Devaney K, Goodman Z D, Ishak K G
Department of Hepatic and Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, D.C.
Am J Surg Pathol. 1994 Nov;18(11):1078-91.
Fifty-two hepatobiliary cystadenomas and 18 hepatobiliary cystadenocarcinomas were drawn from the files of the Armed Forces Institute of Pathology and Rhode Island Hospital and studied in an attempt to correlate light microscopic features of the tumors with immunohistochemical and follow-up data. The cystadenoma patients ranged in age from 2 to 87 years at the time of initial diagnosis (mean, 45 years). All the cystadenomas were multilocular with benign cuboidal to columnar epithelium, and 44 (85%) had densely cellular spindle cell ("ovarian-like") stromata; 96% were female. Fifty-one cystadenomas were macrocystic lesions, typically lined by mucinous epithelium; one of the benign lesions was a serous cystadenoma (microcystic adenoma) reminiscent of the more commonly encountered pancreatic lesion of the same name. The cystadenocarcinoma patients ranged in age from 24 to 90 years at the time of first diagnosis (mean, 59 years); eight patients (44%) were male. All but one of the lesions were multilocular with malignant in situ (one case) or invasive tubulopapillary (15 cases), solid (one case), or adenosquamous (one case) epithelial components. Areas of preexisting benign cystadenoma were found in six (33%), an observation suggesting that benign lesions may evolve into malignant ones in some patients. Most cystadenomas and cystadenocarcinomas arose in the liver, a few in the extrahepatic biliary system (including the gallbladder). On follow-up, the cystadenoma patients in general were successfully treated by surgical excision of the lesions in toto; patients treated by subtotal resection often had persistent symptomatic disease. Four cystadenocarcinoma patients died of their tumors; another two patients were alive with persistent disease at last follow-up. In both the benign and the malignant lesions, most tumor cells were positive on immunohistochemical staining with antibodies to cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen; scattered chromogranin-positive cells also appeared in a few tumors of both types. Immunohistochemistry did not yield a diagnostic immunoprofile to distinguish cystadenoma from cystadenocarcinoma or from other epithelial lesions arising within the abdominal cavity. At least two types of cystadenocarcinoma exist, one developing exclusively in female patients, usually accompanied by an "ovarian-like" stroma, which follows an indolent course; and the other, lacking the distinctive cellular stroma, seen in males, follows a more aggressive course and is more likely to result in the patient's death from tumor. It remains an open question whether the cystadenocarcinomas lacking a mesenchymal stroma, which arise in women, will follow the same aggressive course as similar lesions arising in men.
从武装部队病理研究所和罗德岛医院的档案中选取了52例肝胆囊腺瘤和18例肝胆囊腺癌进行研究，旨在将肿瘤的光镜特征与免疫组化及随访数据相关联。囊腺瘤患者初次诊断时年龄为2至87岁（平均45岁）。所有囊腺瘤均为多房性，上皮为良性立方形至柱状，44例（85%）有密集细胞性梭形细胞（“卵巢样”）间质；96%为女性。51例囊腺瘤为大囊性病变，通常内衬黏液上皮；其中1例良性病变为浆液性囊腺瘤（微囊性腺瘤），类似于更常见的同名胰腺病变。囊腺癌患者初次诊断时年龄为24至90岁（平均59岁）；8例（44%）为男性。除1例病变外，其余均为多房性，有原位恶性（1例）或浸润性小管乳头状（15例）、实性（1例）或腺鳞癌（1例）上皮成分。6例（33%）发现有先前存在的良性囊腺瘤区域，这一观察结果提示在某些患者中良性病变可能演变为恶性病变。大多数囊腺瘤和囊腺癌发生于肝脏，少数发生于肝外胆管系统（包括胆囊）。随访发现，囊腺瘤患者总体上通过手术完整切除病变得到成功治疗；接受次全切除的患者常有持续性症状性疾病。4例囊腺癌患者死于肿瘤；另外2例患者在最后一次随访时仍有持续性疾病存活。在良性和恶性病变中，大多数肿瘤细胞对细胞角蛋白、上皮膜抗原和癌胚抗原抗体的免疫组化染色呈阳性；少数两种类型的肿瘤中也出现散在的嗜铬粒蛋白阳性细胞。免疫组化未产生可区分囊腺瘤与囊腺癌或腹腔内其他上皮性病变的诊断性免疫表型。至少存在两种类型的囊腺癌，一种仅发生于女性患者，通常伴有“卵巢样”间质，病程进展缓慢；另一种缺乏独特的细胞性间质，见于男性，病程更具侵袭性，更可能导致患者死于肿瘤。女性发生的缺乏间充质间质的囊腺癌是否会与男性发生的类似病变有相同的侵袭性病程仍是一个悬而未决的问题。
