Use of Marsupialization as a Definitive Treatment for Large-sized Dentigerous Cysts in a Patient with Mucopolysaccharidosis Type I.

The correct diagnosis is fundamental for the appropriate treatment to be employed in a particular pathology. The best treatment is not the one that solves only local problems, fragmenting the patient, and therefore, it is necessary to integrate the entire systemic condition of the individual before initiating any local treatment. This context inevitably requires dentistry to participate in a multidisciplinary approach, where the role of the dentist is expanded in concepts that encompass ethics, human dignity, and professional valorization. This article describes a clinical case of a patient with mucopolysaccharidosis type I, whose treatment of cystic lesions present in the mandible was exclusively performed through marsupialisation. The objective of this study is to demonstrate, within the complexity of this rare syndrome, the difficulties of diagnosis and the need for evaluation of the patient beyond the limits of the oral cavity, as well as to report two cases of large dentigerous cysts, surgically treated conservatively through marsupialisation, without the need for re-approach for enucleation and without recurrences over a 20-year period.

Glandular odontogenic cyst misdiagnosed and treated as a periapical inflammatory lesion for 6 years in an older adult.

OBJECTIVES: To document the case of a patient who underwent several endodontic treatments due to a glandular odontogenic cyst misdiagnosed as an inflammatory periapical lesion. BACKGROUND: Glandular odontogenic cysts behave more aggressively, while others have an indolent course. There is limited information on this cyst in the gerodontologic literature. MATERIALS AND METHODS: A 76-year-old male patient presented with an asymptomatic expansive lesion in the anterior mandible resistant to several endodontic treatments. Cone-beam computed tomography revealed a multilocular osteolytic lesion measuring 6.0 × 4.0 cm, with cortical bone perforation. RESULTS: Histopathological analysis of a biopsy specimen was consistent with glandular odontogenic cyst. The patient underwent marginal mandibulectomy with preservation of the base of the mandible. CONCLUSION: A strict diagnostic process is important to avoid unwanted consequences, particularly in the geriatric population.

Rare mandibular condition presenting as trismus: an exploration of IgG4-related disease.

A woman in her 30s presented with a 12-month history of reduced mouth opening and swelling on the right side of her mandible. The swelling was non-tender and firm on palpation. The swelling began to increase in size after the extraction of her carious wisdom tooth. Histopathological and serological examinations confirmed the diagnosis of IgG4-related disease, manifested as a mass in the mandible. The patient was prescribed oral corticosteroids at a tapering dosage over 8 weeks. After 3 months, there was an improvement in the patient's mouth opening and a reduction in the size of the swelling. The patient remains in follow-up care. Including IgG4-related disease in the list of potential diagnoses for oral soft tissue masses is crucial, given their positive response to medical treatment, highlighting the significance of an accurate diagnosis to prevent unnecessary surgery, with oral lesions potentially serving as early indicators before multiorgan complications arise.

Mandibular Osteomyelitis as the Earliest Clinical Manifestation of Maxillary Sinus Lymphoma.

Extranodal natural killer/T-cell lymphoma is a distinct subtype of non-Hodgkin lymphoma that originates from natural killer cells or cytotoxic T cells. Its diagnosis is challenging due to the rarity and lack of awareness, especially in cases where osteomyelitis of the jawbone is the initial symptom. This paper reports a case of extranodal natural killer/T-cell lymphoma presenting primarily with oral ulcers. Through analyzing the clinical and pathological characteristics, differential diagnosis, treatment and prognosis, and reasons for misdiagnosis of the disease, this study aims to provide references for clinical diagnosis and treatment.

Central giant cell granuloma in the posterior region of mandible mimicking a fibro-osseous lesion and hemangioma: a case report.

BACKGROUND: A central giant cell granuloma (CGCG) is a benign, proliferative, intraosseous, and non-odontogenic lesion occurring primarily in children and young adults. On the histological level, it is characterized by numerous multinucleated giant cells scattered randomly throughout a sea of spindle-shaped mesenchymal stromal cells which are dispersed throughout the fibrovascular connective tissue stroma containing areas of haemorrhage. When it comes to radiographic features, CGCG can have an array of variations, ranging from well-defined expansile lesions to ill-defined and destructive lesions, with or without expansion. CASE PRESENTATION: This case report reviews an 11-year-old Caucasian patient with a chief complaint of slow-growing swelling involving the right posterior mandibular region. The cone beam computed tomography (CBCT) revealed an ill-defined mixed lesion mimicking both fibro-osseous lesion and hemangioma. However, microscopic examination revealed multinucleated giant cells in a fibrous stroma suggestive of central giant cell granuloma. CONCLUSION: Our intent in reporting this case is to highlight the importance of thorough clinical, radiographical and histopathological examination for accurate diagnosis and therapeutic interventions as well as to emphasize the importance of taking different possibilities into consideration when examining bony swellings in the head and neck region.

Solitary primary intraosseous xanthoma of the mandible in a 15-year-old boy: a case report.

BACKGROUND: A xanthoma is a rare bone condition consisting of a predominant collection of lipid-rich, foamy histiocytes. The central xanthoma of the jaws is a unique benign tumor. CASE REPORT: A 15-year-old Caucasian male has been presented to our department. He had radiological changes in the area of the left mandibular angle, with an area of diffuse osteolysis of 3.0 cm by 2.0 cm. Computed tomography reveals an area of diffuse osteolysis that starts from the distal root of the lower second molar and reaches the ascending process. A bone biopsy was performed, which revealed a benign proliferative process composed of histiocytic cells involving and infiltrating trabecular bone in a background of loose fibrous connective tissue devoid of any other significant inflammatory infiltrate. The size of the formation was 2.9 cm by 2.0 cm. Immunohistochemical staining for CD68 was strongly positive and negative for S-100 and CD1a. From routine blood tests, cholesterol, triglycerides, and blood sugar are within normal values, which excludes systemic metabolic disease. Subsequent to the surgical intervention, the patient underwent postoperative assessments at intervals of 14, 30, 60 days, and a year later, revealing the absence of any discernible complications during the aforementioned observation periods. CONCLUSION: The diagnosis of primary xanthoma of the mandible is rare and can often be confused with other histiocytic lesions. A differential diagnosis should be made with nonossifying fibroma and Langerhans cell histiocytosis, as in our case. In these cases, immunohistochemistry with CD 68, S-100, and CD1a, as well as blood parameters, are crucial for the diagnosis.

Optimal time to definitive enucleation of large cysts following marsupialization: A single center, retrospective study.

OBJECTIVES: Optimal time to enucleation following marsupialization of large odontogenic mandibular cysts is undefined. We aim to assess volume reduction throughout follow-up, to indicate optimal time to enucleation. Secondary objectives include the identification of factors influencing cyst reduction. STUDY DESIGN: We retrospectively enrolled 15 patients with mandibular cysts of different histological types treated with marsupialization at our center between 2018 and 2022. Cyst volume was assessed with cone-beam computed tomography (CBCT) and a semi-automatic segmentation algorithm, at baseline and between 6 and 8 months post marsupialization. RESULTS: The overall mean cyst volume reduction percent (VR%) was 57.7 % or 0.2 % per day. VR% at 8 months was significantly higher than those assessed at 6 and 7 months (67.1% vs 47.1 %, p = 0.003). Time to CBCT was the only independent variable influencing cyst VR%. CONCLUSION: Our study proves that the optimal time to enucleation for mandibular cyst is 8 months, independent of histological cyst type, patient age, baseline cyst volume and the number of pre-operative residual bone walls.

Multiple central giant cell granuloma of the jaws: diagnostic signposts of Noonan syndrome and RASopathy.

Noonan syndrome (NS) is a phenotypically variable inherited multi-system disorder. Maxillofacial findings can be diagnostic, especially in the evaluation of discrete facial dysmorphia. Diagnostic landmark findings of therapeutic relevance for the jaws such as central giant cell granuloma (CGCG) are rare in NS. However, recent molecular genetic studies indicate that these rare, benign lesions are neoplasms and more common in specific syndromes grouped under the umbrella term RASopathies. A specialist surgical diagnosis can be helpful in identifying the underlying disease. This report outlines diagnosis and treatment of a case of CGCG for which jaw diagnosis became the key to identifying a syndromic disease.

Mucormycosis of the Mandible and Tongue: A Systematic Scoping Review.

AIM: Mucormycosis is a rare human infection associated with Mucorales, a group of filamentous moulds found in different environmental niches. Its oral manifestations may occur in the mandible and tongue despite being rare. We aimed to systematically review the data on clinical manifestations, risk factors, diagnostic approaches, treatment options, and outcomes of mandibular and tongue mucormycosis. METHODS: An electronic search of articles published between January 1975 and November 2022 in PubMed, Web of Science, and EMBASE databases was performed. A total of 22 articles met the inclusion criteria and reported 27 cases of oral mucormycosis in total. RESULTS: Fourteen patients had mandibular mucormycosis signs unrelated to COVID-19 infection, 6 had SARS-CoV-2-related mandibular mucormycosis, and 6 had manifestations in the tongue. All published case reports during the COVID-19 pandemic were from India. Patient ages ranged from 4 months old to 82 years, and most patients had important comorbidities, such as blood dyscrasias related to immune deficiency and uncontrolled type 2 diabetes mellitus. The signs and symptoms of mandibular and tongue mucormycosis varied from dental pain, loose teeth, and nonhealing sockets to dysphagia and paraesthesia of the lip. Some patients also reported trismus, draining sinus tract, and facial pain. The diagnosis of oral mucormycosis was based on a combination of clinical, radiographic, and histopathologic findings by demonstrating fungal hyphae in tissue specimens. In most cases, mucormycosis was managed with systemic amphotericin B, strict glycaemic control, and aggressive surgical debridement of infected tissue, minimising the progression of the fungal infection and thus improving the survival rate. In some cases, combined antifungal therapy, antibiotic therapy, and chlorhexidine mouthwashes were used successfully. CONCLUSIONS: Recognition of the signs and symptoms by oral care providers is pertinent for the early diagnosis and treatment of tongue and mandibular mucormycosis, and providers should be aware of the possibility of this opportunistic fungal infection in patients with COVID-19. A multidisciplinary approach is recommended for the management of this lethal infection.

The Moth-Eaten Mandible: Osteomyelitis.

Inflammatory lesions such as osteomyelitis of the jaw may share some of the radiographic features of malignancy; however, a demonstrable dental cause for it usually exists. In addition, inflammatory lesions generally stimulate a sclerotic bone reaction, which is uncommon in malignancy. The imaging modality of choice for aiding in the differential diagnosis is computed tomography imaging because of its ability to clearly delineate sequestra and periosteal new bone formation.

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.

Different presentations of the Buccal Bifurcation Cyst: A case series.

The buccal bifurcation cyst (BBC) is an uncommon odontogenic inflammatory cyst affecting the vestibular aspects of the first or second mandibular molar of pediatric patients. Its etiopathogenesis is not fully understood, but it is hypothesized that food and detritus impacting buccal periodontal pockets in titled tooth would be responsible for inflammation of the pericoronal tissues, leading to proliferation of epithelial rests and subsequent cystic formation. The true prevalence of the BBC is not known, but it is estimated to be less than 1% of all the inflammatory cysts. Most cases are unilateral but bilateral cases may account for up to 30% of all BBCs, which can generate confusion to unfamiliar clinicians. Maxillary cases are extremely uncommon, and to our knowledge, there are no cases published in the English literature. In this case series, we present five BBC cases; two unilateral, two bilateral, and one affecting the maxilla. We included clinical, imaging, and histopathological information to highlight the different presentations that this cyst might have, with the final aim to aid clinicians in its diagnosis and ultimately, its treatment.

[Radiolucency at both lower third molars: the paradental cyst]. / Radiolucentie bij beide derde ondermolaren: een paradentaire cyste.

A 23-year-old man presented with an irritating sensation at the third molar on the left side of the mandible and a bad taste in his mouth. Radiographic and histopathological examination revealed the abnormality was caused by a paradental cyst. The paradental cyst is located distal to a lower third molar and, together with the mandibular buccal bifurcation cyst, belongs to the inflammatory collateral cysts. Treatment consists of enucleation of the cyst and removal of the lower third molar. Recurrences do not occur.

Central giant cell granuloma of the head & neck: A case report and systematic review.

PURPOSE: The purpose of this paper is to describe a recent case of central giant cell granuloma (CGCG) that rapidly progressed post corticosteroid treatment while also providing a review of the existing literature on CGCG of the head and neck (HNCGCG), with particular emphasis on extra-mandibular and maxillary cases. MATERIALS AND METHODS: The investigators designed and implemented a 32-year review of literature, using the online databases: PubMed, Google Scholar, Medline, and Proquest. The total number of cases analyzed was 55 (42 case reports; 3 case series; 8 comparative studies; 1 retrospective cohort). CASE PRESENTATION: We present a case of a CGCG in a 10-year old male. The lesion originated in the right anterior mandibular body and progressed after corticosteroid treatment. Diagnosis was made using a combination of imaging and histology. A timely debulking procedure of the hemi-mandible was performed and there was no recurrence of the lesion at follow up. RESULTS: The average age at the time of diagnosis of CGCG was 27.5 years. HNCGCG was most commonly detected in the jaw (43.1%), but was also found in the temporal bone (33.3%). The most frequently employed treatment modality was complete surgical excision (76.9%). 93.2% of patients were alive with no evidence of disease at follow-up, while 6.8% of patients exhibited recurrence at follow-up. The median follow up was 13 months. CONCLUSION: It is important for clinicians to recognize that CGCGs are capable of manifesting outside of the jaw. CGCG should be considered in the differential diagnosis of non-odontogenic radiolucent lesions, especially in young patients. CGCGs also need to be distinguished from brown tumor of hyperparathyroidism (BTH) and giant cell tumors, which are histologically similar.

Central mucoepidermoid carcinoma arising directly from a glandular odontogenic cyst of the mandible: a case report.

BACKGROUND: Central mucoepidermoid carcinoma (MEC) is a rare salivary gland tumor that affects the jawbone. Glandular odontogenic cyst (GOC) is also a rare odontogenic developmental cyst with glandular differentiation. GOC shares some histological features with central MEC, and a pre-existing GOC can develop into central MEC. Here, we present a rare case of central MEC developed directly from a pre-existing GOC of the mandible. CASE PRESENTATION: A 67-year-old Japanese man presented with a cystic lesion in the right third molar region. Histologically, the biopsy specimen demonstrated both typical findings of a GOC component lined with non-keratinized squamous epithelium and a recognizable component of central MEC consisting of polycystic nests with mucous cells, intermediate cells, and epidermoid cells in the cyst wall. The results from the immunohistochemistry for cytokeratin (CK) profiling demonstrated that, while both central MEC and GOC expressed CKs 7, 14, 18, and 19, CK13 was interestingly exclusively expressed in GOC. Fluorescence in-situ hybridization (FISH) revealed the rearrangement of the Mastermind like (MAML)-2 gene in both the MEC and GOC components. CONCLUSIONS: Our case suggests that central MEC and GOC may be in the same spectrum of diseases caused by the rearrangement of the MAML-2 gene. However, given that the expression profile of CK13 was completely different between central MEC and GOC, they can be considered as separate tumors. Overall, we demonstrated a rare case in which central MEC may have originated directly from the GOC.

Hemimandibulectomía por ameloblastoma multiquístico. A propósito de un caso / Multicystic ameloblastoma hemimandibulectomy. A propos of a case

RESUMEN El ameloblastoma es un tumor odontogénico benigno, localmente agresivo y recidivante, con predilección por la región posterior de la mandíbula. Se caracteriza por su agresividad local con muy baja tendencia a metastizarse. El objetivo fue reportar el caso clínico de un paciente con ameloblastoma multiquístico derecho, tratado a través de hemimandibulectomía. Se presentó un paciente masculino, de 44 años de edad, que refirió aumento de volumen del lado derecho de la mandíbula desde hacía aproximadamente un año, acompañado también de otros síntomas, atendido en el Servicio de Cirugía Maxilofacial del Hospital Militar Principal/Instituto Superior, en Luanda, Angola. Los estudios imagenológicos incluyeron radiografía panorámica y tomografía axial computarizada. El diagnóstico clínico patológico fue de ameloblastoma multiquístico. Este tipo de tumor requiere de un adecuado diagnóstico sobre la base de la presentación clínica, localización, tamaño, edad y tipo histológico; de ahí la importancia de conocer las características clínicas e imagenológicas, pues el tratamiento conlleva gran dificultad (AU).

ABSTRACT Ameloblastoma is a benign odontogenic tumor, locally aggressive and recidivist with predilection for back of the jaw, characterized by local aggressiveness and low tendency to metastasize. The aim was reporting the clinical case of a patient with right multicystic ameloblastoma treated through hemimandibulectomy. We presented a male patient aged 44 years, who referred a volume increase of the jaw right side for around a year, accompanied also by other symptoms; he attended the Maxillofacial Surgery Service of the Main Military Hospital/High Institute of Luanda, in Angola. The image studies included panoramic radiography and computerized axial tomography the clinical pathological diagnosis was multicystic ameloblastoma. This kind of tumor requires an adequate diagnosis based on the clinical presentation, location, size, age and histological kind, therefore the importance of knowing the clinical and image characteristics, because the treatment is very difficult (AU).

A Rare Odontogenic Cyst in an Uncommon Area: Case Report and Review of the Literature / Um cisto odontogênico raro em uma área incomum: Relato de caso e revisão da literatura

Abstract Objective Orthokeratinized odontogenic cyst is a rare developmental odontogenic cyst of the jaws. It is a less aggressive intraosseous cyst identified by an orthokeratinized epithelium. Case Report A 50-year-old male patient with the chief complaint of swelling in the anterior part of his face, and, intraorally, there was diffuse swelling in the palatal cortex. On panoramic radiography, there was a well-defined unilocular radiolucency on the right side of the maxilla and palatal cortical expansion, and thinning of the buccal and palatal cortexes was observed. The histopathological examination revealed a pathologic cyst that was lined by a thick orthokeratinized epithelium. Therefore, the diagnosis was orthokeratinized odontogenic cyst. Conclusion The orthokeratinized odontogenic cyst displays characteristic clinical, histopathological, and biological features that differ significantly from those of keratocystic odontogenic tumor (KCOT), but it has a better prognosis and lower recurrence rate. Thus, other radiolucent lesions of the jaws, including keratocystic odontogenic tumor (KCOT), must be considered in the differential diagnosis.

Resumo Objetivo O cisto odontogênico ortoceratinizado é um raro cisto odontogênico maxilar. É um cisto intraósseo menos agressivo, identificado por um epitélio ortoceratinizado. Relato de caso Um paciente do sexo masculino, de 50 anos de idade, com queixa principal de edema na parte anterior da face, e, intraoralmente, havia edema difuso no córtex palatal. Na radiografia panorâmica, havia uma radioluminescência unilocular bem definida no lado direito da maxila e expansão cortical palatina, e desbastamento dos córtex vestibular e palatino. O exame histopatológico revelou cisto patológico revestido por espesso epitélio ortoceratinizado. Logo, o dignóstico foi de cisto odontogênico ortoceratinizado. Conclusão O cisto odontogênico ortoceratinizado apresenta características clínicas, histopatológicas e biológicas que diferem significativamente das do tumor odontogênico ceratocístico (TOC), mas tem melhor prognóstico e menor taxa de recorrência. Portanto, outras lesões radiolúcidas dos maxilares, incluindo TOC, devem ser consideradas no diagnóstico diferencial.