Altal Omar F, Aleshawi Abdelwahab J, Tashtush Nour A, Alhowary Ala'a
Department of Obstetrics and Gynecology, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Am J Case Rep. 2019 Nov 14;20:1675-1678. doi: 10.12659/AJCR.919488.
BACKGROUND Desmoplastic small round cell tumor (DSRCT) is a rare soft tissue sarcoma that usually arises in the abdomen or pelvis in young boys and adolescents. Presenting symptoms include abdominal pain and ascites. However, DSRCT is often disseminated throughout the peritoneal cavity at diagnosis, and the prognosis is poor. This report is of a case of DSRCT in a 23-year-old Jordanian woman who presented with abdominal pain. CASE REPORT An unmarried 23-year-old woman presented with abdominal pain. On examination, she was found to have ascites. A computed tomography (CT) scan of the abdomen and pelvis showed a complex cystic mass in the left ovary, multiple peritoneal deposits, a large amount of ascitic fluid, two hypodense lesions in the liver, and multiple enlarged lymph nodes. Diagnostic laparoscopy was performed, and multiple tumor biopsies were obtained. Histopathology showed a cellular tumor composed nests of small round cells embedded in desmoplastic stroma. Immunohistochemistry showed positive staining of the tumor cells for pan-cytokeratin, desmin, Wilms tumor 1 (WT1) antigen, epithelial membrane antigen (EMA), and CD56, which supported the diagnosis of DSRCT. After the second cycle of the P6 Protocol, which included seven courses of chemotherapy, the patient developed a severe and fatal infection. CONCLUSIONS It is important to consider the diagnosis of DSRCT that may present atypically, particularly in patients who present with abdominal and pelvic masses. DSRCT has a rapid and aggressive course that requires early and definitive diagnosis with prompt treatment that includes systemic chemotherapy.
背景 促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见的软组织肉瘤，通常发生于年轻男性和青少年的腹部或盆腔。临床表现为腹痛和腹水。然而，DSRCT在诊断时常常已播散至整个腹腔，预后较差。本文报道一例23岁约旦女性DSRCT患者，以腹痛为主要表现。病例报告 一名23岁未婚女性因腹痛就诊。检查发现有腹水。腹部和盆腔计算机断层扫描（CT）显示左卵巢有一复杂囊性肿物、多处腹膜种植、大量腹水、肝脏两处低密度病变以及多处肿大淋巴结。进行了诊断性腹腔镜检查并获取了多处肿瘤活检组织。组织病理学显示肿瘤细胞呈巢状排列，由嵌入促结缔组织增生性基质的小圆细胞构成。免疫组织化学显示肿瘤细胞对泛细胞角蛋白、结蛋白、威尔姆斯瘤1（WT1）抗原、上皮膜抗原（EMA）和CD56呈阳性染色，支持DSRCT的诊断。在包括七个疗程化疗的P6方案第二个周期后，患者发生严重致命感染。结论 必须考虑到DSRCT可能有非典型表现，尤其是对于出现腹部和盆腔肿物的患者。DSRCT病程进展迅速且侵袭性强，需要早期明确诊断并及时进行包括全身化疗在内的治疗。
