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Review
. 2019 Jul 18;48(1):32.
doi: 10.1186/s40463-019-0356-5.

An aggressive central giant cell granuloma in a pediatric patient: case report and review of literature

Yiqiao Wang  1 , Andre Le  2 , Dina El Demellawy  3 , Mary Shago  4   5 , Michael Odell  6 , Stephanie Johnson-Obaseki  6
Affiliations
Review

An aggressive central giant cell granuloma in a pediatric patient: case report and review of literature

Yiqiao Wang et al. J Otolaryngol Head Neck Surg. .

Abstract

Background: Central giant cell granulomas are benign tumours of the mandible, presenting in children and young adults. Divided into non- and aggressive subtypes, the aggressive subtype is relatively rare and can occasionally progress rapidly, resulting in significant morbidity.

Case presentation: We present a case of an aggressive central giant cell granuloma (CGCG) in a six year-old female. The lesion originated in the right mandibular ramus and progressed rapidly to involve the condyle. Diagnosis was made using a combination of imaging and pathology. A timely en bloc resection of the hemi-mandible was performed with placement of a reconstructive titanium plate and condylar prosthesis.

Conclusion: Our case demonstrates the importance of considering CGCG in the differential diagnosis of rapidly progressive mandibular lesions in the pediatric population. Prompt diagnosis and management can greatly improve long-term outcomes.

Keywords: Aggressive; Central giant cell granuloma; Mandible; Pediatrics.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Frontal radiograph of the facial bones at initial presentation to the emergency department. Arrow is pointing to site of lesion
Fig. 2
Fig. 2
MRI Head following initial presentation to the emergency department with coronal and axial views. Arrows are pointing to site of lesion
Fig. 3
Fig. 3
CT Head following initial presentation to the emergency department, with axial and coronal views. Arrows are pointing to site of lesion
Fig. 4
Fig. 4
3D CT reconstruction of skull at second presentation to the emergency department. Arrows are pointing to lesion. Significant obliteration of the right hemimandbile including condyle
Fig. 5
Fig. 5
Gross picture of the second excision showing multicystic lesion filled with clotted blood
Fig. 6
Fig. 6
Low power light microscopic view of the lesion showing multicystic spaces filled with blood (H&E X100)
Fig. 7
Fig. 7
Medium power light microscopic view of the lesion showing fibrous thick septa with reactive woven bony trabeculae. The later are highlighted by back arrow heads (H&E X400)
Fig. 8
Fig. 8
High power light microscopic view of the lesion showing a cystic space and a thick septum formed of myofibroblastic cells. Note the cystic space is devoid of lining (H&E X400)
Fig. 9
Fig. 9
Interphase FISH analysis using a dual colour breakapart probe for the USP6 gene locus (17p13.2; ZytoVision, Bremerhaven, Germany) showing USP6 gene rearrangement in lesional cell nuclei. The abnormal signal pattern has loss of the 5′ USP6 signal with retention of the 3′ USP6 signal
See this image and copyright information in PMC

References

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