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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 5  |  Issue : 2  |  Page : 60-63

Primary gnathic aneurysmal bone cyst in a child patient


Department of OMDR, HPGDC and H, Shimla, Himachal Pradesh, India

Date of Web Publication18-Nov-2019

Correspondence Address:
Dr. Neeta Sharma
Department of OMDR, HPGDC and H, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijohr.ijohr_25_19

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  Abstract 


Aneurysmal bone cyst(ABC) is a benign bone tumour which mainly affects long bones and rarely affects craniofacial bones. It is relatively rare lesion and exhibit variable clinical and radiographic presentations. They are two types “primary” when present solely and “secondary” when preceded by other bony tumors. Radiographically, it exhibits unilocular to multilocular appearance with cortical plates' expansion; and fluid level on computed tomography and magnetic resonance imaging. Histopathologically, it shows three types: solid, mixed, and vascular. The surgical resection is the treatment of choice wing to its high recurrence rate. We present here a rare case of ABC in a 9-year-old male patient present in the anterior mandible crossing midline.

Keywords: Aneurysmal bone cyst, bone neoplasm, mandible


How to cite this article:
Prakash R, Sharma N, Guruprasad R, Verma S. Primary gnathic aneurysmal bone cyst in a child patient. Indian J Oral Health Res 2019;5:60-3

How to cite this URL:
Prakash R, Sharma N, Guruprasad R, Verma S. Primary gnathic aneurysmal bone cyst in a child patient. Indian J Oral Health Res [serial online] 2019 [cited 2019 Dec 10];5:60-3. Available from: http://www.ijohr.org/text.asp?2019/5/2/60/271143




  Introduction Top


The word aneurysmal means “blow out effect” expansion of bone. In 1942, Jaffe and Lichtenstein first described aneurysmal bone cyst (ABC) as benign, expansile osteolytic lesion-containing thin walled sinusoidal cavities. In 1958, Bernier and Bhaskar reported the first case of ABC in the jaws.[1] ABC constitutes 1.5% of nonodontogenic and nonepithelial cyst of the mandible. The WHO has classified ABC under miscellaneous lesions.[2] 12% of ABC affect head-and-neck region, of which 2% occur in jaws. 2/3 of cases occur in the mandible and 1/3 of cases occur in maxilla.[1],[3] Posterior mandible is the most frequent site in the mandible followed by ramus, coronoid, and condyles. The anterior mandible is the least affected site. The peak incidence is seen in the second decade. Males show fewer predilections than females with 1:1.3 sex ratio.[4] When ABC is present de novo it is termed as “Primary ABC” and when it follows other tumors, including fibrous dysplasia, giant cell tumors, chondroblastoma, and nonossifying fibroma, it is termed as “secondary ABC.”[5]

Previously, ABCs were considered as pseudotumor, but features recurrent chromosomal translocation resulting in gene fusion between ubiquitin-specific peptidase 6 and multiple partners, including COL1A1, CDH11, TRAP150, ZNF90, and OMD and is identified as a true neoplasm.[6] Radiographically, it demonstrates variable unilocular and multilocular features and shows fluid level on computed tomography (CT) and magnetic resonance imaging (MRI) imaging.[7] Histopathologically, it shows large cystic spaces separated by fibrous septa, alternating with the solid area with multinucleated giant cells.[8]


  Case Report Top


A 9-year-old male child reported to our dept. with a chief complaint of occasional pain and swelling at the chin for 6 months. The patient developed a small swelling at the chin with occasional pain 6 months back which slowly enlarged to the present size. There was no history of trauma. Medical and dental history was nonrelevant. On general examination, the patient's vitals were within the normal limits. On extra-oral examination, there was a diffuse swelling present at chin in midline, which was 5 cm × 5 cm approximately in size. The swelling was firm and nontender, the overlying skin was normal [Figure 1]. Regional lymph nodes were not palpable. The intra-oral examination revealed a diffuse swelling in anterior mandible obstructing facial vestibule with displaced 31. The cortical plates showed expansion. The swelling extended from the right deciduous first molar to the left deciduous first molar. The overlying mucosa appeared normal. The swelling was firm and nontender. All four mandibular incisors were vital. Routine blood and biochemical investigations were within the normal limits. Radiographic and CT examination revealed an expansile large multilocular osteolytic lesion with internal septation in the anterior mandible [Figure 2], [Figure 3], [Figure 4]. The provisional diagnosis of ameloblastoma with differential diagnosis of central giant cell granuloma, juvenile ossifying fibroma, and ABC were made. The biopsy was done, and histopathological examination revealed sinusoidal spaces which were not lined by endothelial cells, filled with red blood cells and surrounded by fibro-connective tissue. Multinucleated giant cells were also present [Figure 5] and [Figure 6]. The histopathological features were suggestive of ABC. The total enucleation was planned and the gross specimen [Figure 7] showed solid lesion, and there was no history of recurrence in 1-year follow-up [Figure 8].
Figure 1: Swelling at chin region

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Figure 2: Occlusal radiograph showing multilocular osteolytic lesion with facial cortical plate expansion

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Figure 3: Axial computed tomography shows multilocular osteolytic lesion with internal septation and facial cortical expansion

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Figure 4: Three-dimensional-computed tomography shows perforation of cortical plates

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Figure 5: Microphotgraph showing sinusoidal spaces or cavity filled with red blood cells surrounded by fibroconnective tissue and not lined by endothelial cells

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Figure 6: Microphotograph showing multinucleated giant cells

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Figure 7: Gross specimen after enucleation

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Figure 8: Follow-up

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  Discussion Top


ABC is a rare benign expansile osteolytic neoplasm of the bone mainly affecting long bones, flat bones such as vertebrae and pelvis and jawbones. The peak incidence is seen in the second decade.[9] In our case, it has affected mandible of 9-year-old male patient. It crossed the midline also and this makes it a rare case. The etiology of ABC is conflicting. Jeff and Lichenstein suggested that the increased venous pressure and repletion of the vascular bed in the transformed bone caused by the alteration of local hemodynamic was related to resorption, connective tissue replacement, and osteoid formation.[4] Some researchers have reported the previous history of trauma and subperiosteal hematoma formation as a predisposing factor for ABC formation.[3] No history of trauma was reported in our case. Familial incidence is also reported in the literature. Only 3.6% of cases in jaws are reported in the anterior mandible. Clinically and radiographically, ABC demonstrates extreme variability. Pain is the most common clinical symptom at presentation. Local swelling may develop as the lesion increases in size. The other symptoms reported are deformity, disruption of growth plates, neurological symptoms, and pathological fractures. The clinical manifestations may range from a small asymptomatic swelling to a rapidly growing extensive lesion.[10] The painful swelling was also reported in our case.

Radiographically, it shows eccentric expansion of bone, cortical erosion, and destruction, small peripheral areas of periosteal bone reaction. Fluid levels can be detected on CT and MRI. Honeycomb appearance with fluid levels can be appreciated on MRI. MRI is important to distinguish between primary and secondary ABCs. If there is soft-tissue mass present in preoperative MRI, aggressive tumors such as osteosarcoma and giant cell tumor must be considered. In our case, eccentric expansion of bone plates and multilocular appearance was exhibited.[5] Histologically, three types of ABCs are reported: (1) solid- it shows dense stroma with vascular phase. (2) Mixed/transistory - it shows cellular stroma with moderate amount of blood-filled space, and (3) vascular- it shows loose connective tissue stroma with abundant large blood-filled spaces. Cysts and septa are lined by fibroblasts, myofibroblasts and histiocytes and not endothelium. Clusters of osteoclasts like multinucleated giant cells with loose spindly stroma to cellular stroma, reactive woven bone, degenerated calcifying fibromyxoid tissue are present. Variable mitotic figures and hemosiderin with no atypia and no malignant osteoid are present. Molecular abnormality of 17p13.2 loci is reported in 63% of cases.[3] Histopathologically, our case was similar to solid type. Diagnosis is usually made by biopsy.

It is important to differentiate between primary and secondary ABC to institute appropriate treatment plan. Secondary ABCs are usually seen >30 years of age. The age of our patient was in the first decade and no other bone tumor was associated which was confirmed by histopathological examination. The main aim of treatment is to hinder the progression of the lesion, alleviate pain, prevent pathological fracture, and decrease recurrence rate. Management includes complete surgical excision, intralesional curettage, and bone grafting. Less invasive methods include particulate embolization, intralesional injection of alcoholic zein, and radiotherapy.[11] The patient was treated with local surgical excision, and the inferior border was preserved in our case. High-recurrence rate is reported in curettage treatment. Local recurrence rate after surgical removal is about 11.8%–30.8%. No recurrence was reported in our patient in a year of follow-up.


  Conclusion Top


As ABC exhibit extreme variability clinically and radiographically, therefore accurate diagnosis can only be made after histopathological examination. In our case, the lesion was present in the anterior mandible and crossed the midline, which makes it a rare presentation.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Banerji R, Pachisia S, Basu S, Sahu S, Ghosh S. A saga of two aneurysmal bone cyst cases: An institutional case report. Indian J Dent Sci 2018;10:113-7.  Back to cited text no. 1
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2.
Available from: http://www.publications.iarc.fr/Book-And-Report -Series/Who-Iarc-Classificatio-of-Tumours. [Last accessed on 2019 Jul 09].  Back to cited text no. 2
    
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Urs AB, Augustine J, Chawla H. Aneurysmal bone cyst of the jaws: Clinicopathological study. J Maxillofac Oral Surg 2014;13:458-63.  Back to cited text no. 3
    
4.
Devi P, Thimmarasa V, Mehrotra V, Agarwal M. Aneurysmal bone cyst of the mandible: A case report and review of literature. J Oral Maxillofac Pathol 2011;15:105-8.  Back to cited text no. 4
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5.
Sasaki H, Nagano S, Shimada H, Yokouchi M, Setoguchi T, Ishidou Y, et al. Diagnosing and discriminating between primary and secondary aneurysmal bone cysts. Oncol Lett 2017;13:2290-6.  Back to cited text no. 5
    
6.
Warren M, Xu D, Li X. Gene fusions PAFAH1B1-USP6 and RUNX2-USP6 in aneurysmal bone cysts identified by next generation sequencing. Cancer Genet 2017;212-213:13-8.  Back to cited text no. 6
    
7.
Fernández AB, Medina BG, Plaza AM, Aguilar-Salvatierra A, Gómez-Moreno G. Aneurysmal bone cyst of the mandible affecting the articular condyle: A case report. Clin Case Rep 2016;4:1175-80.  Back to cited text no. 7
    
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Simsek HO, Yildirim D, Gormez O, Yuce E, Kapucuoglu FN. Aneurysmal bone cyst of the mandible: Report of a case with a review of the literature. J Oral Maxillofac Radiol 2014;2:64-7.  Back to cited text no. 8
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Bharadwaj G, Singh N, Gupta A, Sajjan AK. Giant aneurysmal bone cyst of the mandible: A case report and review of literature. Natl J Maxillofac Surg 2013;4:107-10.  Back to cited text no. 9
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Sun ZJ, Sun HL, Yang RL, Zwahlen RA, Zhao YF. Aneurysmal bone cysts of the jaws. Int J Surg Pathol 2009;17:311-22.  Back to cited text no. 10
    
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An SY. Aneurysmal bone cyst of the mandible managed by conservative surgical therapy with preoperative embolization. Imaging Sci Dent 2012;42:35-9.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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