腹盆腔促结缔组织增生性小圆细胞瘤磁共振成像检查的影像学特征

MRI features of desmoplastic small round cell tumor of the abdomen and pelvis

  • 摘要:
    目的 探讨腹盆腔促结缔组织增生性小圆细胞瘤(DSRCT)磁共振成像(MRI)检查的影像学特征。
    方法 采用回顾性描述性研究方法。收集2008年1月至2022年6月乐清市人民医院收治3例和温州市人民医院收治5例腹盆腔DSRCT患者的临床病理资料;男5例,女3例;年龄为(43±5)岁。患者均行MRI平扫+增强扫描检查。观察指标:(1)腹盆腔DSRCT影像学特征。(2)腹盆腔DSRCT治疗与病理学检查特征。(3)随访情况。正态分布的计量资料以x±s表示,偏态分布的计量资料以M(范围)表示。计数资料以绝对数表示。
    结果 (1)腹盆腔DSRCT影像学特征。①肿瘤部位:8例患者中,肿瘤分别位于右季肋区肝下缘、右髂区回盲部内侧、右髂区盲肠内侧、左季肋区胃胰间隙、左髂区肠系膜间隙、盆腔膀胱右侧各1例,左季肋区腹膜后间隙2例。②肿瘤大小:8例患者有13个病灶,瘤体最大径为9.1(3.5~20.0)cm;其中单发肿瘤5例,多发肿瘤3例。③肿瘤形状与界限:8例患者中,4例肿瘤呈膨胀性生长,4例肿瘤呈侵袭性生长;5例瘤内坏死囊变,4例瘤内出血,4例瘤内见斑点状钙化,3例瘤周渗出改变(同1例患者可合并多种影像学表现)。④MRI平扫检查影像学特征:8例患者中,MRI平扫检查T1加权成像呈均匀低信号4例,低信号中夹杂斑点状高信号(伴出血) 4例;T2加权成像(T2WI)呈均匀高信号3例,边缘高信号中央斑片状及斑点状更高信号5例;T2WI脂肪抑制序列呈高、等、低混杂信号5例,呈高、低信号3例;弥散加权成像呈均匀高信号3例,呈高、等、低混杂信号5例。⑤MRI增强检查影像学特征:8例患者MRI检查增强后均呈不均匀强化,其中2例动脉期实质部分显著强化,门静脉期持续强化,延迟期强化程度略降低;4例动脉期实质部分中度强化,门静脉期持续强化,延迟期强化缓慢退出;2例动脉期实质部分轻度强化,门静脉期持续强化,延迟期强化缓慢退出。8例患者中,3例肿瘤呈环形强化,瘤内见条索状或网格状强化,3例瘤周见血管增多增粗。⑥肿瘤侵犯周围组织与转移:8例患者中,肿瘤侵犯肠管4例,侵犯周围组织2例,侵犯左肾、脾脏及胰尾1例,侵犯左输尿管下段1例。8例患者中,腹腔、腹膜后及腹股沟淋巴结肿大5例,腹膜多发结节状增厚及腹水4例,肝、肺转移2例,肋骨、股骨及骶骨转移1例(同1例患者可合并多处肿瘤转移)。(2)腹盆腔DSRCT治疗与病理学检查特征。8例患者中,3例肿瘤界限清晰行完整切除术;3例肿瘤与周围血管紧密粘连行部分肿瘤切除术;2例腹膜、网膜、肠系膜及周围肠管广泛转移仅取肿瘤组织行病理学检查。8例患者经显微镜检查、电镜检查、免疫组织化学染色检测、细胞遗传学检测结果确诊为DSRCT。(3)随访情况。8例患者均获得术后随访,随访期间均死亡。
    结论 腹盆腔DSRCT MRI检查特征为单发或多发分叶状肿瘤,界限不清晰,常侵犯网膜、系膜、腹膜及邻近组织;信号混杂,增强扫描检查呈不均匀轻中度强化。

     

    Abstract:
    Objective To investigate the magnetic resonance imaging (MRI) features of desmoplastic small round cell tumor (DSRCT) of the abdomen and pelvis.
    Method The retrospec-tive and descriptive study was conducted. The clinicopathological data of 8 patients with DSRCT of the abdomen and pelvis, including 3 cases admitted in Yueqing People's Hospital and 5 cases admitted in Wenzhou People's Hospital, from January 2008 to June 2022 were collected. There were 5 males and 3 females, aged (43±5)years. All patients underwent MRI plain and enhanced scanning. Observa-tion indicators: (1) imaging features of DSRCT of the abdomen and pelvis; (2) treatment and pathological examination characteristics of DSRCT of the abdomen and pelvis; (3) follow-up. Measurement data with normal distribution were represented as Mean±SD, and measurement data with skewed distri-bution were represented as M(range). Count data were described as absolute numbers.
    Results (1) Imaging features of DSRCT of the abdomen and pelvis. ① Tumor location. Of the 8 patients, there were 6 cases with tumors located respectively at the lower edge of the liver in the right quarter costal region, the medial side of the ileocecal region in the right iliac region, the medial side of the caecum in the right iliac region, the gastro-pancreatic space in the left quarter costal region, the mesenteric space in the left iliac region and the right side of pelvic bladder, and 2 cases with tumors located at retroperitoneal space of left quarter rib region. ② Tumor size. There were 13 lesions in the 8 patients, and the maximum diameter of tumor was 9.1 (range, 3.5‒20.0)cm. Of the 8 patients, there were 5 cases with single tumor and 3 cases with multiple tumors. ③ Tumor shape and boundary. Of the 8 patients, there were 4 cases with tumor in expansive growth and 4 cases with tumor in invasive growth. There were 5 cases with tumor of intratumoral necrosis and cystic degene-ration, 4 cases with tumor of intratumoral hemorrhage, 4 cases with tumor of intratumoral spot calcification, 3 cases with tumor of peritumoral tissue exudation. One patient may combined with multiple imaging manifestations. ④ Imaging characteristics of MRI plain scanning. Of the 8 patients, there were 4 cases with tumor of homogeneous hypointensity signal and 4 cases with tumor of hypointensity mixed with speckled hyperintensity (with hemorrhage) in T1 weighted imaging of MRI plain scanning. There were 3 cases with tumor of homogeneous hyperintensity and 5 cases with tumor of high signal at the edge, patchy and spot-shaped in the center in T2 weighted imaging of MRI plain scanning. There were 5 cases with tumor of high, equal and low confounding signals and 3 cases with tumor of high and low signals in T2 weighted imaging fat suppression sequence of MRI plain scanning. There were 3 cases with tumor of uniform high signals and 5 cases with tumor of high, equal and low mixed signals in diffusion weighted imaging of MRI plain scanning. ⑤ Imaging characteristics of MRI enhanced scanning. All 8 patients had tumor of heterogeneous enhancement in MRI enhanced scanning, including 2 cases with significant enhancement in arterial phase, continuous enhancement in portal phase, slightly reduced enhancement in delayed phase, 4 cases with moderate enhancement in arterial phase, continuous enhancement in portal phase, slowly exited enhancement in delayed phase, 2 cases with mild enhancement in arterial phase, continuous enhancement in portal phase, slowly exited enhancement in delayed phase. Of the 8 patients, there were 3 cases with tumor of annular enhancement with intratumoral strip or grid signals and 3 cases with tumor of peritumoral blood vessels increased and thickened signals. ⑥ Tumor invasion and metastasis. Of the 8 patients, there were 4 cases with tumor invaded bowel, 2 cases with tumor invaded surrounding tissues, 1 case with tumor invaded left kidney, spleen and pancreatic tail, 1 case with tumor invaded distal of left ureter. There were 5 cases with abdominal, retroperitoneal and inguinal lymph nodes enlargement, 4 cases with multiple nodular thickening of peritoneum and ascites, 2 cases with tumor liver and lung metastasis and 1 case with tumor rib, femur and sacrum metastasis. One patient may combined with multiple tumor metastasis. (2) Treatment and patholo-gical examination characteristics of DSRCT of the abdomen and pelvis. Of the 8 patients, 3 patients underwent complete resection as clear tumor boundary, 3 patients underwent tumor partial resection as tight adhesion between tumor and surrounding blood vessels, 2 cases underwent tumor tissue pathological examination as extensive metastasis of peritoneum, omentum, mesentery and surrounding intestine. All 8 patients were diagnosed as DSRCT by microscopic examination, electron microscopic examination, immunohistochemical staining and cytogenetic examination. (3) Follow-up. All 8 patients underwent postoperative follow-up and died during the follow-up.
    Conclusion MRI features of abdominal and pelvic DSRCT include single or multiple lobulated masses with unclear boundaries, invading the omentum, mesentery, peritoneum and adjacent tissues in most cases, mixed signals and heterogeneous mild to moderate enhancement in enhanced scanning.

     

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