Zhou Xunjian, Duan Zhi, Tao Ting, Li Zhen, Wang Ning, Xu Qimei, Wei Meiyan, Zhong Zheng, Liu Ran, Yin Qinghua, Xiong Lixin, Chen Hui
Department of Pathology, The Affiliated Changsha Hospital of Xiangya School of Medicine, Central South University, Changsha, Hunan, China.
Department of Infection and Immunity, The Affiliated Changsha Hospital of Xiangya School of Medicine, Central South University, Changsha, Hunan, China.
Front Oncol. 2023 Aug 18;13:1230021. doi: 10.3389/fonc.2023.1230021. eCollection 2023.
Malignant rhabdoid tumors (MRTs) are rare tumors with high mortality rates and poor prognoses. MRTs occur mainly in the central nervous system, kidneys, and soft tissues, but rarely in the omentum. MRTs occur more commonly in infants and children and less frequently in adults. Here, we report the first observed case of MRT in an adult omentum. A 35-year-old man with abdominal distension and pain was admitted to the emergency department. Previously, several hospitals considered patients with cirrhosis who had not received active treatment. Computed tomography and magnetic resonance imaging revealed diffuse omental thickening and massive ascites. The surgery was performed at our hospital, and the pathological diagnosis was MRT with a SMARCB1(INI-1) deletion. Postoperatively, his symptoms improved, and he underwent five cycles of chemotherapy. However, 6 months after surgery, the tumor developed liver metastases, and the patient subsequently died. Primary MRT of the greater omentum is rare, and its pathological diagnosis usually requires extensive clinicopathological evaluation of various differential diagnoses and an appropriate work-up to exclude other malignancies associated with SMARCB1 deletion. At the same time, the lack of specific signs of omental MRT and its rapid progression should alert clinicians.
恶性横纹肌样瘤(MRTs)是一种罕见的肿瘤,死亡率高且预后不良。MRTs主要发生于中枢神经系统、肾脏和软组织,而发生于大网膜者罕见。MRTs多见于婴幼儿和儿童,成人少见。在此,我们报告首例发生于成人大网膜的MRT病例。一名35岁腹胀、腹痛男性入住急诊科。此前,多家医院考虑为未接受积极治疗的肝硬化患者。计算机断层扫描和磁共振成像显示大网膜弥漫性增厚和大量腹水。在我院进行了手术,病理诊断为伴有SMARCB1(INI-1)缺失的MRT。术后,他的症状有所改善,并接受了5个周期的化疗。然而,术后6个月,肿瘤发生肝转移,患者随后死亡。原发性大网膜MRT罕见,其病理诊断通常需要对各种鉴别诊断进行广泛的临床病理评估,并进行适当的检查以排除与SMARCB1缺失相关的其他恶性肿瘤。同时,大网膜MRT缺乏特异性体征及其快速进展应引起临床医生的警惕。