加深印象 — 食管、胃炎性肌纤维母细胞瘤1例

2022-08-21 华夏影像诊断中心 华夏影像诊断中心

食管、胃炎性肌纤维母细胞瘤病理及病例分析。

1 病例简介

患儿女,11岁,1年半前偶然出现进食后梗阻感,偶有呕吐,无反酸、嗳气,无胸骨后烧灼感,无明显诱因,未行任何治疗,之后进食后梗阻感逐渐加重,且症状持续无缓解,近来上述症状明显加重,行上消化道气钡双对比造影检查,胸透时在胃泡气体衬托下见胃底近贲门侧软组织肿块影,其内见一团不规则钙化(图A);吞钡后食管下段近贲门处见腔内充盈缺损,管腔明显环行狭窄,钡剂通过受阻,胃底部及胃体小弯侧见不规则充盈缺损,局部胃壁僵硬,黏膜面不光整,可见多个小龛影(图B)。

图A.透视点片胃泡影内见胃底内侧软组织肿块影(箭),其内见一团不规则钙化(箭头); B.气钡双对比造影示食管下段、胃底及胃体小弯侧见不规则充盈缺损,黏膜面不光整(箭);

初步诊断:贲门区占位累及食管下段、胃底胃体小弯侧,考虑错构瘤可能,不除外恶性肿瘤,建议进一步检查。CT检查示食管下段食管壁及贲门区胃壁明显不均匀增厚,食管腔狭窄;胃底部见一团高密度影,未见肿瘤向壁外明显生长,周围未见明显肿大淋巴结(图C)。

图C. CT平扫示食管下段食管壁及贲门区胃壁明显不均匀增厚(箭),胃底部见一团高密度影(箭头);

胃镜检查示:食管距门齿30 cm处见结节状占位,表面糜烂,周围管壁略僵硬,管腔狭窄,内镜不能通过。活组织病理检查示食管黏膜组织肌纤维母细胞增生。术中见食管下段-胃底部肿瘤,大小约8 cm×5 cm×4 cm,肿瘤已侵及食管下段肌层及胃底、部分胃体浆肌层,食管腔内见肿瘤几近阻塞食管,食管及胃底、部分胃体黏膜表面糜烂、坏死并存。术后病理镜下见:瘤组织主要由梭形细胞构成,大部分区域细胞排列紧密,少部分呈黏液样,瘤组织间可见散在或局灶性炎细胞浸润(图1D);诊断:食管下段及胃底、胃体炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)。

图D.病理检查见瘤组织主要由排列紧密的梭形细胞(箭)构成,其间可见散在或局灶性炎症细胞(箭头)浸润(HE,×200)

免疫组化示:Cladesmin(-),desmin(-),SMA(+++),ALK(-),CD117(-),S-100(-),P53(-),actin(+),Vim(+),Ki-67指数10%,支持上述诊断。

2 讨论

IMT是一种可发生于多种组织的间叶性肿瘤,曾称为炎性假瘤,WHO软组织肿瘤新分类中建议将其归为纤维母细胞或肌纤维母细胞肿瘤,正式命名为炎性肌纤维母细胞瘤,定义为“由分化的肌纤维母细胞性梭形细胞组成的,常伴有大量浆细胞和(或)淋巴细胞的一种肿瘤”。IMT是一种少见而独特的间叶性肿瘤,常有低度恶性的表现或交界性肿瘤的特点,多见于肺,发生于食管、胃者少见,影像学表现多为个案报道。IMT发病年龄跨度大,几乎可发生于各个年龄段,但以青少年多见,平均发病年龄为10岁,女性稍多于男性;也有报道以中老年多见。

本例患儿11岁,与前者相符。消化道IMT好发于食管下段及贲门部,钡餐检查时向腔内生长者可见不规则充盈缺损,向壁外生长者黏膜面可光整,也可呈腔壁环形增厚。CT及MRI可初步判断良恶性程度,良性多呈边界光滑、境界清楚的单发软组织肿块,有中等程度均匀强化;恶性程度高者可表现为不规则肿块,可呈分叶状,肿块密度不均,较大肿块显示中心低密度坏死,部分可以出现钙化,肿块富含血管,强化形式多样,常有中度或明显不均匀强化,病变还可有包绕或侵蚀邻近组织等恶性肿瘤倾向。

本例患儿消化道钡剂造影见食管下段向心性狭窄,胃底、胃体小弯侧不规则充盈缺损,黏膜面不光整,但无贲门癌等恶性肿瘤在瘤体较大时常见的癌性溃疡所表现的不规则龛影;也无黏膜抬起、黏膜通过瘤体表面的桥形黏膜征等黏膜下肿瘤(如间质瘤、平滑肌瘤)的表现,结合透视时胃底部见一团钙化,考虑错构瘤的可能,不除外恶性肿瘤。在活检、术后病理诊断为IMT后,发现本例临床表现和影像学征象与IMT低度恶性的表现和交界性肿瘤的特点相符,提高了对IMT的认识。总之,在影像学检查中消化道出现兼具良恶性征象的肿瘤时可考虑IMT的可能,最终确诊有赖于病理及免疫组化检查。

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    2022-10-21 okhuali
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    2021-11-07 zhouqu_8
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