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35例胰腺间变性癌的临床病理及免疫组化研究并文献复习

A clinicopathologic and immunohistochemical study of 35 anaplastic carcinomas of the pancreas with a review of the literature.

作者信息

Paal E, Thompson L D, Frommelt R A, Przygodzki R M, Heffess C S

机构信息

Department of Endocrine and Otorhinolaryngic-Head & Neck Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.

出版信息

Ann Diagn Pathol. 2001 Jun;5(3):129-40. doi: 10.1053/adpa.2001.25404.

Abstract

Anaplastic pancreatic carcinomas are rare tumors, frequently displaying a variety of growth patterns. The literature lacks a comprehensive study of this tumor. Thirty-five cases of anaplastic carcinoma of the pancreas diagnosed between 1955 and 1997 were retrieved from the Endocrine Registry at the Armed Forces Institute of Pathology. Histology, immunophenotype, molecular analysis, and patient follow-up were analyzed. The tumors of 10 women and 25 men, aged 34 to 85 years (mean age at presentation, 62.5 years), were studied. Patients had vague symptoms (weight loss, pain, and fatigue, nausea, or vomiting), lasting an average of 13.2 weeks. The tumors, of an average size of 9.2 cm, were usually in the head or tail of the pancreas. The tumors were widely infiltrative, histomorphologically separated into predominantly large, pleomorphic cell, or spindle cell groups. Tumor phagocytosis and necrosis were noted. Immunohistochemical studies confirmed an epithelial origin with at least one epithelial marker in 78% of the tumors. K-ras mutations by sequence analysis were found in eight of 12 cases tested. Surgical biopsy/excision was used in all patients. Twenty-nine of 35 patients died of disease (average, 5.2 months), three died with no evidence of disease (average, 56.9 months), and three patients were alive at last follow-up (average, 94.0 months), one with residual disease. There was no statistically significant difference in survival between patients with and without a K-ras mutation. Anaplastic carcinoma of the pancreas usually occurs in the head of the pancreas in older men. The epithelial nature of the pleomorphic cells (giant or spindled) can usually be documented. Patients with K-ras mutations have a shorter survival time, even though the overall prognosis for all anaplastic carcinomas is fatal (93% fatality; average survival, 448 days). Ann Diagn Pathol 5: 129-140, 2001. This is a US government work. There are no restriction on its use.

摘要

间变性胰腺癌是罕见肿瘤,常呈现多种生长模式。文献中缺乏对该肿瘤的全面研究。从武装部队病理研究所内分泌登记处检索出1955年至1997年间诊断的35例胰腺间变性癌病例。对组织学、免疫表型、分子分析及患者随访情况进行了分析。研究了10名女性和25名男性的肿瘤,年龄在34至85岁之间(就诊时平均年龄62.5岁)。患者有模糊症状(体重减轻、疼痛、乏力、恶心或呕吐),平均持续13.2周。肿瘤平均大小为9.2 cm,通常位于胰头或胰尾。肿瘤具有广泛浸润性,组织形态学上主要分为大细胞、多形性细胞或梭形细胞组。可见肿瘤吞噬和坏死。免疫组化研究证实78%的肿瘤起源于上皮,至少有一种上皮标志物阳性。在检测的12例病例中,8例通过序列分析发现K-ras突变。所有患者均接受了手术活检/切除。35例患者中,29例死于疾病(平均5.2个月),3例死于无疾病证据(平均56.9个月),3例患者在最后一次随访时存活(平均94.0个月),1例有残留疾病。有K-ras突变和无K-ras突变患者的生存率无统计学显著差异。胰腺间变性癌通常发生于老年男性的胰头。多形性细胞(巨细胞或梭形细胞)的上皮性质通常可得到证实。有K-ras突变的患者生存时间较短,尽管所有间变性癌的总体预后均为致命(死亡率93%;平均生存期448天)。《诊断病理学杂志》5: 129 - 140, 2001年。本文为美国政府作品。其使用无限制。

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