PDF(Pubmed)
Abstract：
Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy predominantly affecting adolescents and young adults. We report a case of multifocal DSRCT in an 11-year-old male who presented with complaints of unilateral forehead swelling, proptosis, and ophthalmoplegia for four months along with abdominal pain and dysphagia for six months. A whole-body computed tomography revealed widespread lesions in the skull, orbit, thorax, and abdomen with local infiltration. Ultrasound-guided biopsy of the forehead lump was performed. Based on histopathological and immunohistochemical investigations, it was diagnosed to be a DSRCT with multifocal presentation. The patient underwent chemo-radiation but unfortunately succumbed to neutropenic sepsis and renal failure. DSRCT is a very rare, highly aggressive malignancy with an extremely poor prognosis. Orbital presentations are even rarer, with less than 10 such cases currently described in English medical literature.
摘要：
促纤维增生性小圆细胞瘤(DSRCT)是一种罕见的,高度侵袭性恶性肿瘤主要影响青少年和年轻人。我们报告了一名11岁男性的多灶性DSRCT病例，该患者主诉单侧前额肿胀，突增,和眼肌麻痹四个月，以及腹痛和吞咽困难六个月。全身计算机断层扫描显示颅骨广泛病变，轨道,胸部,和腹部局部浸润。对前额肿块进行超声引导活检。根据组织病理学和免疫组织化学研究，诊断为DSRCT多灶性表现.患者接受了化疗放疗，但不幸的是死于中性粒细胞减少性败血症和肾衰竭。DSRCT是一种非常罕见的，高度侵袭性恶性肿瘤，预后极差。轨道演示更罕见，目前在英国医学文献中描述的此类病例不到10例。
