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A non-ossifying fibroma (NOF) is a benign bone tumor of the osteoclastic, giant cell-rich tumor type. [1] It generally occurs in the metaphysis of long bones in children and adolescents. [ 2 ] Typically, there are no symptoms unless there is a fracture . [ 2 ]
The most common bone tumor is a non-ossifying fibroma. [4] Average five-year survival in the United States after being diagnosed with bone and joint cancer is 67%. [5] The earliest known bone tumor was an osteosarcoma in a foot bone discovered in South Africa, between 1.6 and 1.8 million years ago. [6]
High magnification micrograph of giant cells in a giant-cell tumor of bone, H&E stain. The diagnosis of giant-cell tumors is based on biopsy findings. The key histomorphologic feature is, as the name of the entity suggests, (multinucleated) giant cells with up to a hundred nuclei that have prominent nucleoli.
Metaphyseal tumors or lesions include osteosarcoma, chondrosarcoma, fibrosarcoma, osteoblastoma, enchondroma, fibrous dysplasia, simple bone cyst, aneurysmal bone cyst, non-ossifying fibroma, and osteoid osteoma. [5] One of the clinical signs of rickets that doctors look for is cupping and fraying at the metaphyses when seen on X-ray.
The cystic fibroma (fibroma cysticum) has central softening or dilated lymphatic vessels. The myxofibroma (fibroma myxomatodes) is produced by liquefaction of the underlying soft tissue. The cemento-ossifying fibroma is hard and fibrous, most frequently seen in the jaw or mouth, sometimes in connection with a fracture or another type of injury.
Jaffe–Campanacci syndrome is one of the disorders associated with café au lait macules (CALMs). Presentations may include intellectual disability, disseminated non-ossifying fibromas of the long bones and jaw, hypogonadism or cryptorchidism, or giant cell granulomas of the jaw.
Giant-cell fibroma mostly affects Caucasians and is very rare in other races. Most studies have shown a slight female predominance while however, a few have shown no predominance. [ 6 ] Giant-cell fibromas represent about 1-5% of all biopsied fibrous lesions and around 0.4-1% of total biopsies .
The term fibromatosis refers to a group of soft tissue tumors [1] which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence.