UNLABELLED: Primary solitary plasmacytoma and Ewing's sarcoma of the chest wall are relatively uncommon tumors, and data concerning treatment and results are sparse. To assess the results of therapy we reviewed our 40-year experience. METHODS: Records of 24 patients with solitary plasmacytoma and 62 with Ewing's sarcoma arising in the chest wall who were admitted to our institution from 1949 to 1989 were reviewed. RESULTS: In the group with plasmacytoma (n = 24), ages ranged from 35 to 75 years (median 59 years); male/female ratio was 2.4:1. The presenting complaint was pain or mass or both in 92% (22/24). Primary therapy was local only in 5 (resection in 3, radiotherapy in 2), chemotherapy in 16 (resection in 5, radiotherapy in 10, and chemotherapy alone in 1); 3 patients did not receive therapy. Multiple myeloma developed subsequently in 75% (18/24). Overall 5-year survival was 38% (median 56 months). Age, sex, site of primary tumor, and local therapy did not significantly impact on survival. Ages in the patients who had Ewing's sarcoma (n = 62) ranged from 2 to 39 years (median 16 years); male/female ratio was 1.6:1. Presenting complaint was pain or mass or both in 98% (61/62). Primary therapy was local in 17 (resection in 7, radiotherapy in 7, resection plus radiotherapy in 3) and chemotherapy in 45 (plus resection in 29, resection and radiotherapy in 10, and radiation therapy alone in 3). Overall 5-year survival was 48% (median 57 months). Age, sex, and site of primary tumor did not significantly impact on survival. Patients in whom distant metastases developed (n = 48) had a significantly decreased survival (5 year, 28%) compared with those who did not have metastases (n = 14; 5 year, 100%). CONCLUSION: Plasmacytoma of the chest wall, even if solitary at presentation, should be considered a systemic disease, and therapy should be directed as such. For Ewing's sarcoma, although resection or radiotherapy may offer local control, because of the prevalence of distant metastases (77%), systemic therapy should be considered an integral part of treatment.
UNLABELLED: Primary solitary plasmacytoma and Ewing's sarcoma of the chest wall are relatively uncommon tumors, and data concerning treatment and results are sparse. To assess the results of therapy we reviewed our 40-year experience. METHODS: Records of 24 patients with solitary plasmacytoma and 62 with Ewing's sarcoma arising in the chest wall who were admitted to our institution from 1949 to 1989 were reviewed. RESULTS: In the group with plasmacytoma (n = 24), ages ranged from 35 to 75 years (median 59 years); male/female ratio was 2.4:1. The presenting complaint was pain or mass or both in 92% (22/24). Primary therapy was local only in 5 (resection in 3, radiotherapy in 2), chemotherapy in 16 (resection in 5, radiotherapy in 10, and chemotherapy alone in 1); 3 patients did not receive therapy. Multiple myeloma developed subsequently in 75% (18/24). Overall 5-year survival was 38% (median 56 months). Age, sex, site of primary tumor, and local therapy did not significantly impact on survival. Ages in the patients who had Ewing's sarcoma (n = 62) ranged from 2 to 39 years (median 16 years); male/female ratio was 1.6:1. Presenting complaint was pain or mass or both in 98% (61/62). Primary therapy was local in 17 (resection in 7, radiotherapy in 7, resection plus radiotherapy in 3) and chemotherapy in 45 (plus resection in 29, resection and radiotherapy in 10, and radiation therapy alone in 3). Overall 5-year survival was 48% (median 57 months). Age, sex, and site of primary tumor did not significantly impact on survival. Patients in whom distant metastases developed (n = 48) had a significantly decreased survival (5 year, 28%) compared with those who did not have metastases (n = 14; 5 year, 100%). CONCLUSION:Plasmacytoma of the chest wall, even if solitary at presentation, should be considered a systemic disease, and therapy should be directed as such. For Ewing's sarcoma, although resection or radiotherapy may offer local control, because of the prevalence of distant metastases (77%), systemic therapy should be considered an integral part of treatment.
Authors: Samir Karmali; André Barros; Barbara Rosa; Pedro Campos; Ricardo Gonçalves; Daniel Sá da Costa; Dolores López-Presa Journal: Rare Tumors Date: 2016-04-06