Hondromiksoidny fibroma – very rare hryashcheobrazuyushchy benign tumor. Arises in metafiza and a metadiaphysis of long tubular bones more often, however can develop also other bones of a skeleton. A current usually favorable, however, it is inclined to a retsidivirovaniye and in certain cases can . It is shown by gradually amplifying pains in the field of defeat, in hard cases (especially at children's age) the unsharp or moderate atrophy of muscles of the affected extremity and restriction of movements in a nearby joint is possible. Treatment is surgical, it is performed in a planned order. Radical removal of a tumor for prevention of a recurrence and malignant regeneration is carried out. The forecast is favorable. A recurrence arises at 10-15% of patients, usually – in the first two years after surgery.
Hondromiksoidny fibroma – seldom found good-quality new growth arising in bones and consisting of hryashcheobrazuyushchy fabric. Makes 0,5-1% of total number of bone tumors. Can develop at people of any age, men and women suffer equally often. Rapid growth and the expressed symptomatology is observed at children more often. In 15% of cases proceeds asymptomatically and becomes a casual find of the traumatologist or orthopedist during the radiological research.
It is usually localized in a metafizarny and metaepifizarny zone of long tubular bones of the lower extremities. First place on prevalence is won by hondromiksoidny fibroma of the proximal end of a tibial bone. Quite often it is found in metatarsal bones and a calcaneal bone. Damage of edges, humeral and pelvic bones, and also vertebras, a breast and bones of a skull is possible. 25% of total number of cases are the share of damage of flat bones. The tumor in a backbone differs in the most aggressive growth.
Symptoms of hondromiksoidny fibroma
The clinical picture of a disease substantially depends on age of patients. At adults the disease usually develops slowly and proceeds with the erased symptomatology or at all asymptomatically. More rapid growth of a tumor, progressive strengthening of pains and the expressed clinical symptoms is characteristic of children.
The main complaint of patients is pain which develops during the movement more often, however, can disturb also at rest. Intensity of a pain syndrome significantly differs at different patients – from insignificant, erased to intensive, pronounced. At survey local temperature increase, an atrophy of muscles of the affected extremity, restriction of movements in a nearby joint or pain at the movements can come to light.
Diagnostics and differential diagnosis of hondromiksoidny fibroma
The clinical picture at this disease is not specific therefore the diagnosis is exposed by results of additional researches. On roentgenograms ekzofitny education with accurate scalloped outlines which is usually localized in a metafizarny zone comes to light and grows towards an epifiz. The tumor represents accurately outlined destruction center which length can fluctuate from 1-2 to 6-8 centimeters. The cortical layer over the center is blown up and thinned. On edge of education the sclerous rim comes to light, in the center of destruction impregnations of lime and the trabekulyarny drawing can be found. If the tumor is located subperiostalno, the kortikalny layer becomes uneven. There is no Periostalny reaction usually. At localization of a new growth in vertebras germination of a periosteum is often observed.
The radiological picture at hondromiksoidny fibroma can remind changes at a hondrosarkoma, an enkhondroma, a good-quality hondroblastoma and hondroblastichesky option of osteosarcoma therefore for statement of the final diagnosis the bone trepanobiopsiya is carried out. During differential diagnostics first of all it is necessary to exclude a high-aggressive malignant hondrosarkoma.
Macroscopically fabric of a tumor represents the lobular formation of a dense consistence of cyanotic-gray or grayish-white color which is well delimited from healthy fabrics. By the form can remind a hyaline cartilage. At a microscopic research hondroidny or miksoidny intercellular substance in which the extended or star-shaped cages are located comes to light. Sites of connecting fabric are located mainly in layers between segments. In the same place small vessels are found. Along with collagenic fibers in fabric of a tumor comes to light osteoides and huge multinuclear cages.
The structure of segments within one tumor can significantly differ. Various fibroblasta come to light – both large, and small, some cages have 2 or 3 kernels. Cages settle down mainly on the periphery of segments. In every third case of a cell of benign hondromiksoidny fibroma have atypical structure and by the form remind cages of a malignant hondrosarkoma. In 10% of cases in a bioptata the necrosis centers are found.
During the immunohistochemical research positive reaction with protein S-100 comes to light. The cages located on the periphery of segments show an immunoreactivity with CD 34 and a gladkomyshechny aktin. Cytoplasmatic fibrilla and cellular shoots meet, existence of a glycogen is noted. Along with typical miofibroblasta and hondrotsita intermediate forms of cages are found.
Treatment of hondromiksoidny fibroma
Treatment only surgical. Operation is carried out in a planned order in the conditions of office of oncology. The most effective method of surgical intervention providing the minimum quantity of a recurrence is the regional resection of the site of a bone with the subsequent plasticity of defect auto-or allotransplantaty.
In some cases at this disease the curettage (operation during which the tumor is scraped out the curette – the surgical instrument by the form reminding a spoon) is made. However, this approach is not preferable as at such intervention the probability that in the struck area there will be changed tumor cells increases, and hondromiksoidny fibroma recurs over time.
Data on probability of transformation of hondromiksoidny fibroma into a malignant tumor are ambiguous. It is caused by both a neoplasia rarity, and possible hyper diagnostics (cases when by results of a microscopic research the remote tumor is accepted for malignant because of existence of similar cages). In general the forecast at this disease is considered favorable.