Surgical Management of Haemangiolymphangioma An Infrequent Tumor in Oral Cavity: A Case Report and a Review

Hemangiomas are group of vascular anomalies which are not present at birth. They generally manifest within 3-4 weeks of life, consists of a rapid proliferative phase, stable plateau phase and slow involuting phase. Hemangiomas of the oral cavity are not commonly observed though head and neck are common sites. Histologically it may be evident in some cases that lymphatic channels are present in hemangiomas, and then it is be categorized according to the predominant component either Haemangiolymphangioma or lymphangiohemangiomas. All most all hemangiomas involute with time, but 10-20% of true hemangiomas don’t involute and require post-adolescent surgical treatment. In this article a case of Haemangiolymphangioma of right buccal Case Study mucosa in a 22-year-old male patient is reported. Various treatment modalities and their clinical implication have also been reviewed.


INTRODUCTION
Hemangioma is a common soft tissue head and neck tumor which is relatively rare in the oral cavity [1]. It can be mucosal, with involvement of the lining of oral cavity; cutaneous, involving skin, lips and deeper structures; intra-osseous, involving mandible and / or maxilla or intramuscular, involving masticator and peri oral muscles [2].
According to Mullicken and Glowacki classification vascular anomalies can be subtyped into two main categories: Vascular malformations and tumours [3,4]. Infantile hemangiomas being the most common type of vascular tumor consisting of endothelial cells which are rapidly proliferating [5]. Incomplete blood vessel architecture and hyperplastic cells characterize hemangiomas. The vascular malformations on the contrary do not consist of these hyperplastic cells, but contain progressively enlarging aberrant vessels which can be of any type such as lymphatic vessels, veins, venules, arteries, capillaries or mixed vessel form. Lymphangiomas or lymphatic malformations are ectatic lymph vessels collection congenitally that makes endothelial cell lined cystic spaces. Now sometimes these channels may be filled with blood, thus forming a mixed Haemangiolymphangioma, an uncommon developmental anomaly with an ability to invade and recur locally, which differentiates it from the simple lymphangioma or hemangioma [6,7]. It is a benign disorder, but invasion into local tissues can lead to a deformity [6].
Management and treatment of these lesions depend on factors such as age of the patient, extent and size of the lesion, finally clinical features.

CASE REPORT
A 22-year-old male patient came to Department of Oral and Maxillofacial Surgery, Sri Rajiv Gandhi College of Dental Sciences & Hospital, Bangalore, Karnataka, India, in October 2013, with the complaint of a discolored swelling on inner aspect of right cheek with occasional bleeding since last 10 years. Patient noticed a pea sized swelling 10 years back which remained same for 8 years then gradually increased over the past 2 years to reach the present size. The swelling increased on bending forwards and exercising. The swelling was initially small and increased gradually to the present size in last 2 years. Past medical and family history was not specific and noncontributory. Local examination revealed multiple swelling over right s buccal mucosa in relation to 43, 44, and 45 of size approximately 2 × 1 cm ( Fig. 1).

Fig. 1. Intra oral lesion on right labial mucosa
The 3 swelling were respectively of 2, 1, and 0.5 cm in greatest dimensions. It was not ulcerated. On palpation the lesion was lobulated; soft in consistency, non-tender and pulsatile. On palpation of lesion, bleeding was not elicited. Lymph nodes were not palpable. Provisional diagnosis of slow flow malformation of right buccal mucosa was made. No obvious feeder vessel was identified on ultrasonography, so there was no need for an angiogram study. For crossing out bony involvement intracranially computed tomography to head and Orthopantomogram (OPG) were taken, which showed no abnormalities (Figs. 2 and 3).
Excision of the lesion was carried out under general anesthesia. No feeder vessel was identified intraoperatively. Bleeding was encountered mild to moderately and was successfully controlled by cauterization. Excision was wide and complete and primary closure was achieved. Specimen obtained was soft in consistency and bluish in color (Fig. 4).
Histopathological examination was carried out for specimen. Haematoxyline and eosin stain sections shows atrophic stratified squamous epithelium. The connective tissue shows numerous single layer endothelial lined lymphatic channels, also endothelial lined capillaries without adventitia filled with RBC's. Few sinusoidal spaces are also seen subepithelially. In connective tissue collagen fibres is loosely arranged with normal component of salivary and muscle tissue is minimal inflammatory infiltrate (Fig.  5). A diagnosis of Haemangiolymphangioma was made. Patient was put on continuous follow-up and no recurrence was noted till date.

DISCUSSION
Hemangiomas, a common soft tissue tumor are generally congenital or develop in the neonatal period growing rapidly. They usually cover a large site, and undergo resolution [8,9]. They may occur in the oral region including gingiva, palate, buccal mucosa, lips, jawbone [10,11]. Hemangiomas present with clinical features such as missing teeth, root resorption, asymmetry of the face, delayed eruption, early exfoliation of primary teeth, paresthesia, pulsation, expansion of bone, blanching of tissue, pain, mobility of teeth and spontaneous bleeding [10,12]. These hemangiomas are known to be associated with syndromes such as, von Hippel-Lindau syndrome, Sturge-Weber-Dimitri syndrome, Rendu-Osler-Weber syndrome [13,14]. Bony and intracranial involvement was ruled out by CT Head and OPG in our case.
Histopathologically, hemangioma progresses from a scenario of proliferated endothelial cells to well formed capillaries which appear in lobulated messes in later stages. In some instances intermingled lymphatic channels can also be present and diagnosis is given according to dominant component [7]. In the present case there were interspersed lymphatic channels, but dominant component was of hemangioma, so diagnosis of Haemangiolymphangioma was made.
Diagnosis of the type of vascular lesion influences treatment considerably. Angiographic studies can be used but are not strictly indicated for diagnosis of hemangiomas, and help only to establish the extent and the size of the lesion [12]. CT and MRI could be utilized successfully in diagnosis of the lesion [14]. In the present case after reaching the diagnosis the lesion was planned for wide surgical excision and primary closure. There was no intraoperative complication and healing after primary closure was uneventful.
There are various other treatment modalities like injection of fibrosing agents intralesionally, radiation, cryosurgery, embolization, electrocoagulation, interferon a-2b and laser therapy [5,15]. Chylous Leakage is a rare complication that can occur during surgery and is characterized by the collection of chylous fluid. Postoperative Chylous leakage mainly develops as a result of surgical trauma to lymphatic channels [16].
Bogdan et al. [17] advocated the use of diode laser or Er:YAG laser for effectively treating hemangiomas and vascular malformation by photocoagulation. They stated that this can achieve reduction in size of the lesions without effecting function and cosmetics.
Bonet-Coloma et al. [18] conducted an observational retrospective study, they observed that haemangiomas are usually present in children. Most common location in the oral cavity is lip. Authors stated that surgical excision is the treatment of choice.
Plasma knife (PK) is a latest technique for excision of these lesions. This technology uses the intra and extracellular fluid as a conducting medium to allow the flow of radiofrequency [19]. They used a combination of 90% coagulation and 10% cut mode for the excision of the haemangioma. It had the advantage of hemostasis also and can be applied for small sized and superficial haemangiomas of the oral cavity.

CONCLUSION
It is true that hemangiolymphangiomas are not common in oral cavity but all suspicious cases should be properly investigated for intracranial, bony and systemic involvements before going forward for treatment. Surgical excision with sufficient margins is the mainstay of the treatment for such cases which do not have a feeder vessel. An electrocautery is an advantageous way for excising such vascular lesions as it aids in providing a bloodless field with less incidences of intraoperative bleeding episodes. Nevertheless, for cases of haemangiolymphangioma a long term follow up is a necessity to rule out any recurrence.

CONSENT
All authors declare that 'written informed consent was obtained from the patient for publication of this case report and accompanying images'.

ETHICAL APPROVAL
It is not applicable.