What is Pseudomyogenic Hemangioendothelioma?
Pseudomyogenic Hemangioendothelioma is a very rare type of tumour that comes from the cells lining the blood vessels. It mostly affects young adults, especially men, and often appears in the arms or legs. The word “pseudomyogenic” means that under the microscope, the tumour looks like muscle tissue, even though it is not made from muscles.
It is also called “epithelioid sarcoma-like hemangioendothelioma” because of how the cells appear. This tumour is known to grow slowly and rarely spreads to distant parts of the body, but it often reappears in the same area after treatment.
Symptoms
People usually notice the tumour as one or more small lumps under the skin or in the deeper soft tissues of the arms or legs. These lumps are often painless but may become sore if they press against surrounding tissue. The lumps can grow over time or stay the same size. In some cases, the tumour may appear in different parts of the same limb, which can be confusing for both the patient and the doctor.
Diagnosis
To find out if someone has this tumour, an imaging test like an MRI is usually performed to look at the size and location of the lump. A biopsy is then done, where a piece of the lump is removed and examined in the lab. Under the microscope, the cells look somewhat like muscle cells, but special tests show they are related to blood vessel cells. This helps confirm the diagnosis.
Treatment
The main treatment is surgery to remove the tumour. Because it has a high chance of coming back in the same area, doctors try to remove it completely with clear edges. In some cases, radiation therapy may be used to reduce the chance of recurrence. Chemotherapy is rarely used unless the tumour spreads to other parts of the body.
Prognosis
Most patients do well and live normal lives, especially if the tumour is treated early. However, because it can return, regular follow-up with the doctor is important. Catching any recurrence early gives the best chance for successful treatment again.
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