Acinar Cell Carcinoma of the Pancreas: A Clinicopathologic Study of 28 Cases
Acinar Cell Carcinoma of the Pancreas: A Clinicopathologic Study of 28 Cases
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胰腺腺泡细胞癌28例临床病理分析
DOI：
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发表时间：
1992
影响因子：
5.6
通讯作者：
J. Rosai
中科院分区：
文献类型：
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作者：
D. Klimstra;C. Heffess;J. Oertel;J. Rosai
我们研究了28例胰腺腺泡细胞癌的显微外观、免疫组织化学染色特性和临床行为。其中两个肿瘤发生在儿童身上。成年患者年龄40 ~ 81岁（平均62岁）。男性远远多于女性，而且大多数病人是白人。症状无特异性，黄疸少见。经常报道的由血清脂肪酶水平升高引起的并发症（即关节痛和皮下脂肪坏死）仅出现在16%的患者中。大体而言，肿瘤相对狭窄且肉质，平均为10.8 cm，偶有广泛出血和坏死。显微镜下，肿瘤细胞性很强，缺乏典型的间质。确定了腺泡、实体、小梁和腺状的生长模式；个别肿瘤通常是混合性的。细胞核圆形至卵圆形，多形性最小，核仁单个突出。有丝分裂活性是可变的。一般来说，细胞质丰富，嗜酸性，颗粒状，但许多实体瘤细胞细胞质稀少。90%的病例呈特征性的周期性酸希夫阳性，耐淀粉酶的胞质颗粒，73%的病例呈脂肪酶活性的丁酸酯酶组织化学染色阳性。免疫组化结果显示胰蛋白酶100%阳性，脂肪酶77%阳性，凝乳胰蛋白酶38%阳性，淀粉酶31%阳性。在42%的肿瘤中，有少量的内分泌成分被识别为抗铬粒蛋白或胰岛细胞激素的抗体。超微结构上表现为外分泌特征，极化细胞内可见微绒毛状腔，丰富的粗糙内质网，125- 1000 nm的酶原样颗粒。此外，许多病例显示多形性电子致密颗粒，直径达3500nm，含有纤维状内部结构。88%的病例可获得随访信息。一半的患者在发病时有转移性疾病，另外23%的患者随后发生转移，通常局限于局部淋巴结和肝脏。所有病例的平均生存期为18个月，1年和3年生存率分别为57%和26%。60岁前发病的患者存活时间几乎是老年患者的两倍。分期也影响预后，而肿瘤的组织学亚型和胰腺内的位置与生存仅微弱相关。
We have examined the microscopic appearance, immunohistochemical staining properties, and clinical behavior of 28 cases of acinar cell carcinoma of the pancreas. Two of the tumors occurred in children. The adult patients ranged in age from 40 to 81 years (mean, 62 years). Males greatly outnumbered females, and most of the patients were white. Presenting symptoms were nonspecific, and jaundice was infrequent. The frequently reported complications from increased serum lipase levels (i.e., arthralgias and subcutaneous fat necrosis) were present in only 16% of the patients. Grossly, the tumors were relatively circumscribed and fleshy, averaging 10.8 cm, with occasionally extensive hemorrhage and necrosis. Microscopically, the tumors were very cellular and characteristically lacked a desmoplastic stroma. Acinar, solid, trabecular, and glandular patterns of growth were identified; individual tumors were usually mixed. Nuclei were round to oval, with minimal pleomorphism and single prominent nucleoli. Mitotic activity was variable. In general the cytoplasm was moderately abundant, eosinophilic, and granular, but many of the solid tumors had cells with scanty cytoplasm. Characteristic periodic acid-Schiffpositive, diastase-resistant cytoplasmic granules were demonstrated in >90% of the cases, and the butyrate esterase histochemical stain for lipase activity was positive in 73%. Immunohistochemically, there was positivity for trypsin in 100% of the cases, for lipase in 77%, for chymotrypsin in 38%, and for amylase in 31%. A minor endocrine component was recognized with antibodies against chromogranin or islet cell hormones in 42% of the tumors. Ultrastructurally, exocrine secretory features were present, with polarized cells showing microvillilined lumina, abundant rough endoplasmic reticulum, and 125-1,000-nm zymogen-like granules. In addition, many cases showed pleomorphic electron-dense granules measuring up to 3,500 nm and containing fibrillary internal structures. Follow-up information was available in 88% of the cases. Half of the patients had metastatic disease at presentation and an additional 23% subsequently developed metastases, which were usually restricted to the regional lymph nodes and liver. The mean survival for all cases was 18 months, with 1- and 3-year survivals of 57 and 26%, respectively. Patients presenting before age 60 years survived nearly twice as long as older patients did. Stage also influenced prognosis, whereas the histologic subtype of the tumors and the location within the pancreas correlated only weakly with survival.