2001 CTOS Annual Meeting Posters— Surgery

EPITHELIOID SARCOMA: CLINICAL BEHAVIOUR OF A RARE SOFT TISSUE SARCOMA
Dario Baratti,  Alessandro Gronchi,  Paolo Casali,  Laura Lozza,  Elisabetta Pennacchioli,  Rossella Bertulli,  Palma Dileo,  Mario Santinami
Istituto Nazionale per la Cura e lo Studio dei Tumori

METHODS: Twenty-nine consecutive patients with ES were observed at our institution from June 1985 to June 2001. Twenty-two were male and 7 female; median age at the time of first diagnosis was 29 years (range 9-66). Site of first presentation was the hand in 12 cases, the upper limb in 5, the foot in 2, the lower limb in 6 and the trunk in 4. Fourteen out of 29 patients referred after a median of 3 consecutive relapses (range 1-5), developing multifocal recurrences in 4 cases, loco-regional nodal involvement in 2 and distant metastasis in 1. Two patients underwent amputative surgery before our observation. Primary treatment of choice performed in our department was wide en-bloc excision, with the aim to obtain adequate margin of uninvolved tissue in all directions, and to spare limbs and limbs function as much as possible. Adjuvant RT was administered to high risk patients in a postoperative fashion.

RESULTS: ES presented as small superficial nodules in 18 cases and this feature was prevalent in distal localization (11/14), while deep-seated larger masses were more common in the proximal extremities or in the trunk (8/15). Surgical treatments performed were: wide excision in 15 patients, re-excision in 8, digit or ray amputation in 3, major amputation in 3. Surgical margins were rated as wide in 16 patients and marginal in 5. No residual tumor was found in any patient who underwent re-excision. Adjuvant therapies were the following: postoperative RT in 8 patients, preoperative limb perfusion in 5, preoperative conventional CT in 4 and postoperative CT in 1. Median follow-up of the series was 32 months (range 3-181). Actuarial overall survival at five years was 76.5%; disease free survival at the same interval was 55.8%. Following treatment at our Institution, from 1 to 4 consecutive relapses (median 2) occurred in 12 patients. Recurrence involved loco-regional lymph nodes in five more patients, thus overall lymph nodal involvement rate was 7 out of 29 (24.1%). Distant metastases were detected at the time of first presentation in one case, developed as first recurrence in two and was subsequent to nodal involvement in six. Most common site of distant metastatization was the lung. All the patients with metastatic disease are presently died. Four more amputations were needed to treat local recurrence, thus a total number of 12 such procedures were performed in 8 patients. Relapse occurred in 3 out of 15 (20%) patients treated by primitive neoplasm and in 9 out 14 (64.3%) treated for recurrent tumor.

CONCLUSION: . Epithelioid sarcoma is a rare neoplasm, accounting for less than 1% (29/3376) of all the STS observed during the period of the study. The present study showed a great propensity to recur repeatedly and an high rate of nodal involvement was confirmed. Lack of local control leaded to an increase in amputation rate. Adequacy of treatment was the most relevant prognostic factor, since patients referred for recurrent disease, after repeated attempts of local control, had a worse outcome than patients that underwent primary treatment in our Department.