Multiple jaw radiolucencies in a patient with recurrent skin cancers

Correct answer: A. Multiple odontogenic keratocysts

• A. CORRECT. MULTIPLE ODONTOGENIC KERATOCYSTS are the jaw manifestation of this syndrome and are often the presenting feature. They arise from the epithelial rests of Serres (dental lamina remnants), grow ANTERO-POSTERIORLY along the marrow space with little expansion — which is why the radiolucencies look large for the amount of clinical swelling — and they carry a high recurrence rate from satellite cysts and a friable lining. Enucleation with peripheral ostectomy or Carnoy solution, and long-term radiographic review, are standard.
• B. A dentigerous cyst surrounds the CROWN of an UNERUPTED tooth and attaches at its cementoenamel junction. It is unilocular and, most importantly, is defined by its relationship to a tooth — multiple such cysts are not the pattern here.
• C. A radicular cyst is inflammatory, arises at the apex of a NON-VITAL tooth from the rests of Malassez, and would require multiple non-vital teeth to explain multiple lesions. Nothing in the findings supports pulpal disease.
• D. Cherubism is a bilateral, symmetric, painless multilocular expansion of the POSTERIOR MANDIBLE in a young child, producing a full-cheeked appearance that regresses after puberty. It has no association with basal cell carcinoma or rib anomaly.

Note: What these two items add is the syndrome as the STEM — recognising it from the pattern and knowing what to do next. Nevoid basal cell carcinoma syndrome is autosomal dominant, caused by PTCH1 mutation on chromosome 9q. Features: multiple basal cell carcinomas, multiple odontogenic keratocysts, palmar and plantar pitting, calcified falx cerebri, bifid or splayed ribs, frontal bossing, hypertelorism, and medulloblastoma risk in childhood.

Correct answer: D. Oral and maxillofacial surgery, and dermatology

• A. Periodontics and endodontics treat conditions this patient does not have. The lesions are developmental odontogenic cysts, not inflammatory lesions of pulpal or periodontal origin.
• B. Prosthodontic and orthodontic input may eventually be needed if teeth are lost with the cysts, but neither addresses either active disease.
• C. Paediatric dentistry is not applicable at 26, and while oral medicine may co-manage, it does not replace surgical enucleation or dermatologic oncology care.
• D. CORRECT. The two active disease processes are the JAW CYSTS and the SKIN CANCERS. ORAL AND MAXILLOFACIAL SURGERY manages enucleation of the keratocysts and the long-term surveillance they require, and DERMATOLOGY manages the basal cell carcinomas and screens for new ones — lifelong, since these patients develop them continuously and must avoid sun and, critically, IONISING RADIATION, which accelerates BCC formation. Genetic counselling for an autosomal dominant condition is the third strand.

Note: The radiation caution is the practically important point for a dentist: minimise radiographic exposure in these patients, use the fastest receptors and rectangular collimation, and avoid CT where an alternative exists — but do not abandon the surveillance imaging the keratocysts genuinely need. It is a balance, not a prohibition.