Clinical Characteristics and Treatment Outcomes of Patients with Dermatofibrosarcoma Protuberans Treated with Imatinib at Tikur Anbessa Specialized Hospital:Hospital-based cross-sectional study May-September 2020 G.C.

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Addis Abeba University


Background:DFSP is a rare soft tissue tumor with indolent growth and low probability of distant metastasis. More than 90% of cases are positive for CD34 and are characterized by translocation of chromosome 17 and 22 (t (17; 22)) resulting in the expression of fusion gene-COL1A1-PDGFRb. A TKI, imatinib targets this fusion gene and showed efficacy in treating both locally advanced and metastatic DFSP. Objective:The aim of this study is to describe the clinical characteristics of patients and to evaluate response to imatinib of patients with DFSP treated in Tikur Anbessa Hospital. Methods: We assessed retrospectively 26 patients with DFSP treated with imatinib from 2004-2020 at hematology unit of Tikur Anbessa Hospital, in Addis Ababa. Clinical data was extracted from medical records and patients and analyzed with SPPS version 26. Response to imatinib was evaluated using Response Evaluation Criteria in Solid Tumors (RECIST). Results:18 of the patients were males and 8 were females with a median age of 35 years (range: 22-66). Trunk is the commonest location of primary tumor constituting 61.5%, followed by head and neck. Twelve patients have been treated with imatinib for locally recurrent disease, which was difficult to resect surgically, and 6 (50%) showed partial response, 3 (25%) complete response, 1 (8.33%) patient had stable disease and 2 (16.67%) had disease progression. On the other hand, thirteen patients underwent surgery for locally recurrent disease followed by imatinib therapy for residual disease. Six (6) of these patients had complete response, 3 patients had partial response and 4 patients had progressive disease while on imatinib. One patient had recurrent disease with liver metastasis and he underwent surgery for local recurrence and imatinib was given for liver metastasis which disappeared after 3 months. The overall clinical response rate was 73.1%. The major adverse effects of imatinib were: GI upset in 38.5%, skin depigmentation in 34.4%, anemia in 23%, leukopenia in 23% and edema in 19.2%, which were all transient and self-limiting. Conclusion: DFSP is highly recurrent tumor after surgical resection. We found out that Imatinib is well tolerated and an effective treatment option for locally recurrent and advanced disease that is difficult for complete surgical resection, with over all clinical response of 73.1%.



Dermatofibrosarcoma ,Protuberans , Imatinib,Patients