Dermatofibrosarcoma protuberans (DFSP) is a rare disease in infants and is classified as an intermediate-grade malignancy. The characteristics of the tumor are limited metastasis, slow local but aggressive growth, tendency to recur locally, and rarely a depressed lesion. We report on the case of a 12-year-old boy who developed DFSP on his left chest at five years of age. The patient was suspected to have angioma or lymphangioma and has been followed up at the outpatient clinic in our hospital. Over a period of seven years, the tumor increased in size and showed both protuberant lesions and depressed lesions. We suspected malignant soft tissue tumor, such as sarcoma, on the basis of ultrasonography and magnetic resonance imaging (MRI) findings. The tumor was excised, and its histopathological analysis indicated benign tumor with spindle cells. However, the permanent specimen demonstrated DFSP. We then excised the residual tumor including a wider margin. We found a rare type of DFSP with a depressed epidermis. The boy was surgically treated. DFSP did not recur nor metastasize in the two years of follow up.
"},{"@language":"ja","@value":"隆起性皮膚線維肉腫（Dermatofibrosarcoma protuberans；以下DFSP）は小児ではまれな疾患であり，中間悪性群に分類される．遠隔転移は少ないとされているが，緩徐に進行し，しばしば局所再発することが知られている．我々は，5歳時に病変に気づかれ，12歳で診断に至った左前胸部DFSPの症例を経験した．患者は血管腫もしくはリンパ管腫疑いと診断され経過観察されていた．初診より7年目に，腫瘍は急速に増大し，隆起性部位と陥凹部位を認めた．超音波，MRI検査を行った結果，悪性腫瘍が否定できず，腫瘍切除を施行した．病理診断では紡錘形細胞を伴う良性腫瘍が疑われたが，永久標本ではDFSPと診断されたため，残存腫瘍の追加広範切除術を行った．陥凹病変を合併しているDFSPはまれであるとされている．腫瘤の大きさの変化ならびに外観の変化に気づいてからの対応が早かったことが適切な治療へと結びついたと考えている．完全切除後2年経過しているが，再発，転移は認められていない．
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