|Year : 2016 | Volume
| Issue : 1 | Page : 87-91
Central hemangioma: A case report and review of literature
Sandeep Jain1, Sasidhar Singaraju2, Medhini Singaraju2
1 Department of Oral and Maxillofacial Pathology and Microbiology, Modern Dental College and Research Center, Indore, India
2 Department of Oral and Maxillofacial Pathology and Microbiology, Rishiraj College of Dental Sciences and Research Centre, Bhopal, Madhya Pradesh, India
|Date of Web Publication||2-Feb-2016|
Department of Oral and Maxillofacial Pathology and Microbiology, Rishiraj College of Dental Sciences and Research Centre, Gandhi Nagar, Bhopal, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Hemangioma is a benign self-involuting tumor of endothelial cells. They are the most common benign congenital lesions in humans and are characterized by the proliferation of blood vessels. They are often present at birth or appear soon after, and grow rapidly by endothelial proliferation. This article presents a rare case of central hemangioma occurring in the mandible of a 13-year-old boy, and a brief review on clinical, radiological, and histopathological characteristics of central hemangiomas.
Keywords: Hemangioma, endothelial cells, mandible
|How to cite this article:|
Jain S, Singaraju S, Singaraju M. Central hemangioma: A case report and review of literature. J Indian Soc Pedod Prev Dent 2016;34:87-91
|How to cite this URL:|
Jain S, Singaraju S, Singaraju M. Central hemangioma: A case report and review of literature. J Indian Soc Pedod Prev Dent [serial online] 2016 [cited 2019 Nov 21];34:87-91. Available from: http://www.jisppd.com/text.asp?2016/34/1/87/175525
| Introduction|| |
Hemangioma is the most common benign congenital lesions in humans and are characterized by the proliferation of blood vessels. ,, They are often present at birth or appear soon after, and grow rapidly by endothelial proliferation.  Whether a true neoplasm of the blood vessel system exists remains an open question, and thus today discussion continues on whether they are in fact neoplasms or hamartomas. ,
Hemangioma of bone is histologically classified by Thoma as peripheral type (arising from periosteum) and central or intraosseous type (arising from central spongiosa).  Most central hemangiomas are the cavernous type (large vessels), but can be of capillary type (small vessels) also. Capillary hemangioma are composed of many small capillaries lined by a single layer of endothelial cells supported in a connective tissue stroma of varying density whereas cavernous hemangiomas are formed by large, thin-walled vessels or sinusoids lined with a single layer of endothelium which are separated by thin septa of connective tissues.  The central (intraosseous) type is frequently found in the vertebrae and skull and rarely develops in the jaws. ,, The lesion produces a hard nontender slow-growing swelling. 
Mandible is a very infrequent location although possible. The female to male ratio is 2:1 and the peak of incidence is between the second and fifth decades of life. , In the mandible, the greatest frequency of occurrence has been the body region, but condylar tumors have also been reported.  Wat son and Mc-Carthy  and Matthews  consider hemangiomas to be congenital, but in 50% of the cases a history of trauma is elicited.  Seventy-five percent of hemangiomas are present at birth, whereas 85% are noted by the age of 1-year. Although the head and neck region represents only 14% of the body surface area, 65% of hemangiomas arise in this location. ,
Though its origin is not defined some authors believe that it is a true neoplasm, whereas others state it to be a hamartoma resulting from proliferation of intraosseous mesodermal cells that undergo endothelial differentiation and subsequently canalized and vascularized. ,, Malignant transformation of central hemangioma has been reported in some cases. Most central hemangiomas are the cavernous type (large-calibre vessels), but can be the capillary type (small calibre vessels). 
Clinical and radiographic appearance
Clinically, the patient may be completely symptom-free or may present discomfort, pulsations, bleeding, bluish discoloration, mobile teeth, derangement of the arch form or accelerated dental exfoliation, and agenesis of teeth. ,, Highly expansile lesions cause a sensation of pulsation, audible bruits on extension into the soft tissue, and blanching on pressure. Occasionally patients have paresthesia in the region of the lesion. , Some hemangiomas may be present without any sign or symptom. 
These lesions of bone have been referred to as "the great mimicker" because they can produce many different radiographic images.  The lesion may appear as a radiolucent area which is unilocular or multilocular, reticulated or honeycombed.  The most frequent radiographic finding is a multilocular radiolucent image with honeycomb or soap bubble appearance.  Phleboliths appearing as small rounded or sausage shaped radiopacities having concentric structure with a small radiolucent dot in the center may also be seen.  It often shows a sunburst effect. Computed tomography (CT) imaging shows a poorly delineated, dense, expansile mass, reminiscent of a fibro-osseous lesion. In suspicious cases, scintigraphy with labeled red blood cells, and angiography may be useful. 
Angiographically, hemangiomas appear as well-circumscribed lesions exhibiting intense tissue staining, usually organized in a lobular pattern. Angiography shows the afferent and efferent vessels of these lesions.  Moreover, angiography may be carried out to establish either the extent or the vascular supply of a lesion or to show a therapeutic effect. 
Hemangiomas show intense focal uptake throughout the lesion.  Differential diagnosis by histology alone is often impossible since the hemodynamic properties of the lesion are neglected. Angiography, scintigraphy, and other imaging procedures may help to establish a correct diagnosis, although clinical, radiological, and pathological classification may not only differ, but many even are conflicting.  Therefore, the clinical course may be an important clue in certain situations.
Magnetic resonance tomography, computed tomography, and Doppler color flow imaging
Magnetic resonance imaging (MRI) is the most informative technology for demonstration of both the extent of involvement within tissue planes and rheological characteristics.  CT scanning is useful in defining the spatial relationship of vascular anomalies and alterations in skeletal structures.  The CT-scan allows clear visualization of cortical involvement and is also useful to define the extension of the hemangioma and its relationship with surrounding soft tissues. The classical feature is the "polka-dot" appearance with cortical expansion.  Doppler color flow imaging enables the differentiation of high-flow from low-flow lesions. 
| Case Report|| |
A 13-year-old boy presented with a swelling on mandibular right side of the face that has been present since 2 years. Patient's medical history was noncontributory; however, his dental history revealed extraction of 46 prior to the development of the lesion due to the presence of a deep carious lesion. Initially, the lesion developed as a small painless swelling which gradually increased to the present size. Patient developed pain as the lesion increased in size. Extraoral examination revealed a bony hard oval swelling which extended from the right angle of mouth up to the temporomandibular joint (TMJ) antero-posteriorly and from lower border of the mandible up to the middle one-third of the face measuring about 4 × 5 cm in size [Figure 1].
Oral examination revealed a bony hard swelling extending from mandibular right canine region to TMJ obliterating the vestibular sulcus. Patient exhibited mixed dentition with 45, 46 being absent, but 85 were still present in the arch.
Orthopantomograph revealed multilocular radiolucency with numerous radiopaque trabeculations extending from first premolar up to the neck of the condyle on right side of the mandible. Orthopantomograph (OPG) also showed the expansion of buccal cortical plates. Forty-five was seen floating in the bone [Figure 2]. CT imaging confirmed the radiographic findings [Figure 3].
|Figure 2: Orthopantomograph showing multilocular radiolucency with numerous radiopaque trabeculations|
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Cytopathological and histopathological findings
Fine-needle aspiration cytology showed red blood cells (RBCs) confirming the diagnosis of hemangioma.
An en-bloc resection of the lesion, including 1 cm of healthy bone was performed. A nickel titanium plate was placed over the defect. Eight weeks postsurgery the child was healing well, and the graft was being planned.
Gross specimen received was a mandibular segment with lesion extending from 45 to coronoid process, measuring 7.5 cm × 5 cm × 2.5 cm in size, brownish in color, and irregular shape and surface [Figure 4]. The cut surface of the specimen showed brownish discoloration with multilocular appearance and rich vascular channels [Figure 5].
|Figure 5: Cut surface of the specimen showing brownish discoloration with multilocular appearance and rich vascular channels|
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Under microscopic examination, the H and E stained section showed numerous small capillaries and dilated blood vessels filled with RBC's lined by flattened endothelial cells. The intertwining connective tissue was made of fibrillar collagen fibers. The trabeculae of bone were lined by osteoblast and showed osteocytes in the lacunae [Figure 6] and [Figure 7]. Thus, the diagnosis of central capillary hemangioma was confirmed.
|Figure 6: H and E stained section showing numerous small capillaries and dilated blood vessels|
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|Figure 7: Trabeculae of bone lined by osteoblast and showing osteocytes in the lacunae|
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| Discussion|| |
Hemangioma is a benign self-involuting tumor of endothelial cells. The word "hemangioma" comes from the Greek word hema meaning "blood," angeio meaning "vessel" and the suffix-oma meaning "tumor." Central hemangioma can be either asymptomatic/symptomatic, and can show signs and symptoms like discomfort, oozing or pulsatile bleeding from the gingiva around the teeth in the region of the lesion, bluish discoloration of gingiva and mobile teeth.  In the present case, extraoral swelling was present on right side of the face resulting in facial asymmetry. Intraorally most central hemangiomas present as painless swellings mainly in the premolar-molar area and the teeth in the affected region may show displacement or root resorption.  A similar clinical presentation was seen in the present case wherein the lesion started as a small painless growth, which gradually increased in size and later became tender. OPG revealed 45 was floating in the bone.
Radiographically, central hemangiomas appear as multilocular radiolucent lesions with well-defined margins, wherein the radiolucencies represent enlarged marrow spaces surrounded by coarse, dense, and well-defined radiopaque trabeculae.  However, OPG of the present case showed large areas of radiolucency giving it a typical ground glass appearance, similar to fibrous dysplasia. The CT images had given a better visualization of cortical involvement and extensions of the lesion and its relation to surrounding structures.
Angiography, scintigraphy, and MRI have proved to be useful as a diagnostic tool when clinical and radiographic characteristics suggest a diagnosis of hemangioma. This will demonstrate the presence of a vascular lesion and delineate its boundaries. 
Incisional biopsy is formally contraindicated because of the high risk of bleeding.
Various treatment options are available for central hemangioma depending on size of lesion, location of lesion, and age of patient. The treatment range includes steroid therapy, sclerosing agents, irradiation, and surgical excision with or without ligation of vessels, embolization, laser therapy, and replacement of resected area with iliac bone graft. 
Usually, treatment is indicated only in some conditions like esthetic disfigurement or in case of very large size or unusual location of the lesion as uncontrolled bleeding is the most hazardous complication. ,,,
Radiotherapy and lasers are useful to reduce the volume of tumor. Nevertheless, it has a lot of adverse effects such as damage to the normal adjacent tissues and residual scarring. Hence, radiotherapy is considered the unacceptable therapeutic option.  Simple curettage may lead to an uncontrollable bleeding as well as an incomplete excision of the lesion.  In the present case, hemi-sectioning of the mandible was done due to the size and extent of the lesion. Postoperative follow-up of the patient for 6 months revealed no complications or recurrence.
| Conclusion|| |
The significance of a mandibular hemangioma increases manifold because of its anatomical proximity to the teeth representing a high risk of bleeding due to trauma or extraction. Central hemangioma has a variable radiological appearance. Therefore, it is important for a radiologist and pathologist to differentiate hemangioma from the similar appearing lesions.
When planning a treatment approach, one must bear in mind the age of the patient as majority of childhood lesions spontaneously involute and require no surgical intervention.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Edgerton MT. Steroid therapy of haemangioma. In: Williams HG, editor. Symposium on Vascular Malformation and Melanotic Lesions. St. Louis, MO, Mosby; 1983. p. 74-83.
Watson WL, McCarthy MD. Blood and lymph vessel tumors: A report of 1056 cases. Surg Gyneco Obstet 1940;171:569.
Shafer WG, Hine MK, Levy BM. A Textbook of Oral Pathology. 4 th
ed. Philadelphia: W.B. Saunders Company; 1983. p. 154-7.
Puneet, Khanna R, Khanna AK, Kumar M. Haemangioma of the mandible. Indian J Otolaryngol Head Neck Surg 2004;56:159-60.
Knezevic G. Kirurgija O, Zagreb D. Medicinska Naklada. Oral surgery 2 nd
Medical edi Zagreb.
Knezevic G, Knezevic D, Manojlovic S. Central hemangioma of the mandible: Case report. Acta Stomatol Croat 2007;41:381-7.
White SC, Pharoah MJ. Oral Radiology, Principles and Interpretation. 5 th
ed. Mosby Elsevier publisher, 2004;445-9.
Jindal SK, Sheikh S, Singla A, Puri N. Role of radiology in central hemangioma of jaws. J Clin Exp Dent 2010;2:76-8.
Marwah N, Agnihotri A, Dutta S. Central hemangioma: An overview and case report. Pediatr Dent 2006;28:460-6.
Alves S, Junqueira JL, de Oliveira EM, Pieri SS, de Magalhães MH, Dos Santos Pinto D Jr, et al.
Condylar hemangioma: Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;102:e23-7.
Nagpal A, Suhas S, Ahsan A, Pai K, Rao N. Central haemangioma: Variance in radiographic appearance. Dentomaxillofac Radiol 2005;34:120-5.
Watson WL, McCarthy WD. Blood and lymph vessel tumors: Report of 1056 cases. Surg Gynecol Obstet 1940;71:569.
Matthews DN. Hemangiomata. Plast Reconstr Surg 1968;41:528-35.
Choukas NC, Toto PD, Valaitis J. Sclerosing cavernous hemangioma of the maxilla. Oral Surg 1963;16:17.
Schajowicz F, Rebecchini AC, Bosch-Mayol G. Intracortical haemangioma simulating osteoid osteoma. J Bone Joint Surg Br 1979;61:94-5.
Bunel K, Sindet-Pedersen S. Central hemangioma of the mandible. Oral Surg Oral Med Oral Pathol 1993;75:565-70.
Whear NM. Condylar haemangioma - A case report and review of the literature. Br J Oral Maxillofac Surg 1991;29:44-7.
Zlotogorski A, Buchner A, Kaffe I, Schwartz-Arad D. Radiological features of central haemangioma of the jaws. Dentomaxillofac Radiol 2005;34:292-6.
Savastano G, Russo A, Dell'Aquila A. Osseous hemangioma of the zygoma: A case report. J Oral Maxillofac Surg 1997;55:1352-6.
Burrows PE, Mulliken JB, Fellows KE, Strand RD. Childhood hemangiomas and vascular malformations: Angiographic differentiation. AJR Am J Roentgenol 1983;141:483-8.
Rózy³o TK, Jarzab G. Angiography in the evaluation of cryosurgery efficiency in the treatment of a haemangioma of the face. A case report. J Maxillofac Surg 1980;8:115-8.
Barton DJ, Miller JH, Allwright SJ, Sloan GM. Distinguishing soft-tissue hemangiomas from vascular malformations using technetium-labeled red blood cell scintigraphy. Plast Reconstr Surg 1992;89:46-52.
Niechajev IA, Clodius L. Diagnostic criteria of vascular lesions in the face. Ann Plast Surg 1993;31:32-41.
Meyet JS, Hoffer FA, Barnes PD. MRI correlation with biological classification of soft tissue vascular anomalies. Am J Roentgenol 1991;157:559-63.
Fishman SJ, Mulliken JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993;40:1177-200.
Willinsky RA, Rubenstein JD, Cruickshank B. Case report 216. Intracortical hemangioma of tibia. Skeletal Radiol 1982;9:137-9.
Yoshida H, Yusa H, Ueno E. Use of Doppler color flow imaging for differential diagnosis of vascular malformations: A preliminary report. J Oral Maxillofac Surg 1995;53:369-74.
Ahmed J, Mathur H, Tripathi P, Goel S, Singh MP. Intraosseous hemangioma of the mandible: A case report. Part I. Int J Acad Res 2011;3:163-6.
Kenan S, Abdelwahab IF, Klein MJ, Lewis MM. Hemangiomas of the long tubular bone. Clin Orthop Relat Res 1992;280:256-60.
Shpitzer T, Noyek AM, Witterick I, Kassel T, Ichise M, Gullane P, et al.
Noncutaneous cavernous hemangiomas of the head and neck. Am J Otolaryngol 1997;18:367-74.
Drage NA, Whaites EJ, Hussain K. Haemangioma of the body of the mandible: A case report. Br J Oral Maxillofac Surg 2003;41:112-4.
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