This is a rare type of cancer, which starts from the connective tissue and spreads through different parts of the body. Its name is related to the epithelioid features that are characteristic for this particular type of soft tissue sarcoma. Less than 1% of the general population is diagnosed with this condition, which makes it a rare disease. The condition was described in the 1970s and today it is known that it affects primarily the extremities. It is often seen in young adults and may affect the genital area or the spine in rare cases.
MRI – this investigation allows the doctors to identify the exact location of the sarcoma masses, this being the primary reason why it is performed ahead of the tissue biopsy and the pathologic analysis. The MRI can also be useful after the surgical intervention, so as to determine if the tumor has recurred.
According to the latest studies, the 5-year survival rate in patients diagnosed with epithelioid sarcoma varies between 50-70%. On the other hand, the 10-year survival rate in the same patients is of 42-55%. There are numerous factors that can have a definite influence on the survival rate, including the age of the patient, the gender, the localization of the tumors and the dimensions of the tumors. The histopathologic changes that occurs with the epithelioid sarcoma are going to influence the survival rate as well.
In terms of prognosis, it has been demonstrated that gender plays an essential role. Women have been identified to have a better prognosis than men, which suggests that the survival rate is influenced by gender. Apart from that, it seems that the patients who present proximal lesions have a more negative outcome than those who present distal lesions. The age seems to have an influence on the prognosis as well. For example, the younger the age of the patient, the better the outcome will be.
A negative prognosis is also considered in the patients who presents tumors that are larger in size (over 2 cm diameter). If the tumor has also become affected by necrosis or if the doctor observes vascular invasion in the respective patient, then the outcome is negative as well. In deciding on the prognosis for the epithelioid sarcoma, the doctors will also take into consideration the mitotic index.
In conclusion, this is a rare type of sarcoma, which affects primarily young adults. The lesions are predominantly found on the upper extremities, especially on the hand and forearm. At first, a single mass might be present, being painless and growing quite slowly. However, there are a lot of patients that present multifocal tumors and metastatic disease, with the progression of the condition. Despite the invasive treatments, the condition has a high tendency of recurrence and it often involves the lymph nodes. The most common method of diagnosis is the tissue biopsy but the sentinel node biopsy is also performed, especially if there is a suspicion of lymph node involvement. Genetic testing might guarantee a better diagnosis in the future, eliminating the risk of this condition being misdiagnosed. The most common treatment is represented by the wide resection. Risk factors that lead to a negative prognosis include gender (males are more predisposed to a negative outcome than women), tumor size and vascular invasion.