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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.

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(A) Extraoral profile; (B) General examination showing palmar pits; (C) Panoramic radiograph; (D) Chest radiograph; (F) Cone beam CT scan (axial view); (E) Postoperative panoramic radiograph
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FIG1: (A) Extraoral profile; (B) General examination showing palmar pits; (C) Panoramic radiograph; (D) Chest radiograph; (F) Cone beam CT scan (axial view); (E) Postoperative panoramic radiograph

Mentions: A 16-year-old male reported to the Oral Medicine unit with the complaint of pain and swelling in the right and left posterior mandibular region for two weeks' time. The patient’s medical history and family history were noncontributory. His general examination revealed frontal bossing, wide nasal bridge, hypertelorism (Figure 1A), and palmar and plantar pits (Figure 1B). Extraorally, the facial profile was asymmetrical due to swelling in the right side of the face. The temperature of the overlying skin was normal. Intraorally, an irregular, ill-defined swelling expanding the lingual and buccal cortex was present in the right side of the mandible with bony hard consistency. Blood investigations (CBC, Hb, BT, CT, ESR), serum calcium, phosphorus, and alkaline phosphatase were within normal limits. A panoramic radiograph revealed four well-defined radiolucencies suggestive of a benign odontogenic cyst (Figure 1C). A chest radiograph demonstrated bifid ribs (Figure 1D). Cone beam CT scan illustrated the expansion of buccal and lingual cortical plates, perforation of the lingual cortical plate, and a horizontally impacted mandibular permanent left second molar (Figure 1F). NBCCS was established as a provisional diagnosis, based on the clinical and radiological findings. Mandibular molars involved in the lesion were extracted surgically. 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 a keratocystic odontogenic tumor (KCOT). Healing of the enucleated sites was observed on follow-up visits (six months) without any sign of recurrence (Figure 1E).


Nevoid Basal Cell Carcinoma Syndrome - Clinical and Radiological Findings of Three Cases
(A) Extraoral profile; (B) General examination showing palmar pits; (C) Panoramic radiograph; (D) Chest radiograph; (F) Cone beam CT scan (axial view); (E) Postoperative panoramic radiograph
© Copyright Policy - open-access
Related In: Results  -  Collection

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

FIG1: (A) Extraoral profile; (B) General examination showing palmar pits; (C) Panoramic radiograph; (D) Chest radiograph; (F) Cone beam CT scan (axial view); (E) Postoperative panoramic radiograph
Mentions: A 16-year-old male reported to the Oral Medicine unit with the complaint of pain and swelling in the right and left posterior mandibular region for two weeks' time. The patient’s medical history and family history were noncontributory. His general examination revealed frontal bossing, wide nasal bridge, hypertelorism (Figure 1A), and palmar and plantar pits (Figure 1B). Extraorally, the facial profile was asymmetrical due to swelling in the right side of the face. The temperature of the overlying skin was normal. Intraorally, an irregular, ill-defined swelling expanding the lingual and buccal cortex was present in the right side of the mandible with bony hard consistency. Blood investigations (CBC, Hb, BT, CT, ESR), serum calcium, phosphorus, and alkaline phosphatase were within normal limits. A panoramic radiograph revealed four well-defined radiolucencies suggestive of a benign odontogenic cyst (Figure 1C). A chest radiograph demonstrated bifid ribs (Figure 1D). Cone beam CT scan illustrated the expansion of buccal and lingual cortical plates, perforation of the lingual cortical plate, and a horizontally impacted mandibular permanent left second molar (Figure 1F). NBCCS was established as a provisional diagnosis, based on the clinical and radiological findings. Mandibular molars involved in the lesion were extracted surgically. 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 a keratocystic odontogenic tumor (KCOT). Healing of the enucleated sites was observed on follow-up visits (six months) without any sign of recurrence (Figure 1E).

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.


Related in: MedlinePlus