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Cureus Aug 2022Ameloblastoma is one of the most common benign odontogenic tumors of the jaw that constitutes about 10% of all tumors that arise in the mandible and maxilla. It is a... (Review)
Review
Ameloblastoma is one of the most common benign odontogenic tumors of the jaw that constitutes about 10% of all tumors that arise in the mandible and maxilla. It is a slow-growing but locally invasive tumor that presents with painless swelling of the mandible or maxilla. The World Health Organization (WHO) classification of 2017 describes ameloblastomas of the following four types: ameloblastoma; unicystic ameloblastoma; extraosseous/peripheral ameloblastoma; and metastasizing ameloblastoma. The diagnosis of ameloblastoma requires computerized tomography (CT) imaging as well as a biopsy. A biopsy is helpful in differentiating ameloblastoma from ossifying fibroma, osteomyelitis, giant cell tumor, cystic fibrous dysplasia, myeloma, and sarcoma. The best treatment of ameloblastoma is aggressive en bloc resection with simultaneous reconstruction. The high recurrence rate and large tissue defects have been long-standing issues in the treatment of ameloblastoma. Recent molecular developments strongly suggest the possibility of targeted therapy with better outcomes in ameloblastomas. We present a detailed updated narrative review of our current understanding and management of this enigmatic tumor.
PubMed: 36127985
DOI: 10.7759/cureus.27734 -
Surgical Pathology Clinics Dec 2021The term giant cell-rich tumors of bone refers to a shared morphologic pattern in a group of different osseous lesions, that is, the abundance of osteoclastlike giant... (Review)
Review
The term giant cell-rich tumors of bone refers to a shared morphologic pattern in a group of different osseous lesions, that is, the abundance of osteoclastlike giant cells. Fitting with a broad spectrum of clinical presentations and biological behavior, the recent detection of characteristic molecular alterations in giant cell tumor of bone (H3-3), nonossifying fibroma (KRAS, FGFR1), giant cell granuloma of the jaws (KRAS, FGFR1, TRPV4), and aneurysmal bone cyst (USP6) have contributed significantly to the biological understanding of these morphologically related but clinically distinct lesions and their systematic classification, highlighting differences and pathogenic relationships.
Topics: Bone Cysts, Aneurysmal; Fibroma; Giant Cell Tumors; Giant Cells; Granuloma, Giant Cell; Humans
PubMed: 34742488
DOI: 10.1016/j.path.2021.06.010 -
Ear, Nose, & Throat Journal Jan 2023Cemento-ossifying fibroma (COF) is a rare fibro-osseous tumor. The lesion is most commonly seen in people in the third and fourth decade. There are three variants of...
Cemento-ossifying fibroma (COF) is a rare fibro-osseous tumor. The lesion is most commonly seen in people in the third and fourth decade. There are three variants of ossifying fibroma; juvenile trabecular ossifying fibroma, juvenile psammomatoid ossifying fibroma, and COF. COF is highly cellular and contains a fibrous tissue that has different amounts of calcified tissue. Although histologically benign, it has a significant growth potential.
PubMed: 36708163
DOI: 10.1177/01455613231154038 -
National Journal of Maxillofacial... Aug 2022Ossifying fibroma, also the cement ossifying fibroma radiographically, represents a well-demarcated mixed radiolucency/radio-opacity with smooth and often sclerotic...
Ossifying fibroma, also the cement ossifying fibroma radiographically, represents a well-demarcated mixed radiolucency/radio-opacity with smooth and often sclerotic borders. These lesions are usually solitary and most commonly seen in the mandible; cases in the maxilla have also reported. Histologically, they contain a relatively avascular cellular fibrous stroma with reticular bone trabeculae and cementum-like spherules. We present a case report of an 11-year-old boy with a 2-month history of an asymptomatic swelling seen on the left side of the angle of the mandible. An initial diagnosis of ossifying fibroma was made by an incisional biopsy, and considering the age as a factor surgical resection of the tumor was done under general anesthesia following closure by a two-layer suturing under proper antibiotic and analgesic coverage. The patient has been on regular follow-ups with no adverse effects and satisfactory healing.
PubMed: 36393953
DOI: 10.4103/njms.NJMS_70_18 -
Journal of the Endocrine Society Jul 2021Ossifying fibromas are very rare tumors that are sometimes seen as part of the hyperparathyroidism-jaw tumor syndrome (HPT-JT), which is caused by inactivating mutations...
Ossifying fibromas are very rare tumors that are sometimes seen as part of the hyperparathyroidism-jaw tumor syndrome (HPT-JT), which is caused by inactivating mutations of the tumor suppressor gene mutations have been identified in a subset of sporadic cases but aberrant expression of the encoded protein, parafibromin, has not been demonstrated in ossifying fibroma. We sought to determine if loss of parafibromin regularly contributes to the development of sporadic, nonsyndromic ossifying fibroma. We examined a series of 9 ossifying fibromas, including ossifying, cemento-ossifying, and juvenile active variants, for parafibromin protein expression by immunohistochemistry and for sequence abnormalities by Sanger sequencing and/or targeted AmpliSeq panel sequencing. Four ossifying fibromas showed a complete absence of nuclear parafibromin expression; loss of parafibromin expression was coupled with aberrant cytoplasmic parafibromin expression in 1 case. mutations were detected in 2 cases with aberrant parafibromin expression. These results provide novel evidence, at the level of protein expression, that loss of the parathyroid /parafibromin tumor suppressor may play a role in the pathogenesis of a subset of ossifying fibromas.
PubMed: 34159287
DOI: 10.1210/jendso/bvab087 -
Polish Journal of Pathology : Official... 2021Juvenile psammomatoid ossifying fibroma (JPOF) is an uncommon benign and locally aggressive tumor. We report an unusual head tumour with extremely rare extensiveness and...
Juvenile psammomatoid ossifying fibroma (JPOF) is an uncommon benign and locally aggressive tumor. We report an unusual head tumour with extremely rare extensiveness and aggressivness. The patient was 18-year-old female with three-day-lasting headache and repetitive oral bleeding. Computed tomography revealed a large, well-circumscribed, expansile mass occupying ethmoid cells, nasal cavities and ventral part of the sphenoid sinus, with extention into the anterior cranial fossa. Pterional craniotomy was carried out. On one-year follow-up recurrence of the lesion was identified and the second surgery was performed. The lesion is under supervision now, due to incomplete removal.
Topics: Adolescent; Bone Neoplasms; Female; Fibroma, Ossifying; Humans; Tomography, X-Ray Computed
PubMed: 35048642
DOI: 10.5114/pjp.2021.111779 -
Head and Neck Pathology Jun 2022Peripheral ossifying fibromas (POFs) and peripheral odontogenic fibromas (POdFs) appear clinically similar but of different histogenesis. The novel marker SATB2 is...
Peripheral ossifying fibromas (POFs) and peripheral odontogenic fibromas (POdFs) appear clinically similar but of different histogenesis. The novel marker SATB2 is involved in regulation of osteoblastic differentiation and phenotype. However, SATB2 expression has not been previously explored in POFs and POdFs. Given the potential for mineralized tissue formation in POFs and POdFs, and to more clarify the phenotype of the lesional cells, this study was aimed to immunohistochemically investigate SATB2 expression in POFs and POdFs. Fourteen cases of POF and POdF (7 cases each) were selected, stained for SATB2 immunohistochemically, and scored according to the percentage of positive lesional cells (0, no staining; 1 +, < 5%; 2 +, 5-25%; 3 +, 26-50%; 4 +, 51-75%; and 5 +, 76-100%), and the intensity of staining was graded as weak, moderate, or strong. The control group included the inflammatory fibrous hyperplasia-like area present in two cases, 1 case fibroma, and 1 case giant cell fibroma. Moderate to strong, and diffuse SATB2 nuclear immunoreactivity was detected in the lesional cells of all cases of POFs and POdFs with variable scores; 3-5 + for the POFs and 3-4 + for the POdFs (P = 0.101). The distribution of staining was more prominent in those lesional cells associated with the osteoid/calcification in the cases of POFs. No staining was noted in the control group. The lesional cells in both POFs and POdFs express SATB2 and may exhibit the osteoblastic-like phenotype. SATB2 staining may be useful for diagnosis of subsets of POFs with minimal or absent calcification and some POdFs with unidentifiable odontogenic epithelium.
Topics: Calcinosis; Fibroma, Ossifying; Gingival Neoplasms; Humans; Hyperplasia; Matrix Attachment Region Binding Proteins; Odontogenic Tumors; Transcription Factors
PubMed: 34224081
DOI: 10.1007/s12105-021-01355-z