Ossifying fibroma: the peripheral variant.

Introduction: Peripheral ossifying fibroma (POF) is a solitary gingival growth thought to arise from the gingiva, periosteum or the periodontal ligament. It is a slow-growing, benign, progressive lesion that is limited in size. Case description: This article describes a case of ossifying fibroma of a peripheral variant that occurred in a 26-year-old female in the anterior region of the lower jaw and presented as a growth on the gingiva. Since it was difficult to diagnose clinically, a pathological evaluation was mandatory. Upon pathological confirmation of the diagnosis, the lesion was surgically excised up to the periosteum. This was deemed to be the required treatment yet, since the recurrence rate is high for POF (8% to 20%), the patient must be followed up yearly to check for recurrence.

Introduction: Le fibrome ossifiant périphérique (FOP) est une excroissance gingivale solitaire dont on pense qu'elle provient des gencives, du périoste ou du ligament parodontal. Il s'agit d'une lésion progressive, bénigne et à croissance lente dont la taille est limitée. Description du cas: Cet article décrit le cas d'une variante périphérique du fibrome ossifiant survenue chez une femme de 26 ans, dans la région antérieure de sa mâchoire inférieure et se présentant comme une excroissance sur la gencive. Comme il était difficile d'établir un diagnostic clinique, une évaluation pathologique a été nécessaire. Après confirmation pathologique du diagnostic, la lésion a été excisée jusqu'au périoste lors d'une opération chirurgicale. On a jugé ce traitement nécessaire, puisque le taux de récidive du FOP est élevé (entre 8 % et 20 %). La patiente doit faire l'objet d'un suivi annuel pour rechercher toute récidive éventuelle.

Jaffe-Campanacci syndrome; a case series and review of the literature.

BACKGROUND: Jaffe-Campanacci syndrome is a rare syndrome, characterized by multiple non-ossifying fibromas (NOF) and cafe-au-lait patches. The name was coined in 1982 by Mirra after Jaffe who first described the case in 1958. Although it's suggested there is a relation with Neurofibromatosis type 1, there is still no consensus on whether Jaffe-Campanacci syndrome is a subtype or variant of neurofibromatosis-1(NF-1). CASE PRESENTATION: In this article, we present a case series of 2 patients. The first case is a 13-year-old male with Jaffe-Campanacci syndrome who presented with a distal femur fracture. His father had positive features of both Jaffe-Campanacci syndrome and NF-1, while his sister only had features of NF-1, so we presented both. CONCLUSION: Jaffe-Campanacci has a clear relationship with type 1 neurofibromatosis, which still has to be genetically established. Due to the presence of several large non-ossifying fibromas of the long bones, it is linked to a significant risk of pathological fractures. We concur with previous authors, that an osseous screening program should be performed for all patients with newly diagnosed type 1 neurofibromatosis, to identify non-ossifying fibromas and assess the potential for pathological fracture. Moreover, siblings of patients with NF-1 should be screened for multiple NOFs that may carry a high risk of pathological fractures.

Peripheral Ossifying Fibroma: A Case Report.

Peripheral ossifying fibroma (POF) is a reactive overgrowth that most commonly occurs on the gingiva. It is a benign oral soft tissue tumour. It is most commonly found on the anterior maxilla and has a female predilection. Most commonly found to occur in the second decade of life. This type of lesion originates from the cells of the periodontal ligament. It is often associated with trauma or local irritants, such as subgingival plaque and calculus, dental appliances and poor-quality dental restorations. This entity requires a proper treatment protocol and a regular follow-up. It can cause significant discomfort and irritation in the oral health if left untreated. The recurrence rate of the lesion varies according to the authors. This case report describes a case of POF in an adult female patient which was treated using surgical excision resulting in an uneventful healing during the post-operative period.

Metastatic Fibrosarcomatous Transformation of Dermatofibrosarcoma Protuberans With Lung Metastasis: A Case Report.

Dermatofibrosarcoma protuberans (DFSP) is a low- to intermediate-grade dermal soft tissue malignant tumor (sarcoma) with a high local recurrence rate but low metastatic potential. DFSP is characterized by uniform spindle cell fascicles arranged classically in a storiform pattern and by CD34 immunoreactivity. On gross examination, DFSP usually manifests as a white or yellow soft tissue mass with a smooth outer surface and poor circumscription. In this study, we report a case of DFSP with fibrosarcomatous transformation, a rare but well-known phenomenon encountered in DFSP that is correlated with an increased risk of adverse outcomes in patients with DFSP. A 45-year-old male presented with a progressively enlarging lump on his left shoulder, initially suspected of being a lipoma but diagnosed as a fibrosarcomatous transformation of DFSP. Surgical resection was performed, with the subsequent identification of metastatic sarcoma in pulmonary nodules. Robotic-assisted thoracoscopy excised the nodules, confirming metastatic sarcoma with aggressive behavior. Despite negative adjuvant treatment plans, the patient remains under surveillance with imaging, showing no recurrence in recent scans. Continued follow-up with medical and surgical oncology is planned. DFSP is a rare soft tissue sarcoma characterized by indolent growth and low metastatic potential, except in fibrosarcomatous transformation cases. Molecularly, DFSP is defined by a COL1A1-PDGFB fusion transcript that is targetable with imatinib therapy. Treatment involves wide surgical resection, with adjuvant radiation therapy in select cases. Radiation therapy may be employed in cases with close or positive margins, while conventional chemotherapy has limited utility. Multidisciplinary collaboration is crucial for optimal management. Overall, this case underscores the challenges in diagnosing and managing aggressive sarcomas like fibrosarcomatous DFSP, emphasizing the importance of vigilant surveillance and multidisciplinary collaboration in optimizing patient outcomes. Further research is needed to understand the mechanisms underlying fibrosarcomatous transformation and to explore novel therapeutic avenues for this challenging malignancy.

Maxillofacial Bone Involvement in Fibro-Osseous Lesions: Emphasizing the Significance of Differential Diagnosis.

The World Health Organization's (WHO) 2022 update on the classification of odontogenic and maxillofacial bone tumors has revolutionized diagnostic and treatment paradigms by integrating novel molecular insights. Fibro-osseous lesions of the maxillo-facial bones constitute a heterogeneous group encompassing fibrous dysplasia, Psammomatoid Ossifying Fibroma (PSOF), Juvenile Trabecular Ossifying Fibroma (JTOF), and other variants. Despite histological similarities, their distinct clinical manifestations and prognostic implications mandate precise differentiation. The intricacies of diagnosing fibro-osseous lesions pose challenges for pathologists, maxillofacial surgeons, dentists and oral surgeons, underscoring the importance of a systematic approach to ensure optimal patient management. Herein, we present two cases, fibrous dysplasia and Cemento-Ossifying Fibroma, detailing their clinical encounters and management strategies. Both patients provided informed consent for publishing their data and images, adhering to ethical guidelines.

Craniomaxillofacial Fibro-osseous Lesions in Children.

Craniofacial fibro-osseous lesions represent a diverse spectrum of pathologic conditions where fibrous tissue replaces healthy bone, resulting in the formation of irregular, woven bone. They are more commonly diagnosed in young people, with treatment strategies dependent on clinical behavior and skeletal maturity. This article discusses the examples of craniofacial fibro-osseous lesions, based on the latest classifications, along with their diagnostic criteria and management.

Ossifying fibromyxoid tumor of the soft tissue in the left upper arm and a review of the literature: A case report.

Ossifying fibromyxoid tumor (OFMT) of the soft parts is a mesenchymal neoplasm of uncertain lineage. Fibromyxoid matrix and peripheral metaplastic bone are common histological features of this type of tumor. In the present study, a case of OFMT in a 33-year-old female was reported. The patient was referred to the First Affiliated Hospital of China Medical University (Shenyan, China) in January 2018. The patient had developed a mass in the left upper arm 6 months prior to presentation, which was slowly enlarging. The tumor was 1.5 cm in diameter, with hard texture. Histologically, the tumor showed a clear boundary with no invasion into the adjacent tissue. The majority of tumor cells were round and medium-sized, with abundant pale cytoplasm, without obvious atypia and densely arranged in sheets. The tumor tissue was characterized by cartilage-like morphology and fibromyxoid and hyalinization matrix. Mitotic index was <1/10 high-power fields. Additionally, tumor cells were positive for S-100 and vimentin expression, but negative for smooth muscle actin, CD34, cytokeratin, desmin, human melanoma black 45 and melanoma A. Ki67 index was ~1%. The patient underwent surgery and the tumor was totally removed. No recurrence was observed at the final 6-year follow-up. Based on the aforementioned findings, the patient was diagnosed as typical OFMT. Slow growth and clear boundaries often suggest an indolent nature to this type of tumor. However, close follow-up should be performed due to its malignant potential.

Giant Ossifying Lipoma of the Hand: A Case Report and Review of Literature.

Lipomas are one of the most common benign tumors of the body, characterized by a slow-growing, painless mass that rarely causes symptoms. Bone metaplasia among the mature adipose cells, however, is a rare condition called osteolipoma. In this article, we present a case report of a 61-year-old lady with a giant osteolipoma of the hand. After a surgical extirpation, she showed a fast recovery, and no recurrence during the two-year follow-up period was observed. We aimed to make a literature review of this pathology, discussing the symptoms, diagnosis, and management of this rare condition.

CT-based radiomics analysis of different machine learning models for differentiating gnathic fibrous dysplasia and ossifying fibroma.

OBJECTIVE: In this study, our aim was to develop and validate the effectiveness of diverse radiomic models for distinguishing between gnathic fibrous dysplasia (FD) and ossifying fibroma (OF) before surgery. MATERIALS AND METHODS: We enrolled 220 patients with confirmed FD or OF. We extracted radiomic features from nonenhanced CT images. Following dimensionality reduction and feature selection, we constructed radiomic models using logistic regression, support vector machine, random forest, light gradient boosting machine, and eXtreme gradient boosting. We then identified the best radiomic model using receiver operating characteristic (ROC) curve analysis. After combining radiomics features with clinical features, we developed a comprehensive model. ROC curve and decision curve analysis (DCA) demonstrated the models' robustness and clinical value. RESULTS: We extracted 1834 radiomic features from CT images, reduced them to eight valuable features, and achieved high predictive efficiency, with area under curves (AUC) exceeding 0.95 for all the models. Ultimately, our combined model, which integrates radiomic and clinical data, displayed superior discriminatory ability (AUC: training cohort 0.970; test cohort 0.967). DCA highlighted its optimal clinical efficacy. CONCLUSION: Our combined model effectively differentiates between FD and OF, offering a noninvasive and efficient approach to clinical decision-making.

A giant peripheral ossifying fibroma of the maxilla with extreme difficulty in clinical differentiation from malignancy: a case report and review of the literature.

BACKGROUND: Peripheral ossifying fibroma is a nonneoplastic inflammatory hyperplasia that originates in the periodontal ligament or periosteum in response to chronic mechanical irritation. Peripheral ossifying fibroma develops more commonly in young females as a solitary, slow-growing, exophytic nodular mass of the gingiva, no more than 2 cm in diameter. While various synonyms have been used to refer to peripheral ossifying fibroma, very similar names have also been applied to neoplastic diseases that are pathologically distinct from peripheral ossifying fibroma, causing considerable nomenclatural confusion. Herein, we report our experience with an unusual giant peripheral ossifying fibroma with a differential diagnostic challenge in distinguishing it from a malignancy. CASE PRESENTATION: A 68-year-old Japanese male was referred to our department with a suspected gingival malignancy presenting with an elastic hard, pedunculated, exophytic mass 60 mm in diameter in the right maxillary gingiva. In addition to computed tomography showing extensive bone destruction in the right maxillary alveolus, positron emission tomography with computed tomography revealed fluorodeoxyglucose hyperaccumulation in the gingival lesion. Although these clinical findings were highly suggestive of malignancy, repeated preoperative biopsies showed no evidence of malignancy. Since even intraoperative frozen histological examination revealed no malignancy, surgical resection was performed in the form of partial maxillectomy for benign disease, followed by thorough curettage of the surrounding granulation tissue and alveolar bone. Histologically, the excised mass consisted primarily of a fibrous component with sparse proliferation of atypical fibroblast-like cells, partly comprising ossification, leading to a final diagnosis of peripheral ossifying fibroma. No relapse was observed at the 10-month follow-up. CONCLUSIONS: The clinical presentation of giant peripheral ossifying fibromas can make the differential diagnosis from malignancy difficult. Proper diagnosis relies on recognition of the characteristic histopathology and identification of the underlying chronic mechanical stimuli, while successful treatment mandates complete excision of the lesion and optimization of oral hygiene. Complicated terminological issues associated with peripheral ossifying fibroma require appropriate interpretation and sufficient awareness of the disease names to avoid diagnostic confusion and provide optimal management.

Peripheral Ossifying Fibroma of Maxillary Gingiva with Calcification: A Case Report.

Peripheral ossifying fibroma is presented as an exophytic growth with a smooth surface. It is located mostly on the gingiva, and it is an unusual growth of gingiva that occurs after irritational trauma. It is seen in the later decade of life, with the calcifications in the lesion in advanced stages seen on radiographic images. In the present case, a 50-year-old male reported with a huge gingival growth in the left maxillary posterior gingival region; when radiograph was taken, it showed calcifications. The patient was treated by surgical excision of the growth.

Jaffe-Campanacci syndrome resulted in amputation: A case report.

BACKGROUND: Jaffe-Campanacci syndrome (JCS) is a very rare syndrome. The treatment of JCS is more conservative, and most authors recommend that no surgery should be done in asymptomatic patients. The conventional concept holds that the natural course of non-ossifying fibromas (NOFs) grows with the development of bones, and the osteolytic region gradually stops expanding and self-healing through bone ossifying around the lesion and ossification within the lesion. But in this case, the bone lesions were potentially biologically aggressive, which led to severe limb deformities and pain. CASE SUMMARY: We present the case of a 5-year-old girl with JCS presenting with not only NOF sand café-au-lait macules, but also showed features not mentioned before, severe limb pain, and at last resulted in amputation. She was admitted to our hospital after presenting with claudication and mild pain over her right thigh, which worsened when stretching or being touched. Skin examination revealed multiple café-au-lait macules on the neck, arm, axilla, and torso, including the nipples and perineum. Radiographs revealed multiple lytic lesions in the proximal part of the right humerus, distal part of the right clavicle, proximal and distal parts of the right femur, and proximal parts of the right tibia and fibula. Curettage and biopsy were performed on the distal part of the right femur. At the age of 7, the girl was re-admitted to our hospital for a pathological fracture in the middle in the right femur and underwent Intralesional excision, internal fixation, bone grafting, and spica casting. At the age of 10, the girl came to our hospital again for severe pain of the right leg. Amputation from the middle level of the right femur was performed. We present the case of a 5-year-old girl with JCS presenting with not only NOFs and café-au-lait macules, but also showed features not mentioned before, severe limb pain, and at last resulted in amputation. She was admitted to our hospital after presenting with claudication and mild pain over her right thigh, which worsened when stretching or being touched. Skin examination revealed multiple café-au-lait macules on the neck, arm, armpit, and torso, including the nipples and perineum. Radiographs revealed multiple lytic lesions in the proximal part of the right humerus, distal part of the right clavicle, proximal and distal parts of the right femur, and proximal parts of the right tibia and fibula. Curettage and biopsy were performed on the distal part of the right femur. At the age of 7, the girl was re-admitted to our hospital for a pathological fracture in the middle in the right femur and underwent Intralesional excision, internal fixation, bone grafting, and spica casting. At the age of 10, the girl came to our hospital again for severe pain of the right leg. Amputation from the middle level of the right femur was performed. CONCLUSION: In our opinion, education on preventing pathological fractures and explaining the consequent serious consequences to the parents is a matter of prime significance. At the same time, prophylactic treatment (restricted exercise, support, or surgery) is also considerable for JSC.

A Massive Growth in the Palate: A Case Report.

This case report describes a 67-year-old woman who developed an extensive, slow-growing lesion occupying the whole of the palate in 10 years. Considering clinical and radiographic features, calcifying neoplasms were considered. Correlating microscopic features with clinical features, the lesion was diagnosed as peripheral ossifying fibroma, which seldom presents as an extensive lesion on the palate amongst the elderly age group. This case report will highlight clinicians and pathologists about a rare presentation of a commonly encountered lesion with a comprehensive view of the differential diagnosis of other comparable lesions.

Operative Treatment of a Patient with a Pathological Fracture through a Large Non-ossifying Fibroma: A Case Report.

Introduction: Non-ossifying fibroma (NOF) is a common benign fibrogenic bone lesion commonly found in the metaphysis of long bones. While small NOFs are typically asymptomatic and left untreated, large NOFs are often associated with pathologic fractures that can be treated conservatively or operatively. To our knowledge, the NOF presented in this case report is one of the largest reported in the literature. Case Report: We present a case of a 12-year-old Hispanic female who presented to our institution after falling off a horse and landing on her right leg and wrist. Radiographs revealed a right distal femur fracture through an unusually large lytic bone lesion. The patient was treated with curettage, grafting, open reduction, and internal fixation, given the unstable and pathological nature of the fracture. Conclusion: This case substantiates the literature that large fractures through a NOF can be appropriately managed operatively. However, orthopedic surgeons should be aware of the risks of surgery, including infection, when considering operative management of patients who present with large unstable pathologic fractures through a NOF.

Psammomatoid ossifying fibroma in the frontal sinus: An intriguing clinical encounter-A detailed case report.

BACKGROUND: Psammomatoid ossifying fibroma (POF) is a rare craniofacial neoplasm, primarily affecting the maxillofacial region, and typically observed in adolescents and young adults. This case report presents a unique occurrence of POF in a 50-year-old male, defying the conventional age range and exhibiting an unusual anatomical location within the frontal sinus. CASE: A 50-year-old male with a prior history of cecal adenocarcinoma and colectomy presented with left eye proptosis and new-onset headaches. Imaging revealed a well-defined calcified mass in the left frontal sinus, leading to a diagnosis of POF. Open surgical resection was performed to remove the tumor, and histopathological evaluation confirmed its diagnosis as psammomatoid ossifying fibroma. The patient exhibited no postoperative complications or signs of recurrence. CONCLUSION: This case underscores the diverse clinical presentations and diagnostic challenges associated with POF, emphasizing the importance of accurate diagnosis and multidisciplinary collaboration. Further research is needed to explore the genetic underpinnings and optimal management strategies for this intriguing condition.

Exploring a rare case of juvenile psammomatoid ossifying fibroma in the ethmoid: a case study and review.

Juvenile ossifying fibroma (JOF) and its variants, including juvenile psammomatoid ossifying fibroma (JPOF), represent rare yet clinically significant benign fibro-osseous lesions that primarily occur in children and young adolescents. They can be found in diverse anatomical sites such as the jaw, nasal cavity, paranasal sinuses, and orbit. JOF exhibits an aggressive nature, necessitating early radiological detection and surgical intervention. Similarly, JPOF, with a locally malignant potential, requires surgical removal, typically conducted through endoscopic approaches. We report a case of a 5-year-old girl with JPOF arising in the ethmoid, revealed by recurrent epistaxis and proptosis. The text emphasizes the importance of early diagnosis through histopathology as a diagnostic tool and underscores the need for appropriate management.

Management of ossifying fibroma of the bone of the maxilla: a case report and review of the literature.

Ossifying fibroma (OF) is a slow-growing benign fibro-osseous neoplasm. It is mostly odontogenic in origin, and it arises in the jaws, particularly the mandible. OF is characterized by the production of bone and cementum-like calcifications in a fibrous stroma. OF reports of the bone of the maxilla are uncommon. Diagnosing OF can be challenging due to the considerable overlap of clinico-pathological characteristics with those of other neoplasms. Herein, the authors describe a case report OF in a 26-year-old male who presented with a huge fibro-osseous tumor of the maxilla. Histopathology established the diagnosis of maxillary OF. The tumor was surgically removed via a Weber-erguson approach with satisfactory functional and cosmetic results. No recurrence has been found after one year of follow-up. Clinical, radiological, and pathological characteristics, as well as surgical treatment approaches, are further discussed. This is one of a few documented cases of maxillary OF in our setting.

Cemento-Ossifying Fibroma of the Mandible: A Case Report.

Benign osseous tumors of mesodermal origin that are included within the group of fibro-osseous lesions include cemento-ossifying fibromas (COFs). The fibrocellular component of these diseases originates from the periodontal ligament, which deposits bone and cementum encased in fibrous tissue. It typically appears in the mandible and presents as a solitary, nonaggressive, slowly developing, asymptomatic, expansile lesion, rarely occurring in the maxilla. The only intervention that proved to be successful in producing excellent outcomes and that may be regarded as a final therapeutic option is the complete surgical removal of COFs. Presenting herein is a case report describing a painless and expansile mass in the left mandibular region, histopathologically diagnosed as COF.

Literature Review, Case Presentation and Management of Non-ossifying Fibroma of Right Angle of Mandible: More Than just a Cortical Defect!

Non-ossifying fibroma (NOF) of jaw bones are rare. While NOF is the most common benign bone tumor of long bones with pathognomonic radiological features and bear a tendency for self-regression, gnathic NOF appears to be comparatively larger in size and behave more aggressively. A 16 years old female patient reported with painless swelling of the right side of the face of 4 months duration. Radiographic analysis showed a unilocular radiolucent lesion of right angle of the mandible with ill-defined margins, cortical perforation and thinning of inferior border. The lesion was provisionally diagnosed as odontogenic keratocyst/unicystic ameloblastoma and incisional biopsy was performed. The histopathological features and immunohistochemical characteristics favored a diagnosis of NOF. The lesion was excised and reconstructed. The excised specimen confirmed the diagnosis. There are no signs of recurrence at 18 months follow-up. NOF should be considered in the differential diagnosis of uni-/multilocular radiolucencies of jaws particularly the posterior mandible.