Successful treatment of gallbladder carcinoma producing alpha-fetoprotein with segmental adenomyomatosis

The successful treatment of hepatoid adenocarcinoma of the gallbladder with elevated serum alpha-fetoprotein (1243 ng/ml) and segmental adenomyomatosis in a 58-year-old woman is described. The woman had alpha-fetoprotein (AFP)-producing carcinoma of the gallbladder with regional lymph node metastasis and was treated by extended radical resection and postoperative adjuvant chemotherapy. She is alive, showing normal serum AFP concentration and no recurrence, 57 months after surgery. The tumor cells were stained immunohistochemically for AFP by the peroxidase anti-peroxidase method. Serum AFP reactivity to concanavalin A and lentil lectin was similar to the pattern shown in hepatocellular carcinoma. Only a few cases of AFP-producing gallbladder carcinoma have been reported and there have been no reports of long-term survivors. The combination of aggressive radical resection and chemotherapy seems to have been effective for achieving long-term survival without liver metastasis.     

Carcinosarcoma of the gallbladder producing α-fetoprotein and manifesting as leukocytosis with elevated serum granulocyte colony-stimulating factor: Report of a case

A 69-year-old man was referred to our hospital for investigation of leukocytosis and a persistent fever of 38°C, but we could find no evidence of a specific infection. The leukocyte count was 18 000/mm³, and the serum granulocyte colony-stimulating factor (G-CSF) and α-fetoprotein (AFP) levels were both elevated, at 66.3 pg/ml and 1,495 ng/ml, respectively. Computed tomography (CT) showed a gallbladder tumor and we performed extended cholecystectomy. Postoperatively, the fever subsided and the leukocyte count, serum G-CSF and AFP level normalized. Histologically, the tumor was a carcinosarcoma of the gallbladder. Immunohistochemical staining of the tumor cells was positive for AFP, but negative for G-CSF. This is the first report of a carcinosarcoma of the gallbladder producing AFP. The laboratory findings and clinical course strongly suggested that the tumor produced not only AFP, but also G-CSF.     

肝癌并门静脉癌栓的外科治疗

目的:探讨术中连续门静脉灌注化疗治疗原发性肝癌并门静脉癌栓的治疗效果。方法:将38例肝癌并门静脉癌栓患者随机分为治疗组19例和对照组19例，治疗组术中实施连续门静脉灌注化疗+肝癌切除+癌栓取除术，对照组术中仅行肝癌切除+癌栓取除术。术后两组均行门静脉和/或肝动脉置管化疗。结果:A组与B组术后7 d和30 d的AFP阴性率间明显高于B组(P>0.05)，A组1，3年复发率显著低于B组(P<0.05)，1，3年生存率明显高于B组(P<0.05)。结论:术中连续门静脉灌注化疗+肝癌切除+癌栓取出术+术后门静脉和/或肝动脉置管化疗是治疗肝癌伴门静脉癌栓的一种效果较满意的方法。     

Adeno-endocrine cell carcinoma of the gallbladder

We encountered a rare case of adeno-endocrine cell carcinoma of the gallbladder in an 81-year-old woman. Imaging study revealed a common bile duct stone. Endoscopic sphincterotomy was performed, and the stone was extracted successfully. Thereafter, cholecystectomy was performed. A papillary tumor was found in the neck of the gallbladder. Histologically, the tumor consisted of two components, well differentiated adenocarcinoma and endocrine cell carcinoma. However, no clinical signs of tumor hormonal activity were observed. The tumor cells in the area of the endocrine cell carcinoma were small and round. Histochemical studies of these tumor cells were positive for chromogranin A and Grimelius silver impregnation. The tumor cells in the area of the adenocarcinoma were well differentiated adenocarcinoma and included goblet-type cells with a tubular structure or solid growth pattern. These adenocarcinoma cells stained positively for Alcian blue and periodic acid-Schiff, and both types of tumor cells stained positively for carbohydrate antigen and carcinoembryonic antigen. It was suggested that the histogenesis of the endocrine cell carcinoma of the gallbladder was closely related to that of the adenocarcinoma. Received for publication on May 20, 1999; accepted on Sept. 1, 1999     

Alpha-Fetoprotein-Producing Early Gastric Cancer of the Remnant Stomach: Report of a Case

A 67-year-old man initially underwent a distal gastrectomy for early gastric cancer (T1, N0, M0; Stage IA) in March 1995. During the follow-up period, an elevation of the serum α-fetoprotein (AFP) level (98.8 ng/ml) and a liver tumor (S4) were detected. A left hepatectomy was performed in December 1996. Immunohistochemically, AFP-positive cells were present in both the primary gastric tumor and metastasized liver tumor. The serum AFP level normalized immediately, but it elevated again to 22.4 ng/ml. An endoscopic examination revealed a protruding lesion in the remnant stomach. A total resection of the remnant stomach was performed in February 2005. The tumor was evaluated T1, N0, M0; Stage IA, with positive staining for AFP. The patient has survived without any sign of recurrence for more than 11 years after the first diagnosis of cancer. To the best of our knowledge, this is the first case of a long-term survival of AFP-producing gastric cancer with successfully resected metachronous liver metastasis and gastric remnant carcinoma.     

肿瘤标记物联合检测对胆囊良恶性病变的诊断价值

目的：观察胆囊癌患者血清肿瘤标记物的表达,分析血清肿瘤标记物联合检测在胆囊癌诊断中的作用。方法：实验组50例胆囊癌患者,采用酶联免疫吸附法检测血清AFP和CEA的表达,用荧光免疫分析法检测血清CA19—9的表达,用放射免疫法检测血清CA50的表达,以同期的40例胆囊息肉作对照,分析其临床诊断价值。结果：四种血清肿瘤标记物中CA50、CA19—9和CEA对胆囊癌诊断有较高价值,平行法联合检测可使诊断的灵敏度提高至88％,系列法联合检测可使特异度提高至95％。结论：血清CAS0、CA19—9和CEA的联合检测对胆囊癌的诊断是有临床价值的,联合检测可以增加诊断的特异度和灵敏度。     

Surgical resection for hepatocellular carcinoma with metastasis to the gallbladder: report of a case

A metastatic tumor of the gallbladder is rare. There have so far been no detailed reports of a resection of the metastasis of hepatocellular carcinoma (HCC) to the gallbladder published in the English literature. This report presents the case of a surgical resection for hepatocellular carcinoma with metastasis to the gallbladder. A 48-year-old woman consulted her primary care physician due to chest discomfort. Tumors in the liver and gallbladder were unexpectedly found and she was thus referred to this hospital. The radiologic studies showed two tumors. A round-shaped tumor, 30 mm in diameter, in the fossa of the gallbladder of the liver protruded into the lumen of the gallbladder, and a lobulated shaped tumor, measuring 13 mm, was in S5 of the liver. Angiography demonstrated that these tumors showed a pattern of early enhancement and washout. An en bloc resection of the gallbladder and the liver surrounding the gallbladder was performed, and the final diagnosis was moderately differentiated HCC in segment S5 with gallbladder metastasis. The patient is currently doing well 2 years after surgery, without any signs of recurrence. This case demonstrated that good clinical outcome could be achieved by performing surgery in HCC patients with metastasis to the gallbladder.     

血清甲胎蛋白阳性胆囊癌的临床病理特征及预后分析

目的 分析血清甲胎蛋白(AFP)阳性胆囊癌患者的临床特点及预后分析.方法 回顾性分析第二军医大学东方肝胆外科医院2003年1月-2013年12月收治的20例血清AFP阳性胆囊癌患者资料(研究组),并以同期收治的140例血清AFP正常胆囊癌作为对照组.对两组胆囊癌患者的临床病理特征和随访资料进行统计学分析.结果 研究组与对照组相比,淋巴结转移明显增多(P=0.001),肝侵犯明显增多(P=0.002),手术根治率明显减低(P=0.001).研究组患者的1年、3年、5年生存率分别为45.0％、20.0％、7.5％,中位生存时间10.93个月.对照组患者的1年、3年、5年生存率分别为68.4％、47.6％、36.2％,中位生存时间为27.06个月.研究组患者的1年、3年、5年生存率以及中位生存时间明显低于对照组患者(P=0.007).单因素分析显示,术前血清AFP、TNM分期、组织分化、意外胆囊癌、术前黄疸、肝侵犯、手术方式、肿瘤部位与预后有关(P＜0.05);多因素分析显示:N分期(HR=1.566,95％ CI:1.090 ～2.250,P=0.015)、手术方式(HR=1.450,95％CI:1.053 ～1.997,P=0.023)是胆囊癌患者的独立预后危险因素(P＜0.05).而术前血清AFP水平并不是影响预后的独立危险因素(P＞0.05).结论 血清AFP阳性胆囊癌更易发生淋巴结转移和肝侵犯,手术根治率降低;但血清AFP水平并不是影响患者预后的独立危险因素.     

Alpha-fetoprotein-producing adenocarcinoma of the ureter.

We report a case of primary ureteral tumor producing alpha-fetoprotein (AFP). Computerized tomography, ultrasonography and endoscopy of the bile duct revealed no obvious tumor in the liver, gallbladder, bile duct, pancreas or ovary. Total nephroureterectomy was performed, and histopathological examination revealed adenocarcinoma of the ureter lined with transitional cell carcinoma in its base. The tumor was stained with immunohistological AFP stain, and the high serum AFP level normalized after resection of the tumor.     

High-grade fetal adenocarcinoma of the lung; report of a case

82-year-old man was admitted with an abnormal shadow on the chest roentgenogram. Computed tomography showed a 2.8 x 2.4 cm solid tumor in S3 of the left lung. Transbronchial lung biopsy revealed adenocarcinoma and a left upper lobectomy (ND2a-1) was performed. The tumor consisted mainly of tall columnar clear cells, and no morules were found. Immunohistochemically, the tumor was positive for alpha-fetoprotein (AFP) and p53. Accordingly, we made the histological diagnosis of high-grade fetal adenocarcinoma of the lung, pT2N0M0, stage IB. The patient was not received adjuvant therapy and has been doing well without any tumor recurrence for 3 months postoperatively.     

Induction chemotherapy and radical resection for primary nonseminomatous mediastinal germ cell tumor (NSGCT); report of a case

A 15-year-old male was admitted to our hospital for treatment of an anterior mediastinal tumor. The tumor was visualized by chest radiography 3 months prior to admission. Computed tomography (CT) revealed a heterogeneous solid tumor located in the anterior mediastinum. Although CT-guided needle biopsy had been performed twice, histologic diagnosis could not be confirmed. We believed this tumor to be nonseminomatous mediastinal germ cell tumor (NSGCT) and started intensive chemotherapy with cisplatin (CDDP) without histologic diagnosis because his serum AFP level was rapidly increasing. After 2 courses of chemotherapy, his serum AFP level returned to the normal range and surgical resection of the tumor with part of right lung was performed. Histopathological examination revealed that the tumor consisted of mature teratoma and yolk sac tumor. He underwent 1 course of chemotherapy post-operatively because a small number of viable cells were histopathologically recognized in the yolk sac component. At the time of writing, the patient is alive without any evidence of recurrence.     

A case of alpha-fetoprotein producing rectal cancer

A case of AFP producing rectal cancer was represented in this paper. The patients, 68 years old man, was admitted because of anal bleeding and preoperative examinations revealed rectal cancer without liver metastasis and hepatocellular carcinoma. Serum AFP measured preoperatively was 2750-3500 ng/ml and CEA was 23-33 ng/ml. The patient underwent amputation of the rectum and there were no abnormal masses in the liver at surgery. After operation serum AFP and CEA levels were normalized. Histology of the rectal tumor showed a tubular adenocarcinoma of moderately differentiated type. In the specimen, AFP producing tumor cells were detected by immunoenzymatic labelling (PAP) methods.     

Primary sclerosing cholangitis in which differential diagnosis from gallbladder carcinoma was difficult

We report a case of localized primary sclerosing cholangitis (PSC) which was difficult to distinguish from gallbladder carcinoma. A 75-year-old woman with elevated serum bilirubin was hospitalized and underwent endoscopic nasobiliary drainage (ENBD). There was no history of diseases such as gallbladder stone, pancreatitis, or ulcerative colitis. Cholangiography through the ENBD tube showed localized stenosis of the common bile duct; the gallbladder could not be seen. Angiography showed no encasement of the hepatic artery. Ultrasonography showed a tumor in the cystic duct, and the tumor had invaded the gallbladder and common bile duct. We diagnosed gallbladder carcinoma on radioimaging, and performed an S4aS5 subsegmentectomy of the liver and resection of the extrahepatic biliary tree. Pathologically, no malignant cells were detected, and fibrosis around bile ducts and infiltration of inflammatory cells into hepatic tissue were found. It is well known that PSC is sometimes difficult to differentially diagnose from cholangiocarcinoma. Our case is of high interest because ultrasonography showed findings suggestive of gallbladder carcinoma. It is therefore necessary to keep the possibility of PSC in mind for the diagnosis and treatment of such localized biliary stenosis.     

A Curative Resection Improves the Postoperative Survival Rate Even in Patients with Advanced Gallbladder Carcinoma

The aim of this study was to evaluate the results of our series of 90 operations for gallbladder carcinoma according to the Japanese Society of Biliary Surgery (JSBS) classification system and to clarify the appropriate surgical strategy for advanced gallbladder carcinoma based on the depth of primary tumor invasion and lymph node metastasis. Generally, only a surgical resection can achieve a prognostic improvement of the advanced gallbladder carcinoma. The survival of patients with this neoplasm depends strictly on the depth of histological primary tumor invasion and lymph node metastasis. A retrospective analysis was conducted on 90 patients from 1990 to 2004 who underwent a surgical resection of gallbladder carcinoma. The factors influencing survival were examined. Thirty-nine patients with palliative treatment (not resected cases), which was diagnosed as T3 or T4 by preoperative imagings, were also included in this study. The significance of the variables for survival was examined by the Kaplan-Meier method and the log-rank test followed by multivariate analyses using Cox's proportional hazard model. Portal invasion, lymph node metastasis, the surgical margin (+ vs. -) and the final curability (fCurA, B vs. C) were all found to be independent prognostic factors in the multivariate analysis. In pT2 gallbladder carcinoma, a better survival was achieved in an aggressive surgical approach, in order of a S4a+S5 hepatic resection, an extended cholecystectomy and a cholecystectomy. In pT3 and pT4, although radical extended surgery did not provide the opportunity for good survival even after lobectomy of the liver, the survival of patients with curative surgery was statistically better than in those without curative surgery. In addition, the nodal involvement of pN1 to pN2 was better than that with pN3. A S4a+S5 hepatectomy, therefore, appears to be adequate for the treatment of pT2 gallbladder carcinoma. Even in patients with pT3 and pT4 gallbladder carcinoma, long-term survival can be expected by an operation with a tumor-free surgical margin. The role of radical surgery, however, is considered to be limited in patients with pN3 lymph node metastasis.     

Alpha-fetoprotein-producing renal cell carcinoma

Alpha-fetoprotein (AFP) is recognized as a tumor marker of yolk sac tumors, liver cancer and some other cancers of the digestive organs. Renal cell carcinoma (RCC) producing AFP is a rare entity. A case of AFP-producing RCC with solitary bone metastasis, but without liver involvement, is reported. The stain specific to AFP proved the presence of AFP in the cytoplasms of more cells of the renal tumors. Additionally, the other published cases are reviewed. These cases indicate that mesoderm-originating malignant tumors such as RCCs can produce AFP in some situations. So, AFP is probably more universal than believed, although it is generally a popular and useful tumor marker for hepatocellular carcinomas and yolk sac tumors.     

A case of renal cell carcinoma producing alpha-fetoprotein

Renal cell carcinoma (RCC) producing alpha-fetoprotein (AFP) is a rare condition with only 11 cases reported in Japan to our knowledge. A 69-year-old man was admitted to our hospital for further examination of an incidental right renal tumor. Laboratory tests showed markedly increased serum level of AFP whereas both HBs antigen and anti-HCV antibody were negative. Computed tomography and magnetic resonance imaging imagings showed a right renal tumor but no tumor in liver, testis or lymph node. We performed right radical nephrectomy. Serum level of AFP declined within the normal range 7 weeks after nephrectomy according to its half-life curve. The tumor specimen was composed mainly of granular cells. Immunohistochemical examination of the tumor cells proved the presence of AFP in the cytoplasm. The possibility of AFP as a tumor marker of renal cell carcinoma in this case was presented.     

A case of a choledochal cyst associated with a lymphatic infiltration of a hyperplastic gallbladder epithelium

A 4-year-old girl with a congenital choledochal cyst (Todani IV-A, Komi type A) underwent a resection of the dilated common bile duct and gallbladder. Histologic studies of the gallbladder showed a general hyperplastic change associated with cribriform proliferation at the gland base of the gallbladder. In this region, clusters of cribriform glands were found within the lymphatic vessels, compatible with lymphatic infiltration of tumor cells. However, careful histologic studies did not reveal any apparent neoplastic changes in the gallbladder and common bile duct, so a final diagnosis of epithelial atypism with reactive hypertrophy was made. The displacements observed in the lymphatics are just an incidental finding in a proliferative process of the hyperplastic gallbladder epithelium. In the follow-up observation for 3 years, the patient is doing well without evidence of tumor recurrence. These results suggest that a mere lymphatic infiltration of hyperplastic gallbladder epithelium should not be directly considered as evidence of carcinogenesis.     

Clinicopathological study of AFP producing gastric cancer--significance of AFP in gastric cancer

We experienced fifteen cases (3.9%) out of 387 with gastric cancer showing elevation of alphafetoprotein (AFP) in serum. In these cases AFP producing cells in primary lesion demonstrated positive for anti-AFP by means of immunohistochemical study. In this report, clinicopathological feature and clinical significance as a tumor marker of gastric cancer were discussed. Gross type showed Borrmann II and III which were located in atrophic area in the stomach. Histological type revealed papillary, moderately differentiated and poorly differentiated adenocarcinoma, all of which showed medullary growth in stroma. In clinical field, liver metastasis was observed in 12(80%) of 15 cases. In these cases, elevation of serum AFP was already observed before the detection of liver metastases by CT or Echo. Immunohistochemical study showed no difference between primary tumor and metastases, and no AFP positive cells were seen in 68 early gastric cancers without elevation of serum AFP.     

肝切除联合经导管化学药物治疗伴有门静脉癌栓的肝癌40例报告

目的 探讨非规则性肝切除及癌栓摘除术联合肝动脉化学药物治疗（化疗）（hepatic artery chemotherapy,HAC)及门静脉化疗（portal vein chemotherapy,PVC)治疗肝细胞癌（hepatocellular carcinoma,HCC)合并门静脉癌栓（portal vein tumor thrombi,PVTT)的价值。方法 总结1987年1月－1996年12月采用非规则性肝切除及癌栓摘除术治疗HCC合并PVTT的患者62例,其中,40例在术后联合HAC和PVC。59例获随访3年。结果 6例在术后3个月内死于肝、肾功能衰竭,56例术后恢复良好。术后1、2、3年的复发率和生存率,在手术后应用HAC和PVC组分别为46％（18／39）、59％（23／39）、79％（31／39）和69％（27／39）、51％（20／39）、31％（12／39）,而在未化疗组分别为80％（16／20）、90％（18／20）、100％（20／20）和30％（6／20）、10％（2／20）、5％（1／20）。结论 非规则性肝切除和癌栓摘除术是HCC合并PVTT有效的治疗方法,术后联合HAC和PVC可降低复发率、提高生存率。