Dermatofibrosarcoma Protuberans in a 12-Year-Old Child: A Rare Case.

IF 1.9 4区 医学 Q3 DERMATOLOGY
Clinical, Cosmetic and Investigational Dermatology Pub Date : 2024-08-27 eCollection Date: 2024-01-01 DOI:10.2147/CCID.S480616
Eva Krishna Sutedja, Endang Sutedja, Kartika Ruchiatan, Yogi Faldian, Yuri Yogya, Risa Miliawati Nurul Hidayah, Rafithia Anandita, Yohana Azhar, Anglita Yantisetiasti, Bethy Suryawathy Hernowo, Yovan Rivanzah
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引用次数: 0

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor involving the dermis and subcutaneous fat that rarely occurs in children, manifested as a slowly growing firm plaque on the trunk. A 12-year-old girl patient presented with dark patch on the nasal root after finishing 25 sessions of radiotherapy. Initially, patient came to Oncology Surgery Clinic at Hasan Sadikin General Hospital Bandung with the chief complaint of a large exophytic mass located in the nasal area, which was neither itchy nor painful. A large, firm, painless mass with no sign of localized heat or redness was found on physical examination. There were no palpable cervical or axillary lymph nodes. Wide local excision and frontal flap procedure were performed by Oncology Surgery Department leaving a pedicle with 2×1.5×1 cm on size was observed. Upon histopathological examination, tumor mass was found in the subepithelium and consisted of oval to spindle-shaped cells that were hyperplastic, compacted, diffuse, forming fasciculus, whorled, and cartwheel. Cell nuclei were pleomorphic (oval to wavy), hyperchromatic, with clear nucleolus, and occasion mitotic figures. Hyalinisation was seen between the tumor masses. On immunohistochemical stains, there were diffuse positivity for epithelial membrane antigen (EMA) and vimentin. Based on the histological and immunohistochemical findings, the diagnosis of stage II DFSP was made. Until now, there is no established algorithm for treatment of DFSP. Wide local excision and radiotherapy for 25 sessions was performed on this patient, resulting in complete tumor mass removal. After three months of observation, the second surgery was done to remove a pedicle; however, there is no recurrence of tumor growth. Despite its rarity, DFSP should be considered as a differential diagnosis to avoid underdiagnosis or misdiagnosis.

一名 12 岁儿童的皮肤纤维肉瘤(Dermatofibrosarcoma Protuberans):罕见病例。
皮纤维肉瘤(DFSP)是一种不常见的软组织肿瘤,累及真皮和皮下脂肪,很少发生在儿童身上,表现为躯干上缓慢生长的坚实斑块。一名12岁的女孩在完成25次放疗后,鼻根部出现黑斑。患者最初来到万隆哈桑-萨迪金综合医院肿瘤外科门诊就诊,主诉是鼻腔部位有一个巨大的外生肿块,既不痒也不痛。体格检查发现肿块巨大、坚实、无痛,局部无发热或发红迹象。未触及颈部或腋窝淋巴结。肿瘤外科对其进行了大范围局部切除和额部皮瓣手术,术中观察到一个 2×1.5×1 厘米大小的蒂。组织病理学检查发现,肿瘤肿块位于上皮下,由椭圆形至纺锤形细胞组成,这些细胞增生、密集、弥漫,形成束状、轮状和车轮状。细胞核呈多形性(椭圆形至波浪形),高色素,核仁清晰,偶有有丝分裂。瘤块之间可见透明化。免疫组化染色显示,上皮膜抗原(EMA)和波形蛋白呈弥漫阳性。根据组织学和免疫组化结果,诊断为 DFSP II 期。迄今为止,DFSP 的治疗还没有既定的算法。该患者接受了广泛的局部切除术,并接受了 25 次放疗,肿瘤块被完全切除。经过三个月的观察,患者接受了第二次手术,切除了肿瘤蒂,但肿瘤没有复发。尽管 DFSP 非常罕见,但仍应将其作为鉴别诊断,以避免漏诊或误诊。
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来源期刊
CiteScore
2.80
自引率
4.30%
发文量
353
审稿时长
16 weeks
期刊介绍: Clinical, Cosmetic and Investigational Dermatology is an international, peer-reviewed, open access journal that focuses on the latest clinical and experimental research in all aspects of skin disease and cosmetic interventions. Normal and pathological processes in skin development and aging, their modification and treatment, as well as basic research into histology of dermal and dermal structures that provide clinical insights and potential treatment options are key topics for the journal. Patient satisfaction, preference, quality of life, compliance, persistence and their role in developing new management options to optimize outcomes for target conditions constitute major areas of interest. The journal is characterized by the rapid reporting of clinical studies, reviews and original research in skin research and skin care. All areas of dermatology will be covered; contributions will be welcomed from all clinicians and basic science researchers globally.
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