Pathology · patient case question
What are the jaw lesions most likely to be?
Patient case: Multiple jaw radiolucencies in a patient with recurrent skin cancers
Patient: Female, 26 years old
Chief Complaint: “My dentist saw some dark areas on my x-ray.”
Background: Chart records excision of FOUR basal cell carcinomas from the face and trunk since age 19. Chest imaging previously reported a BIFID RIB. Frontal bossing and hypertelorism noted. Mother had similar skin lesions.
Vital Signs: BP 116/70 mmHg · HR 74 bpm
Findings: Panoramic radiograph shows MULTIPLE well-defined, corticated MULTILOCULAR radiolucencies in the posterior body and ramus of the mandible bilaterally, and one in the maxilla. Minimal buccolingual expansion for the size of the lesions. Palmar pitting is present.
Show answer and explanation
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.