Wenig B M, Albores-Saavedra J, Buetow P C, Heffess C S
Department of Otolaryngic and Endocrine Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000, U.S.A.
Am J Surg Pathol. 1997 Jan;21(1):70-80. doi: 10.1097/00000478-199701000-00008.
Three patients with pancreatic mucinous cystic neoplasms having a sarcomatous stroma are reported. The tumors occurred in two women and one man, aged 48, 66, and 67 years, respectively. Symptoms included epigastric pain or a palpable abdominal mass or both. Radiographically, all the tumors were large, multicystic, and located in the tail of the pancreas. Histologically, the cystic component was lined by mucin-producing, columnar to cuboidal epithelium composed of benign to atypical to overtly malignant cells (cystadenocarcinoma). Immunohistochemistry showed the epithelial component of all three tumors to be positive with cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, and the pancreatic epithelial mucin antibodies CA 19-9 and DUPAN 2. In all three cases, an undifferentiated malignant spindle cell (sarcomatous) stroma was intimately associated with the epithelial component. The sarcomatous component showed variable reactivity with vimentin (all three cases), muscle-specific and smooth-muscle actin (all three cases), S-100 protein (1/3), Leu-7 (one case), and estrogen and progesterone receptors (in two cases). Two patients died with widespread abdominal disease within 15 months of diagnosis, one of whom had an omental metastasis entirely composed of the sarcomatous stroma. The third patient was alive and free of disease at 16 months after diagnosis. Our findings document the existence of a sarcomatous component in pancreatic mucinous cystic neoplasms; this component appears to be responsible for the highly malignant behavior of the tumors.
本文报告了3例伴有肉瘤样间质的胰腺黏液性囊性肿瘤患者。这些肿瘤分别发生于2名女性和1名男性，年龄分别为48岁、66岁和67岁。症状包括上腹部疼痛或可触及的腹部肿块，或两者皆有。影像学检查显示，所有肿瘤均较大，为多囊性，位于胰尾。组织学检查显示，囊性成分内衬有产生黏液的柱状至立方上皮，由良性至非典型至明显恶性的细胞组成（囊腺癌）。免疫组化显示，所有3例肿瘤的上皮成分对细胞角蛋白、上皮膜抗原、癌胚抗原以及胰腺上皮黏液抗体CA 19-9和DUPAN 2均呈阳性。在所有3例病例中，未分化的恶性梭形细胞（肉瘤样）间质与上皮成分紧密相连。肉瘤样成分对波形蛋白（所有3例）、肌肉特异性和平滑肌肌动蛋白（所有3例）、S-100蛋白（1/3）、Leu-7（1例）以及雌激素和孕激素受体（2例）显示出不同的反应性。2例患者在诊断后15个月内死于广泛的腹部疾病，其中1例患者的网膜转移完全由肉瘤样间质组成。第3例患者在诊断后16个月时存活且无疾病。我们的研究结果证明了胰腺黏液性囊性肿瘤中存在肉瘤样成分；该成分似乎是肿瘤高度恶性行为的原因。
