Hemangioma is a benign tumor characterized by proliferation of blood vessels. Its occurrence within the mandible and maxilla is considered relatively rare, and very few cases have been reported to date. Clinically, cavernous haemangiomas may be locally aggressive and destructive by virtue of the pressure exerted. Despite their malignant-type evolution, these tumors do not undergo malignant transformation. Almost half of the haemangiomas are found in the neck and head regions, their most common locations including the face, the scalp, the orbit, and the oral and nasal cavities. Batsakis classified haemangiomas according to the predominant type of vasculature involved in the lesion and described them as capillary, cavernous, or mixed. In children, the most common manifestation is capillary hemangioma, whereas in adults the cavernous variation is more frequent. To date, the pathogenesis of hemangioma is unknown. It evolves with rapid proliferation involving pericyte and endothelial cell hyperplasia and then enters a steady regression phase [1]. Haemangiomas are considered as benign tumors, being characterized by 3 stages: Endothelial cell proliferation, rapid growth and at last spontaneous involution. The pathophysiology of haemangiomas is attributed to genetic and cellular factors, mainly to monocytes, which are considered the potential ancestors of hemangioma endothelial cells. Imbalance in the angiogenesis, which causes an uncontrolled proliferation of vascular elements, associated with substances such as vascular endothelial growth factor (VEGF), basic fibroblast growth factor (BFGF) and indole?amine 2,3?dioxygenase (IDO), which are found in large amount during proliferative stages, are believed to be the cause [2]. Haemangiomas may occur as a part of a variety of syndromes such as Osler-Weber-Rendu syndrome, Klippel-Trenaunay syndrome, Kasabach-Merritt syndrome, Sturge-Weber syndrome, Parkes Weber syndrome, CLOVES syndrome, Maffucci syndrome, Cobb syndrome, PHACES syndrome and Blue rubber bleb nevus syndrome [3].

A 52-year-old male patient reported to our hospital with a complaint of growth in his mouth. The growth was initially small & started two years back but slowly increased over time & interfered with occlusion. The Patient complained of bleeding from the mouth & inability to chew. The Patient presents a history of tobacco quid chewing (Gutkha) since many years. Previous biopsy report ruled out malignancy & on intra-oral examination the growth of 5cm x 5cm x3.5cm seem to arise from right maxillary alveolus covering the right maxillary occlusal & alveolar surface. The Colour of mucosa covering the growth appears to be pale pink & continuous bleeding was seen from previous biopsy site. The swelling was soft, pedunculated arising from the edentulous process of maxilla. Surface ulceration & bleeding from the biopsy site was seen. Depending upon the clinical feature, a working diagnosis of peripheral giant cell granuloma was made with differential diagnosis of inflammatory granuloma, carcinoma was made. Routine blood examination was carried out & patient was reported to be anemic with hemoglobin concentration of 5.3gm/dl. Patient was examined by hematologist & physician & planned for preoperative blood transfusion before definitive surgery. He was given 2 units of blood & then he showed improvement in hematology status. After the 2^nd transfusion the results showed Hb 8.8 g/dl, RBC 3.41 million cells/microliter, WBC 9100/microliter, Platelets 239,000/microliter, PCV 29.1%, MCV 78.6 fl, MCH 27.1 pg, MCHC 31.9 g/dl. After 3^rd transfusion the results showed Hb 9.7g/dl, RBC 3.85 million cells/microliter, WBC 10900/microliter, Platelets 239,000/microliter, PCV 29.7%, MCV 78.6fl, MCH 27.2 pg, MCHC 32.1 g/dl. Patient was scheduled for surgery under General Anesthesia. Weber-Ferguson incision was placed to completely access the site of the tumor and wide excision of tumor with the surrounding alveolar bone was done. Tumor was completely excised with surrounding healthy bone and the feeder vessels arising from the branches of greater palatine artery were cauterized. An impression was taken Intraoperatively and a temporary obturator was prepared & placed. Wound was closed with 3-0 polyglactin & 4-0 nylon sutures. The healing was uneventful & patient was scheduled for permanent obturator 3 months later.

Figure 1: Weber Ferguson Incision was placed to completely access the site of the tumor.

Figure 2: Tumor was completely excised with surrounding bone and the feeder vessels arising from the branches of greater palatine artery was cauterized.

Figure 3: Grossing reveals the excised specimen size to be approx. 4.5 x 4 x 4 cm3.

Figure 4: Intraoperatively an Impression was taken and a temporary obturator was prepared & placed (A). Immediate post-operative picture with closed surgical wound (B).

Figure 5: H&E staining reveals large dilated blood sinuses (a) with thin endothelial lining (A). (40X) H&E staining reveals blood sinuses filled with RBC’s (b) and chronic inflammatory cell infiltrate. (40X) (B). H&E staining reveals numerous large dilated blood sinuses (c) with thin endothelial lining (10X) (C).

Figure 6: Coronal view of CECT NECK + FACE shows the growth medially extending into oral cavity & in close contact with the tongue (a). Laterally it is closely abutting the buccal mucosa (A).  Axial view of CECT NECK + FACE shows Ill –defined heterogeneously enhancing locally infiltrative solid soft tissue growth visualized epicentered in right maxilla (b) associated with full thickness bony erosion of floor of right maxillary sinus & erosion of right maxillary alveolus (B) .

H & E- Stained section reveals large dilated blood sinuses with thin endothelial lining as walls which are filled with RBC’s also present areas of chronic inflammatory cell infiltrate. Immunohistochemistry results shows CD-34 marker to be positive in endothelial cells lining the vascular channels and D2-40 marker to be negative in endothelial cells lining the vascular channels. Thus, the above histopathology and immunohistochemistry results in correlation to clinical findings favor vascular origin of lesion over lymphatic.

CECT neck and face reports show ill-defined heterogeneously enhancing locally infiltrative solid soft tissue growth visualized epi centered in right maxilla associated with full thickness bony erosion of floor of right maxillary sinus & erosion of right maxillary alveolus.

Growth measures 2.7 x 4.6 x 3.2 cm (APxTRxCC). Medially growth was extending into oral cavity & seen in close contact with the tongue with maintained fat plane. Laterally it was closely abutting the buccal mucosa. Infratemporal fossa is involved & associated with sclerotic changes in pterygoid plate & base.

The most frequent location of hemangioma is the molar premolar region [4]. Pathogenesis is still debatable [5]. and several theories are postulated. Some authors describe haemangiomas as congenital lesions whereas others believe that the inferior dental canal is the origin of the lesion, based on its widening in the majority of these patients [5, 6]. The initial diagnosis is usually complicated because of the absence of symptoms and the unspecific radiological findings [5, 7]. Radio graphically, a differential diagnosis of ameloblastoma, cavernous hemangioma, giant cell lesion, cyst, and myxomas could be made due to the characteristic sunburst appearance [8, 9, 10].Clinical history, examination findings, radiographs, and scanning examination illustrates many features which show characteristic of central hemangioma. The CT-scan allows clear visualization of cortical involvement [6, 11]. And is also useful to define the extension of the hemangioma and its relationship with surrounding soft tissues [5]. the classical feature is the “polka-dot” appearance with cortical expansion. Honeycombed appearance and periostic reaction are extremely rare presentations [5, 12].A CT angiography is needed to rule out any feeding vessel. Preoperative arteriography is usually unnecessary because a vascular flow cannot be identified in the majority of the cases [5, 13].Nevertheless, it should be performed together with a presurgical embolization in big lesions to minimize the surgical bleeding [7, 14].There are two types of hemangioma: Peripheral and central. Peripheral hemangioma is originated in the periostic vessels that grow into the medullar bone, while central hemangioma is originated into the medullar bone and grow toward the cortical bone. Histologically, hemangioma can be divided into three groups: Cavernous, is the most frequent one and is located into the mandible [5]. capillary and mixed. Treatment is indicated only in some conditions: Esthetic disfigurement, repetitive bleeding, and palpable mass. Clinical observation is only indicated in two conditions: Asymptomatic patients or minimal facial deformity [5]. Therapeutic alternatives include: Surgery, curettage, radiotherapy, and embolization [14]. Systemic or intralesional corticosteroids can also be used along with angiogenesis inhibitors, such interferon in selected cases [7]. Intralesional injections of sclerosing agents would not have been effective because of the lesion’s bony nature. Surgical excision with reconstruction of mandible remains the preferred treatment. Prognosis after complete excision is excellent and recurrence is usually rare [15, 16].Simple curettage may lead to an uncontrollable bleeding as well as an incomplete excision of the lesion [5]. Radiotherapy was not the treatment of choice, considering the age of the patient and the retarding effects of radiation on oral and perioral tissues. Percutaneous embolization has been defended by several authors, although technical risks are greater than benefits obtained [17].

Anemia induced from hemangioma have been mostly reported due to bleeding from cavernous hemangioma of GI tracts [18].

Severe Anemia has also been reported in a patient with Cavernous Hemangioma presented with gigantic tongue. Bleeding in a 5yr child which required blood transfusion for correction of anemia [18].

Recurrent epistaxis has also been reported from cavernous hemangioma of left nasal cavity and maxillary sinus in two cases [19].

The elective treatment should be a wide excision of the lesion including healthy surrounding bone, as well as ligature of the nutritional vessels, if present [6, 14]. The identification of feeding vessels is very important for the management of vascular lesions. In cavernous hemangioma of bone, though, conventional radiographs give us adequate information regarding the extent and nature of the lesion, specialized radiographic techniques such as CT angiography would help in detecting the inner component of the lesion and identification of the feeder vessels if present. Because of the serious consequences, haemangiomas must always be considered in the differential diagnosis and proper precautions must be taken in establishing the final diagnosis before any surgical treatment is undertaken.

Conflict Of Interest

There are no conflicts of interest.

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