Epithelioid sarcoma: analyses of 15 cases treated in INCA

Abstract

Objectives: To relate the clinical characteristics and evaluations of patients with epithelioid sarcomas treated in Instituto Nacional de Cancer comparing them with the results obtained in the literature. Method: Careful analyses of 15 epithelioid sarcoma cases registered in Instituto Nacional do Cancer between 1984 - 2003. Results: The average age was 28, and ranged from 3 to 69. Of the 15 cases, 8 were female and 7 male. The most frequent location of the sarcoma was the hand (six cases). The size of the tumor was given in six cases, with the average size being 5 cm, while they ranged from 4 - 10 cm. The clinical manifestation referred to in 100% of the cases was the presence of a node, with lethargic growth. The presence of histopatologically confirmed lymph nodes was seen in 3 of the 15 cases. In one of the patients the appearance of the tumor was multicentrical with primary disease on the forearm, with the lumbar and thoracic regions also being affected. In 5 of the cases the surgery was conservative. In 8 of the patients radical surgery with mutilation was used, of which 7 were amputations and one was disarticulation. Radiotherapy was performed in 4 patients, 3 of which were adjuvant and 1 palliative. In 2 cases palliative chemotherapy was used, in another adjuvant chemotherapy was necessary and another case it was neoadjuvant. In both cases there was reoccurrence in the lymph nodes, which were treated by inguinal evacuation. At present, 9 patients are alive and cured, one still has the disease and is being treated with chemotherapy and 3 have died. Conclusion: Epithelioid sarcoma, a rare subset of soft-tissue sarcoma, presents an atypical clinical behavior when compared to other high degree sarcomas and occurs predominantly in young patients, mainly on the superior member extremities. It is a multifocal disease, has a high reoccurrence rate and metastasis for regional lymph nodes. The treatment is multidisciplinary and surgery must involve large resections with margins, when feasible, while amputation can be employed in some cases.