15例鼻腔鼻窦横纹肌肉瘤的临床分析

目的 探讨鼻腔鼻窦横纹肌肉瘤(RMS)的临床特征、治疗方式及预后的影响因素。方法 回顾性分析2012年1月—2022年1月收治的15例鼻腔鼻窦RMS患者资料。15例RMS患者多为青少年,胚胎型12例,腺泡型3例。其中单纯化疗1例,鼻内镜手术+术后化疗3例,鼻内镜手术+术后放化疗7例,术前诱导化疗+鼻内镜手术+术后放化疗4例。应用SPSS 26.0软件进行描述性统计分析,用Kaplan-Meier法评估患者的临床特征、病理类型、肿瘤分期、治疗方式等因素与临床预后的关系。结果 随访3～113个月。治疗后6例无复发,4例局部复发,5例全身转移。8例生存,7例死亡。1年生存率约为93.3%,5年生存率约为43.6%。病理类型、淋巴结转移、远处转移、治疗方式及美国RMS研究组(IRS)分期是影响RMS预后的危险因素(P<0.05)。结论 鼻腔鼻窦RMS临床罕见,易侵及眼眶、颅底,具有高复发率及死亡率。多学科联合诊疗是目前有效的治疗方式,手术联合辅助放化疗可降低复发率、改善预后。提高对该病的临床及病理认识,进行分子学及基因组学研究,寻找新的治疗靶点,有望提高患者的生活质量及生存率。     

肝未分化胚胎性肉瘤的CT及MRI表现

目的 探讨肝未分化胚胎性肉瘤(UESL)的螺旋CT及高场强MR的影像学表现,提高UESL诊断准确率.方法 回顾性分析14例经手术病理证实的UESL患者.螺旋CT检查5例,MR检查9例.结果 5例患者CT扫描均表现为平扫边界清晰的囊状低密度影,其内含有不规则的软组织影,增强后动脉期软组织强化,门脉期及延迟期继续强化.MRI 9例患者中6例T1WI表现为囊状高低信号混杂影,T2WI病灶以高信号为主.增强后动脉期病灶边缘强化,门脉期及延迟期继续强化;3例患者T1WI表现为囊状低信号影,T2WI呈高信号.增强后动脉期病灶边缘强化,门脉期及延迟期继续强化.结论 平扫和动态增强螺旋CT及高场强MRI能反应UESL的影像学特点,提高了UESL诊断准确率.     

CONGENITAL SPINDLE CELL RHABDOMYOSARCOMA OF THE URINARY BLADDER

Spindle cell rhabdomyosarcoma is a recently described variant of embryonal rhabdomyosarcoma that carries a relatively favorable prognosis when compared with other types of rhabdomyosarcoma. We report the first case of a congenital spindle cell rhabdomyosarcoma in the urinary bladder of a newborn. The patient was diagnosed with obstructive uropathy and cystic kidneys in the antenatal period. The tumor was composed primarily of mature strap-shaped rhabdomyoblasts with cross-striations; it involved focally the full thickness of the bladder wall. No extension into adjacent organs or distant metastases were identified. The patient died of complications of urinary tract obstruction shortly after birth.     

重组人干扰素α1b抗肠道病毒71型的作用机制研究

目的 研究重组人干扰素α1b(IFN-α1b)体外对肠道病毒71型(EV71)复制的抑制作用,并初步探讨其抗病毒机制.方法 测定IFN-α1b对RD细胞的毒性和IFN-α1b在EV71感染前后给药对EV71感染所致的RD细胞病变的抑制作用,检测IFN-α1b对EV71 RNA和VP蛋白表达量以及病毒复制的影响,并通过构建瞬时表达干扰素诱导的跨膜蛋白3(IFITM3)的RD细胞探索IFN-α1b 通过促进IFITM3的表达,抑制EV71侵入的作用机制.结果 在EV71感染前12h和感染后1h给药,IFN-α1b 抑制EV71细胞病变的IC50值分别为258.53IU/ml和2113.58IU/ml,选择指数SI分别为>16497和>3271,提示IFN-α1b具有明显的抗EV71活性,且在EV71感染前给药效果更明显.机制研究显示,与对照组相比,IFN-α1b可显著抑制EV71的RNA复制、蛋白合成和子代病毒释放,且可能通过对IFITM3 的诱导表达阻止EV71侵入.结论 IFN-α1b具有抗EV71活性,可通过影响病毒生命周期的侵入、复制、装配和释放过程达到抗病毒作用.     

Rhabdomyosarcoma of the breast: Report of a rare malignancy

Rhabdomyosarcoma (RMS) is an uncommon type of soft-tissue malignancy which mainly influences children. RMS rarely occurs in breast and little of the clinical behavior and treatment strategies were reported. Here, we describe a case of adult female patient with breast RMS. A 34-years-old Chinese woman visited to our hospital complaining palpable mass in her left breast. Seven months ago, the patient was diagnosed as lymphocytic mastitis and received surgical excision in the left breast. Five months later, she noticed a palpable mass again in the left breast. Ultrasonography suspected a malignant lesion and a diagnosis of RMS was made after segmental mastectomy and immunohistochemical staining. Therapy consisted of mastectomy and following neoadjuvant chemotherapy. The patient has remained disease free 30 months post-operatively. We should consider RMS when we see a breast mass. Tissue biopsy and immunohistochemical staining are recommended for diagnosis of RMS in young women. Oncologists should take immediate and active treatment on RMS.     

Rhabdomyosarcoma of nasopharynx

Rhabdomyosarcoma is a highly invasive malignant tumour arising from the mesenchymal tissue. Here, we reported a rare case of embryonal rhadomyosarcoma of nasopharynx with extension to different spaces of base of skull and orbit. Rarity of the case difficulty in diagnosis encourages its reporting.     

Spindle cell rhabdomyosarcoma of the retroperitoneum: an unusual case developed in a pregnant woman but obscured by pregnancy

Spindle cell rhabdomyosarcoma (RMS) is an uncommon histiologic variant of RMS that has spindle cell morphology. This tumor occurs almost exclusively in childhood and more rarely in adults. Only a few adult cases, including two retroperitoneal cases in male patients, have been documented previously. We describe a rare case of spindle cell RMS of the retroperitoneum in a 37-year-old woman developed during pregnancy and incidentally discovered after vaginal delivery. Computed tomography showed a huge tumor mass, measured 20 × 20 × 15 cm in size, arising in retroperitoneal space. Histologically, the tumor consisted of spindle cells arranged in a fascicular or herringbone growth pattern, morphologically mimicking adult fibrosarcoma, intermingled with scattered rhabdomyoblasts. Mitotic activity ranged from 20 to 28 mitoses per 10 high-power fields and tumor necrosis was evident. Immunohistochemically, tumor cells were stained diffusely positive for muscle specific actin, desmin, and vimentin, scattered positive for myogenin, MyoD1 and myoglobin, with a Ki-67 (MIB-1) proliferative labeling index of 46.11%. This tumor also stains positively for CD99, strong cytoplasmic WT1, and nuclear p53. Other markers such as S100 protein, smooth muscle specific actin, CD34, cytokeratin, and epithelial membrane antigen were all negative in the tumor cells. On the basis of the findings, a spindle cell RMS was diagnosed. The neoplasm was incompletely excised because of encasement of major vessels and invasion to adjacent structures, and additional chemotherapy was given.