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Nevoid Basal Cell Carcinoma Syndrome - Clinical and Radiological Findings of Three Cases

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ABSTRACT

Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder, characterized by skeletal anomalies and multiple keratocystic odontogenic tumors of the jaws. The skeletal anomalies of this syndrome are mandibular prognathism, bossing of frontal and parietal bones, high-arched palate, and bifid rib. We report three cases with NBCCS, emphasizing the clinical and radiographic findings, the importance of the early diagnosis of NBCCS, and a preventive multidisciplinary approach in the management of NBCCS.

No MeSH data available.


(A) Extraoral profile; (B) Intraoral examination; (C, D) Physical examination; (E) PA mandible radiograph; (F) Chest radiograph; (G) Panoramic radiograph; (H) Postoperative radiograph
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FIG2: (A) Extraoral profile; (B) Intraoral examination; (C, D) Physical examination; (E) PA mandible radiograph; (F) Chest radiograph; (G) Panoramic radiograph; (H) Postoperative radiograph

Mentions: A 10-year-old male reported to the Oral Medicine unit with the complaint of pain on the left side of the face. The patient’s medical history and family history were noncontributory. The general examination revealed macrocephaly, ocular hypertelorism (Figure 2A), palmar and plantar pits (Figure 2C), and a Sprengel scapular deformity (Figure 2D). On extraoral examination, facial symmetry was noted. Intraorally, swelling in the mandibular right parasymphyseal region and high arched palate (Figure 2B) was observed. Routine blood investigations (CBC, Hb, BT, CT, and ESR), serum calcium, phosphorus, and alkaline phosphatase were normal. A posterior to anterior (PA) mandible radiograph revealed mild calcification of the falx cerebri (Figure 2E). A chest radiograph demonstrated bifid ribs (Figure 2F). A panoramic radiograph revealed three well-defined radiolucencies suggestive of a benign odontogenic cyst (Figure 2G). NBCCS was established as a provisional diagnosis, based on the clinical and radiological findings. The patient was managed surgically under general anesthesia. Surgical extraction of an inferiorly displaced mandibular permanent left canine was performed. Multiple mandibular lesions were enucleated, and Carnoy’s solution was applied to peripheral osseous walls. The histopathologic examination of the enucleated tissue demonstrated features suggestive of KCOT. Healing of the enucleated sites was observed on follow-up visits (six months) without any sign of recurrence (Figure 2H).


Nevoid Basal Cell Carcinoma Syndrome - Clinical and Radiological Findings of Three Cases
(A) Extraoral profile; (B) Intraoral examination; (C, D) Physical examination; (E) PA mandible radiograph; (F) Chest radiograph; (G) Panoramic radiograph; (H) Postoperative radiograph
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5016041&req=5

FIG2: (A) Extraoral profile; (B) Intraoral examination; (C, D) Physical examination; (E) PA mandible radiograph; (F) Chest radiograph; (G) Panoramic radiograph; (H) Postoperative radiograph
Mentions: A 10-year-old male reported to the Oral Medicine unit with the complaint of pain on the left side of the face. The patient’s medical history and family history were noncontributory. The general examination revealed macrocephaly, ocular hypertelorism (Figure 2A), palmar and plantar pits (Figure 2C), and a Sprengel scapular deformity (Figure 2D). On extraoral examination, facial symmetry was noted. Intraorally, swelling in the mandibular right parasymphyseal region and high arched palate (Figure 2B) was observed. Routine blood investigations (CBC, Hb, BT, CT, and ESR), serum calcium, phosphorus, and alkaline phosphatase were normal. A posterior to anterior (PA) mandible radiograph revealed mild calcification of the falx cerebri (Figure 2E). A chest radiograph demonstrated bifid ribs (Figure 2F). A panoramic radiograph revealed three well-defined radiolucencies suggestive of a benign odontogenic cyst (Figure 2G). NBCCS was established as a provisional diagnosis, based on the clinical and radiological findings. The patient was managed surgically under general anesthesia. Surgical extraction of an inferiorly displaced mandibular permanent left canine was performed. Multiple mandibular lesions were enucleated, and Carnoy’s solution was applied to peripheral osseous walls. The histopathologic examination of the enucleated tissue demonstrated features suggestive of KCOT. Healing of the enucleated sites was observed on follow-up visits (six months) without any sign of recurrence (Figure 2H).

View Article: PubMed Central - HTML - PubMed

ABSTRACT

Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder, characterized by skeletal anomalies and multiple keratocystic odontogenic tumors of the jaws. The skeletal anomalies of this syndrome are mandibular prognathism, bossing of frontal and parietal bones, high-arched palate, and bifid rib. We report three cases with NBCCS, emphasizing the clinical and radiographic findings, the importance of the early diagnosis of NBCCS, and a preventive multidisciplinary approach in the management of NBCCS.

No MeSH data available.