Villous tumors of the duodenum: Reappraisal of local vs. extended resection

Benign villous tumors of the duodenum are often managed by transduodenal local excision. Risk of local recurrence, coupled with improving safety of radical pancreaticoduodenectomy, has prompted reexamination of the roles of conservative and radical operations. The aim of this study was to determine long-term outcome after local and extended resection in order to identify factors to consider in planning operative strategy. Eighty-six patients (mean age 64 years) with villous tumors of the duodenum managed surgically from 1980 to 1997 were reviewed. Histologic findings, size, presence of polyposis syndromes, and extent of resection were correlated with outcome. Villous tumors were benign adenomas in 64 patients (74%), contained carcinoma in situ in three (4%), and invasive carcinoma in 19 (22%). The presence of cancer was not known preoperatively in 9 (47%) of the 19 with invasive carcinoma. Operative treatment included transduodenal local excision in 53 patients, pancreaticoduodenectomy in 20, pancreas-sparing duodenectomy in five, full-thickness excision in four, and other in six. Among the 50 patients with benign tumors managed by local excision, 17 had a recurrence with actuarial rates of 32% at 5 years and 43% at 10 years; four of the recurrences (24%) were adenocarcinomas. The recurrence rate was influenced by the presence of a polyposis syndrome but not by tumor size. Recurrence of benign villous tumors after local excision is common and may be malignant. Pancreaticoduodenectomy is appropriate for villous tumors containing cancer and may be considered an alternative for select patients with benign villous tumors of the duodenum. If local excision is performed, regular postoperative endoscopic surveillance is mandatory. Presented at the Fortieth Annual Meeting of The Society for Surgery of the Alimentary Tract, Orlando, Fla., May 16–19, 1999, and published as an abstract in Gastroenterology 116:A1310, 1999.     

Primary tumors of the small bowel

Primary tumors of the small bowel are uncommon, representing less than 6 per cent of all gastrointestinal tumors and less than 2 per cent of all malignant gastrointestinal tumors. This report concerns a twenty-five year survey of our clinical records from 1946 to 1971 which revealed 140 primary small bowel tumors, excluding periampullary tumors. Fifty-two of the neoplasms (37 per cent) were benign; eighty-eight (63 per cent) were malignant and included twenty-eight adenocarcinomas (31.8 per cent), twenty-four lymphosarcomas (27.3 per cent), nineteen carcinoids (21.6 per cent), and ten leiomyosarcomas (11.4 per cent). The average age at the time of diagnosis was 56.9 years for patients with benign tumors and 55.9 years for those with malignant tumors. The illusive and obscure nature of small bowel tumors is illustrated by the fact that 63.3 per cent of patients with benign lesions and 47.6 per cent of those with malignant lesions had symptoms for more than six months before the diagnosis was made. Bleeding was the most common present complaint in patients with benign neoplasms (52.9 per cent) whereas patients with malignant lesions more often had symptoms of obstruction (50.6 per cent). Most of the benign lesions were located proximally in the small bowel (duodenum, 34.6 per cent; ileum, 11.5 per cent), and most of the malignant lesions were located distally (duodenum, 17.0 per cent; ileum, 61.4 per cent). Treatment of patients with malignant lesions was radical excision whenever possible. Adjunctive radiation therapy was used for those with lymphoma. A second benign or malignant tumor occurred in 42.9 per cent of the patients with primary small bowel tumors. The average period of survival after diagnosis of a malignant small bowel tumor was 5.03 years: for patients with adenocarcinoma, 3.6 years; lymphosarcoma, 1.3 years; carcinoid, 6.8 years; and leiomyosarcoma, 8.3 years.     

Villous adenomas of the duodenum and an unusual variant

We report two cases of villous adenoma of the duodenum, one arising from the main papilla and the other from the accessory papilla. Both were managed by local resection. In one case endoscopic biopsies and intraoperative frozen sections were negative for carcinoma but histology of the locally resected specimen revealed a focus of invasive adenocarcinoma. Villous adenomas of the duodenum have a high risk of malignant change and foci of carcinoma can be missed on endoscopic biopsy. The literature is reviewed and the clinical, diagnostic, pathological and therapeutic aspects of villous adenomas of the duodenum are discussed.     

Villous tumors of the duodenum.

Villous tumors of the duodenum are rare, but treatment may be problematic because of their association with invasive adenocarcinoma. Two cases of villous tumor of the duodenum are described and 39 other reported cases are reviewed. Presenting symptoms were bleeding 27%; obstruction 24%; jaundice 22% and vague dyspepsia 20%. Diagnosis may be made by radiographic barium contrast evaluation of the duodenum, especially with the addition of air contrast hypotonic studies and by fibro-optic endoscopy. Twenty-seven per cent of villous tumors of the duodenum are associated with adenocarcinoma. Invasive tumor is more common in patients over 50 years old (35%), in tumors of the third and fourth portions of the duodenum (44%) and in tumors over 4 cm in diameter (30%). Local excision is the treatment of choice for benign lesions. Pancreatico-duodenectomy is recommended for tumors which include invasive carcinoma in patients without distal metastases.     

Villous tumor of the doudenum (author's transl)

Villous tumors of the duodenum are rare, but treatment may be problematic because of their association with invasive adenocarcinoma. One case of villous tumor of the duodenum is described and 44 others reported cases are reviewed. Diagnosis may be made by radiographic barium contrast evaluation of the duodenum, especially with the addition of air contrast hypotonic studies and by fibro-optic endoscopy with biopsies. 32 p. cent of villous tumors of the duodenum are associated with adenocarcinoma. Local excision is the treatment of choice for benign lesions. Duodenectomy or pancreatico-duodenectomy are recommended for tumors which include invasive carcinoma.     

Main-duct intraductal papillary mucinous neoplasms of the pancreas: clinical predictors of malignancy and long-term survival following resection

OBJECTIVE: To describe clinical characteristics and outcomes of a large cohort of intraductal papillary mucinous neoplasms (IPMNs) of the pancreas affecting the main pancreatic duct. SUMMARY BACKGROUND DATA: IPMNs are being diagnosed with increasing frequency. Preoperative determination of malignancy remains problematic, and reported results of long-term survival following resection are conflicting. METHODS: The combined databases from the Massachusetts General Hospital and the Pancreatic Unit of the University of Verona were analyzed. To avoid confusing overlap with mucinous cystic neoplasms, only patients with tumors of the main pancreatic duct (with or without side branch involvement) were included. A total of 140 tumors consecutively resected between 1990 and 2002 were classified as either benign (adenoma and borderline tumors) or malignant (carcinoma in situ or invasive cancer) to compare their characteristics and survival. RESULTS: Men and women were equally affected (mean age 65 years). Seven patients (12%) had adenomas, 40 (28%) borderline tumors, 25 (18%) carcinoma in situ, and 58 (42%) invasive carcinoma. The median age of patients with benign IPMN was 6.4 years younger than those with malignant tumors (P = 0.04). The principal symptoms were abdominal pain (65%), weight loss (44%), acute pancreatitis (23%), jaundice (17%), and onset or worsening of diabetes (12%); 27% of patients were asymptomatic. Jaundice and diabetes were significantly associated with malignant tumors. Five- and 10-year cancer-specific survival for patients with noninvasive tumors was 100%, and comparable survival of the 58 patients with invasive carcinoma was 60% and 50%. CONCLUSIONS: Cancer is found in 60% of patients with main-duct IPMNs. Patients with malignant tumors are 6 years older than their benign counterparts and have a higher likelihood of presenting with jaundice or new onset diabetes. No patients with benign tumors or carcinoma in situ died of their disease following resection, and those with invasive cancer had a markedly better survival (60% at 5 years) than pancreatic ductal adenocarcinoma. These findings support both the concept of progression of benign IPMNs to invasive cancer and an aggressive policy of resection at diagnosis.     

经胆总管刮取组织法诊断壶腹癌（附13例报告）

经胆总管刮取组织法诊断壶腹癌13例,11例刮取组织中腺癌9例,腺瘤伴高度不典型增生、原位癌1例,绒毛乳头状瘤样增生1例。2例疑壶腹癌者刮取未得组织。常规胆总管探查术中适时应用此法有助于诊断未经注意的壶腹癌。在疑壶腹癌病例中采用本法诊断时多能得到阳性结果,但对刮取未得组织者应改用其它方法明确诊断。刮取组织病检诊为腺癌或绒毛肿瘤时有可能遗漏隐藏的恶性病变,故仍应将其完全切除病检。     

Villous adenomas of the colon and rectum

Our thirteen year experience with villous adenomas of the colon and rectum, embracing 264 patients, is reported. The average age of patients was sixty-two years with an almost equal sex distribution. Seventy-two per cent of the lesions occurred in the rectum and rectosigmoid and the most frequent symptoms were rectal bleeding and diarrhea. A positive correlation between size of adenoma and incidence of malignancy was noted. Although 55 per cent of the lesions were malignant, only 22 per cent harbored invasive carcinoma and only 10 per cent of the deaths were due to metastatic carcinoma. It is concluded that in the absence of invasive carcinoma, the clinical behavior of this tumor is relatively benign and a more conservative approach to the surgical management of this neoplasm should be encouraged.     

Villous tumors of the duodenum.

Nineteen cases of villous tumors of the duodenum are reported. They have a predilection for the ampullary region, tend to present with obstructive jaundice, especially if malignancy is present, and have a high prevalence of cancer (12 of 19, or 63%). Even when biopsies are available, the diagnosis of cancer is frequently missed (5 of 9 proven cancers, 56% false-negative rate), and it may be impossible to assess the presence of carcinoma in situ or invasive carcinoma without complete excision of the lesion. The authors' experience suggests that some small benign ampullary villous adenomas or those with carcinoma in situ can be excised locally but that pancreaticoduodenectomy is preferable in the fit patient for better local control both of extensive benign lesions and cancers without distant metastases.     

Squamous cell carcinoma of the thymus. An analysis of eight cases

Eight cases of squamous cell carcinoma of the anterior mediastinum, most likely derived from the thymus, are presented. Seven were male and one female ranging in age from 39 to 65 years; the average was 55.5 years. There were no cases associated with any paraneoplastic syndromes. They possessed common morphological characteristics. Grossly, the tumors resembled malignant thymoma. Invasion of the lung and metastases to regional lymph nodes were frequent. Often observed microscopically were foci of sharply defined keratinization resembling Hassall's corpuscles, no radial arrangement of tumor cells at the periphery of nests, and broad, fibrotic, or hyalinized stroma. Admixture of a few lymphoid cells and some features transitional to thymoma were also observed in some parts of tumors. However, undoubtedly carcinomatous areas were present in some or large parts of all the tumors, where individual cells possessed a vesicular nucleus and a prominent round nucleolus. These features were distinct from those of bronchogenic squamous cell carcinoma and other thymic tumors, although they appeared to be related to thymoma. Treatment of choice is radical surgery and postoperative radiotherapy, because of relatively high radiosensitivity. Prognosis of patients was relatively good. From analyses of cases it is concluded that squamous cell carcinoma of the thymus should be separated from ordinary thymoma of the epithelial type, and that squamous cell carcinoma involving both the thymus and lungs should be carefully examined for the primary site of growth.     

Carcinoma of the fourth part of the duodenum: Report of a case

Primary carcinoma of the duodenum is rare, accounting for only 0.35% of all gastrointestinal carcinomas, and carcinoma of the fourth part of the duodenum constitutes approximately 10% of duodenal carcinomas. Since the predominant symptoms of tumors in this part of duodenum are related to upper intestinal obstruction, the diagnosis is usually made late. We report the case of a 66-year-old man who presented with anemia, weight loss, and upper intestinal obstruction, and was found to have advanced primary carcinoma of the fourth part of the duodenum by upper gastrointestinal series and computed tomography (CT). A review of the literature indicates that survival, which is related to nodal status, the grade of the tumor, and surgical results, seems to be longer in patients with carcinoma in this part of the duodenum compared to those with carcinomas in other parts of the duodenum. Moreover, segmentary resection has been reported to achieve a favorable outcome.     

Villous adenomas in the duodenum

Five patients with villous adenomas in the duodenum are described. In one patient malignant degeneration had occurred at the time of diagnosis. This patient was treated with a pancreaticoduodenal resection and is doing well 2 years postoperatively. The other four patients all had a duodenotomy and a local excision of the tumour. One patient had a recurrence with malignant degeneration within 1 year. The other three patients are doing well without signs of recurrence 1-4 years postoperatively. It is concluded that duodenal villous adenomas are potentially malignant. The strategy of surgical treatment is discussed.     

原位微波灭活术治疗骨盆恶性骨肿瘤的临床研究

目的 探讨原位微波灭活术治疗骨盆原发恶性肿瘤及转移瘤的疗效.方法 2000年2月至2009年4月对18例骨盆原发恶性肿瘤及转移瘤患者采用原位微波灭活及肿瘤全部或部分切除,男11例,女7例;年龄16～72岁,平均45岁.原发恶性骨肿瘤12例,转移瘤6例.肿瘤累及Ⅰ区6例、Ⅱ区10例、Ⅲ区2例.根据肿瘤所在区域采用传统髂腹股沟切口或髂腹股沟"T"形切口进行显露,以铜网保护周围软组织,用2450 MHz微波将肿瘤局部加热至50℃,持续20 min,全部或部分剥离坏死肿瘤组织,保留骨盆环的解剖连续性.结果 手术时间60～180min,平均110min;术中失血量400～800ml,平均480 ml.无严重的术中及术后并发症.随访0.5～7年,平均3.5年.1例软骨肉瘤患者术后8个月复发,随访6个月带瘤生存.1例恶性纤维组织细胞瘤患者术后24个月出现肺、脑和全身多发转移死亡.1例骨肉瘤患者术后18个月出现肺转移死亡.6例骨盆转移瘤患者中5例分别于术后6～19个月出现其他部位转移而死亡.术后3个月18例患者患侧髋关节屈曲80°～130°,后伸0°～10°,外展25°～35°,内收18°～23°.结论 原位微波灭活术具有操作简便、疗效可靠、创伤小的特点,可用于或辅助用于骨盆恶性骨肿瘤的治疗,能保持骨盆环的完整性.

Abstract：

Objective To investigate the clinical outcomes of microwave heliotherapy in situ on the primary or metastatic malignant tumors of the pelvis.Methods From February 2000 to April 2009,18 patients with primary or metastatic malignant tumors of the pelvis were treated with microwave heliotherapy in situ,and followed a total or partial tumor resection.There were 11 males and 7 females with an average age of 45 years(range,16-72).Twelve cases were diagnosed as primary malignant tumor and 6 as metastases.Locations of tumors involved:the Ⅰ region 6 cases.the Ⅱ region 10 cases.and the Ⅲ region 2 cases.The exposures of all tumors were via a"T"type or ilioinguinal approach.The lesions were heated at 50℃ for 20 min by 2450 MHz microwave,with surrounding soft tissue protected by copper.mesh.The necrotic tumor tissues were total or partial excised after treatment,with preservation of the anatomical continuity of the pelvic ring.Results The duration of surgery was 60-180 min (110 min on average).The blood loss was 400-800ml(480 ml on average).All patients were followed-up for 0.5-7 years(3.5 years on average).Tumor local recurred in 1 case with chondrosarcoma,and was survival in tumor-bearing after 6 months follow up.One case with malignant fibrous histiocytoma died due to brain,pulmonary,and all body metastases.One case with osteosarcoma died due to pulmonary metastases.Five cases with the metastases died due to non-pelvis metastases.Functions of hip joint in 18 patients were as follows:flexion 80°-130°,extension 0°-10°,abduction 25°-35°,and adduction 18°-23°.Conclusion The clinical result demonstrated that the advantages of microwave heliotherapy in situ were quick increase of temperature,sensitive responses,easy control of temperature,and effective inactivation of tumor cells in the malignant bone tumors of pelvis.     

Duodenal villous tumors

The treatment of 36 patients with duodenal villous tumors was reviewed to determine the long-term outcome of various surgical treatment options for specific adenoma histopathology. Duodenal villous tumors were typically solitary and periampullary in location. Villous adenomas contained epithelial atypia in 30% of patients, in situ carcinoma in 14%, and invasive carcinoma in 33%. Treatment consisted of transduodenal submucosal excision in 19 patients and radical pancreaticoduodenectomy in 15. There was no perioperative mortality. Perioperative morbidity for transduodenal excision and pancreaticoduodenectomy was 16% and 47%, respectively. Benign adenomas recurred more than 5 years postoperatively in 17% of patients undergoing transduodenal excision. Five-year survival following radical resection for invasive cancers was 45%. Overall median follow-up was 5.8 years. We conclude that duodenal villous tumors without invasive cancer can be managed successfully by local submucosal excision, but invasive carcinoma requires radical resection.     

Large periampullary villous tumor of the duodenum

A 67-year-old woman, who had symptoms of epigastric pain and abdominal distension, was found, on endoscopy, to have a large sessile villous adenoma of the periampullary duodenum. Despite the lack of evidence of malignancy, a pancreaticoduodenectomy procedure was performed, mainly because of the tumor size and site, involving the ampulla of Vater. The presence of the carcinoma was diagnosed only in the resected specimen by definitive histology. Because there is no general consensus on the optimal surgical procedure for the treatment of villous tumors of the duodenum, especially for the early stages, the indications for the operative procedure are discussed, based on a review of the literature.     

原发性十二指肠肿瘤86例的诊断和治疗

目的:探讨原发性十二指肠肿瘤的诊断及治疗方法。方法:回顾分析26年间收治的86例原发性十二指肠肿瘤患者的资料，其中良性18例，恶性68例。诊断依赖于肠镜和影像学检查，治疗有赖于外科切除术。结果:临床表现包括胃肠道症状、胆道梗阻症状和非特异性的发热贫血及消瘦等。术前确诊率十二指肠镜为91.3％，气钡造影为88.5％，磁共振胰胆管造影为84.6％，B超为31.9％，CT为51.9％，磁共振为76.2％，ERCP为100%；联合十二指肠镜、气钡造影检查确诊率可达95.0％。71例发生于十二指肠降部。18例原发性十二指肠良性肿瘤全部被切除，5年生存率为92.3％；68例原发性十二指肠恶性肿瘤中35例行胰十二指肠切除，8例行十二指肠节段切除，切除率为63.2％；切除术后5年生存率为31.0％；25例行旁路手术，5年生存率为0。结论:原发性十二指肠肿瘤缺乏特异性症状，经内镜、气钡造影等联合检查，可提高术前阳性诊断率。对良性肿瘤可行十二指肠节段切除或局部切除，恶性肿瘤首选胰十二指肠切除术。     

Large villous adenoma of the colon in carcinomatous transformation: colonic resection after endoscopic excision

Large colonic villous adenomas are benign neoplasms capable of malignant transformation with a higher frequency than other adenomas. Such transformation often requires surgical therapy after endoscopic resection. The aim of the present study was to establish the indications for surgery in a series of 13 cases of large colonic villous adenomas initially submitted to endoscopic resection. The patients (8 males and 5 females; mean age; 62 years) were observed over the period 1993-2000. All endoscopic resections were performed using the piecemeal technique. In 7 cases there were 2 endoscopic sessions and in one case 3; a single case of post-endoscopic bleeding was treated conservatively. In 5 cases, endoscopic resection was deemed not to be radical and these patients were submitted to surgical resection. Histology on the surgical specimens revealed 2 cases of carcinoma (T1 and T2, respectively), confirmation of colonic villous adenoma in 2 cases and the presence of inflammatory tissue in 1 case. Among the patients treated with endoscopic resection alone one death occurred at two years due to lung and systemic metastases probably due to the malignant adenoma. After a review of the literature and on the basis of their own experience, the authors stress the importance of a combined pathological and endoscopic approach to establish when surgery is required.     

Clinical observations and the planning of therapy for sacrococcygeal tumors

Amongst sacrococcygeal tumors, teratoma is seen often while other histologic types are rare. It is apparent that the less obvious intrapelvic tumors can cause obstructive symptoms and are usually diagnosed late. Twenty cases of sacrococcygeal tumor, including 14 teratomas (9 benign, 5 malignant) and 6 others (3 benign, 3 malignant) were treated at Tokyo Medical College and Shizuoka Children's Hospital over the last 23 years. The majority of the benign tumors were readily excised, but one case of benign teratoma was difficult to excise and died of bleeding. The resectability of malignant tumors was low, but aggressive multiagent chemotherapy, radiotherapy and second look operation made it possible to increase the survival. One case is alive and has been disease free for 11 years. Recently combined therapy by chemotherapy and surgery has improved the survival rate although some problems still remain.     

乳腺典型髓样癌临床分析

目的:研究乳腺典型髓样癌的临床特征和预后。方法:分析发病年龄、肿块大小、部位、淋巴结转移与生存率关系。结果:本文58例典型髓样癌占同组女性乳腺癌的3.1%。平均发病年龄52.6岁,腋淋巴结转移率30.9%,五年和十年生存率分别为87.9%、85.2%。结论:典型髓样癌属较低度恶性肿瘤,预后较好。但也有个别病例死于脑转移。     

Villous Adenocarcinoma of the Duodenum Invading the Ampulla of Vater: Case Report and Review of Literature

We report a case of villous adenocarcinoma of duodenum arising from the ampulla of Vater with a review of the literature. Although preoperative endoscopic biopsies were performed, no malignancy was identified. Because of the pathological uncertainty we decided to perform a pylorus-preserving pancreatoduodenectomy. Microscopic examination demonstrated glandular dysplasia with aspects of villous adenoma and well differentiated adenocarcinoma. We conclude that both in malignant cases and in cases with uncertain diagnosis a pylorus-preserving pancreatoduodenectomy is the best surgical treatment because it results in better 5 year survival.