists from Oral medicine, Oral surgery, Oral Pathology, Interventional Radiology and Anesthesia has been shown in this article. Successful management of this lesion was possible due to this multidisciplinary approach. Surgeon should not hesitate to seek help from other specialties whenever needed.