B Del Frari1, B G Zelger, H Piza-Katzer. 1. Universitätsklinik für Plastische und Wiederherstellungschirurgie Innsbruck, Osterreich. Del-Frari@uibk.ac.at
Abstract
BACKGROUND: The epithelioid sarcoma of the hand is often misdiagnosed. This tumor occurs most frequently in children and young adults, has an uncharacteristic course, and carries a high risk for local recurrence and metastases. The characteristic histopathology is helpful in establishing the correct diagnosis. Differential diagnosis includes synovialoma, fibromatosis and Dupuytren's contracture. Multinodular appearance is not uncommon. PATIENTS AND METHOD: Three young patients (one male and two female) were operated on the hand for treatment of Dupuytren's contracture, synovialoma and fibromas, respectively. The necessity for surgical revision in these patients led to a reevaluation of the primary histological data. The original diagnosis having been found to be incorrect, the correct diagnosis was established namely, epithelioid sarcoma. RESULTS: Surgical revision was performed, which involved partial amputation of the extremity. None of the patients underwent adjuvant radio- or chemotherapy. The patients were followed-up for ten, four and two and a half years, respectively. They are currently free of recurrences. CONCLUSION: Prognosis depends on the size and the location of the tumor. Adequate treatment requires early radical excision; amputation may be required if the primary tumor is located in the fingers or treatment of recurrent growth. The tumor tends to metastasize via the lymphatic system. Patients must be monitored with yearly lung X-rays since recurrence or metastasis may occur many years after the initial diagnosis and treatment.
BACKGROUND: The epithelioid sarcoma of the hand is often misdiagnosed. This tumor occurs most frequently in children and young adults, has an uncharacteristic course, and carries a high risk for local recurrence and metastases. The characteristic histopathology is helpful in establishing the correct diagnosis. Differential diagnosis includes synovialoma, fibromatosis and Dupuytren's contracture. Multinodular appearance is not uncommon. PATIENTS AND METHOD: Three young patients (one male and two female) were operated on the hand for treatment of Dupuytren's contracture, synovialoma and fibromas, respectively. The necessity for surgical revision in these patients led to a reevaluation of the primary histological data. The original diagnosis having been found to be incorrect, the correct diagnosis was established namely, epithelioid sarcoma. RESULTS: Surgical revision was performed, which involved partial amputation of the extremity. None of the patients underwent adjuvant radio- or chemotherapy. The patients were followed-up for ten, four and two and a half years, respectively. They are currently free of recurrences. CONCLUSION: Prognosis depends on the size and the location of the tumor. Adequate treatment requires early radical excision; amputation may be required if the primary tumor is located in the fingers or treatment of recurrent growth. The tumor tends to metastasize via the lymphatic system. Patients must be monitored with yearly lung X-rays since recurrence or metastasis may occur many years after the initial diagnosis and treatment.