An unusual clinical and histopathologic presentation of a maxillofacial ameloblastoma: a literature review and case report.

The objectives of this article are to describe an unusual clinical and histopathologic presentation of an ameloblastoma affecting the right maxilla, maxillary sinus, and nasal cavity and to discuss the difficulty of establishing a clinical classification based on the most recent edition of Head and Neck Tumours in the WHO Classification of Tumours series (2022). A 74-year-old man presented with a 6 × 6-cm expansile, ulcerated mass on the right lateral palate. A clinical diagnosis of squamous cell carcinoma was rendered. A biopsy was performed, and the specimen showed multiple histologic patterns of ameloblastoma inconclusive of odontogenic or sinonasal origin. Cone beam computed tomographic imaging demonstrated a well-defined unilocular mass in the right maxilla extending up to the nasal cavity. A surgical resection was performed and confirmed the diagnosis of maxillary ameloblastoma with extension into the nasal cavity. This dilemma in delayed diagnosis led to a literature search for similar maxillary ameloblastoma cases with extension into vital structures. In 45 cases previously reported in the literature, the median age of patients with maxillary ameloblastoma was 50 years, and there was extensive involvement of adjacent vital structures. The nasal cavity/sinonasal region (24/45), orbit/orbital floor (12/45), multiple fossae (5/45), and base of the skull (4/45) were the most common extensions of maxillary ameloblastoma. Fifteen patients had lesions with multiple extensions, and 1 patient showed lung metastasis. The most common histologic presentation was the follicular pattern, followed by the plexiform pattern or mixed follicular and plexiform patterns. Surgical interventions were performed on most patients, with the majority undergoing maxillectomy. Differentiating primary sinonasal ameloblastoma from gnathic ameloblastoma with sinonasal extension is challenging, and this article discusses subtle radiographic criteria and symptoms that aid in the distinction of both types. The authors suggest that variants of maxillary ameloblastoma with extensive involvement of the sinonasal region, orbit, or base of the skull be classified with a clinical diagnosis of maxillofacial ameloblastoma, regardless of the tumor origin.

Squamous Cell Carcinoma Arising in a Squamous Odontogenic Tumor of the Maxilla: Case Report and Review of the Literature.

Squamous odontogenic tumor (SOT) is an exceedingly rare, benign epithelial odontogenic tumor showing squamous differentiation. It is composed of variably sized and shaped islands of cytologically bland, mature squamous epithelium within a fibrous stroma. In this report, we present a rare transformation of a squamous odontogenic tumor (SOT) of the maxilla into a well-differentiated squamous cell carcinoma (SCC) with involvement of the pterygoid plates. To the best of our knowledge, only two cases of malignant transformation of SOT has been reported in the literature. Herein, we seek to report this extremely rare occurrence to raise awareness of oral and maxillofacial surgeons and pathologists of this unusual, but serious event and perform a literature review of squamous odontogenic tumors.

Odontogenic Myxomas Harbor Recurrent Copy Number Alterations and a Distinct Methylation Signature.

Odontogenic myxoma is a rare, benign, and locally aggressive tumor that develops in the tooth-bearing areas of the jaw. The molecular mechanisms underlying odontogenic myxomas are unknown and no diagnostic markers are available to date. The aim of this study was to analyze DNA methylation and copy number variations in odontogenic myxomas to identify new molecular signatures for diagnostic decision-making. We collected a cohort of 16 odontogenic myxomas from 2006 to 2021 located in the mandible (n = 10) and maxilla (n = 6) with available formalin-fixed paraffin-embedded or fresh frozen tumor tissue from a biopsy or resection material. Genome-wide DNA methylation and copy number variation data were generated from 12 odontogenic myxomas using the Illumina Infinium Methylation EPIC array, interrogating >850,000 CpG sites. Unsupervised clustering and dimensionality reduction (Uniform Manifold Approximation and Projection) revealed that odontogenic myxomas formed a distinct DNA methylation class. Copy number profiling showed recurrent whole-chromosome gains (trisomies) of chromosomes 5, 8, and 20 in all cases, and of chromosomes 10, 12, and 17 in all except one case. In conclusion, odontogenic myxomas harbor recurrent copy number patterns and a distinct DNA methylation profile, which can be used as an additional diagnostic tool in the appropriate clinical and radiologic context. Further research is needed to explain the genetic mechanisms caused by these alterations that drive these locally aggressive neoplasms.

Is an Elective Neck Dissection Needed in Squamous Cell Carcinoma of the Maxillary Alveolus and Hard Palate?

Background Squamous cell carcinoma (SCC) of the maxillary alveolus and hard palate is a rare site for oral cavity carcinoma. Much controversy is there regarding the management of this site and elective neck dissection due to rarity and complex lymphatic drainage. Objective To estimate the prevalence of neck nodal metastasis in squamous cell carcinoma of maxillary alveolus and hard palate and the factors influencing the nodal metastasis. Method This retrospective cohort study includes patients diagnosed with squamous cell carcinoma of maxillary alveolus and hard palate and who underwent surgical intervention between March 2017 and March 2022. Result The study included 53 patients among them majority were men (73.6%). Prevalence of neck nodal metastasis was 36.6% and occult nodal metastasis was noted in 16%. On multivariate analysis, clinical nodal positivity increases the odds of pathological nodal positivity by 9.4 times compared to no nodal involvement (95% CI 2.07-42.57, p < 0.004). A depth of invasion (DOI) of more than 10 mm increases risk by 7.4 times for pathological nodal positivity compared to less than 10 mm invasion (95% CI 1.53- 35.27, p=0.013). Conclusion Squamous cell carcinoma of maxillary alveolus and hard palate has a high risk of nodal metastasis. Depth of invasion is an important predictor for nodal metastasis. Due to the high risk of nodal metastasis elective neck dissection would be recommended in advanced stages. Squamous cell carcinoma of maxillary alveolus and hard palate with nodal metastasis has a poor survival.

Operative Technique for Extirpation of Large Melanotic Neuroectodermal Tumor of Infancy Involving Alveolus and Anterior Maxilla in the Region of the Premaxilla.

BACKGROUND: Melanotic neuroectodermal tumors of infancy are rare, benign neoplasms predominantly affecting the craniofacial region, and they are typically managed through resection with minimal need for reconstruction. However, in exceptional cases, larger or more complex tumors may necessitate open craniofacial approaches, with limited literature detailing the surgical strategies for these scenarios. The authors report a distinctive case of an aggressively expanding melanotic neuroectodermal tumor of infancy in a pediatric patient, describing their approach for the tumor's resection. METHODS: A 10-week-old male presented to the hospital with a reported 2 weeks of swelling of the left upper gum line noted by his mother and pediatrician. Preoperative biopsy revealed a soft, fluid-filled lesion, prompting surgical planning for complete excision. Intraoperative assessment with a nasal speculum by ENT, and utilization of a piezoelectric saw by plastic surgery facilitated precise tumor resection. The surgical technique required meticulous dissection, ensuring clear margins while preserving surrounding structures. RESULTS: The tumor, encompassing the alveolus and anterior maxilla, was completely excised with negative margins. Utilization of the piezoelectric saw facilitated safe bone cutting, preserving vital structures while ensuring comprehensive tumor removal. The patient tolerated the procedure well, with no immediate postoperative complications. CONCLUSIONS: Complete resection is essential in minimizing the risk of recurrence in MNTI. The utilization of piezoelectric surgery enhances precision and safety in tumor resection, preserving anatomic integrity and optimizing patient outcomes. This case underscores the importance of meticulous surgical techniques in managing craniofacial tumors, advocating for the adoption of advanced surgical tools to improve clinical outcomes.

Modern Oncologic Maxillary Reconstruction.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: (1) Have a broad understanding of the oncological principles relating to cancers involving the maxilla. (2) Define anatomically the various types of maxillectomy defects and their associated morbidity. (3) Understand the goals and principles of maxillary reconstruction. (4) Demonstrate an understanding of the strengths, limitations, and alternative reconstructive options for the various types of maxillectomy defects. SUMMARY: Reconstruction of the maxilla following cancer resection has evolved over the past decade. Underpinned by advances in virtual surgery technology and an increased focus on dental rehabilitation, more sophisticated workflows using free bone flaps have become more commonplace. There are few reconstructions anywhere in the body that require the restoration of similarly intricate bone and soft-tissue relations with implications for both form and function, like the maxilla. Success demands careful anatomical definition of the defect, an understanding of the goals and principles of reconstruction, and the understanding of relative strengths and limitations of the various reconstructive options. Equally, as no one technique is without shortcomings and maxillary surgery can be associated with a significant complication profile, it is just as important to match the patient and oncologic context to the reconstruction. This article is intended to provide the reader with a broad overview of these core factors.

Synchronous cemento-ossifying fibromas: a systematic review.

BACKGROUND: This systematic review aimed to incorporate published data regarding synchronous cemento-ossifying fibromas (COF), with an analysis of their demographic and clinicopathological characteristics. MATERIAL AND METHODS: Case reports and case series of synchronous COF were searched in PubMed, Web of Science, Scopus, EMBASE, and LILACS according to the PRISMA (2020) statement. Also, a manual search was carried out and the grey literature was assessed. A descriptive statistical analysis was performed. RESULTS: Nineteen studies comprising 20 cases of synchronous COF were included. The mean age at diagnosis was 35 years (±13.8), with a predominance of female patients (n=12/60%). In 13 cases (65%) the mandible and the maxilla were affected simultaneously. In two cases (10%) first-degree relatives (parents or siblings) had been previously diagnosed with COF. The diagnostic hypotheses were reported in 8 cases (40%), with florid cemento-osseous dysplasia, ameloblastic fibroodontoma, calcifying cystic odontogenic tumor, osteoma and cementoblastoma being cited in the differential diagnosis. Among the cases with details about management (n=17), eleven were treated by surgical enucleation and/or excision (64.7%). Follow-up was provided for 10 cases (50%), with a mean period of 44.7±62.19 months. Recurrence occurred in three of informed cases. CONCLUSIONS: Synchronous manifestation of COF is rare. Female patients around the 3rd decade of life are more commonly affected. Bilateral involvement of the mandible and maxilla is the most common clinical presentation.

Clinical course and vascular endothelial growth factor signaling system expression in maxillary angiosarcoma: A case report.

Maxillary angiosarcoma, an aggressive tumor derived from vascular endothelial cells, is very rare. Recently, antivascular endothelial growth factor (VEGF) therapies have attracted considerable attention. We describe the clinical course of a patient with maxillary angiosarcoma and discuss the expression of VEGF signaling molecules assessed via immunohistological analysis. An 81-year-old man presented with an aggressive tumor in the left maxillary sinus. Biopsy revealed atypical nuclear cell proliferation, and the tumor was suspected to be a sarcoma. The maxillary malignancy was treated using a multidisciplinary approach with a combination of surgery, radiotherapy, and regional chemotherapy. Examination of the specimen obtained in the first surgery revealed maxillary angiosarcoma, found to be positive for CD31, while negative for CD34, D2-40, and factor Ⅷ. Although no pathological residual tumor was observed after the planned wide surgery, cervical lymph node and distant metastases occurred. The patient died 24 months after the first surgery. Staining revealed VEGF receptor (VEGFR) 1, VEGFR2, phosphorylated Ak strain transforming, mitogen-activated protein kinase, and signal transducer and activator of transcription 3 positivity. Although our findings do not indicate that anti-VEGF therapy is beneficial for treating maxillary angiosarcomas, we found that VEGFR signaling pathways were activated in maxillary angiosarcomas similar to angiosarcomas originating at other sites. Herein, we report a case of maxillary angiosarcoma, focused on VEGFR and signaling pathway activation. To our knowledge, this is the first report to describe VEGFR system immunostaining findings in maxillary angiosarcoma.

Central dentinogenic ghost cell tumor of the maxilla: a case report with new imaging findings and review of the literature.

A dentinogenic ghost cell tumor (DGCT) is a rare benign odontogenic tumor that commonly shows characteristics of solid proliferation and has a relatively high risk of recurrence after surgical treatment. We herein report a case of a central DGCT that occurred in the maxilla and resulted in bone expansion. This study highlights new imaging findings (particularly magnetic resonance imaging) along with histopathological observations. In addition, we conducted a review of the existing literature on this rare tumor. A 37-year-old man developed swelling around the right cheek. A benign odontogenic tumor such as ameloblastoma was suspected based on the imaging examination findings (including bone expansion and the internal characteristics of the tumor) on panoramic imaging, computed tomography, and magnetic resonance imaging. The lesion was surgically excised from the right maxilla. Postoperative histopathological examination led to a definitive diagnosis of central DGCT. The tumor comprised epithelial neoplastic islands, resembling ameloblastoma, inside tight fibroconnective tissue; masses of ghost cells and formation of dentin were also observed. We had suspected that the minute high-density region around the molars on the imaging examinations represented alveolar bone change; however, it represented dentin formation. This led to difficulty diagnosing the lesion. Although DGCT may present characteristic findings on imaging examinations, its occurrence is infrequent, and in some cases, the findings may include the presence or absence of an impacted tooth without obvious calcification. The present case suggests that we should consider the possibility of an odontogenic tumor with calcification when high-density structures are observed inside the lesion.

Postoperative Radiotherapy in Advanced Stage Squamous Cell Carcinoma Requiring Maxillectomy.

OBJECTIVE: To evaluate whether postoperative radiotherapy (PORT) improves survival among patients who received maxillectomy for pT4aN0 maxillary gingival or hard palate squamous cell carcinoma (SCC) with respect to tumor size. STUDY DESIGN: Retrospective analysis. SETTING: National Cancer Database from 2004 to 2019. METHODS: Included adult patients who received maxillectomy (partial, subtotal, or total) and neck dissection for treatment-naive margin negative pT4aN0 SCC of the maxillary gingiva or hard palate. Adjusted for age, gender, race, insurance status, income, education, urban/rural, facility type, region, comorbidity index, tumor grade, and tumor extension. Inverse probability weights were incorporated into a multivariable Cox proportional hazards model. A priori post hoc subgroup analysis was performed according to tumor size. RESULTS: We included 416 patients who underwent maxillectomy for pT4aN0 SCC of the maxillary gingiva or hard palate (mean [standard deviation] age, 71.5 [11.3] years; male, 190 [45.7%]; tumor size 2 cm, 362 [87%]). Overall, 49.3% of patients received PORT (205 patients). PORT was associated with a 50% improvement in survival compared to surgery alone (adjusted hazard ratio [aHR], 0.50; 95% confidence interval [95% CI], 0.32-0.81). On subgroup analysis, PORT was associated with improved survival for tumors 2 cm (aHR, 0.47; 95% CI, 0.29-0.77), but not for tumors < 2 cm (aHR, 1.15; 95% CI, 0.33-4.08). CONCLUSION: The vast majority of patients with pT4aN0 bone-invading SCC of the maxillary gingiva and hard palate benefit from PORT. Patients with tumors < 2 cm did not demonstrate a survival benefit from adjuvant treatment, suggesting that bony invasion alone may not be sufficient criteria for treatment escalation.

Solitary intraosseous neurofibroma of the oral cavity: rare localization in the maxilla.

BACKGROUND: Neurofibroma is a common benign tumor of neuronal origin that can occur as a solitary tumor or as a component of the generalized syndrome of neurofibromatosis. Neurofibromas are primarily located in the subcutaneous soft tissues and commonly involve extra-oral sites. Solitary intraosseous neurofibromas of the oral cavity are infrequent, with occurrences in the maxilla being exceedingly rare. CASE PRESENTATION: A 22-year-old male patient presented with an asymptomatic mass in the maxilla. Cone-beam computed tomography revealed a round, well-outlined, radiolucent lesion with expansive growth. The neoplasm with the complete capsule was completely removed and confirmed as a neurofibroma based on histopathological and immunohistochemical findings. The reported cases of solitary intraosseous neurofibromas located in the maxilla published in the English literature were compiled to assist in the diagnosis of solitary intraosseous neurofibromas of the maxilla. Nine months after the surgery, there were no signs of tumor recurrence or malignant transformation. CONCLUSIONS: This report emphasizes that rare locations of neurofibromas, such as solitary intraosseous neurofibromas in the maxilla, typically demonstrate nonspecific clinical and radiological features. Clinicians should consider solitary intraosseous neurofibromas as possible differential diagnoses and recognize the histopathological and immunohistochemical features to confirm the correct diagnosis. A longer follow-up period is required because of the potential for local recurrence and malignant transformation of these tumors.

Ameloblastic fibrosarcoma of the maxilla arising in an old woman, a rare case report and literature review.

BACKGROUND: Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone. CASE PRESENTATION: A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up. CONCLUSION: Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.

Central Myoepithelioma of the Maxilla.

Myoepithelioma is a benign salivary gland tumor. Central myoepitheliomas are very rare. The aim of this report was to describe a case of maxillary myoepithelioma. A 14-year-old female patient presented with an multilocular lesion in the anterior maxilla, with nearly 8 months of duration. The lesion was asymptomatic, and the patient's dental history was unremarkable. The diagnostic hypothesis was an odontogenic tumor. Biopsy specimen consisted of nests of plasmacytoid cells in a myxoid stroma without duct formation. No cellular atypia or bone and cartilage formation were noted. The neoplastic cells were positive for Pan-cytokeratin, S100, CK7, and CK8. The final diagnosis was myoepithelioma. The patient was treated by surgical excision followed by bone curettage, and no signs of recurrence were found after 8 years of treatment.

Prosthetic rehabilitation of patients with maxillary oncology defects using zygomatic implants.

PURPOSE: Prosthetics for patients after oncological resection of the upper jaw is a complex problem associated with the physiological and anatomical separation of the oral cavity and the nasal/paranasal region. This study reports the clinical results of the use of the zygomatic implants for prosthetic rehabilitation in patients with maxillectomy due to upper jaw tumors. MATERIALS AND METHODS: The study included 16 patients who underwent prosthetic rehabilitation using a zygomatic implant after maxillectomy period from 2021 to 2023. After the tumor was removed, immediate surgical obturators were placed. Main prosthetic rehabilitation was performed 6-12 months after tumor removal, but before that, a temporary obturator was made and used. Six-twelve months after tumor resection, 1-4 zygomatic implants were inserted into the zygomatic bone unilaterally or bilaterally. A total of 42 zygomatic implants were installed, 2 of which were unsuccessful and were removed in 1 patient. The implants were placed using the surgical guide, which was planned and prepared digitally. RESULTS: No postsurgical complications were seen, and the patients were discharged from the hospital after 7-10 days. The patients were able to return to a normal diet (hard food) after just 7 days following surgery, with no further complaints regarding function or pain, apart from the residual edema caused by the intervention. CONCLUSIONS: The use of prostheses fixed on zygomatic implants in patients with maxillary defects is an effective method of prosthodontic rehabilitation in complex clinical cases after maxillectomy.

Neck dissection of cN0 maxillary oral squamous cell carcinoma: A study based on SEER database.

OBJECTIVE: For patients with clinical nodal-negative (cN0) maxillary oral squamous cell carcinoma (MOSCC), neck dissection (ND) and clinical observation are the main two management strategies for the neck. However, the indications corresponding to these two options remain controversial. This study aimed to elucidate the clinical factors affecting ND treatment and to identify clinical characteristics of the population that may benefit from ND based on a retrospective analysis of cN0 MOSCC patient data from the Surveillance, Epidemiology, and End Results (SEER) database. METHODS: 8846 MOSCC patients were identified in the SEER database from 2000 to 2020. The Kaplan-Meier method was utilized to examine overall survival (OS) and disease-specific survival (DSS), while the hazard ratio (HR) was estimated using the stepwise multivariate Cox regression model. Furthermore, multi-subgroup analyses of DSS and OS were performed to compare ND and No ND. RESULTS: We included 2,512 cN0 MOSCC patients. Basic survival analysis and Cox regression modeling showed that ND was an independent prognostic factor that promoted DSS and OS. Additional subgroup analyses revealed that the primary site and T-stage might influence the efficacy of ND modality. Moreover, patients with T3/T4 stage of upper gingival squamous cell carcinoma (UGSCC) (DSS p = 0.009, OS p = 0.004), hard palate squamous cell carcinoma (HPSCC) (DSS p = 0.001, OS p < 0.001), and soft palate squamous cell carcinoma (SPSCC) (p = 0.029) showed a better survival benefit with ND in OS and DSS. Nonetheless, no differences were observed in OS and DSS between ND and No ND at the T1/T2 stage of the abovementioned primary tumor sites. Additionally, the DSS outcomes for T1/T2 stage upper lip squamous cell carcinoma (ULSCC) patients were significantly worse in the ND group than in the No ND group (p = 0.018). However, no significant differences were noted in OS (p = 0.140) as well as OS (p = 0.248) and DSS (p = 0.627) for T1/T2 and T3/T4 patients, respectively. CONCLUSION: Active surveillance might be a feasible strategy for managing all T-staged ULSCC as well as early-stage (T1/T2) UGSCC, SPSCC, and HPSCC, provided regular and meticulous follow-up is performed. Hence, concurrent ND is recommended for patients with intermediate to advanced (T3/T4) stage UGSCC, SPSCC, and HPSCC.

Maxillary reconstruction with horizontally oriented scapular tip free flap: outcomes in orbital support and palate closure.

BACKGROUND: Current indications of maxillary reconstruction with scapular tip free flap (STFF) are palatoalveolar defects associated with zygomaticomaxillary buttress and/or orbital floor defects. STFF can be placed either horizontally or vertically. Horizontal placement usually allows ideal palatal conformation, preventing oronasal communication, but has been argued to compromise orbital support and projection of the midface, whereas vertical placement is advocated for midface support but may be insufficient for the complete closure of the palate. The present study focuses on the horizontal placing of STFF to allow complete palate reconstruction and fistulae prevention while still obtaining optimal midface projection and orbital support. MATERIALS AND METHODS: This study included 21 case complex maxillary reconstructions with this flap, in which the horizontally placed scapular tip component replaced the palate, a muscular flap component was included for midface volume restoration, and an alloplastic implant was utilized for supporting the orbital content when needed. RESULTS: None of the patients presented palatal fistulas or alterations in the orbital support. CONCLUSION: A multilevel approach was proposed according to the maxillectomy defect. This experience supported the horizontal insetting of STFF to allow palatal fistulae prevention while still obtaining an optimal midface projection and orbital support.

[Komplexes Odontom im Unterkieferfrontzahnbereich bei einer 16-jährigen Patientin - Diagnostik, Therapie und Nachsorge].

Odontome gelten zusammen mit den Amelo- blastomen als die häufigsten odontogenen Tumoren. Sie entstehen während der embryo- nalen Zahnkeimentwicklung durch fehlerhaft differenziertes Keimgewebe und werden daher auch als Hamartome bezeichnet. Somit sind sie also strenggenommen keine klassischen Neoplasien.