They are benign lesions and malignant degeneration is rare. They are usually treated with curettage which however have a high recurrence rate of 25%. As such if an en-bloc resection is possible this is advisable

Plain film

often seen as a lobulated, eccentric radiolucent lesion

long axis parallel to long axis of long bone

no periosteal reaction (unless a complicating fracture present)

geographic bone destruction: almost 100%

well defined sclerotic margin: 86%

there can be presence of septations (pseudotrabeculation): 57% 2

there can be presence of matrix calcification in a small proportion of cases: 12.5%1

MRI

MR features are often not particularly specific. Signal characteristics include

T1 - low signal

T1 C+ (Gd) -

the majority (~70%) tend to show peripheral nodular enhancement

~ 30% diffuse contrast enhancement and this can be either homogeneous or heterogeneous 19

T2 - high signal

Bone scan

A scintigraphic "doughnut sign" has been described in this tumour type 11. However, this is very non-specific and can be found in a plethora of other bone lesions.

Diagnosis is usually made by ultrasonography showing a solid ovarian lesion, or, on some occasions, mixed tumors with solid and cystic components. Computed tomography and magnetic resonance imaging can also be used to diagnose fibromas.

In a series of 16 patients, 5 (28%) showed elevated levels of CA-125.

The standard treatment of COC is enucleation and curettage (E&C). Recurrence following E&C is rare.

Usually the lesion is surgically removed. Primarily, there is concern that the lesion identified in a patient could be cancerous, but there is also the risk of torsion, and possibly the development of symptoms. A stable lesion, however, could be clinically followed.

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.

Unilocular radiolucency may be seen and mutilocular also, and mixed too .irregular calcifications may be seen in some cases.

The most conclusive test for a patient with a potential neurofibrosarcoma is a tumor biopsy (taking a sample of cells directly from the tumor itself). MRIs, X-rays, CT scans, and bone scans can aid in locating a tumor and/or possible metastasis.

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.

Although the origin of the disease is unknown, there is speculation that it is an aggressive healing response to small tears in the plantar fascia, almost as if the fascia over-repairs itself following an injury. There is also some evidence that it might be genetic.

In the early stages, when the nodule is single and/or smaller, it is recommended to avoid direct pressure to the nodule(s). Soft inner soles on footwear and padding may be helpful.

MRI and sonogram (diagnostic ultrasound) are effective in showing the extent of the lesion, but cannot reveal the tissue composition. Even then, recognition of the imaging characteristics of plantar fibromatoses can help in the clinical diagnosis.

Surgery of Ledderhose's disease is difficult because tendons, nerves, and muscles are located very closely to each other. Additionally, feet have to carry heavy load, and surgery might have unpleasant side effects. If surgery is performed, the biopsy is predominantly cellular and frequently misdiagnosed as fibrosarcoma. Since the diseased area (lesion) is not encapsulated, clinical margins are difficult to define. As such, portions of the diseased tissue may be left in the foot after surgery. Inadequate excision is the leading cause of recurrence.

Radiotherapy has been shown to reduce the size of the nodules and reduce the pain associated with them. It is approximately 80% effective, with minimal side-effects.

Post-surgical radiation treatment may decrease recurrence. There has also been variable success in preventing recurrence by administering gadolinium. Skin grafts have been shown to control recurrence of the disease.

In few cases shock waves also have been reported to at least reduce pain and enable walking again. Currently in the process of FDA approval is the injection of collagenase. Recently successful treatment of Ledderhose with cryosurgery (also called cryotherapy) has been reported.

Cortisone injections, such as Triamcinolone, and clobetasol ointments have been shown to stall the progression of the disease temporarily, although the results are subjective and large-scale studies far from complete. Injections of superoxide dismutase have proven to be unsuccessful in curing the disease while radiotherapy has been used successfully on Ledderhose nodules.

Benign fibromas may, but need not be, removed. Removal is usually a brief outpatient procedure.

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.

Giant-cell tumor of the tendon sheath, also known as giant-cell synovioma and localized nodular tenosynovitis, is a firm lesion, measuring 1 to 3 cm in diameter, and is most commonly attached to the tendons of the fingers, hands, and wrists, with a predilection for the flexor surfaces. Giant-cell tumor of tendon sheaths most often affect the wrist and fingers of males and females from the ages of 20-50 . These tumors are typically painless and can cause cortical erosion. Surgery to remove the tumor is a common treatment, though the tumors tend to recur.

Patient response to treatment will vary based on age, health, and the tolerance to medications and therapies.

Metastasis occurs in about 39% of patients, most commonly to the lung. Features associated with poor prognosis include a large primary tumor (over 5 cm across), high grade disease, co-existent neurofibromatosis, and the presence of metastases.

It is a rare tumor type, with a relatively poor prognosis in children.

In addition, MPNSTs are extremely threatening in NF1. In a 10-year institutional review for the treatment of chemotherapy for MPNST in NF1, which followed the cases of 1 per 2,500 in 3,300 live births, chemotherapy did not seem to reduce mortality, and its effectiveness should be questioned. Although with recent approaches with the molecular biology of MPNSTs, new therapies and prognostic factors are being examined.

Neurothekeoma is a benign cutaneous tumor first described by Gallager and Helwig, who proposed the term in order to reflect the presumed origin of the lesion from nerve sheath. Microscopically, the lesions described closely resembled the tumor, "nerve sheath myxoma", an entity first described by Harkin and Reed. The latter had, through the years, been variously described as "Bizarre cutaneous neurofibroma", "Myxoma of nerve sheath", and "Pacinian neurofibroma".

Clinically, neurothekeomas present as a solitary nodule of the skin. The most common sites of occurrence are the head and neck and the extremities. The lesions range in size from about 0.5 cm. to more than 3 cm. The average patient age is about 25 years, but neurothkeomas may occur at any age. Women are affected about more often; the male to female ratio is approximately 1:2.

Microscopically, neurothekeoma consists of closely aggregated bundles or fascicles of spindle-shaped cells. The fascicles may or may not have a myxoid background.

Since the time of their first description, it has been reported that neurothekeomas are likely not of nerve sheath origin, as implied by the term. Consequently, neurothekeoma and nerve sheath myxoma are likely not related histogenetically, although they are similar in appearance and in behavior.

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.

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%.

The cause of development for cardiac fibroma is still unknown or unexplained. Some of these cases are observed to be linked to Gorlin syndrome; a complex genetic disorder causing the formation of tumors in various parts of the body. Research is currently being undertaken to identify relevant casual factors. Currently, there are no known methods for preventing cardiac fibroma.

The most common locations are the shaft and epyphises of long bones (fibula and humerus) but the spine, metatarsal bones, and ilium have been involved as well. Radiologic examination evidences osteolytic areas with a lobulated framework comprising radiolucent and radiodense foci admixed to speckled calcification. Cortical destruction is a common finding with no soft tissue expansion in many cases. Histopathology of the lesion shows large areas of mature fibrous stroma undergoing hyaline cartilage metaplasia resulting in conspicuous lobules or gradual transformation into chondroid foci. Both hyaline cartilage and chondroid in turn undergo calcification and endochondral cancellous bone formation mimicking epiphyseal plate-like cartilage.

Differential diagnosis is concerned with fibrocartilaginous dysplasia of bone, desmoplastic fibroma, low-grade fibrosarcoma, chondromyxoid fibroma and low-grade chondrosarcoma.

A full account of imaging findings on radiography, bone scan, CT and magnetic resonance has been provided by Sumner et al.

PCNA and Ki67 immunoreactivity happens in case of fibroma and peripheral granuloma.

Ganglion cysts are diagnosed easily, as they are visible and pliable to touch.

Radiographs in AP and lateral views should be obtained to exclude any more serious underlying pathology. Ultrasonography (US) may be used to increase diagnostic confidence in clinically suspected lesions or to depict cysts, because intratendinous ganglia are readily distinguished from extratendinous ganglia during dynamic ultrasonography, as microscopically, ganglionic cysts are thin-walled cysts containing clear, mucinous fluid.

The histological and ultrastructural features of Ledderhose and Dupuytren's disease are the same, which supports the hypothesis that they have a common cause and pathogenesis. As with Dupuytren's disease, the root cause(s) of Ledderhose's disease are not yet understood. It has been noted that it is an inherited disease and of variable occurrence within families, i.e. the genes necessary for it may remain dormant for a generation or more and then surface in an individual, or be present in multiple individuals in the same generation with varying degree.

There are certain identified risk factors. The disease is more commonly associated with -

- A family history of the disease

- Higher incidence in males

- Palmar fibromatosis 10-65% of the time.

- Peyronie's disease

- Epilepsy patients

- Patients of diabetes mellitus

There is also a suspected, although unproven, link between incidence and alcoholism, smoking, liver diseases, thyroid problems, and stressful work involving the feet.

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.

A schwannoma is a usually-benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves.

The histology of CGCG is one that is composed of many multinucleated giant cells. There is evidence that these giant cells represent osteoclasts (bone-eating cells); others suggest they are more like macrophages. The giant cells may be diffusely located throughout the lesion or focally aggregate in the lesion. The giant cells are typically either large and round, or small and irregular, and can vary greatly in size and shape. Close examination may reveal some hemosiderin deposits as well.