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Year : 2021  |  Volume : 64  |  Issue : 2  |  Page : 334-338
Central hemangioma of Zygoma - A rare case report with CT and MRI features

1 Department of Oral Medicine and Radiology, Kamineni Institute of Dental Sciences, Narketpally, Nalgonda, Hyderabad, Telangana, India
2 Government Area Hospital, Nampally, Hyderabad, Telangana, India
3 Tatva Dental Clinic, Hyderabad, Telangana, India

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Date of Submission26-Jun-2020
Date of Decision07-Aug-2020
Date of Acceptance15-Oct-2020
Date of Web Publication9-Apr-2021


Intraosseous hemangiomas are uncommon, constituting less than 1% of all osseous tumors. The most frequent sites are the calvaria and the vertebral column. The involvement of the facial bones is rare, and if occurs, it can involve maxilla, mandible, nasal bones and zygomatic bone. Zygomatic hemangioma is a benign, slow-growing tumor occurring mostly in adult women. The radiographic findings are diagnostic. Total excision of the tumor with the primary reconstruction of the defect is the preferred treatment modality. Here, we are reporting a case of a 37-year-old woman who presented with a painless hard swelling in the right zygomatic prominence, which was diagnosed as intraosseous hemangioma after the radiological examination because of its characteristic radiological picture. An Excisional biopsy also proved the swelling to be a cavernous hemangioma.

Keywords: Arteriovenous malformations, cavernous, hemangioma, vascular malformations, Zygoma

How to cite this article:
Pokala A, Devulapally RV, Avinash Tejasvi M L, Nedunooru S. Central hemangioma of Zygoma - A rare case report with CT and MRI features. Indian J Pathol Microbiol 2021;64:334-8

How to cite this URL:
Pokala A, Devulapally RV, Avinash Tejasvi M L, Nedunooru S. Central hemangioma of Zygoma - A rare case report with CT and MRI features. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 16];64:334-8. Available from: https://www.ijpmonline.org/text.asp?2021/64/2/334/313262

   Case Report Top

A 37-year-old woman presented with a painless hard swelling in the right zygomatic prominence [Figure 1]. The patient gave a history of trauma 2 years back. 6 months later after trauma, a small swelling developed below the right lower eyelid which slowly progressed to the present size. On examination, a solitary 3 cm × 4 cm dome-shaped swelling was present in relation to the body of right zygoma with normal overlying skin. On palpation, swelling was tender, bone hard, and fixed to the underlying bone. No palpable thrill was identified. A provisional diagnosis of osteoma of zygoma was made.
Figure 1: Worm's view of clinical picture of the patient with swelling in the right zygomatic region

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Further, radiographic investigations were planned, and the following set of radiographs were taken. In tangential projections taken on Intra Oral Periapical film showed a characteristic spoke wheel appearance with internal radiopaque striae, radiating from the center of the lesion. The axial section of Computed Tomography showed a trabeculated mass of size 1.8 cm × 1.8 cm at its greatest dimension, arising from the body of the right zygoma and extending onto the anterior one-fourth of the zygomatic arch. The internal structure appeared to have trabeculae radiating from the center of the lesion like a cartwheel or spoke wheel. There is displacement and thinning of outer and inner cortices and it resulted in a slight breach of the inner cortex. The coronal section of Computed Tomography showed the location of trabeculated mass in the inferolateral aspect of orbit and superolateral aspect of maxillary sinus causing no deformity in the bony margins of these two structures [Figure 2]. MRI confirmed the CT localization of the lesion and showed an overall intermediate T1 signal intensity and a high T2 signal intensity with flow voids within the lesion suggestive of fluid within the lesion [Figure 3]. Based on the history, clinical and imaging study, a differential diagnosis of Central hemangioma and AV malformation were considered.
Figure 2: Axial section of CT shows hypodense and hyperdense areas within the lesion. Coronal section of CT shows location of lesion with respect to orbit and maxillary sinus

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Figure 3: T1 axial MRI view shows hypo to intermediate signal intensity within the lesion. T2 axial MRI view shows hyperintense signal area with flow voids of hypointensity within the lesion

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Considering this differential diagnosis, surgical complications like profuse bleeding were anticipated and to prevent this, necessary precautions were taken and surgical excision of the mass with a 0.5 mm safe margin was done. Considering facial esthetics, approach to the mass was planned through both intra-oral 'uccal vestibule) and extra-oral incisions (through infraciliary approach). Reconstruction of the surgical defect was done using iliac crest graft.

The excised gross specimen appeared to be bluish and bony hard [Figure 4]. The radiographs of the gross specimen showed the characteristic spoke wheel appearance [Figure 4]. Specimen was subjected to histopathological evaluation. The photomicrograph of 4 × and 10 × microscopic views [Figure 5] showed mature lamellar trabecular bone in which the inter-trabecular space was completely occupied by fibro-vascular connective tissue with large, dilated, thin-walled vessels and endothelial-lined sinusoidal spaces are filled with blood, consistent with a cavernous hemangioma.
Figure 4: Bluish colored gross specimen and its characteristic spoke wheel type of radiographic appearance

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Figure 5: Histopathology picture in 4x view shows blood filled sinusoid spaces surrounded by fibrovascular connective tissue. Histopathology picture in 10x view clearly showing the sinusoidal spaces with RBC and the surrounding epithelium and fibro vascular connective tissue

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Post-operative follow up after 3 months showed improved facial esthetics and reduced facial asymmetry. Patient is still under follow-up, with no functional or aesthetic complaints.

   Discussion Top

Hemangiomas are common benign vascular tumors occurring anywhere in skin or soft tissues like muscles, tendons, connective tissue, fatty tissue, synovium or bone.[1] Hemangiomas represent an anomalous proliferation of endothelial-lined vessels and are regarded as a hamartoma rather than a real neoplasia.[2] The most frequent sites include the calvaria and the vertebral column. Involvement of facial bones is rare, and if occurs involves most commonly the maxilla, mandible, and nasal bones. Matthews[3] and Watson and McCarthy[4] consider hemangiomas to be congenital, but in 50% of the cases a history of trauma is elicited, particularly with those located in vulnerable areas such as the nasal bones.[5] Although they have been described in the facial skeleton, those occurring in the zygomatic bone are extremely rare with a male to female rate of 1:3. It occurs most commonly in the second decade of life, but cases have also been reported in newborn babies and ninth decade of life. The left zygoma is more frequently involved (60%) than the right (40%).[2]

The radiographic appearance of central hemangioma is variable, so it is considered as a “great mimicker”. The radiopaque striations usually constituted several or many locules, known as a soap bubble or honeycomb pattern. Less commonly, the multilocular lesion exhibits internal radiopaque striae, which radiate from the center of the lesion and resemble spokes of a wheel. But few authors claim that a spoke-like appearance is a common radiological manifestation of central hemangioma. Central hemangiomas can also appear as a unilocular appearance[6] [Figure 6].
Figure 6: Various radiographic appearances of Central Hemangioma. (a) Unilocular, (b) Multilocular with few septa, (c) Multilocular with many septa, (d) Sparse Trabeculae, (e) Spoke-like, (f) Hghly trabeculated resembling a honeycomb, (g) Tube-like appearance. Small, rounded radiolucent areas are due to the axial projection of some of the tubular areas. (h) Radiating bony spicules most often seen on skull tangential projection. (i) Direct projection of rays passing axially through tubular bony spaces. Of great importance is the marginal cortex of compact bone which points to be a benign condition. (j) Cancellous spaces slightly enlarged. (k) Cancellous spaces reduced in size and with increased density of trabeculae. (l) Mixture of normal and enlarged cancellous spaces, some of which are elongated. (Courtesy of Drs. H.M. Worth and D. Stoneman, Toronto, Canada, and Eastman Kodak Company, Rochester, N.Y.)

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Radiographic differential diagnosis includes osteosarcoma, fibrous dysplasia, central giant cell granuloma, ameloblastoma, odontogenic myxoma, multiple myeloma, dentigerous cyst, and aneurysmal bone cyst.[6] Central hemangiomas that are diagnosed based on clinical and radiographic appearance, Angiography proves to be a useful diagnostic tool which demonstrates the presence of vascular lesions and delineates its boundaries. But, past experience suggests that the diagnostic and therapeutic benefits obtained from preoperative angiography are overshadowed by the potential risks of the procedure. Diagnosis is readily made by plain-film and CT radiographic examination.

During any surgical procedure either tooth extraction or biopsy, the most hazardous complication in hemangioma patients is uncontrolled bleeding and consequently even death. Profuse intraoperative bleeding was reported in all previously reported cases which was because of either incisional biopsy or curettage directly into the tumor.[7] A review of the previously reported cases revealed no evidence of troublesome intraoperative bleeding when a margin of normal bone was resected along with the specimen.[8]

Reconstruction of the zygoma after excision of an intraosseous hemangioma requires the reestablishment of normal facial symmetry and contour. A variety of reconstructive modalities have been reported previously. These include

  1. Tumor excision without defect repair[9]

  2. Repair with autologous rib[10]
  3. Repair with pedicled infratemporal fat[11]
  4. Repair with Surgical pack[12]
  5. Repair with autogenous iliac crest bone graft[12]
  6. Repair with a silicone implant[13]
  7. Repair with split calvarial bone graft.[14]

Whatever might be the method of reconstruction, the final goal should be the facial esthetics that has to be maintained because the zygoma is a corner stone for facial esthetics. Most commonly and successful approaches used for the treatment of hemangiomas besides conventional surgical excision are cryosurgery, laser surgery after Sclerosing solution and intravascular embolization with plastic spheres.[15]

   Review of Literature Top

   Conclusion Top

The significance of Central haemangioma increases manifold in this particular case report is because of the location of the lesion i.e., in the zygoma. Central haemangioma has got varied radiographic appearances, therefore proper diagnosis by radiologist and histopathologic confirmation by pathologist is very important to prevent complications like bleeding. Central haemangioma should be considered in the differential diagnosis and proper precautions must be taken in establishing the final diagnosis before any surgical treatment is planned.

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 Top

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Correspondence Address:
Archana Pokala
Department of Oral Medicine and Radiology, Kamineni Institute of Dental Sciences, Narketpally - 508 254, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_707_20

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