What Is a Hemangioendothelioma?

Hemangioendothelioma is a malignant tumor originating from vascular endothelial cells. Generally according to the tumor tissue morphology, the cell differentiation is divided into two levels: one is intermediate (low-grade malignant), the cells are well differentiated, and most are non-metastatic; high. Both can have single and multiple performances. WHO statistics: Intermediate hemangioendothelioma accounts for 0.28% of primary bone tumors, and hemangiosarcoma accounts for 0.23%.

Hemangioendothelioma

Visiting department: Cardiac Surgery
Multiple groups: Young and middle-aged

Common causes: still uncertain.
Common symptoms: Swelling, pain, and limited joint movements.
Contagious: no

Clinical manifestations of hemangioendothelioma

Incidence has been reported in all age groups, but it is mainly seen in young and middle-aged people. The male to female ratio is basically 1: 1. The tumor can occur in bones throughout the body, and is more common in long tubular bones. Long cases are more common in the lower extremities, especially on the right.

Clinically, patients often complain of swelling, pain, and even limited joint movement. Tumors in the spine can also cause neurological symptoms. Once the symptoms of angiosarcoma appear, the disease usually develops more quickly, with local spread and metastasis in the later stages.

Hemangioendothelioma imaging

On X-rays, intermediate-type hemangioendothelial tumors show flaky or irregular osteolytic swollen bone destruction at the metaphysis of long bones, with well-defined boundaries, and cancellous and cortical bones can be involved. Residual trabeculae can be seen in the bone destruction area, and periosteal reaction is rare. Angiosarcoma has bony destruction of plaques, foams, or large pieces, unclear boundaries, partial or complete disappearance of the bone cortex, radial needles in the vicinity, periosteal reactions, and sometimes soft tissue masses. Angiography can clearly show the tumor bone boundaries and soft tissue boundaries. There are a large number of tortuous irregular tumor arteriovenous fistulas in the tumor. Neovascular disorders are more common in angiosarcomas.

Hemangioendothelioma pathological changes

The tumor was soft to the naked eye, without capsules, and filled with blood clots or sponge-like gels. Microscopically, the tumor was composed of proliferating capillaries. Vascular cavity is mostly immature, and there is anastomosis between blood vessels. Intermediate tumor vascular endothelial cells can still be differentiated, showing a round or oval shape, the nucleus is slightly deep stained, no or mild atypia, and rare divisions. Angiosarcoma shows a large number of new capillaries. Endothelial cells are arranged in multiple layers and piles. Cells are poorly differentiated. Nuclei and deep-stained mitosis are common. Cells infiltrate into the stroma, and bone trabecula is difficult to see.

Differential diagnosis of hemangioendothelioma

It should be differentiated from osteolytic osteosarcoma, giant cell tumor of bone, chondrosarcoma, reticulosarcoma and metastatic tumor.

Hemangioendothelioma treatment and prognosis

Mainly surgical treatment. Intermediate hemangioendothelial cell tumors that occur in the limb can be extensively resected by tumor margins, autologous or allogeneic bone grafts, and appropriately internally fixed. In general, the tumor is completely removed by surgery. The tumor is grade , and the five-year survival rate is 95 to 100%. Grade can reach 60 to 70%. Multiple tumors can be prepared according to the situation, and amputation is used if necessary. In case of recurrence, surgery can be performed again, and metastasis generally does not occur. Angiosarcoma, high degree of malignancy, rapid growth and soft tissue involvement, many patients have metastasized when found. Various effective limb salvage techniques can be used in early cases, and amputations or joint dissections are used in later stages, but the prognosis is generally poor. The five-year survival rate is less than 20%. This type of tumor is more sensitive to radiotherapy and can be used as an adjuvant treatment before and after surgery. Radiation therapy that occurs in the vertebral body or pelvis is of greater significance. It has been reported that the use of radiotherapy-surgery-radiotherapy improves the cure rate and improves the quality of life.