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Biomolecules Apr 2022Fibrous dysplasia (FD) and hyperparathyroidism-jaw tumor syndrome (HPT-JT) are well-characterized benign bone fibro-osseous lesions. The intracellular mechanism leading...
Fibrous dysplasia (FD) and hyperparathyroidism-jaw tumor syndrome (HPT-JT) are well-characterized benign bone fibro-osseous lesions. The intracellular mechanism leading to excessive deposition of fibrous tissue and alteration of differentiation processes leading to osteomalacia have not yet been fully clarified. Tissue Microarray (TMA)-based immunohistochemical expression of β-catenin, CK-AE1/AE3, Ki-67, cadherins and P-Runx2 were analyzed in archival samples from nine patients affected by FD and HPT-JT and in seven controls, with the aim of elucidating the contribution of these molecules (β-catenin, cadherins and P-Runx2) in the osteoblast differentiation pathway. β-catenin was strongly upregulated in FD, showing a hyper-cellulated pattern, while it was faintly expressed in bone tumors associated with HPT-JT. Furthermore, the loss of expression of OB-cadherin in osteoblast lineage in FD was accompanied by N-cadherin and P-cadherin upregulation (p < 0.05), while E-cadherin showed a minor role in these pathological processes. P-Runx2 showed over-expression in six out of eight cases of FD and stained moderately positive in the rimming lining osteoblasts in HPT-JT syndrome. β-catenin plays a central role in fibrous tissue proliferation and accompanies the lack of differentiation of osteoblast precursors in mature osteoblasts in FD. The study showed that the combined evaluation of the histological characteristics and the histochemical and immunohistochemical profile of key molecules involved in osteoblast differentiation are useful in the diagnosis, classification and therapeutic management of fibrous-osseous lesions.
Topics: Adenoma; Cadherins; Core Binding Factor Alpha 1 Subunit; Fibroma; Humans; Hyperparathyroidism; Jaw Neoplasms; beta Catenin
PubMed: 35454175
DOI: 10.3390/biom12040587 -
Head and Neck Pathology Dec 2017Meningiomas are benign extraaxial tumors of the central nervous system (CNS). Extracranial meningiomas are extremely rare (2%) and can develop as a direct extension from...
Meningiomas are benign extraaxial tumors of the central nervous system (CNS). Extracranial meningiomas are extremely rare (2%) and can develop as a direct extension from a primary intracranial meningioma or as true primary extracranial meningioma originating from ectopic arachnoid cells. Only eight cases of primary meningioma in the jaw have been reported to date. Extracranial meningiomas are frequently misdiagnosed, resulting in inappropriate clinical management. The aim of this article was to describe the case of a man with an asymptomatic swelling in the right retromolar area over a period of 2 months. Cone beam computed tomography was performed to determine the extension and involvement of the adjacent structures. Histopathological findings and immunohistochemical analysis aided in the diagnosis of primary extracranial meningioma in the mandible and several aspects of this unusual neoplasm are reviewed. The treatment of choice was a partial resection of the mandible and reconstruction with autogenous iliac tricortical bone. Five years after surgery, the patient remains free of disease.
Topics: Adult; Humans; Male; Mandibular Neoplasms; Meningioma
PubMed: 28401439
DOI: 10.1007/s12105-017-0813-2 -
Head and Neck Pathology Dec 2021Dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. The... (Comparative Study)
Comparative Study
Dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. The aim of this study was to perform a comparative systematic review of the clinicopathological, genetic, therapeutic, and prognostic features of DGCT and GCOC. The electronic search was performed until December 2020 on seven electronic databases. Case reports, series, and research studies with enough histopathological criteria for diagnosis and all genomic studies were included. Both DGCT and GCOC showed a male prevalence (p = 0.043), with mandibular and maxillary predilections, respectively (p = 0.008). Peripheral DGCT (DGCTp) affected most elderly people (p < 0.001), and central DGCT (DGCTc) and GCOC occurred mainly in younger individuals. Unilateral enlargement of maxilla or mandible was the most common clinical sign associated with a radiolucent or mixed image. Ameloblastomatous epithelium was often present in both neoplasms. Basaloid and large cells with vesicular nuclei were also frequently seen in GCOC. β-catenin expression and mutations (CTNNB1 gene) were found in DGCT and GCOC. Conservative surgery was mostly used for DGCTp, while radical resection was chosen for DGCTc and GCOC. High recurrence rates were found in DGCTc and GCOC. Metastasis occurred in 16.7% of GCOC cases and the 5-year survival rate was 72.6%. DGCT and GCOC share numerous clinicopathological features and demand a careful histopathological evaluation, considering the overlap features with other odontogenic tumors and the possibility of malignant transformation of DGCT. A strict regular post-operative follow-up is mandatory due to high recurrence rates and metastatic capacity in GCOC.
Topics: Age Factors; DNA Copy Number Variations; Humans; Jaw Neoplasms; Keratins; Ki-67 Antigen; Mutation; Neoplasm Recurrence, Local; Odontogenic Tumors; Sex Factors; Tumor Suppressor Protein p53; beta Catenin
PubMed: 34128137
DOI: 10.1007/s12105-021-01347-z -
Prognostic Factors of Primary Intraosseous Squamous Cell Carcinoma (PIOSCC): A Retrospective Review.PloS One 2016To delineate clinical and pathological features and determine the prognostic factors of primary intraosseous squamous cell carcinoma (PIOSCC).
OBJECTIVES
To delineate clinical and pathological features and determine the prognostic factors of primary intraosseous squamous cell carcinoma (PIOSCC).
MATERIALS AND METHODS
Patients diagnosed with PIOSCC, attending the department of oral and maxillofacial surgery, Nanjing stomatological hospital between 2005 and 2015, were identified and retrospectively reviewed for clinical and pathological characteristics. Therapeutic modalities were measured and related follow-up data recorded, in order to determine prognostic factors of PIOSSC.
RESULTS
A total of 77 patients with PIOSCC were included in the study. Mean age at diagnosis was 58.8 years, (range, 37-81 years). Of the 77 patients, there were 58 men and 19 women. The most common location of disease was the mandible (71.42%), particularly the posterior mandible. The common presenting symptoms included jaw swelling (79.2%) and ulceration (42.65%). The estimated 2-year and 5-year overall survival were 68.9% and 38.8%, respectively. Univariate analysis identified the following as negative prognostic factors: histological grade, N classification, nodal status and treatment modalities. However, multivariate analysis determined positive nodal status, high histological grade and advanced N classification as the independent significant prognostic factors.
CONCLUSION
Our results demonstrate several clinical and pathological features of PIOSCC and identify important prognostic factors associated with overall survival in PIOSCC. These prognostic factors include nodal status, histological grade, N classification, and treatment modalities, all of which are important for patient counseling and may be useful for the development of new treatment approaches.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Male; Mandibular Neoplasms; Maxillary Neoplasms; Middle Aged; Odontogenic Tumor, Squamous; Prognosis; Retrospective Studies
PubMed: 27074366
DOI: 10.1371/journal.pone.0153646 -
BMJ Case Reports Dec 2012Odontomas are the most common odontogenic tumours of the jaws, characterised by their slow growth and non-aggressive behaviour. They usually remain asymptomatic, and are... (Review)
Review
Odontomas are the most common odontogenic tumours of the jaws, characterised by their slow growth and non-aggressive behaviour. They usually remain asymptomatic, and are diagnosed on routine radiographs. Clinically, they are often associated with delayed eruption or impaction of permanent teeth and retained primary teeth. The purpose of this paper is to review the literature and report two cases of odontomas. In the first case, a compound odontoma was associated with an unerupted maxillary permanent right central incisor, in an 11-year-old boy. In the second case, a 12-year-old girl had retained mandibular primary left central incisor and its unerupted successor was associated with a compound odontoma, a site considered rare for compound odontoma to occur. The clinical features, diagnosis and treatment of these cases have been discussed.
Topics: Child; Female; Humans; Incisor; Male; Mandibular Neoplasms; Maxillary Neoplasms; Odontoma; Tooth, Impacted; Tooth, Unerupted
PubMed: 23242095
DOI: 10.1136/bcr-2012-007666 -
Medicine Sep 2023Ghost cell odontogenic carcinoma is a rare malignant odontogenic carcinoma characterized by the presence of ghost cells. It has a nonspecific clinical and radiographic... (Review)
Review
RATIONALE
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.
PATIENT CONCERNS
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.
DIAGNOSES
The histopathological examination was morphologically suggestive of ghost cell odontogenic carcinomas.
INTERVENTIONS
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.
OUTCOMES
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.
LESSONS
This case presented that Toripalimab and chemotherapy may be a safe and effective systemic therapy for ghost cell odontogenic carcinoma.
Topics: Male; Humans; Adult; Quality of Life; Jaw Neoplasms; Odontogenic Tumors; Mouth Neoplasms; Carcinoma
PubMed: 37746979
DOI: 10.1097/MD.0000000000035225 -
Hematology/oncology and Stem Cell... Mar 2019Synovial sarcoma is a high-grade soft tissue sarcoma that rarely arises in the head and neck region. It affects the parapharyngeal space and the hypopharynx most... (Review)
Review
Synovial sarcoma is a high-grade soft tissue sarcoma that rarely arises in the head and neck region. It affects the parapharyngeal space and the hypopharynx most commonly and it has different presentations based on the affected site. In extremely rare occasions, it involves the hard palate such as in our case where a 24-year-old female patient presented with a mass lesion involving the left hard palate, which was identified clinically and by imaging studies. The histopathological assessment confirmed that it was a monophasic synovial sarcoma which was also confirmed with further molecular studies. The patient underwent surgical excision and postoperative radiotherapy. Her close follow up over a 6-year period that followed her curative treatment has demonstrated no evidence of disease recurrence or distant metastasis. Surgical excision is the mainstay of treatment for synovial sarcoma and adjuvant radiotherapy is advised. Long-term follow up is recommended because of the remote possibility of late recurrence of the tumor.
Topics: Adult; Female; Humans; Jaw Neoplasms; Palate, Hard; Sarcoma, Synovial
PubMed: 28183682
DOI: 10.1016/j.hemonc.2016.12.005 -
International Journal of Environmental... Feb 2022(1) Background: Many radiolucent jaw lesions exist, and they often show a radiographic resemblance, rendering diagnosis a challenging act. Closely related lesions should...
(1) Background: Many radiolucent jaw lesions exist, and they often show a radiographic resemblance, rendering diagnosis a challenging act. Closely related lesions should be frequently mentioned together in the academic literature, which might be helpful for junior practitioners in determining their differential diagnosis. The usefulness of bibliometric analysis in this respect has yet to be demonstrated. (2) Methods: This study evaluated academic publications on radiolucent jaw lesions, as indexed by the Web of Science Core Collection database. The mentions of radiolucent jaw lesions were extracted from the complete bibliographic records of the publications, and co-word analyses were conducted with the aid of VOSviewer. (3) Results: Based on 1897 papers, visualization maps were synthesized to evaluate co-occurrences of the radiolucent jaw lesions. Ameloblastoma was frequently mentioned together with odontogenic keratocyst, dentigerous cyst, and radicular cyst. Osseous dysplasia was co-mentioned with osteomyelitis, ossifying fibroma, odontoma, fibrous dysplasia, and apical periodontitis. (4) Conclusions: The co-word analysis, a form of bibliometric analysis, could demonstrate a relatedness of radiolucent jaw lesions that could be considered at differential diagnosis.
Topics: Diagnosis, Differential; Fibrous Dysplasia of Bone; Humans; Jaw; Jaw Neoplasms; Odontogenic Tumors
PubMed: 35206118
DOI: 10.3390/ijerph19041933 -
European Annals of Otorhinolaryngology,... Feb 2017Ameloblastoma is a rare, benign odontogenic tumour associated with a high recurrence rate. It accounts for 1% of all tumours of the jaws. The purpose of this study was...
INTRODUCTION
Ameloblastoma is a rare, benign odontogenic tumour associated with a high recurrence rate. It accounts for 1% of all tumours of the jaws. The purpose of this study was to compare the ameloblastoma recurrence rate according to the type of treatment: radical or conservative.
PATIENTS AND METHODS
All patients with a diagnosis of ameloblastoma between 1991 and 2013 were retrospectively identified in order to extract topographic, radiological, and histological data and the type of treatment: conservative (marsupialization, enucleation, curettage) or radical (segmental resection) and to compare the recurrence rate according to the type of treatment.
RESULTS
Twenty-seven patients were included, managed by conservative treatment (CT) in 22 cases and radical treatment (RT) in 14 cases. The recurrence rate was 90.9% in the CT group and 9.1% in the RT group (P=0.025) with a mean follow-up of 56.2 months.
DISCUSSION
The recurrence rate after conservative treatment was higher than that after radical treatment. These results are similar to those reported in the literature. The choice of treatment must be adapted to the macroscopic and histological characteristics of each tumour and to the patient.
Topics: Ameloblastoma; Conservative Treatment; Female; Follow-Up Studies; Humans; Jaw Neoplasms; Male; Middle Aged; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 27793625
DOI: 10.1016/j.anorl.2016.09.004 -
World Journal of Surgical Oncology Nov 2012In the head and neck region, osteosarcoma is the most common primary malignant bone tumor, representing 23% of total head and neck malignancies. Osteosarcomas of the... (Review)
Review
BACKGROUND
In the head and neck region, osteosarcoma is the most common primary malignant bone tumor, representing 23% of total head and neck malignancies. Osteosarcomas of the jaws are nevertheless rare lesions, representing only 2 to 10% of all osteosarcomas. This report reviews a single-center histopathology experience with craniofacial osteosarcomas, and reports the management of unusually large synchronous mandibular and maxillary osteosarcomas in a patient.
PATIENTS AND METHODS
A search of the hospital pathology database for specimens with a histological diagnosis of osteosarcomas submitted between July 1992 and May 2011 was made. A chart review of a patient with large synchronous maxillary and mandibular osteosarcomas was performed, and is reported.
CASE PRESENTATION
A 21-year-old African man with large maxillary and mandibular tumors under palliative care presented with increasing difficulties with eating, speech, and breathing. Surgical debulking was performed, with histology confirming synchronous osteosarcomas of the mandible and maxilla. The patient is well after one year, with no evidence of recurrence, having undergone no further treatment.
CONCLUSION
Osteosarcomas of the jaw remain enigmatic, and a number of difficulties related to their diagnosis and treatment are yet to be resolved. True synchronous multicentric osteosarcomas of the jaws are extremely rare but, like other osteosarcomas of the jaws, have a favorable outcome, and palliative resection of such lesions, though challenging, can therefore lead to an enormously improved quality of life and self-image, and may even offer the opportunity for cure.
Topics: Adult; Humans; Jaw Neoplasms; Male; Neoplasms, Multiple Primary; Osteosarcoma; Radiography
PubMed: 23140538
DOI: 10.1186/1477-7819-10-240