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Salivary gland neoplasms account for 3% of all head and neck tumours. Pleomorphic adenoma (PA) is the most common salivary gland tumour that mainly occurs in the parotid gland, followed by minor salivary glands of the oral cavity, however, the occurrence of PA inside the jaw bones is exceedingly rare and very few cases have been reported in the literature. Inside jaw bones these lesions tend to imitate large osteolytic lesions encompass a diagnostic challenge. An exhaustive review of the literature revealed only 10 cases of central pleomorphic adenoma. We present a rare case of primary PA that occurred inside the mandible and was provisionally diagnosed as ameloblastoma.

UNASSIGNED: Ewing\'s sarcoma of bone is a rare malignant round cell tumor of the head and neck. The jaw, particularly the mandible, accounts for 3% of occurrences in the head and neck area. These tumors have been reported more frequently in men than in women, and they are usually between 5- and 20-year-old. It is difficult for clinicians and pathologists to make a diagnosis based solely on clinical and microscopic findings. Immunohistochemistry presents a plausible tool that can help the pathologist to arrive at a confirmatory diagnosis. This jaw tumor has the best prognosis when detected and treated early in the disease course, so the importance of timely, and accurate diagnosis cannot be overemphasized. The highlighting feature of this case report of an 18-year-old male that was primary detected by immunohistochemically as Ewing\'s sarcoma and is localized to the impacted left mandibular molars.

Hereditary primary hyperparathyroidism (PHPT) accounts for 5-10% of all PHPT cases, necessitating genetic testing for diagnosis and management. Among these, hyperparathyroidism-jaw tumor syndrome (HPT-JT) is an autosomal dominant disorder caused by CDC73 mutations with variable clinical presentations and incomplete symptoms.
The proband, diagnosed with PHPT, underwent parathyroidectomy at the age of 41 with pathological examination of parathyroid carcinoma (PC). Hereditary PHPT was initially suspected due to the early-onset PHPT and family history. Genetic testing identified a heterozygous CDC73 mutation, NM_024529.4: c. 687_688delAG (p. Arg229Serfs*37). Even in the absence of jaw tumors, the diagnosis of HPT-JT was confirmed based on the discovery of renal cysts. A secondary thyroidectomy was performed to reduce the risk of recurrence.
Genetic testing is strongly recommended in cases of early-onset PHPT, family history, jaw tumors, renal and uterine involvement, atypical parathyroid tumors, and PC. This testing provides valuable information for personalized management, and counseling is available for affected families.

BACKGROUND: Ghost cell odontogenic carcinoma is a rare malignant odontogenic carcinoma characterized by the presence of ghost cells. It has a nonspecific clinical and radiographic presentation and can be locally destructive and invasive, sometimes with distant metastases. However, no effective systemic therapy is currently recommended for such patients.
METHODS: The patient has been unable to undergo surgery or radiotherapy again. Therefore, he was referred to our department for a more aggressive, multimodal systematic treatment program.
METHODS: The histopathological examination was morphologically suggestive of ghost cell odontogenic carcinomas.
METHODS: We report a case of locally invasive primary inoperable odontogenic shadow cell carcinoma in a 31-year-old Chinese man who achieved treatment with Toripalimab and chemotherapy, followed by Toripalimab maintenance therapy after 6 cycles.
RESULTS: He achieved partial remission after treatment. The quality of life significantly improved after treatment. There were no grade 3/4 treatment-related adverse events during treatment.
CONCLUSIONS: This case presented that Toripalimab and chemotherapy may be a safe and effective systemic therapy for ghost cell odontogenic carcinoma.

Intraosseous schwannoma of the mandible is rare, with diagnostic and therapeutic challenges. The aims of this study were to report new cases of intraosseous schwannoma of the mandible and to propose a clinical classification, providing suggestions for treatment methods. The cases of 13 patients treated at the authors\' hospital and 86 cases reported previously in the literature were reviewed. The most common clinical feature was facial swelling (60/93). The rate of cortical thinning or expansion was 44.8% (43/96); widening of the inferior alveolar nerve canal on radiographs was observed in 15 patients.

Ghost cell odontogenic carcinoma (GCOC) is an extremely rare intraosseous malignant odontogenic tumor with prominent ghost cell keratinization and dentinoid formation. Here, we present the first case of GCOC arising in dentinogenic ghost cell tumor (DGCT), peripheral. The patient was a man in his 60s with an exophytic mass in the anterior part of lower gingiva. The resected tumor measured 4.5 cm in maximum diameter. Histologically, the nonencapsulated tumor proliferated in the gingiva without bone invasion. It was predominantly composed of ameloblastoma-like nests and islands of basaloid cells with ghost cells and dentinoid in the mature connective tissue, suggesting DGCT, peripheral. As minor components, sheets of atypical basaloid cells and ameloblastic carcinoma-like nests with pleomorphism and high proliferative activity (Ki-67 labeling index up to 40%) consistent with malignancy were identified. CTNNB1 mutation and β-catenin nuclear translocation were observed in both benign and malignant components. Final diagnosis was GCOC arising in DGCT, peripheral. GCOC shows similar histological features to DGCT. In this unique case without invasion, the cytological atypia and high proliferative activity supports the diagnosis of malignant transformation from DGCT.

The aim of this case report is to review and compare the clinical, radiologic, histopathologic, and immunohistochemical features, along with the treatment of a case of ghost cell odontogenic carcinoma. In addition, a report of the existing published literature with an emphasis on treatment will be described to provide information on this rare but aggressive tumor. The family of odontogenic ghost cell tumors comprises a spectrum of lesions characterized by odontogenic epithelium with ghost cell keratinization and calcifications. It appears that early detection is vital in proper treatment due to the high possibility of malignant transformation.

Odontogenic fibroma (OF) is a rare benign odontogenic tumor of ectomesenchymal origin, mostly affecting the tooth-bearing portions of the jaws in middle-aged patients. Whilst small lesions tend to be clinically asymptomatic, varying unspecific clinical symptoms occur with an increase in size and may mimic odontogenic or other maxillofacial bone tumors, cysts, or fibro-osseous lesions of the jaws.
A 31-year-old female patient presented with a hard, non-fluctuating protrusion in the vestibule of the upper right maxilla. It was visualized on cone beam computed tomography (CBCT) as space-occupying osteolysis with the displacement of the floor and facial wall of the maxillary sinus, mimicking a cyst-like lesion. The tissue was surgically removed and identified as an OF in the histopathological examination. One year after the surgery, restitution of regular sinus anatomy and physiological intraoral findings were observed.
This case report emphasizes that rare entities, like the maxillary OF presented, often demonstrate nonspecific clinical and radiological findings. Nevertheless, clinicians need to consider rare entities as possible differential diagnoses and plan the treatment accordingly. Histopathological examination is essential to conclude the diagnosis. OF rarely recur after proper enucleation.

Ameloblastoma is a benign tumor of odontogenic epithelium which is slow growing, locally invasive, expansive that may result with asymmetries of the face. Conservative treatment of odontogenic tumors with marsupialization is not common but can be done successfully in those with cystic pattern. The aim of this case and literature review is to propose marsupialization as an initial procedure for its treatment. We have presented a case of an ameloblastoma involving 56-year-old male patient. Orthopantomography and computed tomography showed the lesion extended between the right molar region and left molar region of mandible with both buccal and lingual expansion and root resorptions. On clinical examination, expansions of mandible and slight tooth mobilities were seen. An incisional biopsy was carried out and histopathologic examination revealed ameloblastoma although clinical features of lesion were more suggestive of cyst. Our case was treated by obturator marsupialization with a good healing. This case and review of the literature discusses the available treatment option according to macroscopic features of jaw lesions and emphasizes on the importance of the most conservative treatment option. Conservative treatment preserves integrity of bones while radical treatment can leave major cosmetic and functional sequelae such as pathologic fractures, complication with reconstruction plates.