Cystic Fibrous Dysplasia Research Articles

Case of unicystic ameloblastoma with review of literature

AbstractIntroductionAmeloblastoma is one of the most frequent benign odontogenic tumours of the jaw, accounting for approximately 10% of all tumours in the mandible and maxilla. It is a slow‐growing, locally invasive tumour that causes painless swelling of the jaw or maxilla.DiagnosisAmeloblastoma is diagnosed through computerised tomography imaging and a biopsy. A biopsy can help distinguish ameloblastoma from other cancers, such as ossifying fibroma, osteomyelitis, giant cell tumour, cystic fibrous dysplasia, myeloma, and sarcoma.TreatmentAmeloblastoma is best treated with vigorous en bloc excision and concomitant repair. Ameloblastoma therapy has long been plagued by high recurrence rates and extensive tissue abnormalities. Recent molecular findings strongly imply that ameloblastoma patients may benefit from targeted treatment. We offer a case report of unicystic ameloblastoma with a step‐by‐step customised therapeutic plan that prioritises patients’ desire.

Ameloblastoma: An Updated Narrative Review of an Enigmatic Tumor.

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.

Cystic Fibrous Dysplasia of the Humerus

The patient was a 39-year-old woman with a 3-month history of worsening right distal humerus pain that was insidious in onset. At the time of the initial physical therapy visit, the patient complained of weakness and numbness throughout the entire right upper extremity, with an inability to perform daily activities. Magnetic resonance imaging revealed extensive bone marrow signal abnormalities extending from the proximal humeral epiphysis to the distal humeral diaphysis. Further evaluation with radiographs and computed tomography revealed multiple lytic lesions through the humerus. Tissue biopsy confirmed a diagnosis of cystic fibrous dysplasia.

A rare case of psammomatoid ossifying fibroma in the sphenoid bone reconstructed using autologous particulate exchange cranioplasty

Psammomatoid ossifying fibroma (POF), a variant of ossifying fibroma, is a benign fibroosseous lesion typically arising within the nasal cavity, paranasal sinuses, and orbit. Cranial vault involvement is exceedingly rare, with very few cases reported in the literature. The authors report a case of POF in the neurocranium of an 11-year-old child, 4 years after chemotherapy and radiation therapy for acute lymphoblastic leukemia. This case is reported in view of its rarity, novelty of presentation, and the difficulty in diagnosis due to its radiological resemblance to aneurysmal bone cyst or monostotic cystic fibrous dysplasia, further aggravated by the clinical scenario. A novel technique of cranial reconstruction called autologous particulate exchange cranioplasty was used following tumor excision.

Cystic fibrous dysplasia in mandible

Fibrous dysplasia is a benign, rare and idiopathic skeletal disorder characterized by replacement and expansion of medullary bone by disorganized fibroosseous tissue. Radiographically, the lesions found as unilocular or multilocular in radiolucency or a spotted radiolucent/radiopaque pattern or dense radiopaque mass. The most typical radiographic feature is an opacification with weak defined margins interfered into the surrounding bone. Histologically, it is divided as osteolytic, osteosclerotic and cystic type although fibrous dysplasia shows irregularly shaped trabeculae of immature woven bone in a cellular fibroblastic stroma.

Cystic Fibrous Dysplasia in the Long Bone

Prominent osteolysis associated with "ground glass" density of fibrous dysplasia may indicate cystic change or sarcomatous transformation. This complication has been reported only sporadically in the long bones. This article presents clinical, radiographic, and pathologic findings, and outcome of simple curettage and bone graft observed in a series of 8 patients with prominent cystic fibrous dysplasia of the long bone. Magnetic resonance imaging features provide a basis for separation of benign cystic change from malignant transformation. However, biopsy is necessary to distinguish nonspecific cystic degeneration from secondary aneurysmal bone cyst. Simple curettage with allo-chip-bone graft is an effective treatment for cystic fibrous dysplasia.

Cystic fibrous dysplasia of the skull with intracystic haemorrhage

Cystic fibrous dysplasia of the skull with intracystic haemorrhage

Large cystic fibrous dysplasia of the temporal bone: case report and review of literature

Fibrous dysplasia is an uncommon benign disorder of unknown aetiology. It most likely represents a disorder of normal bone development. As it may occasionally involve the temporal bone, and encroach on the middle cranial fossa, it is of interest to the neurosurgeon. Fibrous dysplasia of the cystic variety is very uncommon. We report a case of a large cystic fibrous dysplasia involving predominantly the squamous temporal bone. The radiology and histology are discussed, and its differentiation from other lesions.

Visual loss associated with fibrous dysplasia of the anterior skull base

The authors present a case of visual loss associated with fibrous dysplasia of the anterior skull base and the surgical management of this case. Preoperative computerized tomography scanning in this patient demonstrated a patent optic foramen and a rapidly growing cystic mass within the orbit, which was responsible for the patient's visual loss. A literature review revealed that this case is typical, in that cystic mass lesions of various types are frequently responsible for visual loss associated with fibrous dysplasia. The authors did not find significant evidence in the literature to support the notion that visual loss associated with fibrous dysplasia is the result of progressive optic canal stenosis, thus raising questions about the value of prophylactic optic canal decompression. Instead, as demonstrated by this case and those uncovered in the literature review, most instances of visual loss result from the rapid growth of mass lesions of cystic fibrous dysplasia, mucoceles, or hemorrhage. Findings of the literature review and the present case of fibrous dysplasia of the anterior skull base support a role for extensive surgical resection in these cases and indicate a need for additional prospective analysis of a larger number of patients with this disease.