Noguchi Aya, Imoto Hirofumi, Yoshida Hiroshi, Asano Shigeyuki, Unno Michiaki, Shinya Fumiaki
Department of Surgery, Iwaki City Medical Center, 16 Kuzehara, Uchigou Mimaya-machi, Iwaki City, Fukushima, 973-8555, Japan; Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.
Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.
Int J Surg Case Rep. 2021 Feb;79:386-389. doi: 10.1016/j.ijscr.2021.01.081. Epub 2021 Jan 22.
Malignant rhabdoid tumours (MRTs) were first described as an infrequent variant of Wilms' tumour and have been reported in several organs. The small intestine is a rare site for MRTs.
A 70-year-old man presented with appetite loss and melena. Haemorrhagic small intestinal tumours, swollen mesenteric and paraaortic lymph nodes, a tumour in the left kidney, and multiple tumours in the lung were found. He underwent partial resection of two haemorrhagic small intestinal tumours classified as MRTs based on the results of a pathological examination. However, melena appeared again on postoperative day 6. We performed another operation and resected approximately 180 cm of the small intestine that contained multiple tumours. All lesions were classified as MRTs. Unfortunately, melena appeared again 4 days after the second operation. He did not want invasive therapy and died from massive melena 2 months after the initial surgery.
MRTs of the small intestine are uncommon and have an extremely poor prognosis. Although curative resection is an important treatment, cases of metastasis at diagnosis and postoperative early recurrence have been observed, as was the case for the patient described herein. In these cases, effective systemic therapy is necessary. Recently, tumour suppressor genes were shown to be involved in the occurrence of MRT, and new therapies for MRT have been studied.
We herein conclude effective systemic therapy is necessary for MRTs with multiple organ involvement. The development of new drugs for this disease is ongoing.
恶性横纹肌样瘤（MRTs）最初被描述为Wilms瘤的一种罕见变体，已在多个器官中被报道。小肠是MRTs的罕见发病部位。
一名70岁男性出现食欲减退和黑便。发现有出血性小肠肿瘤、肠系膜和腹主动脉旁淋巴结肿大、左肾肿瘤以及肺部多发肿瘤。基于病理检查结果，对两个出血性小肠肿瘤进行了部分切除，这两个肿瘤被分类为MRTs。然而，术后第6天再次出现黑便。我们又进行了一次手术，切除了约180厘米包含多个肿瘤的小肠。所有病变均被分类为MRTs。不幸的是，第二次手术后4天又出现了黑便。他不希望接受侵入性治疗，在初次手术后2个月死于大量黑便。
小肠MRTs并不常见，预后极差。尽管根治性切除是重要的治疗方法，但如本文所述患者的情况，在诊断时出现转移和术后早期复发的病例已被观察到。在这些病例中，有效的全身治疗是必要的。最近，肿瘤抑制基因被证明与MRT的发生有关，并且已经对MRT的新疗法进行了研究。
我们在此得出结论，对于多器官受累的MRTs，有效的全身治疗是必要的。针对这种疾病的新药研发正在进行中。
