Comparison of the histopathological characteristics of diffuse sclerosing osteomyelitis of the mandible, chronic suppurative osteomyelitis, and craniofacial fibrous dysplasia.

BACKGROUND: There are relatively few reports on the histopathological characteristics of diffuse sclerosing osteomyelitis of the mandible (DSOM), which is difficult to distinguish from chronic suppurative osteomyelitis (CSO) and craniofacial fibrous dysplasia (CFD). This study aimed to summarize and compare the histopathological characteristics of DSOM, CFD, and CSO. MATERIALS AND METHODS: In this study, hematoxylin and eosin-stained sections of patients with DSOM, CSO, and CFD at the Peking University Hospital of Stomatology from 2015 to 2020 were retrieved. The histopathological characteristics were summarized, including new bone formation, inflammatory cell infiltration, bone trabecular morphology, osteoclasts, sequestrum, bacterial mass, and calcified spherules, similar to cementicles. The histopathological characteristics of DSOM, CSO, and CFD were compared, and the results were statistically analyzed. RESULTS: In total, 50, 13, and 10 patients with DSOM, CSO, and CFD were included in this study, respectively. In terms of new bone formation, both DSOM and CSO showed reactive bone formation (p = 1), whereas CFD mainly showed fiber osteogenesis (p < 0.001). The inflammatory cells of DSOM were mainly lymphocytes and plasma cells, whereas those of CSO were mainly lymphocytes and neutrophils (p < 0.001), and there was usually no inflammatory cell infiltration in the CFD specimens (p < 0.001). DSOM, CSO, and CFD showed irregular bone trabeculae (p = 0.045, p = 0.703) and active osteoclasts (p1 = 0.189, p2 = 0.256). DSOM showed a small amount of bacterial mass but no sequestrum; neither of which was found in CFD (p = 1, p = 1), but it was common in CSO (p = 0.011 and p = 0.025). DSOM and CSO showed smooth and regular basophilic lines (p = 0.308), whereas CFD showed a rough and irregular basophilic line (p < 0.001). CONCLUSIONS: The histopathological characteristics of the three diseases were partly similar, but there were evident differences. The main differences are the type of new bone formation, types and distribution of inflammatory cells, and presence of sequestrum and bacterial masses. These differences will help clinicians diagnose DSOM.

[Craniofacial fibrous dysplasia: about six cases]. / Dysplasie fibreuse osseuse crânio-faciale: à propos de six observations.

Fibrous dysplasia (FD) of bone is a benign, congenital and rare disease in which normal bone is replaced by fibrous bone tissue, resulting in bone deformities. It can affect any bone in the body, however craniofacial fibrous dysplasia is characterized by specific clinical manifestations, progression and therapeutic issues. The purpose of our study was to describe the diagnostic, therapeutic and evolutionary features of craniofacial FD. This study involved six patients with craniofacial FD followed up in the Department of Otolaryngology at the Principal Hospital of Dakar. The average age of patients was 26.16 years, ranging from 11 to 58 years. Sex ratio favoured women (83% of the cases). Bone deformity was the main feature of craniofacial FD leading to diagnosis. One patient presented with unilateral nasal obstruction with epistaxis. In all cases, scanner enabled diagnosis and topographic balance. Two female patients underwent surgery. One case of recurrence was reported. Craniofacial FD is a rare bone disease that can manifest as serious sensory and functional disorders. It poses real therapeutic issues; hence adequate interdisciplinar management is essential.

Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports.

BACKGROUND: McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throughout. The clinical phenotype of MAS is highly variable and no definite treatment is available. CASE PRESENTATION: This article describes two cases, a 10-year-old girl and an 11-year-old boy, both with MAS comprising deforming craniofacial FD. Challenges related to diagnosis and management included late reporting with big lesions, involvement of multiple craniofacial bones, mutilating surgeries and ultimately high degree of morbidity. CONCLUSION: Delayed diagnosis and management of MAS results in devastating physical disabilities and severe morbidity after treatment.

Fibrous dysplasia imitating malignancy.

Fibrous dysplasia is a benign bone disease, presenting as monostotic or polyostotic lesions, or as part of a syndrome (McCune-Albright/Mazabraud). Its clinical course shows a variegated picture and the progression of its growth is unpredictable. In the workup of 39 fibrous dysplasia cases in the cranio-facial area, four cases presented fast growth tendencies, of which two patients with McCune-Albright syndrome showed malignant-like rapid growth. This local aggressive form is extremely rare, and the concept of this issue has not been clearly defined. With regard to the speed of growth a volumetric-time analysis in one of our cases demonstrated a 74 days tumor doubling rate with an exponential growth curve. According to the literature the aggressive form presented extra-cranially mainly at an adult age, whereas its appearance in our cranio-facial patient collective was much younger. Distinguishing nonmalignant and malignant aggressive forms is difficult and highly inconsistent in the literature. We therefore implemented a quantitative growth measure analysis to define aggressive forms based on progression and speed of growth and impartial of type of FD, localization or functional incapacity. Due to our study findings and literature review we state a prevalence of an aggressive form might be possibly about 5 %.