Central haemangioma: variance in radiographic ... - BIR Publications

Dentomaxillofacial Radiology (2005) 34, 120–125 q 2005 The British Institute of Radiology http://dmfr.birjournals.org

CASE REPORT

Central haemangioma: variance in radiographic appearance A Nagpal*, S Suhas, A Ahsan, KM Pai and NN Rao Department of Oral Medicine and Radiology, College of Dental Surgery, Manipal, Karnataka-576104, India

Central haemangioma, a “great mimicker” which, fortunately, is a relatively rare condition, may pose a lethal risk for the patient. The diagnosis may become apparent only during biopsy or tooth extraction, which poses a risk of lethal exsanguination; therefore a correct diagnosis is desirable before any biopsy is undertaken. The clinician may not anticipate the severe haemorrhage because of vague clinical history, physical findings and ambiguous radiographic characteristics of the lesion. We report a case of central haemangioma of the mandible whose clinical and radiographic features were equivocal. In addition, an attempt is made to discuss all possible radiographic presentations of central haemangioma and consider differential diagnosis. This case is significant for the reason that it had diverse radiographic appearances in various areas of the lesion in different projections. Dentomaxillofacial Radiology (2005) 34, 120–125. doi: 10.1259/dmfr/14940087 Keywords: haemangioma, capillary, mandible, aspiration, radiography Case report A 13-year-old female patient presented to the Department of Oral Medicine, complaining of a swelling in the left mandibular body for a duration of 1 year. She reported a similar swelling 10 years ago following trauma. That lesion was surgically treated. Clinical examination The swelling on the left side of the face resulted in facial asymmetry (Figure 1). It was approximately 4 cm £ 5 cm in size over the left body of the mandible with diffuse margins. The skin over the lesion appeared normal. The swelling had bony hard consistency and it was tender on palpation. Intraoral examination showed obliteration of the buccal sulcus extending from the distal aspect of the mandibular left lateral incisor to the mesial aspect of the mandibular left second molar. There was expansion of the lateral aspect of the mandible with an irregular raised erythematous submucosal mass involving the attached gingiva, vestibule and buccal mucosa adjacent to mandibular left canine and first premolar (Figure 2). This area blanched on pressure but was without any pulsations. There was no evidence of bleeding from the gingival sulcus. The crowns *Correspondence to: Dr Archna Nagpal, Department of Oral Medicine and Radiology, College of Dental Surgery, Manipal, Karnataka-576104, India; E-mail: [email protected] Received 19 February 2004; revised 21 December 2004; accepted 28 December 2004

of the mandibular left second premolar, first and second molar were displaced lingually with loss of occlusion on the left side. The crowns of the mandibular left canine and first premolar were rotated and a diastema of 4 – 5 mm was evident between the mandibular left first and second premolar. The mandibular left canine was shifted labially and a diastema of 2 mm existed between the mandibular left lateral incisor and canine. None of the teeth were tender or mobile. The teeth related to the lesion were vital. The overall appearance of the lesion gave a clinical impression of vascular malformation in the body of the mandible.

Radiographic examination A panoramic radiograph (Figure 3) disclosed coarse trabeculations causing areas of increased radiodensity with ground glass appearance of the bone surrounding the mandibular left canine, first and second premolar. Bone surrounding the mandibular left first molar and mesial root of second molar showed small circular radiolucencies separated by bony septa giving a honeycomb appearance. There were multiple rounded loculations with fine bony septa located 4 – 5 mm below the roots of mandibular left second molar. Multiple tube-like radiopaque striae placed parallel to each other were evident distal to this region. Multiple scattered circular radiolucent areas without sclerotic border were seen in between the roots of mandibular left first and second molar. A sketch of the

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Figure 1 Swelling on the left side of the body of the mandible, with diffuse margins

panoramic radiograph showing left side of the mandible is demonstrated in Figure 3b. Resorption of the root of the mandibular left premolar along with divergence of the mandibular left first and second premolar was seen. The outline of the mandibular canal was not traceable beyond the mesial aspect of the left mandibular second molar and it was displaced inferiorly. There was an increase in the vertical height of the mandible on the left side. This was suggestive of increase in the size of the involved bone, while keeping a relatively normal morphology. Since the dense sclerotic pattern in the anterior mandible could be the overlapping density of cervical spine, an intraoral periapical radiograph of the left mandibular premolar region was taken to confirm the presence of sclerotic pattern in that area. It showed coarse trabeculations in between the first and second premolar (Figure 4). A mandibular occlusal radiograph and its sketch (Figure 5) showed expansion of the buccal cortical plate in the left mandibular body and displacement of the mandibular left first premolar, first and second molar

Figure 2 Obliteration of the buccal sulcus extending from distal aspect of 32 to mesial aspect of 37. An irregular raised erythematous submucous mass involving the attached gingiva, vestibule and buccal mucosa in relation to 33 and 34, 35, 36 and 37 are displaced lingually. Crowns of 33 and 34 are rotated. 33 is shifted buccally

Figure 3 (a) A panoramic image shows coarse trabeculations in the bone surrounding 33, 34 and 35 causing areas of increased radiodensity. Bone beneath 36 and mesial root of 37 shows small circular radiolucencies separated by bony septa giving a honeycomb appearance. Multiple rounded locules with fine bony trabeculations traversing them 4– 5 mm below the roots of 37. Multiple tube like radiopaque striae placed parallel to each other were evident distal to this region. Inferiorly displaced mandibular canal whose outline not traceable beyond the mesial aspect of 37. Divergence of roots of 33, 34 and 35 with resorption of root of 34. (b) Sketch of the panoramic radiograph showing left side of the mandible. A, Areas of coarse trabeculations; B, Honeycomb appearance; C, Multiple rounded locules; D, Parallel tube like trabeculations; E, Inferiorly displaced mandibular canal, outline not traceable beyond the mesial aspect of 37

Figure 4 A periapical radiograph showing coarse trabeculations in between 34 and 35 Dentomaxillofacial Radiology


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lingually. Also evidence of fine trabeculations or spiculated bone was seen emanating from the buccal aspect of the mandibular cortex. There was enlargement of the adjacent bone. There was a large variation in radiographic appearance and based on clinical impression of a vascular lesion, an aspiration was performed which yielded fresh blood. Review of previous records Clinical notes from the previous records indicated the presence of a bony hard swelling over the body of the left mandible when the child was 3 years old, which had occurred 2 months after an episode of trauma to the left side of lower jaw. There was a history of bleeding for a duration of 1 day from the associated tooth region subsequent to the trauma. Radiographic notes described an ill-defined radiolucency in relation to the mandibular left deciduous canine, first and second molar and a mandibular occlusal radiograph showing a typical “sunburst” effect. Surgical notes revealed that an excisional biopsy was done under general anaesthesia. Profuse bleeding encountered during surgery was controlled by local measures. Histopathological examination (Figure 6) of the decalcified section after the surgery, 10 years ago, had shown broad lamellar bony trabeculae with marrow spaces exhibiting poorly arranged fibrillar stroma with varying number of mesenchymal cells, numerous vascular channels lined by endothelial cells and extravasated red blood cells. Soft tissue sections showed highly cellular connective tissue stroma with endothelial cells, budding capillaries, and areas of haemorrhage with haemosiderin pigment. The lesion was diagnosed as capillary haemangioma. The archived histopathological slide (Figure 6) was reviewed. On the basis of a diagnosis of capillary haemangioma, angiography and pre-operative embolisation were advised but due to financial constraints patient did not comply. No other treatment modality was performed, as she was lost to follow up.

Figure 5 (a) Cropped occlusal radiograph showing expansion of buccal cortical plate and displacement of 35, 36 and 37 lingually. Fine spiculation extending from the cortex. (b) Sketch of cropped occlusal radiograph showing expansion and spiculation Dentomaxillofacial Radiology

Figure 6 Decalcified section showing lamellated bone with marrow spaces exhibiting endothelial cells; numerous vascular channels lined by endothelial cells and extravasated blood. (Haematoxylin and eosin stain, 10 £ magnification)


Discussion Haemangiomas of the jaws are quite rare;1 – 5 when they occur two thirds are found in the mandible,3 with a female:male ratio of 2:1,2,3,6,7 and peak incidence in the second decade of life.1 – 3,6,7 In the mandible, the greatest frequency of occurrence has been the body region,2,3,7 but condylar tumours have also been reported.2,7 The origin of central haemangioma is debatable.1,3 Some believe that it is a true benign neoplasm,6 – 8 while others state that it is a hamartoma1 – 3,6 – 8 resulting from proliferation of mesoderm cells that undergo endothelial differentiation and subsequently are canalized and vascularized. In our case the swelling was noticed 2 months following trauma, which might have accentuated the progression of an already existing lesion rather than causing it.9 – 11 Malignant transformation of central haemangioma has been reported in some cases.2,3,8,12,13 The initial signs and symptoms of central haemangioma are said to vary. It may be asymptomatic resulting in death following tooth extraction or biopsy.1,2,6 Other reported signs and symptoms include discomfort, oozing or pulsatile bleeding from the gingiva around the teeth in the region of the lesion,2 bluish discoloration of gingiva,2 mobile teeth,1,2,6,8,13,14 derangement of arch form,4,13 accelerated exfoliation and agenesis of teeth.4 Highly expansile lesions cause sensation of pulsation,1 audible bruits on extension into the soft tissue2,12 and blanching on pressure.4 Cases of spontaneous bleeding have also been reported.1,2,4,8,12 Occasionally patients have paraesthesia in the region of the lesion.13 In the present case, the erythematous area blanched on pressure, suggestive of a vascular lesion. In the current case the lesion recurred over a period of 10 years. The possibility of continuation of the original lesion cannot be excluded, since excision biopsy was done during the previous surgery. The ideal treatment would have been segmental resection of the mandible. Review of literature showed that following surgery the lesion generally recurs over a period of 4 months to 2 years.4 – 6,8 One case has been reported where the swelling reappeared 7 years after surgery.13 In most cases the radiographic appearance is certainly not pathognomonic and only a working diagnosis of central haemangioma of the bone can be made from radiographs.15 Nearly any combination of lesion shape, location and pattern can develop. There are, however, a greater number of variations and combination of changes that may lead the clinician to suspect a vascular lesion as in the current case.16 Lamberg et al2 reported a similar case of central haemangioma of the mandible with variation of radiographic appearance in different parts of the lesion and in a variety of projections. Careful evaluation of radiographs is emphasised since cases have been reported in which such a lesion was originally detected on a bitewing radiograph.10 Early lesions may not be visible on radiographs.2,3,5,7 In some cases the lesion produces an alteration in trabecular pattern4 which may be thin or lost in some areas while in others it can be thicker or coarser as seen in the present case.

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In some instances the periphery is well defined showing corticated areas with scalloped margins2,7 and in other cases it may be ill defined. Unilocularity, multilocularity3,9,14 and heterogeneous degree of radiolucency are possible. In about 50% of cases a multilocular appearance of honeycomb1,2 type can be detected that result from fine trabeculations within a loculation. A similar kind of presentation is evident in the left mandibular region in the case reported here. Less commonly a soap bubble2,12 and tennis racket appearance have been reported.17 The corticated cyst-like spaces are visible because of alignment of vessels in the direction of Xray beam.1 This feature is also evident in the current case. Langland16 suggested that presence of parallel or tube like arrangement of radiopaque striae could be indicative of the lesion. This feature is evident in the case mentioned here. Haemangiomas can influence the growth of bone and teeth. The involved bone may be enlarged and have coarse internal trabeculae. Also, developing teeth may be larger and erupt earlier, when in an intimate relationship with haemangioma.19 The case mentioned here demonstrates the feature of enlargement of bone. Worth18 described a pattern whereby the trabeculae are arranged in a manner resembling spokes of a wheel radiating outward from the centre of the lesion towards the periphery. The periphery is rounded, irregular and usually not well defined. An expansile lesion with spiculated appearance6,15 caused by trabeculae of bone between blood vessels can sometimes be appreciated in occlusal radiographs. This description is diagnostic. In the case reported here, there is a similar presentation. Phleboliths appearing as small rounded or sausage shaped radiopacities having concentric structure with small radiolucent dot in the centre may be seen.6,18 When present it is highly suggestive if not absolutely indicative of vascular lesion; but unfortunately they rarely appear when the tumour originates in bone. In addition, teeth in the area may be displaced or demonstrate root resorption,2,3,8,14 as in the present case. A multilocular appearance may result from serpiginous deformity of mandibular canal. In the mandible the lesion is often located within the inferior alveolar canal.19 The canal may also be enlarged along its entire length.18 Mandibular canal may be widened or not seen in some cases.13 Hence it is important to examine carefully the relation of the lesion to mandibular canal to help with differential diagnosis.19 Enlargement of mental foramen has been noted in some cases.6,13 The lesion may also show discrete punched out areas,6,15 as depicted in the present case. It is very obvious from the preceding features that the most illustrious attribute of haemangioma radiographically is lack of a characteristic picture. Radiographic differential diagnosis includes osteosarcoma, fibrous dysplasia, central giant cell granuloma, ameloblastoma, odontogenic myxoma, multiple myeloma, dentigerous cyst and aneurysmal bone cyst.2,3,6,8,13 – 15 Osteosarcoma produces similar sunburst appearance. It may be entirely radiolucent, mixed radiolucent – radiopaque or quite radiopaque. There may be asymmetric broadening of periodontal ligament space and onion skin Dentomaxillofacial Radiology


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growth of periosteal bone. Irregular margins of a lesion raise a question of malignant tumour like osteosarcoma15,19 but this must be discounted where the other appearances are sufficiently suggestive of a haemangioma. Central haemangioma with areas of increased radiopacity can be mistaken for ground glass appearance of fibrous dysplasia. The other radiolucent patterns of central haemangioma can be misdiagnosed as intermediate stages of fibrous dysplasia but this lesion does not show sunburst appearance. Multilocular regions of rarefaction that accompany expansion and thinning of the cortex11,13,19 may mimic a central giant cell granuloma of the jaws. However some investigators believe that loculations produced by a central haemangioma are smaller with fine fibrillar network.19 Gibilisco5 suggests that bony spicules can be seen to extend at right angles from the bone into the lesion with the surrounding bone intact, a feature that is pathognomonic of haemangioma. He mentioned this feature in cavernous haemangioma of the maxilla. When present this feature serves to differentiate it from ameloblastoma, odontogenic myxoma and other lesions that may have a honeycomb appearance but, unfortunately, it is not a consistent finding.5 Tennis racket appearance can mislead to the diagnosis of odontogenic myxoma but central haemangioma produces varied type of radiographic appearance.17 Cystic lesions are more difficult to diagnose radiographically and an aneurysmal bone cyst may be suspected, but radiographically this lesion does not resorb the adjacent teeth.16 Also, this neoplasm lacks the multiple non-corticated foraminae that are sometimes seen in central haemangiomas and arteriovenous malformations.16 Central haemangioma, like multiple myeloma, may also present with punched out radiolucencies but multiple punched out skull lesions are highly characteristic of the latter. Mandibular lesions of multiple myeloma are well delineated without a cortical outline. Perforation of the cortex is more common than expansion.16

Since haemangioma of bone occurs early in life, the lesion can be in close proximity to the erupting teeth and it can be mistaken for a dentigerous cyst. In contrast to haemangioma, a dentigerous cyst will be associated with a well corticated pericoronal radiolucency attached to the cementoenamel junction of impacted tooth.16 According to Langland et al,16 a difference in vertical depth of the mandible from one side to the other is suggestive of central haemangioma, but it may be seen also in lymphangioma and neurofibroma. The central haemangioma of bone may be clinically and radiographically indistinguishable from another vascular condition known as central arteriovenous fistula, shunt or aneurysm.16 Angiography has proved to be useful as a diagnostic tool when clinical and radiographic characteristics suggest a diagnosis of haemangioma. This will demonstrate presence of a vascular lesion and delineate its boundaries.12 Also it will be helpful in differentiating arteriovenous fistula and central haemangioma.12 Haemangiomas of the bone are dangerous, particularly in the jaws where proximity of the teeth to the lesion may lead to catastrophe. Extraction of a tooth may terminate fatally on account of excessive bleeding.5 In almost all the earlier reported cases the haemorrhage was completely unexpected, and it is for this reason the simple technique of needle aspiration must be emphasised.13 The choice of treatment depends on the size and location of the lesion, age of the patient and anticipated problems.4 The range of treatment includes steroid therapy, carbon dioxide and argon laser therapy, sclerosing agents, irradiation, surgical excision with and without ligation of vessels and embolisation.3 In conclusion, because of the variation in the radiographic appearance of vascular lesions and the importance of a correct diagnosis for the welfare of the patient, the radiologist should include these in the differential diagnosis of similar appearing lesions.

References 1. Thorn JJ, Worsaae N, Gyldensted C. Arterial embolisation in treatment of central hemangiomas of the maxilla-report of two cases. Br J Oral Maxillofac Surg 1986; 24: 114 – 121. 2. Lamberg MA, Tasanen A, Jaaskelainen J. Fatality from central hemangioma of the mandible. J Oral Surg 1979; 37: 578 – 584. 3. Bunel K, Sindet-Pedersen S. Central hemangioma of the mandible. Oral Surg Oral Med Oral Pathol 1993; 75: 565 –570. 4. Greene LA, Freedman PD, Friedman JM, Wolf M. Capillary hemangioma of the maxilla. A report of two cases in which angiography and embolization was used. Oral Surg Oral Med Oral Pathol 1990; 70: 268 – 273. 5. Gibilisco JA. Non odontogenic tumors of the jaw bone. In: Stafne’s oral and radiographic diagnosis (V edn). Philadelphia: W B Saunders; 1985, pp 224 – 226. 6. Piercell MP, Waite DE, Nelson RL. Central hemangioma of the mandible: intraoral resection and reconstruction. J Oral Surg 1975; 33: 225 – 232. 7. Whear NM. Condylar haemangioma-a case report and review of literature. Br J Oral Maxillofac Surg 1991; 29: 44 –47.

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8. Sadowsky D, Rosenberg RD, Kaufman J, Levine BC, Friedman JM. Central hemangioma of the mandible. Literature review, case report and discussion. Oral Surg Oral Med Oral Pathol 1981; 52: 471 –477. 9. Kelly DE, Terryand BC, Small EW. Arteriovenous malformation of the mandible: report of a case. J Oral Surg 1977; 35: 387 –393. 10. Kula K, Blakely G, Wright JT, Terry BC. High flow vascular malformations: literature review and case report. Pediatr Dent 1996; 18: 323 –327. 11. Wood NK, Goaz PW. Multilocular radiolucencies. In: Wood NK, Goaz PW (editors). Differential diagnosis of oral and maxillofacial lesions (III edn). St Louis, MO: Toronto: Princeton: The CV Mosby company, 1985, pp 430 – 433. 12. Shira RB, Guernsey LH. Central cavernous hemangioma of the mandible: report of a case. J Oral Surg 1965; 23: 636 –642. 13. Weinstein I, Yamanaka H, Fuchihata H. Resection and reconstruction of the mandible for removal of central hemangioma. Oral Surg Oral Med Oral Pathol 1963; 16: 2 – 13. 14. Noreau G, Landry PP, Morais D. Arteriovenous malformation of the mandible: review of literature and case history. J Can Dent Assoc 2001; 67: 646 –651.


15. Topazian RG. Central hemangioma of the mandible. Oral Surg Oral Med Oral Pathol 1964; 18: 1 –5. 16. Langland OE, Langlais RP, McDavid WD, Delbalso AM. Multilocular radiolucencies. In: Langland OE, Langlais RP, McDavid WD, Delbalso AM (editors). Panoramic radiology (II edn). Philadelphia, PA: Lea and Febiger, 1989, pp 288 – 290. 17. Wood NK, Goaz PW. Multilocular radiolucencies. In: Wood NK, Goaz PW (editors). Differential diagnosis of oral and maxillofacial lesions (III edn). St Louis, MO: Toronto: Princeton: The CV Mosby Company, 1985, pp 433 –437.

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18. Worth HM. Benign tumors of the jaw. In: Worth HM (editor). Principles and practices of oral radiographic interpretation. Chicago, IL: Yearbook Medical Publishers Inc., 1969, pp 522 – 528. 19. White SC, Pharoah M. Benign tumors of the jaws. In: White SC, Pharoah M (editors). Oral radiology: principles and interpretation (IV edn). St Louis Philadelphia Sydney, Toronto: Mosby, 2000, pp 411 – 414.

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