Surgical management and outcome of metachronous Krukenberg tumors from gastric cancer

BACKGROUND AND OBJECTIVES: The question of whether resection should be performed in Krukenberg tumors from gastric cancer has yet to be adequately examined. Despite some reports on the surgical treatment of Krukenberg tumors, the outcomes after resection are not well characterized. PATIENTS AND METHODS: Using a gastric cancer database, a total of 34 patients who underwent a resection of metastatic ovarian tumors after curative surgery for gastric cancer were identified. A prospective database of these patients was reviewed for the presentation, clinical features, and outcomes after resection. RESULTS: The median age of 34 patients was 44 years (range, 24-66). The majority of patients was in the premenopausal state and had bilateral ovarian involvement. The most common presenting symptom was an abdominal mass (35.3%). Tumor size ranged from 3.5 to 20 cm with 61.8% measuring larger than 10 cm. In 17 patients who had metastatic disease confined to the pelvis, a complete gross resection (R0) was achieved. In the other 17 with the disease beyond the pelvis gross residual tumors remained after the resection (R1). The median survival of all patients was 11 months (95% confidence interval [CI] 8-14), and that of the patients rendered R0 was 18 months (95% CI, 14-22), in comparison with 9 months (95% CI, 3-15) for those with R1 resection (P = 0.0001; log-rank test). The median progression free survival was also significantly longer for the patients with R0 resection than those with R1 resection (8 months, 95% CI, 5-11 vs. 5 months, 95% CI, 4-6, P = 0.0103). Multivariate analysis identified R0 resection as the only significant factor predictive of survival. CONCLUSIONS: In the management of Krukenberg tumors after gastric cancer, a metastasectomy may significantly improve the overall and progression free survival if it could render a complete gross resection. To define the patient group that benefits most from resection, the extent of disease and resectability must be carefully evaluated before surgery.     

胰腺头部实性假乳头状瘤的手术治疗

目的:观察手术切除术治疗胰腺头部实性假乳头状瘤的临床疗效。方法:回顾性分析2008年11月至2014年7月间,盛京医院普通外科14例因患胰腺头部实性假乳头状瘤而行手术治疗的患者临床资料,女性12例,男性2例,平均年龄36.2岁。8例行胰十二指肠切除术,2例行保留幽门的胰十二指肠切除术,2例行胰腺部分切除术,1例行胰十二指肠切除术联合门静脉切除术,1例行胰腺部分切除术联合胰体尾空肠Roux-en-Y吻合术。评价手术效果,分析影像特点,并随访远期效果。结果:肿瘤平均7.1cm×6.1cm,所有患者术后病理及免疫组化证实胰腺实性假乳头状瘤,平均住院时间26天,有5例发生术后胰瘘,发病率35.7%,无死亡病例。术后随访8~82个月,中位随访时间27.5个月,有 1例于术后66个月复发,其余13例均无复发。结论:手术治疗胰腺头部实性假乳头状瘤是安全的,完整切除肿物远期疗效确切。     

Solid-pseudopapillary tumor of the pancreas (Frantz tumor) in children: report of four cases and review of the literature

BACKGROUND: Solid-pseudopapillary tumor of the pancreas (SPT) is an exceptionally rare neoplasm in children. Its origin remains enigmatic. It is of low malignant potential and occurs most frequently in young females. PATIENTS AND METHODS: A cumulative review of the tumor's clinicopathological characteristics from the world's literature is presented. The clinical course, pathohistologic data and outcome of surgery of four Austrian children treated at the general hospital of Vienna are analyzed. RESULTS: Between 1987 and 1999, four girls (age: 12--16 years) with SPT were diagnosed at our institution. All patients presented with an abdominal mass and uncharacteristic abdominal pain. Two tumors were located in the tail, one in the body and tail and one in the head of the pancreas (diameter: 7--15 cm). Surgical procedures included three distal pancreatectomies and one partial duodenopancreatectomy (Whipple procedure). One patient had two recurrences with metastases that could only be partially resected. Chemotherapy was initiated for this patient. In the follow-up period (range: 6 months to 12 years) all patients are alive with no evidence of recurrence. CONCLUSIONS: SPT is a rare differential diagnosis of a pancreatic mass in children. It is mandatory to establish this diagnosis since complete surgical removal of the tumor even in case of metastases or local invasion offers an excellent prognosis.     

胰腺乳头状囊实性肿瘤的病理与临床特点

目的：探讨胰腺乳头状囊性实性肿瘤（solid cystic papillary tumor,SCPT）的病理与临床特点。方法：回顾性分析在小儿外科手术及病理确诊的8例患儿,同时分析同期在成人外科手术及病理确诊的7例患者的临床资料。结果：15例患者中,男1例,女14例。10-20岁8例,20-30岁4例,30-40岁3例。平均年龄15.8岁,中位年龄16岁。该肿瘤多以腹胀、腹痛和腹部包块为首发症状,多数肿瘤位于胰头或胰尾。3例行肿瘤切除术,8例行远端胰腺切除术,3例行胰十二指肠切除术,1例仅取病理行活检术。随访3个月-4年,15例患者生存至今,2例复发。镜下观察15例肿瘤均以实性区、囊性区及乳头状突起为其特征。α抗胰蛋白酶免疫组化阳性率100%（15/15）,波形蛋白80%（12/15）,神经元特异性烯醇化酶53.3%（8/15）。15例均诊断为胰腺囊性实性乳头状瘤。结论：胰腺乳头状囊性实性肿瘤多见于青春期女性和年轻妇女,组织病理来源不清,属低度恶性,具有良性临床病程,手术治愈率高,预后良好。     

Analysis of 50 cases of solid pseudopapillary tumor of pancreas: Aggressive surgical resection provides excellent outcomes

IntroductionThis study reports the clinicopathological characteristics and the perioperative and long-term treatment outcomes after aggressive surgical resection in solid pseudopapillary tumor (SPT) of the pancreas performed at a high volume center for pancreatic surgery in India.Materials and methodsWe analyzed a prospectively maintained database of the patients operated for SPT at Tata Memorial Hospital, India over a period of 11 years from February 2007 to February 2018.ResultsFifty consecutive patients operated for SPT, during the study period were included. The median age at presentation was 24 years. Majority of the patients (43/50) were female (86%). Disease was predominantly localized in the head and uncinate process of pancreas (66%). Median tumor size was 7.7 cm (Range 1.6–15 cm). Tumor extent was radiologically defined as borderline resectable or locally advanced in 48% (n = 24) patients. Forty-six major pancreatic resections were performed, which included 10 (21%) vascular resections, 2 synchronous liver metastasectomies, 1 multi visceral resection and 5 total pancreaticosplenectomies. Five of these resections were reoperations in patients deemed inoperable on exploration at other centers. R0 resection was achieved in 47 patients (98%). Postoperative major morbidity was 19% and there was no mortality. At a median follow-up of 29 months (Range, 1–121 months), all patients were alive without any recurrence.ConclusionAggressive complete surgical resection of SPT achieves excellent long-term survival. Surgery, especially for large and borderline resectable tumors, can be potentially complex and should be performed at high-volume centers to provide the best chance of cure.     

74例原发性气管腺样囊性癌的临床分析

目的探讨原发性气管腺样囊性癌的临床特点。方法74例患者均经病理或细胞学证实。T分期中T18例（10.8%）,T219例（25.7%）,T323例（31.1%）,T424例（32.4%）。纵隔淋巴结阳性8例（10.8%）。远处转移2例（2.7%）。62例（83.8%）接受手术治疗,27例手术完全切除,R1或R2切除35例。单纯手术24例,单纯放疗12例,综合治疗38例。结果复发或转移的患者共43例（58.1%）,局部复发20例,远处转移17例,局部复发＋远处转移6例。除2例外,所有远处转移患者均有肺转移。第一次复发或转移的中位时间为32月,第二次、第三次复发或转移的中位时间分别为12月和6月。所有患者中位生存时间55月,5年、10年总生存率分别为69.7%和35.9%。结论原发性气管腺样囊性癌治疗效果较好。病变长度、T分期以及包含手术的治疗可能是影响预后的因素。患者治疗后出现多次复发或转移,积极治疗仍能获得长期生存的机会。远处转移中肺转移最多见。     

Primary Mediastinal Germ Cell Tumors—The University of Western Ontario Experience

Extragonadal germ cell tumors account for 2–5.7% of germ cell tumors (GCTs). Of these, primary mediastinal GCTs (PMGCTs) are responsible for 16–36% of cases. Given the rarity of these tumors, specific treatment strategies have not been well defined. We report our experience in treating these complex patients. In total, 318 men treated at our institution with chemotherapy for GCTs between 1980 and 2016 were reviewed. PMGCT was defined as clinically diagnosed mediastinal GCT with no evidence of testicular GCT (physical exam/ultrasound). We identified nine patients diagnosed with PMGCT. All patients presented with an anterior mediastinal mass and no gonadal lesion; four patients also had metastatic disease. Median age at diagnosis was 30 years (range, 14–56) and median mass size at diagnosis was 9 cm (range, 3.4–19). Eight patients had non-seminoma and one had pure seminoma. All patients received cisplatin-based chemotherapy initially. Surgical resection was performed in four patients; three patients had a complete resection and one patient was found to have an unresectable tumor. At a median follow-up of 2 years (range, 3 months–28 years) six patients had progressed. Progression-free survival was short with a median of 4.1 months from diagnosis (range 1.5–122.2 months). Five patients died at a median of 4.4 months from diagnosis. One and 5-year overall survivals were 50% and 38%, respectively. PMGCT are rare and aggressive. Our real-life Canadian experience is consistent with current literature suggesting that non-seminoma PMGCT has a poor prognosis despite prompt cisplatin-based chemotherapy followed by aggressive thoracic surgery.     

胃间质瘤26例临床诊治分析

背景与目的:胃间质瘤(gastric stromal tumor,GST)是起源于间叶组织的肿瘤,胃是消化道间质瘤的最常见的发生部位,以往外科手术是唯一的治疗手段,随着酪氨酸激酶受体CD117在肿瘤中的表达的发现,胃间质瘤的诊治出现了重大的进展.本研究回顾性分析26例胃间质瘤的患者的临床资料,探讨胃间质瘤的病理特点、治疗方式以及影响预后的凶素.方法:回顾性分析2000年1月至2008年12月期间本院普外科收治的26例胃间质瘤患者的临床资料,所有患者均经手术治疗,其中3例术后口服甲磺酸伊马替尼,剂量为400 mg/d.分析全部病例的临床病理结果及随访资料.结果:全部患者肿瘤切除率100%,发现肝转移3例,一并手术切除转移灶.复发转移6例,其中低度危险组1例,中度危险组1例,高度危险组4例.肿瘤大小从2～15 cm(中位数5.5 cm).术后病理以及免疫组织化学结果显示CD117阳性24例(92.3%)、CD34阳性21例(80.8%),Vimentin阳性25例(96.2%).术后3例口服甲磺酸伊马替尼6个月以上,1例目前无瘤生存,1例肝脏带瘤生存,1例6个月后死亡.本组随访时间4～36个月,术后失访4例(15.3%),平均随访时间28个月;全组病例1、3生存率分别为96.2%和84.6%.结论:肿瘤大小、核分裂相、免疫组织化学指标是判断胃间质瘤预后的重要指标,发生转移者预后差.手术仍是胃间质瘤的首选方式,靶向治疗可改善其预后.     

Desmoid tumors: a novel approach for local control

BACKGROUND AND OBJECTIVES: After resection, desmoid tumors are associated with a recurrence rate that is typically 25-50%. Although this is an unusual problem, we instituted a prospective cohort study with neoadjuvant chemotherapy and radiation, followed by surgical resection, in an effort to improve local control. METHODS: Between 1985 and 1999, 13 patients with potentially resectable disease were managed with a treatment protocol of preoperative doxorubicin (30 mg continuous infusion daily for 3 days) and radiotherapy (10 x 300 cGy). Resection was performed 4-6 weeks later. All lesions were resected with an intended margin of 1 cm, but clear adventitial margins were accepted in order to preserve critical structures. RESULTS: The median follow-up was 71 months (range, 22-109). Six patients (46%) presented after failure of a previous surgery. Clear microscopic margins were obtained in 11 patients, and 2 patients had positive margins. There were two local recurrences (15% local recurrence). Both recurrences followed resection of large thigh lesions, which appeared at 30 and 49 months of follow-up. In one patient with a chest wall tumor, two new primary desmoid tumors developed outside the treatment area, in the ipsilateral arm and forearm. Eleven patients have been disease free for a median of 71 months (range, 22-109). CONCLUSIONS: For potentially resectable lesions, this protocol provides excellent local control, even in those with recurrent disease. Neoadjuvant treatment with doxorubicin and radiotherapy appears to be a better option than surgery alone, or surgery and adjuvant radiotherapy. These results need to be confirmed in larger, prospective randomized trials.     

35例食管腺样囊性癌的临床特征及预后分析

目的 分析食管腺样囊性癌（esophagus adenoid cystic carcinoma, EACC）的临床特点、诊断、治疗方式及预后。方法 回顾性分析35例EACC患者的病例资料,分析EACC的临床病理特征及预后影响因素。结果 35例腺样囊性癌多发于食管中段,平均年龄60.6岁,男女比例4:1,病理形态以蕈伞型和息肉型为主。早期EACC神经受侵发生率低,实性型较经典筛状型复发转移率高,患者中位生存期为38月,术后1、3、5年生存率分别为88.1%、46.6%和27.1%。结论 发生于食管的腺样囊性癌是一种罕见的恶性肿瘤,其预后与肿瘤大小、病理分期相关。     

Outcome after surgical treatment of suspected gastrointestinal stromal tumors involving the duodenum: Is limited resection appropriate?

INTRODUCTION: Present surgical opinion is divided regarding the optimal method for the treatment of duodenal gastrointestinal stromal tumor (GIST) with some supporting the selective use of limited resection (LR) versus others who prefer pancreaticoduodenectomy (PD). METHODS: A retrospective review of 22 patients who underwent surgery for suspected GIST involving the duodenum. RESULTS: There were 15 GISTs, 1 leiomyosarcoma and 6 other non-GIST benign submucosal tumors. Seven patients underwent LR and seven underwent PD for GIST. The median follow-up was 42 (range, 2-174) months. Patients who underwent LR versus PD had similar mean disease-specific survival [144 (95% CI, 92-196) vs. 130 (95% CI, 82-127) months, P = 0.808] and recurrence rates (14% vs. 29%, P = 0.515). All recurrences occurred at distant sites. Comparison between LR versus PD demonstrated that LR was associated with a significantly shorter operation time [125 (range, 50-305) vs. 350 (range, 210-465) min., P = 0.001] but similar morbidity rate (23% vs. 43%, P = 0.357). Comparison between GIST and other benign tumors demonstrated that size was the only statistically significant distinguishing factor [8.5 (range, 2.5-18.0) vs. 2.5 (range, 1.5-8.0) cm, P = 0.014]. CONCLUSION: Benign non-GIST tumors may be distinguished from duodenal GIST as they are smaller in size. LR is a viable treatment option for suspected GIST involving the duodenum.     

Value of CT criteria in predicting survival in patients with potentially resectable pancreatic head carcinoma

BACKGROUND AND OBJECTIVE: Survival is often poor after resection of pancreatic tumors. We correlated the pre-operative CTs with survival to find criteria that have prognostic value. To establish the prognostic value of CT in patients with potentially resectable pancreatic head carcinoma. METHODS: In 71 consecutive patients with potentially resectable pancreatic head carcinoma, prognostic factors on CT were scored, for example, tumor size, peripancreatic infiltration, grades of vascular encasement, and local irresectability. All patients underwent surgical exploration. CT findings were compared with results of surgery and histopathology. Prognostic factors for resected and unresected tumors were analyzed using single and multivariate analysis. RESULTS: Forty-one of 71 tumors were resected (24 radical). The sensitivity, specificity, and positive predictive value of CT for surgical irresectability were 0.67, 0.63, and 0.57, respectively. For a non-radical resection, these were 0.62, 0.75, and 0.83, respectively. The median survival was 21 months for resectable tumors and 9.7 months for unresectable tumors. For resected tumors, a tumor diameter of > 3 cm (relative hazard 3.8) and CT signs of local unresectability showed a poor survival. The median survival of resected tumors <2 cm was nearly 30 months. CONCLUSION: CT signs of local irresectability and a tumor diameter of >3 cm predict a poor survival after resection.     

Iodine125 interstitial brachytherapy in the treatment of carcinoma of the lung.

In a prospective study, 14 patients with primary non-oat cell lung carcinoma were treated with intraoperative Iodine125 (I125) implantation of the lung tumor via lateral thoracotomy or median sternotomy. Staging mediastinal node dissection was performed in each case. Patients were selected when wedge or segmental resections were not technically feasible, such that lobectomy or completion pneumonectomy would have been required or pulmonary function studies were poor. Doses ranged from 8,000 cGy at the periphery to 20,000 cGy at the center. With a minimum 12 month follow-up, mean and median survivals were 16.7 and 15.1 months, respectively. Local control was achieved in 10 of 14 patients (71%) with all local failures occurring in pathologic stage III patients. When separated according to tumor size, local control was obtained in six of seven tumors of less than 3 cm and four of five tumors of 3-5 cm. Both cases with masses greater than 5 cm failed locally. There was one operative mortality and two postoperative complications. All other patients were discharged within one week of surgery. There was no radiation pneumonitis. I125 lung brachytherapy is an excellent alternative treatment for T1 and T2 tumors when medical conditions preclude curative resection.     

Multimodal treatment of malignant sacrococcygeal germ cell tumors: a prospective analysis of 66 patients of the German cooperative protocols MAKEI 83/86 and 89.

PURPOSE: To evaluate a multimodal approach including surgery and cisplatinum chemotherapy for treatment of children with malignant sacrococcygeal germ cell tumors (GCT) and to compare adjuvant and neoadjuvant strategies in advanced tumors. PATIENTS AND METHODS: Between 1983 and 1995, 71 patients with malignant sacrococcygeal GCT were prospectively enrolled onto the German protocols for nontesticular GCT Maligne Keimzelltumoren 83/86 and 89. Five patients who received no chemotherapy (n = 2) or nonplatinum chemotherapy (n = 2) or who did not undergo tumor resection (n = 1) were excluded from this analysis. Among the 66 patients analyzed were 14 boys and 52 girls. The median age was 17.4 months (range, 7 months to 119 months). Median follow-up was 79 months (range, 4 months to 145 months). RESULTS: Fifty-two patients presented with locally advanced stage T2 tumors, and 30 patients had distant metastases at diagnosis. Patients received a median of eight cycles (range, four to nine cycles) of cisplatinum-based chemotherapy. Thirty-five patients underwent tumor resection at diagnosis and received adjuvant cisplatinum-based chemotherapy (group A). Thirty-one patients received up-front chemotherapy followed by delayed tumor resection (group B). Group B included more metastatic tumors than group A (group B, 19 of 31 patients; group A, 11 of 35 patients, P =.01). Preoperative chemotherapy facilitated complete tumor resections (group B, 20 of 31 patients; group A, five of 35 patients, P <.001) and avoided second-look surgery. Metastases at diagnosis and completeness of the first attempt of tumor resection were significant prognostic predictors; however, metastases were not predictive for patients treated with up-front chemotherapy. At 5 years follow-up, event-free survival was 0.76 +/- 0.05 (50 of 66 patients), and overall survival was 0.81 +/- 0.05 (54 of 66 patients). Four patients died as a result of therapy-related complications, and eight patients died of their tumors. Patients with locally advanced and metastatic tumors (T2b M1) fared better with neoadjuvant treatment [overall survival: 0.83 +/- 0.09 (16 of 19 patients) versus 0.45 +/- 0.15 (five of 11 patients), P =.01]. CONCLUSION: Even locally advanced and metastatic sacrococcygeal GCT can be successfully treated with up-front cisplatinum-based chemotherapy followed by delayed but complete tumor resection.     

胃肠道外间质瘤临床病理特征、治疗方法对预后的影响

目的:分析胃肠道外间质瘤(extra-gastrointestinal stromal tumors,EGIST)的临床特征、手术方式和伊马替尼治疗对患者生存率的影响。方法:回顾性总结2010年01月至2018年12月我院收治的40例胃肠道外间质瘤患者的临床资料。计量和计数资料分别采用t检验和χ2检验,生存情况采用寿命表法和Kaplan-Meier法。结果:全组胃肠道外间质瘤（EGIST）患者术后中位随访时间为24个月（3~76个月）,1、3、5年生存率分别为75%、42%、35%。R0术后服用伊马替尼组3例患者1、2、3生存率100%、100%、100%;R0术后未服用伊马替尼组14例患者1、2、3生存率100%、91.0%、91.0%;非R0术后服用伊马替尼组12例患者1、2、3生存率91.7%、50.3%、33.5%;非R0术后未服用伊马替尼组11例患者1、2、3年生存率18.2%、9.0%、0;差异有统计学意义（P=0.000)。结论:EGIST具有低的发病率,预后差。手术方式、肿瘤是否破裂、有无复发转移、危险度分级、术后靶向药物治疗等是影响 EGIST 预后的重要因素。     

Liver resection for intrahepatic cholangiocarcinoma

AIMS AND BACKGROUND: Intrahepatic cholangiocarcinoma (IHCC) is the second most common primary liver cancer, representing 10% of all primary liver malignancies. Despite the increase in its incidence, this tumor remains extremely rare in Western countries and few reports detailing experience with surgical resection have been published. The aim of this study was to analyze the experience with resection of IHCC in our center. METHODS: From 1987 to 2003 we observed 35 patients with IHCC; 15 of them (42.8%) were submitted to hepatic resection. IHCCs accounted for 13% of all liver resections for primary liver tumors carried out at our center during this period. According to the classification of the Liver Cancer Study Group of Japan, the tumors were classified as "mass-forming" in 14 cases and as "periductal" in one case. Major resections were performed in ten cases and minor resections in five cases. In the patient with a periductal tumor a major resection was performed along with excision of the main biliary confluence. In 14 cases (93.3%) tumor-free resection margins were obtained. RESULTS: The intraoperative mortality was nil and the postoperative mortality 6.6%. The postoperative morbidity rate was 21.4%. The mean overall survival was 38.4 months, with 86% and 49% one- and three-year survival rates, respectively. Patients with mass-forming tumors and curative resections (R0) (mean survival 40.8 months; one- and three-year survival rates 92.3% and 52.7%), and those with TNM stage I-II tumors (mean survival 43.7 months; one- and three-year survival rates 100% and 66.7%) had a longer survival. The patient with the periductal tumor and R1 resection died after seven months. CONCLUSIONS: These results support a surgical approach based on accurate selection of patients with IHCC and aimed at radical resection whenever possible. The good survival rates observed in R0 resections emphasize the role of radical surgery as the only chance of cure for patients with this tumor.     

Pancreatic resection for metastatic tumors to the pancreas

BACKGROUND AND OBJECTIVES: The incidence of metastases to the pancreas is very low. The benefit of resection of pancreatic metastasis is poorly defined. In this review we evaluated the outcome of patients undergoing pancreatic resection for metastatic tumors to the pancreas. METHODS: Eight patients underwent pancreatic resection for metastatic tumor from December 1980 to June 2001. The primary cancer was colon carcinoma (n = 4), renal cell cancer (n = 2), duodenal leiomyosarcoma (n = 1), and malignant fibrous histiocytoma (n = 1). The median interval between primary treatment and detection of pancreatic metastases was 36 months. In two cases pancreatic metastases were synchronous with the primary tumor. RESULTS: Four patients underwent pancreatoduodenectomy, two distal pancreatectomy, one total pancreatectomy, and one median pancreatectomy. Associated resection of extrapancreatic lesions was performed in four patients, including two left hepatectomy and two left colectomy. There was no postoperative mortality, but two patients had a pancreatic and a biliary fistula, respectively. Survival averaged 23 months (range 14-42 months): four patients died for metastatic disease from 14 to 42 months after operation, while four patients are alive and well 14 to 31 months after surgery. CONCLUSIONS: Pancreatic resection for metastatic disease to the pancreas should be considered even in selected patients with limited extrapancreatic disease. Long-term survival or good palliation may be achieved.     

A Case of Osteoclast-like Giant Cell Tumor of the Pancreas Associated with Borderline Mucinous Cystic Neoplasm

A 34-year-old, previously healthy female presented with severe acute upper quadrant abdominal pain and an 11-cm cystic mass in the tail of the pancreas. The patient underwent distal pancreatectomy with total gross excision of the mass. Grossly, the mass consisted of a multiloculated cystic lesion measuring 11.7 cm in its greatest dimension. An irregular solid lobulation at the lateral aspect of the cyst was visible, measuring 3 cm in the largest dimension. Histologically, there were two distinct components: a mucinous, neoplastic epithelial cyst with few foci of moderate atypia, and nodular spindle cell areas containing multinucleated tumor giant cells. Immunohistochemically, the multinucleated giant cells were positive for vimentin, CD68 and CD45, and negative for cytokeratin and epithelial membrane antigen (EMA). The spindle cells of hypercellular stroma were stained for vimentin, but not for EMA or carcinoembryonic antigen (CEA). Neuron-specific enolase (NSE), S100 and Ki-67 showed no reactivity. The histological diagnosis “osteoclast-like giant cell tumor of the pancreas associated with borderline mucinous cystic neoplasm” was made. The patient recovered and is free of disease 4 years after the diagnosis.     

头颈部唾液腺腺样囊性癌术后调强放疗预后分析

目的 探讨头颈部唾液腺腺样囊性癌(ACC)的综合治疗模式及预后因素。方法 收集2016—2018年间上海交通大学医学院附属第九人民医院放疗科收治的头颈部唾液腺ACC完成术后放疗患者资料进行回顾性分析。Kaplan-Meier法生存分析,log-rank检验单因素预后分析,Cox模型多因素分析。结果 共166例患者被纳入研究,其中男70例,女96例,平均年龄53岁(18~71岁)。中位随访期31.2个月(8.6~63.1个月)。接受根治手术者66例(肿瘤床外跨解剖区域扩大切除,追求各类切缘阴性),接受保守手术者100例(仅病灶扩大切除,不追求神经切缘阴性)。术后放疗中位剂量66Gy (54~70Gy)。有73例患者因切缘阳性或近切缘,9例患者因颈淋巴结转移行TP方案同期化疗。全组患者3年和5年总生存(OS)率、局部区域无复发生存(LRRFS)率、无远处转移生存(DMFS)率、无进展生存(PFS)率分别为95%和95%、93%和93%、74%和66%、73%和65%。全组病例共40例出现治疗失败,其中原发灶复发7例,区域淋巴结复发5例,远处转移38例。T3-T4期、N (+)、Ⅳ期、病理分型实体型、Ki67高表达、神经侵犯是影响PFS、DMFS的预后不良因素,多因素分析只有病理分型是影响OS、LRRFS、PFS、DMFS的独立预后因素。结论 头颈部唾液腺ACC术后辅助调强放疗提高了局控率,推荐放疗剂量≥66Gy。实体型病理分型是最重要的预后不良因素。     

External-beam radiotherapy for localized extrahepatic cholangiocarcinoma

PURPOSE: The role of radiation therapy (RT) in extrahepatic cholangiocarcinoma (EHCC) is not clear and only limited reports exist on the use of this modality. We have reviewed our institutional experience to determine the pattern of failure in patients after potentially curative resection and the expected outcomes after adjuvant RT and in unresectable patients. METHODS AND MATERIALS: After institutional review board approval, 81 patients diagnosed with EHCC (gallbladder 28, distal bile duct 24, hilar 29) between June 1986 and December 2004 were identified and their records reviewed. Twenty-eight patients (35%) underwent potentially curative resection with R0/R1 margins. Fifty-two patients (64%) were unresectable or underwent resection with macroscopic residual disease (R2). All patients received three-dimensional planned megavoltage RT. The dose for each patient was converted to the equivalent total dose in a 2 Gy/fraction, using the linear-quadratic formalism and alpha/beta ratio of 10. The median dose delivered was 58.4 Gy (range, 23-88.2 Gy). 54% received concomitant chemotherapy. RESULTS: With a median follow-up time of 1.2 years (range, 0.1-9.8 years) 75 patients (93%) have died. Median overall survival (OS) and progression-free survival (PFS) were 14.7 (95% CI, 13.1-16.3) and 11 (95% CI, 7.6-13.2) months, respectively. There was no difference among the three disease sites in OS (p = 0.70) or PFS (p = 0.80). Complete resection (R0) was the only predictive factor significantly associated with increase in both OS and PFS (p = 0.002), and there was no difference in outcomes between R1 and R2 resections. The first site of failure was predominantly locoregional (68.8% of all failures). CONCLUSION: Local failure is a major problem in EHCC, suggesting the need for more intense radiation schedules and better radiosensitizing strategies. Because R1 resection appears to convey no benefit, it appears that surgery should be contemplated only when an R0 resection is likely. Borderline-resectable patients might be better served by neoadjuvant therapy.