PEMPix is the American Academy of Pediatrics Section on Emergency Medicine’s annual visual diagnosis competition. This year, in addition to the 10 finalists I will be presenting (virtually) at the National Conference and Exhibition I will be sharing three cases online in advance of the conference. This is the first of the three cases.

This case was submitted by…

An 11-year-old boy presented to the emergency department with left-sided facial swelling. He and his parents noticed this about two weeks ago. He first thought it was a pimple, but it was mobile and nontender. It has since become firmer and continues to enlarge at a relatively rapid pace. He has not had fever or recent illnesses. His past medical history of asthma with a couple of cases of remote sinusitis. Despite the swelling, he has not had any pain when he pushes on the area or when he moves his facial muscles or chews..

On exam he is well appearing and afebrile with normal vital signs. There is a distinct area of prominence over his left maxilla that is firm and nontender. He has normal dentition including no dental caries. His oropharynx is normal. His cranial nerve function and sensation throughout his face is normal. 

His labs were notable for a normal CBC with differential, ESR, and CRP.

To better figure out what this swelling was the team obtained a CT scan of his face, and here is a sagittal view.

A. Odontogenic Abscess

B. Dentigerous Cyst

C. Giant Cell Tumor

D. Adenomatoid Odontogenic Tumor

E. Periapical Cyst

D. Adenomatoid Odontogenic Tumor

The CT imaging of the maxilla revealed a round, expansile lesion in the left maxilla with well-circumscribed margins which contains an uninterrupted left maxillary incisor tooth as well as circumferential-oriented calcific deposits.

This imaging is consistent with an adenomatoid odontogenic tumor. These are uncommon, benign, slow-growing lesions. These lesions are referred to as “two-thirds tumor” as 66% of these present within the first two decades of life, 66% in females, 66% surround an impacted tooth, and 66% involve the canine. From an imaging perspective, these cysts typically involve the crown and part of the root of the tooth as seen in our case involving the canine. 66% of these will also reveal calcifications within the cyst as demonstrated in our case.

Periapical cysts are an inflammatory odontogenic cysts and are the most common cyst of the jaw. These are associated with the apex of a nonvital tooth that typically has had dental trauma or caries. The cyst comes from the residual epithelial remnants of the tooth and grows due to increase blood flow from the surrounding inflammation. As described in its name, the cyst sits only at the apex of the tooth.

Dentigerous cysts are the second most common cyst of the jaw and are associated with the crown of an impacted or partially erupted tooth. Permanent third molars and canines are the most likely to fail to erupt, which make them the most likely to involve dentigerous cysts. These cysts typically involve the crown but in contrast to an adenomatoid odontogenic tumor, these never involve the root of the tooth.

Odontogenic abscess is an infection of the tooth and typically is associated with dental caries. These are painful and may be associated with fever, two features not appreciated in this case.

Lastly, giant cell tumors are typically a benign but painful tumor of the bone typically in the lower extremities. These very rarely involve the mandible and occur in the second to fourth decade of life.

Patient Follow-Up

Given the findings of these exams, the oral maxillofacial surgeons evaluated the patient in the emergency department and scheduled a biopsy as an outpatient to confirm the diagnosis. Staged therapy planning is ongoing.

References

Grover S, Rahim AM, Parakkat NK, Kapoor S, Mittal K, Sharma B, Shivappa AB. Cystic Adenomatoid Odontogenic Tumor. Case Rep Dent. 2015;2015:503059. doi: 10.1155/2015/503059. Epub 2015 Oct 22. PMID: 26579317; PMCID: PMC4633555.

Bilodeau EA, Collins BM. Odontogenic Cysts and Neoplasms. Surg Pathol Clin. 2017 Mar;10(1):177-222. doi: 10.1016/j.path.2016.10.006. Epub 2016 Dec 29. PMID: 28153133.