Isse Hamdi Mohamed, Sereke Senai Goitom, Erem Geoffrey
Department of Radiology and Radiotherapy, School of Medicine, Makerere University College of Health Sciences, Kampala, Uganda.
Department of Radiology, Nsambya Hospital, Kampala, Uganda.
Int Med Case Rep J. 2022 Aug 29;15:457-462. doi: 10.2147/IMCRJ.S363320. eCollection 2022.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor that involves the skin with a low metastatic rate; however, this tumor has a tendency to recur locally. Myxoid DFSP is a rare variant, with a few cases mentioned in the literature, and may present diagnostic difficulties on histopathological examination.
We present a 9-year-old male with a history of a left scrotal mass for 3 years. An enhanced computed tomography (CT) scan of the pelvis showed a heterogeneously enhancing mass in the scrotum with extension into both inguinal regions. Histology showed spindle-shaped cells forming a storiform pattern in the hypercellular area and myxoid stroma with prominent thin-walled vessels. Immunohistochemically, the cluster of differentiation 34 (CD34) was positive. The mass was removed by surgical excision with negative resection edges, and then adjuvant radiotherapy of 60 Gy was given. The patient had a good outcome with treatment and was doing well on follow-up.
This variant of sarcoma is rare and often presents a diagnostic challenge. Early detection is crucial to prevent both undertreatment and overtreatment.
隆突性皮肤纤维肉瘤（DFSP）是一种累及皮肤的罕见肿瘤，转移率低；然而，该肿瘤有局部复发的倾向。黏液样DFSP是一种罕见的变异型，文献中仅有少数病例报道，在组织病理学检查中可能存在诊断困难。
我们报告一名9岁男性，有左侧阴囊肿物3年病史。骨盆增强计算机断层扫描（CT）显示阴囊内有一不均匀强化肿物，延伸至双侧腹股沟区。组织学检查显示，在细胞丰富区域，梭形细胞形成车辐状结构，伴有黏液样间质和明显的薄壁血管。免疫组织化学检查显示，分化簇34（CD34）呈阳性。通过手术切除肿物，切缘阴性，随后给予60 Gy的辅助放疗。患者治疗效果良好，随访情况良好。
这种肉瘤变异型罕见，常带来诊断挑战。早期检测对于防止治疗不足和过度治疗至关重要。
