Central Pleomorphic Adenoma of Mandible Mimicking Ameloblastoma - A Rare Case Report.

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.

Ameloblastic Fibro-Odontoma.

Introduction: Ameloblastic fibro-odontoma (AFO) is a benign odontogentic tumor without an aggressive behavior, unlike the similar ameloblastic fibroma. Case Presentation: A 9-year-old boy, with tooth eruption failure, underwent enucleation and curettage of a well-defined variable radiolucent and radio-opaque right mandible lesion. There was odontogenic epithelium with peripheral palisading in a loose myxoid stroma as well as a disorganized component of dentin, enamel, and cementum, features of an AFO. Conclusion: AFO is an odontogenic mixed tumor of epithelium and mesenchyme.

Giant Mandibular Ameloblastoma with Rare Hypercalcemia: A Case Report and Literature Review.

Ameloblastoma is the most common benign odontogenic tumor with local invasion and high recurrence, which generally occurs in the jaw bones. Hypercalcemia is a common paraneoplastic syndrome that is commonly observed in patients with malignancies but rarely encountered in patients with benign tumors. Thus far, not many cases of ameloblastoma with hypercalcemia have been reported, and the pathogenic mechanism has not been studied in depth. This paper presents a case report of a 26-year-old male diagnosed with giant ameloblastoma of the mandible, accompanied by rare hypercalcemia. Additionally, a review of the relevant literature is conducted. This patient initially underwent marsupialization, yet this treatment was not effective, which indicated that the selection of the appropriate operation is of prime importance for improving the prognosis of patients with ameloblastoma. The tumor not only failed to shrink but gradually increased in size, accompanied by multiple complications including hypercalcemia, renal dysfunction, anemia, and cachexia. Due to the contradiction between the necessity of tumor resection and the patient's poor systemic condition, we implemented a multi-disciplinary team (MDT) meeting to better evaluate this patient's condition and design an individualized treatment strategy. The patient subsequently received a variety of interventions to improve the general conditions until he could tolerate surgery, and finally underwent the successful resection of giant ameloblastoma and reconstruction with vascularized fibular flap. No tumor recurrence or distance metastasis was observed during 5 years of follow-up. Additionally, the absence of hypercalcemia recurrence was also noted.

A Recurrent Case of Ameloblastic Fibroma in 37-year Old Male.

Ameloblastic fibroma (AF) is a benign mixed epithelial and mesenchymal odontogenic tumor. This was previously grouped in odontogenic tumor showing odontogenic epithelium with odontogenic ectomesenchyme, with or without hard tissue formation. This report describes a case of ameloblastic fibroma in a 37-yearold male who came with the complain of swelling in the left side of lower jaw since one year. Enucleation of the mass followed by reconstruction was done six years back. However, after two years of initial treatment; radiographic findings suggested recurrence. Histopathological examination confirmed the diagnosis of ameloblastic fibroma. Patient had no clinical and radiographic evidence of recurrence in three and six months' follow-up. Because of the higher proliferative capacity and malignant degree of the mesenchymal component in the recurrent neoplasm, sarcomatous transformation may occur. Hence, a long term clinical and radiographical follow-up is essential due to its transformation into ameloblastic fibrosarcoma.

Differential diagnosis for a mandibular mass - a rare case of an odontoameloblastoma in a red deer (Cervus elaphus elaphus).

BACKGROUND: Mandibular masses caused by inflammatory processes due to bacterial infections, most common with Actinomyces bovis, are well known in herbivors. This case represents a rare differential diagnosis to common inflammatory processes which cannot be distinguished from neoplasia without detailed histopathological examination. CASE PRESENTATION: A large unilateral mandibular mass of a free-ranging female adult red deer (Cervus elaphus elaphus) was submitted for pathological examination. The animal had been shot due to its poor body condition. Grossly, the mandibular mass showed gingival ulceration and necrosis. Histologically, irregular strands and islands of odontogenic epithelial cells and a matrix of dentin and osteoid-like material were found, leading to the diagnosis of an odontogenic tumor. Considering the animal's age the tumor was classified as odontoameloblastoma with secondary chronic purulent osteomyelitis. CONCLUSIONS: Odontogenic tumors are rare in domestic and wildlife species and so far have not been reported in red deer. In addition to the more common inflammatory processes of the mandibula and other neoplastic diseases of the oral cavity, odontogenic tumors represent a rare differential diagnosis that must be kept in mind especially when masked by inflammatory lesions.

Primary hyperparathyroidism presenting as a brown tumor in the mandible: a case report.

BACKGROUND: Primary hyperparathyroidism is characterized by hypercalcemia and elevated or inappropriately normal serum levels of parathyroid hormone. Brown tumor of bone is a rare non-neoplastic lesion resulted from abnormal bone metabolism in hyperparathyroidism. However, nowadays, skeletal disease caused by primary hyperparathyroidism is uncommon. We report a case of brown tumor in the mandible as the initial exhibition of primary hyperparathyroidism associated with an atypical parathyroid adenoma. CASE PRESENTATION: The patient was a 49-year-old female, she had a pain mass on the right mandible a year ago and was treated with root canal therapy and marginal resection. After seven months, the mass recurred and enlarged. Enhanced CT scan, laboratory examination, Ultrasonography, 99mTc-MIBI SPECT-CT scintiscan and pathological examination were used to confirm the diagnosis of brown tumor. The patient's symptom improved after parathyroidectomy. CONCLUSIONS: 99mTc-MIBI SPECT/CT scintigraphy is a highly sensitive examination of the localization diagnosis of hyperparathyroidism. Brown tumors should be considered in the differential diagnosis of osteolytic lesions to avoid unnecessary and harmful interventions.

Spontaneous Regression of a Mandibular Plasmacytoma in a Juvenile Dog: A Case Report.

A 4 mo old female Finnish lapphund presented for further investigation of a swelling of the right rostral mandible. A computed tomography scan showed the swelling to be an expansile and osteolytic mandibular lesion. Histopathology revealed a poorly differentiated, moderately well-demarcated, unencapsulated, highly infiltrative round cell neoplasm, and immunohistochemistry was supportive of a plasmacytoma. Performance of a rostral partial mandibulectomy was initially discussed with the owners, but the lesion improved spontaneously both clinically and on repeated computed tomography scanning before surgery could be performed. It subsequently almost completely resolved 6 mo after diagnosis. Hypotheses for spontaneous regression of the lesion are discussed and the human literature is briefly reviewed.

Metastatic renal cell carcinoma to the mandible: a case report with clinical and histologic findings.

Metastatic disease to the oral cavity is rare. Patients with metastasis to the oral cavity may present with swelling, pain, and paresthesia and require evaluation by providers trained in managing pathoses of the oral cavity and surrounding structures. This report describes the case of a 78-year-old man with painful enlargement of the right posterior mandible that caused paresthesia. An open biopsy procedure resulted in significant blood loss and the need for percutaneous needle biopsy. Immunohistochemical analysis was used to make the diagnosis of metastatic clear cell renal cell carcinoma in this patient, whose primary malignancy was previously unknown. Composite resection of the metastatic lesion and reconstruction were performed with the use of virtual surgical planning, an osteomyocutaneous free tissue transfer, and a custom reconstruction plate. This case highlights the importance of dental professionals in the diagnosis and management of lesions of the head and neck and adds to the literature on metastatic lesions to the region.

Sarcomas of the mandible.

INTRODUCTION: Sarcomas of the mandible are extremely rare tumors, with osteosarcoma being the most common, followed by Ewing's sarcoma MATERIALS AND METHODS: A retrospective review of the clinical records, imaging studies, and pathology slides of patients with sarcoma of the mandible at a Tertiary Care Cancer Center from 1998 to 2014 was undertaken. The impact of neoadjuvant chemotherapy and postoperative radiotherapy with or without chemotherapy was studied, and factors impacting upon local control and disease-specific survival were analyzed. RESULTS: Twenty-two patients were treated over the study period, comprising of 15 males and seven females. External swelling, intraoral growth, or facial numbness were the presenting symptoms. Eighteen patients had osteosarcoma and four had the Ewing's sarcoma. Nine patients received neoadjuvant chemotherapy. All but one patient underwent surgery. Eleven had negative margins, with 90% recurrence-free survival at 3 years, compared to 10 with positive or close margins, leading to 67% recurrence-free survival. None of the patients receiving neoadjuvant chemotherapy developed recurrence and all were alive at 3 years. The impact of postoperative radiotherapy or adjuvant chemotherapy was not statistically significant. CONCLUSIONS: Wide surgical resection with negative margins remains the hallmark of surgical treatment.

Malignant transformation of craniomaxillofacial fibro-osseous lesions: A systematic review.

The purpose of this study was to perform a systematic review of the literature concerning all documented cases of malignant transformation of craniomaxillofacial fibro-osseous lesions (FOLs). Three electronic databases were searched. Data were evaluated descriptively. Kaplan-Meier survival curves were constructed and compared using the log-rank test. A critical appraisal of included articles was performed through the Joanna Briggs Institute tool. A total of 19 studies including 27 patients were selected for data extraction. Twenty-six cases were initially diagnosed as fibrous dysplasia and one as ossifying fibroma. The mean age at the time of malignant transformation was 38.11 years, and the average time from initial diagnosis to malignant transformation was 18.2 years. The male:female ratio was 1:1.2, and the maxilla:mandible ratio was 1.5:1. The histological type of the malignant tumor was predominantly osteosarcoma. Follow-up was available for 21 patients. The 3-year overall survival rate was 51%. Mandible tumors and diagnoses other than osteosarcoma tended to have poor survival rates, but no significant difference was identified. We concluded that between all FOLs, only fibrous dysplasia seems to have a considerable increased risk of malignant transformation. Thus, a regular and long follow-up period is advised.

Young Adult With Osteosarcoma of the Mandible and the Challenge in Management: Review of the Pediatric and Adult Literatures.

Neoadjuvant chemotherapy for osteosarcoma of the jaw (OSJ) remains controversial despite being a standardized treatment in osteosarcoma of the long bones. We present a case of a 22-year-old male with OSJ and performed a retrospective systemic review of previously published literatures of OSJ. We identified 27 articles: 7% recommended neoadjuvant chemotherapy, 22% recommended adjuvant chemotherapy, 19% recommended both neoadjuvant and adjuvant chemotherapy, 33% recommended against chemotherapy and 19% stated the role of chemotherapy is unknown. The lack of consensus regarding the use of chemotherapy in OSJ, despite its benefits, demonstrates the need to establish a standardized algorithm for OSJ.

Heterozygosity for CMT Type 4 Predicts a Severe Vincristine-induced Polyneuropathy Phenotype: A Case Report and Review of Literature.

Vincristine (VCR) is a common chemotherapeutic agent used in the treatment of multiple types of pediatric tumors. VCR's adverse effects are well documented and commonly involve peripheral neuropathy via axonal degeneration. Neuropathic severity is dose-dependent, with sensory deficits occurring with as little as 4 mg cumulative dose. Severe peripheral neuropathy is generally rare, but its effects become additive when given to patients with undiagnosed hereditary peripheral neuropathy such as Charcot-Marie-Tooth. We report a case of an effect of VCR administration given to a patient who developed grade 4 neuropathy and was found to be a carrier of Charcot-Marie-Tooth disease type 4.

Sclerosing Epithelioid Fibrosarcoma of the Bone With Rare EWSR1-CREB3L3 Translocation Driving Upregulation of the PI3K/mTOR Signaling Pathway.

Sclerosing epithelioid fibrosarcoma (SEF) is an uncommon neoplasm that rarely presents in bone. It is characterized by epithelioid cells arranged in nests and single-file cords within a sclerotic stromal background which may mimic neoplastic bone. SEF harbors an EWSR1 translocation, which may complicate its distinction from Ewing sarcoma in cases with histomorphologic overlap. We present a diagnostically challenging case of SEF in the mandible of a 16-year-old girl. Our experience highlights the lack of specificity of traditional morphology and EWSR1 break-apart fluorescent in situ hybridization. Open-ended RNA-based fusion gene testing coupled with MUC4 immunohistochemistry aided the eventual diagnosis in this case. Herein, we report the third case of SEF with EWSR1-CREB3L3 translocation and show that this fusion leads to aberrant upregulation of the phosphoinositide 3-kinase/mammalian target of rapamycin signaling pathway in heterologous cell models.

Ameloblastic Carcinoma In Situ: Review of Literature and a Case Presentation in a Pediatric Patient.

Ameloblastic carcinomas are rare malignant lesions with 3 mandibular pediatric cases reported in the literature. We present a case of ameloblastic carcinoma in situ in a 15-year-old male with a right mandibular cystic lesion on computed tomography. The incisional biopsy revealed plexiform ameloblastoma. Due to the infiltrating and aggressive nature of the tumor, the patient underwent hemimandibulectomy and immediate reconstruction with a vascularized osteocutaneous fibula free flap. The final pathology was read as ameloblastic carcinoma in situ. Given the rarity of this disease in the pediatric population, this case report may be a valuable addition to the current literature.

Recurrent peripheral ameloblastoma in an elderly patient: A case report.

OBJECTIVE: To report a case of recurrent peripheral ameloblastoma (PA) in an elderly patient. BACKGROUND: PA is a benign tumour that affects soft tissues of gingiva or edentulous alveolar areas, exhibiting histopathological characteristics of ameloblastoma. METHODS: A 79-year-old man showed a nodule in the edentulous right mandibular alveolar ridge diagnosed as recurrent PA. CONCLUSION: Clinicians should consider PA as a differential diagnosis of routine nodular lesions affecting the oral mucosa of geriatric patients.

Unicystic Ameloblastoma in a Child Treated with a Combination of Conservative Surgery and Orthodontic Treatment: A Case Report.

Unicystic ameloblastoma (UAM) is a variant of intraosseous ameloblastoma that occurs as a single cystic cavity. This report describes a case of UAM of the mandible in a seven-year-old girl. The lesion radiographically mimicked a dentigerous cyst. Under the primary diagnosis of a dentigerous cyst, marsupialization was performed to erupt the first molar involved in the cystic lesion and to obtain a definitive diagnosis. The biopsy specimen revealed ameloblastoma. During careful observation, orthodontic treatment, which was performed to upright and promote the eruption of the first molar involved in the tumor, maintained the space needed for enucleation of the tumor. Finally, the second primary molar was extracted, and the lesion was enucleated at 3 years and 4 months after marsupialization. The results of the histological examination revealed UAM. Conclusively, the treatment course not only avoids a resection of the mandible but also induces eruption of the teeth involved in the tumor. Thus, the combination of conservative surgery and orthodontic treatment was effective in the management of UAM that mimics a dentigerous cyst.

Intraosseous Schwannoma of the Jaws: An Updated Review of the Literature and Report of 2 New Cases Affecting the Mandible.

Schwannomas are benign nerve sheath neoplasms composed almost entirely of Schwann cells. These tumors most often arise in the soft tissues of the head and neck. However, they seldom occur within bone. This article presents a rare case of a recurrent intraosseous schwannoma of the anterior mandible and another case of a posterior intraosseous mandibular schwannoma accessed through a sagittal split ramus osteotomy. Furthermore, an updated review of the literature on intraosseous schwannomas affecting the mandible and maxilla is provided.

Intraosseous intraneural perineurioma derived from the inferior alveolar nerve with an abnormality of chromosome 22 and expression of the BCR-ABL fusion gene: report of a case and review of recent literature.

BACKGROUND: Perineurioma (PN) is a peripheral nerve disease that primarily develops in the limbs and trunk and very rarely occurs in the oral cavity. PN is classified into two types: intraneural perineurioma (INPN) and soft tissue perineurioma (extraneural perineurioma, ENPN). In this article, we report a patient with mandibular body INPN derived from the perineurium of the inferior alveolar nerve. CASE PRESENTATION: The patient was a 43-year-old male. He consulted our department for a detailed examination of the right mandibular body. A biopsy was performed at another hospital and he was diagnosed with a schwannoma. At his first visit, hypesthesia extending from the right lower lip to the mental region was recognized and enlargement of the right mandibular canal was confirmed with X-ray CT and MRI. Considering the possibility of future tumor growth, we extirpated the tumor under general anesthesia. Cystic tumor was seen continuously in the inferior alveolar nerve. Immunohistologically, the tumor cells were positive for Glut-1, weakly positive for EMA, and weakly positive for Claudin-1, and the histopathological diagnosis was INPN. In addition, absence of the BCR region of chromosome 22 and expression of the BCR-ABL fusion gene were observed by fluorescent in situ hybridization (FISH), and a chromosome 22 abnormality was confirmed. These findings indicated that the disease was a neoplastic lesion. CONCLUSION: Expression of the BCR-ABL fusion gene in INPN that develops in the oral cavity is thought to be very rare, and to the best of our knowledge, ours is the first case to be reported in the literature. About three postoperative years have passed, but findings suggestive of recurrence have not been observed.

Recurrent Retinoblastoma With Mandibular Metastasis: A Case Report.

Retinoblastoma (RB) is an aggressive intraocular tumor arising from cells of the retina. Infrequently, distant metastasis occurs in advanced stages of the disease. This case report describes the metastasis of the tumor to the mandible, an extremely rare phenomenon. This condition was observed for a recurrent tumor in a patient who initially underwent chemotherapy, making this case report unique and providing new insights into the behavior of this tumor. An overview of the management of a metastatic RB also is discussed.

Ghost Cell Tumors.

Ghost cell tumors are a family of lesions that range in presentation from cyst to solid neoplasm and in behavior from benign to locally aggressive or metastatic. All are characterized by the presence of ameloblastic epithelium, ghost cells, and calcifications. This report presents the cases of a 14-year-old girl with a calcifying cystic odontogenic tumor (CCOT) and a 65-year-old woman with a peripheral dentinogenic ghost cell tumor (DGCT) with dysplastic changes, a rare locally invasive tumor of odontogenic epithelium. The first patient presented with a 1-year history of slowly progressing pain and swelling at the left body of the mandible. Initial panoramic radiograph displayed a mixed radiolucent and radiopaque lesion. An incisional biopsy yielded a diagnosis of CCOT. Decompression of the mass was completed; after 3 months, it was enucleated and immediately grafted with bone harvested from the anterior iliac crest. The second patient presented with a 3-month history of slowly progressing pain and swelling at the left body of the mandible. Initial panoramic radiograph depicted a mixed radiolucent and radiopaque lesion with saucerization of the buccal mandibular cortex. An incisional biopsy examination suggested a diagnosis of DGCT because of the presence of ghost cells, dentinoid, and islands of ameloblastic epithelium. Excision of the mass with peripheral ostectomy was completed. At 6 and 12 months of follow-up, no evidence of recurrence was noted.