Amyloid Variant of Central Odontogenic Fibroma in the Mandible: A Case Report and Literature Review

Abstract

Patient: Male, 35-year-old Final Diagnosis: Central odontogenic fibroma Symptoms: Asymptomatic Medication:— Clinical Procedure: Enucleation • surgery removal Specialty: Dentistry Objective: Rare disease Background: Odontogenic fibroma is a rare mesenchymal odontogenic tumor. It can be classified as central odontogenic fibroma (COF) or peripheral odontogenic fibroma (POF) based on clinical features. There are several variants of COF, including amyloid, ossifying, and giant cell. It grows slowly and exhibits painless cortical expansion of the jawbone. Radiographically, COF appears as a radiolucent unilocular or multilocular lesion with well-defined borders. COF may be associated with unerupted or displaced teeth and root resorption. Case Report: A 35-year-old man was referred to our hospital for submandibular swelling. Panoramic radiography and contrast-enhanced computed tomography revealed a unilocular area of bone resorption with a well-defined border and equal enhancement from the canine to first molar on the right side of the mandible. Root resorption of the first premolar and root separation of the first and second premolars were also evident. The lesion was asymptomatic. Histopathological examination of a biopsy of the lesion was suggestive of OF. Enucleation of the tumor, curettage of the bone around the tumor, and extraction of the premolar were then performed. Histopathological examination of the tumor showed fibrous connective tissue with inactive-looking odontogenic epithelial islands and strands, amyloid deposit, intercalation of Langerhans cells into the tumor epithelium, and no calcification. The final diagnosis of amyloid variant of COF was made. The postoperative course is going well. Conclusions: Herein we describe and discuss the clinical, radiological, and pathological features of the amyloid variant of COF. This report will enhance understanding of this extremely rare variant.

Non-calcifying Langerhans Cell Rich Variant of Calcifying Epithelial Odontogenic Tumor and Amyloid Rich Variant of Central Odontogenic Fibroma: A Unique Entity or a Spectrum?

“…As NCLC-CEOT nomenclature indicated, numerous Langerhans cells immunostained by different markers, such as S-100 [ 9 , 16 , 18 , 19 ], lysozyme [ 9 ], MT1 [ 9 ], LN3 [ 9 ], LKT6 [ 9 ], Langerin [ 18 ], and CD1a [ 17 – 20 ] and those detected by electron microscopy [ 9 , 16 ], have been discovered in the epithelial nests in the NCLC-CEOT cases. Numerous Langerhans cells were also reported in the AR-COF cases [ 13 – 15 , 21 ] with an approximately 40% mean ratio of positive Langerhans cells to tumor epithelial cells. The odontogenic epithelial cells in the NCLC-CEOT cases are positive for pan-cytokeratin [ 9 , 16 , 20 ], but are negative for epithelial membrane antigen (EMA) [ 16 ], involucrin [ 16 ], vimentin [ 16 ], collagen IV [ 16 ], and laminin [ 16 ].…”

“…In clinical outcome, all cases either treated with conservative curettage or enucleation or radical resection showed no recurrence. One exception in the AR-COF group was a case described with a recurrent history at the time of biopsy [ 21 ]. However, no information regarding the previous lesion was provided, and no recurrence in this specific case was noted after the treatment.…”

“…The odontogenic epithelial cells in the NCLC-CEOT cases are positive for pan-cytokeratin [ 9 , 16 , 20 ], but are negative for epithelial membrane antigen (EMA) [ 16 ], involucrin [ 16 ], vimentin [ 16 ], collagen IV [ 16 ], and laminin [ 16 ]. The odontogenic epithelial cells in AR-COF cases and in two previously reported as NCLC-CEOT cases [ 15 ] have been shown to be positive for CK (AE1/AE3) [ 21 ], CK5/6 [ 21 ], CK19 [ 21 ], and CK10/13 [ 15 ], but are negative for CK7 [ 15 ] and CK8/18 [ 15 ]. In NCLC-CEOT cases, the amyloid-like products might or might not display green birefringence by polarized microscopy after Congo red staining [ 9 , 16 , 19 , 20 , 33 ].…”

“…In this study, the focused diseases were intraosseous NCLC-CEOT and AR-COF. All the cases from case reports or case series named NCLC-CEOT [ 9 , 16 – 20 ] or AR-COF [ 13 – 15 , 21 , 22 ] were reviewed. The cases included in review articles focusing on NCLC-CEOT or AR-COF and review articles including information about the NCLC-CEOT or AR-COF variants were also reviewed [ 22 – 24 , 26 – 29 ].…”

“…However, some variants of CEOT and COF had been reported in the literature during the past decades. Among the variants, the overlapping clinicopathological features of non-calcifying Langerhans cell rich variant of CEOT (NCLC-CEOT) [ 9 , 16 – 20 ] ( Figures 1E,F ) and the amyloid rich variant of COF (AR-COF) [ 13 – 15 , 21 , 22 ] have been recognized recently. Thus, the disease diagnosed as either NCLC-CEOT or AR-COF started to be considered as the same disease [ 13 , 15 , 23 ].…”