What Is a Pineoblastoma?

Desmoplastic fibroma, also known as hard fibroproliferative fibroma or ligament-like fibroma, is a fibroma that originates in benign fibrous tissue of bone. It is similar to hard fibroids of soft tissue and is easy to relapse.

Basic Information

nickname

Hard fibroproliferative fibroma

Visiting department

orthopedics

Common symptoms

Moderate pain, pathological fracture, occasionally considerable bone swelling

Fibroblastic fibroma pathology

As seen by the naked eye: typical fibroblasts are dense, bright white tendon-like, bunched or swirling, and the boundaries are unclear.

Clinical manifestations of fibroblasts

This disease is rare. No significant gender difference. It can occur in all ages, but at the time of diagnosis, 75% of patients are under 30 years of age. There is no clear onset site, and it can occur in any bone of the entire skeletal system. The long bones (especially the femur, humerus, and tibia) are more common in the metaphysis, and can also be found in the backbone, often invading the metatarsus in adulthood. Symptoms are mild and occur late, including moderate pain, pathological fractures, and occasionally considerable bone swelling.

Fibroblastic fibroma test

Due to the lack of symptoms seen on X-rays, fibroblasts often reached a considerable volume when the X-ray film was taken for the first time. The images showed expansive osteolytic lesions, sometimes regular and sometimes due to parietal epiphysis The presence of a foamy image. The cortical bone can be eccentrically thin, or the entire cortical bone can be thinned. Fibroblasts only moderately and diffusely enhance staining in arteriography. It is a relatively "cold area" on the bone scan. CT is better than X-rays in judging the continuity of bone cortex. MRI is the best choice to evaluate soft tissue invasion and extent. Generally, the boundaries of the lesions are relatively clear on MRI. The T ₁ weighted image shows moderate signal intensity, and the T ₂ weighted image shows mixed signal intensity.

Fibroblastic fibroma treatment

Current treatments include surgery, radiation therapy, chemotherapy and hormone therapy.

1. About half of the cases can be recurred after curettage. Therefore, marginal or extensive mass resection is preferred for the treatment of fibroblasts. With this resection method, severely swollen cases can be cured.

2. In some cases, surgical treatment can cause important dysfunction of the body. At this time, radiotherapy is also an effective treatment. But for young patients, radiation is harmful to growth and development, and increases the risk of disease deterioration. For cases where surgery is difficult to completely remove, postoperative radiotherapy is necessary.

3. Low-dose vincristine and methotrexate proved effective. Low-dose chemotherapy plus surgery is the best choice for children with head and neck lesions.

4. The estrogen antagonist tamoxifen has a certain effect in treating this tumor, but it is still controversial.

Prognosis of fibroblasts

The prognosis of bone ligament-like fibroids is closely related to the tumor site and treatment. Because of its high invasiveness and high recurrence rate, local curettage or intracapsular resection has a poor prognosis, and extensive resection has a good prognosis.