原发胰腺囊性肿瘤少见,Remine于1987年根据文献报道统计共计500余例,其中囊腺瘤为少数,胰腺囊性肿瘤(cystic neoplasms of pancreas)由胰腺导管上皮发生。病理上胰腺囊性肿瘤进一步分为两大组:①微小囊性腺瘤(浆液性囊腺瘤);②大囊性肿瘤(黏液囊性肿瘤和囊腺癌),包括黏液囊腺瘤和囊腺癌。这种分类对临床和病理十分重要。
微小囊性腺瘤是常见的胰腺囊腺肿瘤,可发生于胰腺各个部位,肿瘤为良性,无恶变趋向。肿瘤直径一般<2cm,边界清楚,内有许多小囊,囊液为无色清亮的液体,富含糖原,呈多房性小囊,偶尔肿瘤中心有瘢痕形成。大小为1~12mm,平均5mm大小,肿瘤边缘常规则光滑,囊内壁可见壁结节。约有20%患者合并有肝、肾及中枢神经系统的囊肿。
大囊性肿瘤起源于胰管上皮,具有高度潜在恶性,瘤体愈大,癌的可能性也愈大。50%患者年龄在40~60岁,肿瘤多数位于胰体尾处,直径常超过10cm。少数胰管扩张型可位于钩突。肿瘤一般较大,外有包膜,内有分隔,呈多个小囊,小囊的数目一般少于6~10个,病理上细胞浆内和囊内有大量黏液。
临床上,两类肿瘤均多见于女性,临床无特异症状,一般发病缓慢,可有腹痛、腹泻、乏力等。微小囊腺瘤位于胰头部时,压迫胆总管可出现黄疸,但发生率明显低于胰腺癌。黏液性囊腺瘤体积较大时,于上腹部可扪及包块,质硬而有压痛。
【影像学表现】
1.X线表现 约有10%的病可见上腹部胰腺部位大、中肿块影,伴有不定性粗乱的钙化斑。
2.CT 微小囊性腺瘤由许多小囊和间隔混合组成,一般<2cm,偶尔>2cm,病灶轮廓清楚,增强后扫描可清楚显示囊壁和间隔,囊壁很薄。有时还可见病灶中央和囊壁钙化约占38%,典型者可见囊的中央有纤维瘢痕,呈辐射状或蜂窝状。基于CT表现,相当一部分微小囊腺瘤能与巨囊性腺瘤鉴别。
黏液囊腺瘤或囊腺癌多位于胰体尾部,常为单发,少数多发;在CT上表现为圆形或不规则卵圆形低密度影,边界清楚,病灶较大,直径为3~14cm,一般为10cm左右,肿瘤呈多房,CT可显示肿瘤的壁和间隙,其厚度不一,为2~10cm。有时还可见壁或间隙钙化。少数病例肿瘤呈多囊均匀水样密度,伴有囊壁上乳头状赘生物,增强后扫描可见壁结节强化。囊壁太薄时,CT不易显示,酷似单房假性囊肿。黏液囊性肿瘤若伴有淋巴转移,肝转移的征象有利于恶性肿瘤(囊腺癌)的诊断。另外,肿瘤具有较多的实质部分,且在大囊附近有多个子囊存在,亦常提示为囊腺癌。黏液囊性肿瘤可压迫胃、脾,结肠和小肠使其移位,但一般不侵犯它们(图4-3-18)。
图4-3-18 胰腺小囊腺瘤(病理证实)CT表现
A.平扫CT:胰腺颈部可见类圆形低密度灶,直径1.7cm,CT值约10Hu;B.增强扫描:病灶几乎无强化,正常胰腺实质均匀强化,两者界限清晰
3.MRI 微小囊性腺瘤MRI表现为肿瘤在T1WI像上为低信号,轮廓光滑规则,不侵犯周围脏器;T2WI像上似蜂窝状的高信号,其内多个小囊肿和间隔清晰可见,尤其在单次激励的SE序列T2WI像上,由于是屏气扫描,图像质量更清晰。胰腺囊腺肿瘤的多个小囊性成分聚集在一起,其小囊直径往往<1cm,常常提示为小中型肿瘤。小囊直径在1~2cm者,既可见于小囊性腺瘤,也可见于大囊性腺瘤;如果子囊的直径>2cm往往提示为大囊性腺瘤或囊腺瘤。一般而言,小囊性腺瘤的子囊直径偶尔也可达或超过2.0cm,但相当薄的间隔和没有邻近脏器的侵犯,常是小囊性腺瘤区别于大囊性腺瘤的特征;肿瘤间隔常常有轻度增强,延迟增强扫描中心的瘢痕偶尔可观察到。囊内分隔和壁结节以T2WI和动态增强图像上显示最佳。
大囊性肿瘤,由于黏液产生,故肿瘤在T1WI像上呈混合的高、低信号,T2WI像上均表现为高信号。囊内分隔在T2WI像上可清楚显示,增强后囊壁和分隔可强化,一般T1WI增强像上肿瘤内壁不规则,外壁规则。在影像学上判断良、恶性困难,如果有周围脏器侵犯,提示为恶性。如果出现肝转移,则肯定为恶性。其肝转移病灶也含黏液,故在T2WI像上呈高信号,在T1WI像上可呈混合的高、低信号;或者由于肝转移病灶血供丰富,在增强图上,呈边缘环状强化表现。囊壁不规则,分隔厚而不均匀,出现壁结节,强化较明显者,从影像学上提示恶性可能。由于MRI较CT能更好地显示肿瘤囊性成分的形态和大小,所以MRI更容易鉴别微小囊性肿瘤和(或)大囊性肿瘤,微小囊性腺瘤子囊的直径常不超过2cm,这是鉴别的要点。
乳头状囊性肿瘤(papillary cystic tumor)是胰腺囊性肿瘤中比较罕见的一种类型,肿瘤直径为2.4~17cm,平均10.8cm。肿瘤可呈局部浸润性生长,发生恶变后也少有转移。在MRI上主要表现为囊性肿物内壁上见多个结节,也可见分隔,外壁较光整,尤其在增强图像上,壁结节强化甚明显,提示为乳头状囊性肿瘤(图4-3-19)。
【鉴别诊断】
1.慢性胰腺炎合并假性囊肿 慢性胰腺炎引起的假性囊肿,边缘光滑,中央密度均匀,一般为多房蜂窝状,无瘤结节,胰腺弥漫肿大,炎性浸润至胰周及吉氏筋膜,甚至小网膜囊。胰腺炎不包埋腹腔动脉或肠系膜上动脉,无实性肿块。
2.胰周淋巴结结核 为腹腔淋巴结结核的一部分,胰周淋巴结肿大聚集成块,中央有干酪坏死、液化,形成多灶低密度胰腺肿块,类似胰腺囊性肿瘤,但其位置往往为与胰腺边缘一侧。对比增强后,边缘增强,中央因干酪坏死为低密度,联系结核病史、临床表现、年龄、腹腔及胰周散在淋巴结钙化斑,可以做出诊断。
3.胰腺癌 当胰腺癌出现较大的中央液化、坏死灶时,与黏液囊腺癌鉴别困难。若瘤体实性成分多,壁厚更不均匀,囊变区内无分隔现象以及囊变区内密度混杂不均等征象时,胰腺癌的可能性大。
图4-3-19 乳头状囊腺瘤(病理证实)
A、B.平扫CT:胰头区可见一类圆性囊性密度肿块,边界清晰,可见囊壁,CT值约20Hu,肿块邻近结构明显受压;C、D.增强扫描:胰头部病灶轻度强化,肿块厚壁显示更清晰;E.MPR重建:可详细观察肿瘤与周边关系
4.胰包虫病 典型的CT表现为多囊和囊内分隔,囊内存在子囊,囊周结构强化明显,囊壁可有钙化。结合牧区生活史以及合并的肝、肺包虫囊肿,有助于鉴别诊断。
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