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Case Reports
. 2021 Mar 19;14(3):e240952.
doi: 10.1136/bcr-2020-240952.

Juvenile Psammomatoid Cemeto-ossifying Fibroma of Mandible: a Diagnostic dilemma

Santhosh Rao  1 , Vandita Singh  2 , Abdul Hafeez A  3 , Subham S Agarwal  3
Affiliations
Case Reports

Juvenile Psammomatoid Cemeto-ossifying Fibroma of Mandible: a Diagnostic dilemma

Santhosh Rao et al. BMJ Case Rep. .

Abstract

Psammomatoid Juvenile ossifying fibroma (PsJOF) is a rare benign fibro-osseous lesion characterised to grow to unusually large size very rapidly. Its usual presentation is in younger age group mostly children and predominately involving the Sino-Naso-Orbital region. Its aggressive nature gimmicks a malignant lesion but it is rather a benign lesion with a higher recurrence rate than the conventional ossifying fibroma but lacking metastatic potential. The high recurrence rate makes it essential that the lesion is not reconstructed immediately and thorough monitoring in the follow-up period. Lesion of such clinical importance needs to be diagnosed preoperatively to provide a better and radical surgical treatment option, but the variability in its presentation as seen in this case makes it even harder to diagnose. We aim to draw attention to the rare phenomena that PsJOF presents to help readers broaden their purview in diagnosis and thereby manage them accordingly.

Keywords: head and neck cancer; oral and maxillofacial surgery; otolaryngology / ENT.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Clinical presentation of the patient—frontal view.
Figure 2
Figure 2
Clinical presentation of the patient—lateral view.
Figure 3
Figure 3
Pedunculated mass extruding out of the oral cavity through the breach created in the cheek.
Figure 4
Figure 4
Hyperemic mucosa covering the oral surface of the tumour.
Figure 5
Figure 5
Contrast-enhanced CT revealing egg mass appearance.
Figure 6
Figure 6
Multiple septa with flecks of calcification was seen within the tumour mass on contrast-enhanced CT.
Figure 7
Figure 7
Fig; 3 CT angiography image showing the single facial artery as a feeder vessel.
Figure 8
Figure 8
Tumour prepared to be excised, frozen section biopsy was sent for confirmation of the pathology.
Figure 9
Figure 9
Tumour excision through lip split approach.
Figure 10
Figure 10
Tumour specimen measuring over 17 cm.
Figure 11
Figure 11
Oral layer closure using pedicled skin flap and lingual mucosa.
Figure 12
Figure 12
Harvested iliac crest corticocancellous graft.
Figure 13
Figure 13
Mandible reconstruction using Iliac crest corticocancellous graft.
Figure 14
Figure 14
Deltopectoral flap to close the defect.
Figure 15
Figure 15
Photomicrograph at 10× showing psammoma bodies in the stroma of fibrous proliferation characteristic of juvenile psammomatoid ossifying fibroma.
Figure 16
Figure 16
Photomicrograph at 40× showing spindle shaped fibroblastic cells with highly rich fibrous stroma giving a whorled appearance with relatively acellular psammomatoid ossicles.
Figure 17
Figure 17
12-Month follow-up picture of the patient.
See this image and copyright information in PMC

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References

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