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Recurrence rate of solid form of tumour is lower than classic form.
Osteofibrous dysplasia is treated with marginal resection with or without bone grafting, depending on the size of the lesion and the extent of bony involvement. However, due to the high rate of recurrence in skeletally immature individuals, this procedure is usually postponed until skeletal maturity.
It is common in age group of 10–30 years. It is second most common tumor of spine and commonest benign tumor of pelvis in pediatric population. Incidence is slightly more in males than females (1.3:1).
A nonossifying fibroma (also called fibroxanthoma) is a common benign bone tumor in children and adolescents. However, it is controversial whether it represents a true neoplasm or rather a developmental disorder of growing bone. Radiographically, the tumor presents as a well marginated radiolucent lesion, with a distinct multilocular appearance. These foci consist of collagen rich connective tissue, fibroblasts, histiocytes and osteoclasts. They originate from the growth plate, and are located in adjacent parts of the metaphysis and diaphysis of long bones, most often of the legs. No treatment is needed in asymptomatic patients and spontaneous remission with replacement by bone tissue is to be expected.
Multiple nonossifying fibromas occur in Jaffe-Campanacci syndrome in combination with cafe-au-lait spots, mental retardation, hypogonadism, ocular and cardiovascular abnormalities.
Treatment usually involves surgical removal of the lesion down to the bone. If there are any adjacent teeth, they are cleaned thoroughly to remove any possible source of irritation. Recurrence is around 16%.
Osteofibrous dysplasia (also known as ossifying fibroma) is a rare, benign non-neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. This condition should be differentiated from Nonossifying fibroma and fibrous dysplasia of bone.
The majority of cases occur in the second and third decades, with approximately 75% of cases occurring before the age of 30 years 1,12-15. There is no recognised gender predilection. Examples have however been seen in patients up to the age of 75 years. In some series there is a male predilection 12 whilst in others no such distribution is found 2
The standard treatment of COC is enucleation and curettage (E&C). Recurrence following E&C is rare.
Chondromyxoid fibroma is a type of cartilaginous tumor.
Most cases are characterised by GRM1 gene fusion or promoter swapping. It can be associated with a translocation at t(1;5)(p13;p13).
A chondromyxoid fibroma (CMF) is an extremely rare benign cartilaginous neoplasm which accounts for < 1% bone tumours.
NBCCS has an incidence of 1 in 50,000 to 150,000 with higher incidence in Australia. One aspect of NBCCS is that basal-cell carcinomas will occur on areas of the body which are not generally exposed to sunlight, such as the palms and soles of the feet and lesions may develop at the base of palmar and plantar pits.
One of the prime features of NBCCS is development of multiple BCCs at an early age, often in the teen years. Each person who has this syndrome is affected to a different degree, some having many more characteristics of the condition than others.
This is a very rare phenomenon (50 years of age), and more commonly in women than men (5:1). There is an increased frequency in Okinawa, Japan, but this may be a reporting bias.
The tumor develops very specifically in the subscapular or infrascapular area, deep to the muscle, sometimes even attached to periosteum of ribs. It is usually between the shoulder blade and the lower neck, with rare tumors reported in the chest wall.
Fibrocartilaginous mesenchymoma of bone is (FCMB) is an extremely rare tumor first described in 1984. Fewer than 20 cases have been reported, with patient ages spanning from 9 to 25 years, though a case in a male infant aged 1 year and 7 months has been reported. Quick growth and bulky size are remarkable features of this tumor.
Surgery is curative despite possible local relapses. Wide resection of the tumor and resection arthrodesis with an intramedullary nail, vertebrectomy and femoral head allograft replacement of the vertebral body, resection of the iliac wing and hip joint disarticulation have been among the performed procedures.
The close resemblance of FCMB to fibrocartilaginous dysplasia has suggested to some scholars that they might be closely related entities, although the latter features woven bone trabeculae without osteoblastic rimming, which is a quite distinctive aspect. Instead the occurrence of epiphyseal plate-like cartilage is peculiar of the former.
In medicine, a desmoplastic fibroma is a benign fibrous tumor of bone, affecting children and young adults, potentially resulting in cortical bone destruction.
It can also occur in the hand.
A famous occurrence of this particular form of the disease involved Italo-Australian Riccardo Torresan in 2011, with 18 cm of femur needing to be removed with the now widely recognized method of "aggressive curettage" being employed.
There are several theories about origin:
- There is support for a genetic predisposition, as there are alterations of short arm of chromosome 1;
- Multifocality may suggest systemic enzymatic defect, resulting in abnormal elastogenesis;
- Repeated trauma or friction seems unlikely, but is still a possibility.
Peripheral ossifying fibroma “a gingival nodule which is composed of a cellular fibroblastic connective tissue stroma which is associated with the formation of randomly dispersed foci of mineralised products, which consists of bone, cementum-like tissue, or a dystrophic calcification. The lesion is considered part of an ossifying fibroma, but that is usually considered to be a gnathic tumor. Because of its overwhelming incidence on the gingiva, the condition is associated with two other diseases, though not because they occur together. Instead, the three are associated with each other because they appear frequently on gingiva: pyogenic granuloma and peripheral giant cell granuloma. Some researchers believe peripheral ossifying fibromas to be related to pyogenic fibromas and, in some instances, are the result of a pyogenic granuloma which has undergone fibrosis and calcification.
The term "peripheral ossifying fibroma" has been criticized as this lesion is not related to the ossifying fibroma of bone and is not a fibroma. This term is used in America, however in Britain, this lesion would be termed a fibrous epulis containing bone.
The calcifying odotogenic cyst or the Gorlin cyst, now known in the WHO Classification of Tumours as the calcifying cystic odontogenic tumor, is a benign odontogenic tumor of cystic type most likely to affect the anterior areas of the jaws. It is most common in people in their second to third decades but can be seen at almost any age. On radiographs, the calcifying odontogenic cyst appears as a unilocular radiolucency (dark area). In one-third of cases, an impacted tooth is involved. Microscopically, there are many cells that are described as "ghost cells", enlarged eosinophilic epithelial cells without nuclei.
Benign fibrous histiocytomas (also known as dermal dendrocytoma, dermatofibroma, fibrous dermatofibroma, fibrous histiocytoma, fibroma simplex, nodular subepidermal fibrosis, and sclerosing hemangioma) are benign skin growths.
Central giant-cell granuloma (CGCG) is a benign condition of the jaws. It is twice as likely to affect women and is more likely to occur in 20- to 40-year-old people. Central giant-cell granulomas are more common in the mandible and often cross the midline.
Fibroma of tendon sheath is a benign tumor that presents as a small subcutaneous nodule that slowly increases in size. The tumors often have a multinodular growth pattern, with individual nodules being composed of bland, slender, spindle-shaped cells (myofibroblasts) in a dense, fibrous matrix.” A common microscopic finding is the presence of elongated, slit-like blood vessels. The lesions nearly always arise in the distal portions of the extremities. They often occur on the fingers, hands, toes, or feet. Although they are benign, they may recur in up to 40% of cases.
Although they may be regarded as a tumor of the skin, the lesions arise from tendons and aponeuroses in superficial sites, and are therefore properly classified as in the category "soft tissue tumor."
The biological nature of Fibroma of tendon sheath is not known, but the category appears to comprise a number of different pathologic processes. It is considered that about one-third of the lesions in this category may be acral variants of the entity, nodular fasciitis.
The central odontogenic fibroma is a rare benign odontogenic tumor. It is more common in adults, with the average age being 40. It is twice as likely to affect women than men. It is usually found either in the anterior maxilla or the posterior mandible. Radiographically it presents with either radiolucency or mixed radiolucency/opaque. The simple type is characterized by delicate fibrillar stroma of collagen containing fibroblasts; the WHO type is characterized by more mature fibrillar stroma of collagen. Treatment is by surgical removal
The treatment for CGCG is thorough curettage. A referral is made to an oral surgeon. Recurrence ranges from 15%–20%. In aggressive tumors, three alternatives to surgery are undergoing investigation:
- corticosteroids;
- calcitonin (salmon calcitonin);
- interferon α-2a.
These therapeutic approaches provide positive possible alternatives for large lesions. The long term prognosis of giant-cell granulomas is good and metastases do not develop.
Dermatofibromas are hard solitary slow-growing papules (rounded bumps) that may appear in a variety of colours, usually brownish to tan; they are often elevated or pedunculated. A dermatofibroma is associated with the dimple sign; by applying lateral pressure, there is a central depression of the dermatofibroma. Although typical dermatofibromas cause little or no discomfort, itching and tenderness can occur. Dermatofibromas can be found anywhere on the body, but most often they are found on the legs and arms. They occur most often in women; the male to female ratio is about 1:4. The age group in which they most commonly occur is 20 to 45 years.
Some physicians and researchers believe dermatofibromas form as a reaction to previous injuries such as insect bites or thorn pricks. They are composed of disordered collagen laid down by fibroblasts. Dermatofibromas are classed as benign skin lesions, meaning they are completely harmless, though they may be confused with a variety of subcutaneous tumours. Deep penetrating dermatofibromas may be difficult to distinguish, even histologically, from rare malignant fibrohistocytic tumours like dermatofibrosarcoma protuberans.
Dermatofibromas typically have a positive "buttonhole sign", or central dimpling in the center.
An ossifying fibromyxoid tumour is a type of myxoma. It presents in the extremities more frequently than the trunk. It is derived from mesenchyme. Appearance in the head and neck is rare, but has been reported. Their malignancy has been characterized as "intermediate".
Treatment is usually supportive treatment, that is, treatment to reduce any symptoms rather than to cure the condition.
- Enucleation of the odontogenic cysts can help, but new lesions, infections and jaw deformity are usually a result.
- The severity of the basal-cell carcinoma determines the prognosis for most patients. BCCs rarely cause gross disfigurement, disability or death .
- Genetic counseling