婴儿纤维性错构瘤:一项意大利多中心试验

来源 :世界核心医学期刊文摘(皮肤病学分册) | 被引量 : 0次 | 上传用户:zsj_bj
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Background: Fibrous hamartoma (FH) of infancy is a benign mesenchymal tumor, occurring as a superficial mass. Complete excision is curative. Objective and Methods: The clinical features and treatment results of 18 children with FH are described. Results: Local excision was the most common procedure. Surgery was radical in 10 patients, with microscopic residual disease in 6; all of them are alive with no evidence of disease 2 to 49 months after diagnosis. One patient, treated with a local reexcision for macroscopic residual disease (and chemotherapy for a synchronous desmoid fibromatosis) is well 83 months after diagnosis; the last patient, with a lesion of the labia majora, only underwent biopsy and is doing well, awaiting plastic surgery. Limitations: The results did not reach statistical significance due to difficulties in collecting cases. Conclusions: FH should be treated by complete excision; in our experience a nonradical excision was also able to achieve the cure. An aggressive approach should be avoided, as the overall prognosis is excellent. Objective: Methods: The clinical features and treatment results of children with FH are described. Results: Local excision was the Most common procedure. Surgery was radical in 10 patients, with microscopic residual disease in 6; all of them are alive with no evidence of disease 2 to 49 months after diagnosis. One patient, treated with a local reexcision for macroscopic residual disease (and chemotherapy for a synchronous desmoid fibromatosis) is well 83 months after diagnosis; the last patient, with a lesion of the labia majora, only underwent biopsy and is doing, awaiting plastic surgery. Limitations: The results did not reach statistical significance due to difficulties in collecting cases. Conclusions: FH should be treated by complete excision; in our experience a nonradical excision was also able to achieve the cure. An aggressive approach should be avoided, as the overall prognosis is excellent.
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