Capillary hemangioma: An occasional growth of attached gingiva.

The capillary hemangiomas represent developmental hamartomatous lesions of vascular tissue and their growth stops after certain period of time, following which some of the hemangiomas may involute. Capillary hemangiomas are common tumors of infancy and adolescents. Although head and neck are the most prevalent sites for origin of hemangiomas, they are very rarely observed arising on attached gingiva. This paper presents a rare case report of capillary hemangioma on attached gingiva of posterior maxilla in an adult female. Surgical management was done by Widman's periodontal flap operation with satisfactory uneventful healing. The characteristic clinical and histo-pathologic features of vascular tumors and malformations, especially capillary hemangiomas are discussed.

INTRODUCTION

The term hemangioma incorporates heterogeneous group of clinical benign vascular lesions with similar histologic characters.[1] Hemangiomas are characterized by rapid endothelial cell proliferation, followed by involution over time. The proliferating mass of vessels does not undergo malignant transformation. Though the lesion usually develops in children, older age individuals may also occasionally be affected.[12] Although there are conflicting reports regarding its gender predilection, but many clinicians observed that this lesion has higher incidences in female subjects than in male subjects.

Hemangiomas are classified into capillary and cavernous types on the basis of the size of vascular spaces and histology. The capillary hemangiomas have numerous proliferating small thin walled blood filled vessels composed of single layer of flattened or plump endothelial cells, surrounded by discontinuous layer of pericytes and reticular fibers. The cavernous hemangiomas on other hand consists of deep, irregular, dermal tangles of large thin walled vessels or sinusoids separated by scanty connective tissue and surrounded by discontinuous layer of endothelial cell. Capillary hemangioma (CH) as a term has been commonly practiced to describe a large number of vasoformative tumors (VFT). CH exhibits both, a proliferative phase and an involution phase, where as vascular malformations are more stable and fail to regress.[3]

Hemangiomas have been described to be presenting clinically as a soft tissue mass, smooth or lobulated, sessile or pedunculated and with variable size.

They appear as pale halo surrounding an area of telangiectasis. Sometimes CH may be confused with vascular appearing lesions of face or oral cavity similar to Sturge-Weber syndrome. Most true CH involute with time, but certain small percentage does not, and may present with complication that require surgical management.

Although the literature suggests CH as a common soft tissue tumor of head and neck,[4] but contrarily it is observed as a relatively rare and uncommon pathologic entity in the oral cavity. These tumors are mostly seen on face, fingers, and occasionally on oral mucosa. Occurrence of CH with its primary location on gingiva seems extremely rare. Periodontally, these growths often appear to arise from the gingival papilla and spread laterally to involve adjacent tissues.[5] This paper describes the case of a patient who had a growth similarly arising from gingival papilla between maxillary left first and second molars on palatal side, which was successfully managed by periodontal Widman's flap surgery, with two years uneventful follow-up for observing any recurrence.

CASE REPORT

A 30-year-old female reported with a gradually increasing, painless swelling in the upper left posterior palatal region since childhood. There was history of frequent bleeding from the lesion, and difficulty in swallowing and speaking.

Extra-oral examination including the lymph nodes was insignificant. Intra-oral examination revealed a 2×2.5 cm growth which was red in color with bluish hue and with slightly pale periphery, arising from the buccal attached gingiva of maxillary left first and second molar, which extended palatally [Figure 1]. The lesion appeared sessile imperceptibly with the papilla and attached gingiva. Although two surface ulcerations were noticed, probably due to trauma during mastication, the remaining entire surface was smooth with several visible small vessels. The growth was gently soft to palpate and showed blanching on application of pressure, and was pulsatile but not reducible [Figure 2]. Intra-oral periapical radiograph of the region exhibited a horizontal pattern of bone loss due to chronic periodontitis [Figure 2]. Carotid angiogram could not be possible because of under-privileged socio-economic status.

Figure 1

Bluish hue colored growth in the maxillary left posterior region, occupying space in the posterior region of oral cavity

Figure 2

Blanched palatal extension of the growth with heavy calculus on adjacent molars

A provisional diagnosis hemangioma was laid down due to clinical appearance and characteristics of the growth. Preoperative hematological examination revealed all findings within normal parameters. Widman's periodontal flap of maxillary left quadrant was carefully raised under local anesthesia (posterior superior alveolar block) and the growth was excised [Figure 3]. Bleeding spots were cauterized and intermittent sutures were given and periodontal dressing was applied. The excised growth [Figure 4] was sent for histo-pathological examination (HPE) and patient was given necessary hygiene instructions for home care of wound. Satisfactory uneventful healing occurred and follow-up was done for two years to monitor any recurrence.

Figure 3

Excised growth

Figure 4

HP section under low magnification, showing multiple vessels

The excised soft tissue mass was subjected to routine processing and several sections were obtained. HPE of the sections obtained from the lesion showed numerous capillaries filled with RBC in a delicate fibrillar connective tissue which was predominantly full of proliferating endothelial cells. Few inflammatory cells were also evident in the stroma. The connective tissue component was covered by parakeratinized stratified squamous epithelium which showed ulceration at few places. Areas of ulceration were covered with fibrin meshwork and were infiltrated by RBCs and mixed inflammatory cells. Correlating the microscopic features with the clinical picture, HPE confirmed the diagnosis as “Capillary hemangioma” [Figures 5–7].

Figure 5

Periodontal flap reflected and curetted

Figure 7

Higher magnification showing proliferating endothelial cells and dense distribution of plasma cells and lymphocytes. (H and E ×400)

Micro-photograph of the capillary hemangioma, showing multiple vessels with RBCs occluding the lumen and inflammatory cells uniformly distributed in the stroma. (H and E ×100)

DISCUSSION

The term hemangioma has been used traditionally to describe a variety of developmental vascular anomalies, and may often be referred by terms like hamartoma, malformations, or true benign neoplasm/tumors. It is necessary to distinguish it from inflammatory growths (reactive hyperplasia) which have excessive proliferation of reparative tissue. The hemangiomas (both solitary or when found with other anomalies/angiomatosis syndromes) are true hamartomas.[6]

The hamartomas are tumor-like malformations characterized by the presence of cellular proliferation that is native to the part but that manifests growth cessation without potential for further growth. Hamartomas are usually congenital and simultaneously grow along the growth of rest of the body. Once they achieve their adult size, they do not extend to involve more tissue unless there is trauma, infection, edema, inflammation, or filling of new vascular channels. However, it is different from malformations (eg., extra digits) in which excessive tissue is present in its usual histological relationship with almost no growth potential. On the other hand, true benign neoplasm/tumors have a relatively unlimited capacity for expansive growth which may continue even after the stimulus has ceased to operate.[6]

The term - VFT, encompasses the hemangiomas and the vascular malformations and the difference between them are elaborated in the Table 1. In 1982, Mulliken and Glowacki described classification scheme that is ubiquitously accepted [Table 2].[3]

Table 1

Comparison of hemangiomas and vascular malformations

Table 2

Types of hemangiomas and vascular malformations

Although, the etiology of VFTs is unclear, a hypothesis had been postulated that the placental cells such as trophoblasts may be the cell of origin of hemangiomas, and therefore, they arise secondary to some event in utero. It has been suggested that the endothelial cells of hemangiomas are derived from a distant population of endothelial precursors carried by existing vascular pathways to a receptive environment, and its potential sources include bone marrow and placenta. A small embolic nidus of placental endothelial cells could reach fetal tissues through the permissive right-to-left fetal shunt of fetal circulation. Possibly, local placental injury may predispose the shedding of cells into the fetal circulation. However, further investigations are required to confirm this hypothesis.[7]

The pathogenesis of the common capillary hemangioma involves three stages of development similar to development of any other VFT.

The first stage is that of undifferentiated capillary network stage. It represents an arrest in the development of mesenchymal primordia which is being nourished by an interlacing system of blood spaces without distinguishable arterial and venous channels. As the development progresses to the second stage i.e., the retiform developmental stage, the primitive vessels penetrate deeper into the subcutaneous layers (to muscle or bone) and gives rise to capillary hemangiomas. As this stage ends, an arterial, venous, and capillary system is established. Finally in the third stage, i.e., the final developmental stage there is gradual replacement of the immature plexiform network of vessels by the mature vascular channels.[7]

During the early proliferative phase CH can be shown immunohistochemical expression of proliferating cell nuclear antigen and vascular endothelial growth factor (VEGF), localized to endothelium and pericytes, while type IV collagenase only along endothelium. All these three are required for growth and proliferation of the vessels and are reduced during involution phase. Contrarily, the tissue inhibitors of metalloproteinases (TIMP), and anti-angiogenic factors are markedly increased. A number of growth factors, including VEGF, basic fibroblast growth factor (bFGF), transforming growth factor-beta (TGF-beta), and interleukin-6 (IL- 6), have been demonstrated as regulators of angiogenesis.[8] Takahashi et al.[9] outlined a number of cellular markers that distinguish the phases of hemangiomas; these markers include metalloproteinase (TIMP-1), bFGF, proliferating cell nuclear antigen, type IV collagenase, VEGF, and urokinase. Proliferating hemangiomas have been shown to have estradiol-17 beta-receptors in the cytoplasm.[10] Corticosteroid treatment has been theorized to block these receptors. Lack of estradiol receptors in the stable or involuted lesions have supported this theory; therefore, steroid treatment has become a first line of treatment for proliferating lesions.

Hemangiomas are common tumors of infancy, affecting as many as 12% whites, but it rarely occurs in dark-skinned individuals. The vascular malformations have also been commonly observed in the whites. Hemangiomas can occur at any age, but the incidences are more among infants and children. They occur in 5-10% of 1-year-old children[11] and their incidence increases to 23% in premature infants with birth weight less than 1000 grams.[7] Vascular malformations have a much broader range for age of incidence. Barette and Speight[12] observed 35 oral vascular malformations over a 48 years period at their institution. The mean age was 52.6 years with a range of 12-90 years. The peak incidence of central vascular malformation of the jaws is in the second decade of life. Hemangiomas are approximately 3-5 times more common in females than in males.[13] But for vascular malformations, the gender ratio is reported to be 1:1. Although 80% of hemangiomas occur as single lesions, about 20% of affected patients have multiple tumors.[11]

The most common location for occurrence of hemangiomas is head and neck region, and it accounts for almost 60% of all cases.[11] Despite this, intraoral hemangiomas are not common pathologic entities. Furthermore, if at all CH are found, the bones, muscles, mucosa, and the skin are affected, and the most frequent site of occurrence of intraoral hemangiomas are lips, tongue, buccal mucosa, and palate.[7] It is very rare to find hemangiomas on attached gingiva as has been observed in the present case discussed here. When found, the hemangiomas of gingiva are often covered by rugose epithelium and may be connecting to central hemangiomas/lesions. The incidence of intra-osseous hemangiomas varies from 0.5-1.0% of all intra-osseous neoplasms.[1314] The most commonly affected facial bones are the mandible and the maxilla, with a ratio of 2:1 reported in the study.[15] Involvement of zygoma has been reported rarely.[16] Intramuscular hemangiomas in the oral region are most commonly seen in the masseter, comprising 5% of all intramuscular hemangiomas.[17] The location of the lesion does not generally influence its behavior, but lesions of the lip are less favorable. Occasionally, CH may be accompanied by other deformities at other sites on the body. Patient's sex and size of the hemangioma does not influence the speed or the completeness of the involution.[18]

Histologically, the early hemangiomas are characterized by numerous plump endothelial cells and often in distinct vascular lumina. As the lesions mature, the endothelial cells become flattened. Finally, small capillary sized vascular spaces lined by single layer of endothelial cells supported by a connective tissue stroma of varying density become evident. Mast cells and factor-VIII- positive interstitial cells are the consistent features of these tumors. Mast cells are important producer of angiogenic factors that regulate the growth of these tumors. Myriade tiny vessels are encircled by connective tissue fibers. The histologic picture has considerable resemblance to young granulation tissue. It is identical to some cases of pyogenic granuloma – which in fact, histopathologically is a lobulated capillary hemangioma more aptly. Clinically pyogenic granuloma is non-pulsatile and does not blanch on pressure application.

Capillary hemangiomas appear as pale halo surrounding an area of telangiectasis. An interval of 6-12 months often follows the growth period. Then, a slow spontaneous involution often begins in the center of the lesion with darkening of color followed by numerous fibrous septa within the lesion.

Capillary hemangiomas are not common in this age group people as discussed in this our case and specifically hemangioma arising on attached gingival is extremely rare entity. Clinical findings in correlation with histo-pathologic picture supported the diagnosis of capillary hemangioma. The surgical management of this case was preferred therefore Widman's periodontal flap surgery was performed. Spontaneous and profuse bleeding as well as possibility of post operative recurrence, regular clinical observation of the affected site should be mandatory for certain time and for this patient follow-up for clinical evaluation of site was done for two years with satisfaction.