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.     

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.     

Villous adenoma of the urinary tract: a report of 23 cases, including 8 with coexistent adenocarcinoma.

Villous adenoma originating in the urinary tract is uncommon. We present the first study of a large number of cases of villous adenoma of the urinary tract with clinical follow-up. Our series consisted of 15 patients with isolated villous adenoma and 8 patients with coexistent adenocarcinoma. The tumors occurred in elderly patients and had a predilection for the urachus, dome, and trigone of the urinary bladder. The typical clinical presentation was hematuria and irritative symptoms, and endoscopic examination usually identified a tumor growth. There was no gender predominance. Light microscopic examination showed morphologic similarity to colonic villous adenoma in all cases. Each tumor was composed of pointed or blunt finger-like processes lined by pseudostratified columnar epithelium. The epithelial cells displayed nuclear stratification, nuclear crowding, nuclear hyperchromasia, and occasional prominent nucleoli and mitotic figures. There was intense carcinoembryonic antigen immunoreactivity on the luminal surfaces (89%). Most cases (78%) contained cytoplasmic acid mucin, demonstrated by Alcian blue periodic acid-Schiff stain. Cytokeratin 20 was positive in all cases, cytokeratin 7 was positive in 56% of cases, and epithelial membrane antigen was positive in 22% of cases. Recurrence or invasive adenocarcinoma did not develop in any patient with isolated villous adenoma during a mean follow-up of 9.9 years. Lung metastasis developed in one patient with coexistent adenocarcinoma and multiple recurrences in another (mean follow-up, 3 years). We conclude that the prognosis is excellent in patients with isolated villous adenoma, and complete surgical resection is curative. Patients with coexistent adenocarcinoma may experience recurrence or distant metastasis, and more aggressive treatment may be indicated.     

Villous adenocarcinoma of the colon and rectum: a clinicopathologic study of 36 cases

Some colorectal adenocarcinomas show villous architecture with morphologic similarities to tubulovillous or villous adenomas. We reviewed 420 consecutive colorectal adenocarcinoma resection specimens and found that 95 tumors (23%) showed areas of villous architecture. Thirty-six tumors (8.6%) in 35 patients showed more than 50% villous architecture and were designated villous adenocarcinomas. Only 42% of the villous adenocarcinomas showed severe atypia and only 44% of the available pre-resection biopsies of these tumors were diagnosed as adenocarcinoma. Epithelial islands in desmoplastic stroma (EIDS) may be helpful in the diagnosis of these tumors. EIDS were found in 97% of the resection specimens for villous adenocarcinomas and none of 62 resection specimens for tubulovillous or villous adenomas. The presence of EIDS showed a 67% sensitivity, 100% specificity, and 100% predictive value in the diagnosis of villous adenocarcinoma in a blinded review of villous tumors. On review of the pre-resection biopsies of villous adenocarcinoma without a final diagnosis of adenocarcinoma, 40% showed EIDS. Clinical follow-up of the 35 patients with villous adenocarcinoma showed that only one died of colorectal adenocarcinoma (median follow-up, 46 months). This sole patient dying of colorectal adenocarcinoma showed a synchronous advanced stage of nonvillous adenocarcinoma at the time of diagnosis. Villous adenocarcinoma is a diagnostically challenging subset of colorectal adenocarcinoma, which appears to be associated with a favorable prognosis. Classifying these tumors as a special type of colorectal cancer may facilitate the development of diagnostic adjuncts and optimal treatment protocols.     

Results of surgical treatment of periampullary tumors: a thirty-five-year experience

Data on 126 consecutive patients with periampullary tumors resected at the Cleveland Clinic between January 1950 and December 1984 were reviewed. One hundred five patients underwent pancreatoduodenal resection, 10 patients total pancreatectomy, and 11 patients local resection of the tumor. The site of tumor was ampulla of Vater (59), head of the pancreas (30), duodenum (20), and distal common bile duct (11). Six patients had benign disease. The operative mortality rate for radical resection for the entire period was 7.8%; it has declined to 5.4% since 1974. The operative mortality rate for local resection was 9.1% (one patient). The overall 5-year survival rate for all malignant tumors of the periampullary area was 28% and 25.5% for invasive adenocarcinoma. Survival was affected primarily by location and histologic findings. The 5-year survival rate for adenocarcinoma of the ampulla of Vater was 37.2%, 27.5% for the duodenum, 16.7% for the distal common bile, and 4.3% for the pancreas (p = 0.0001). Papillary adenocarcinoma had a 5-year survival rate of 49.2% in contrast to 18.4% for nonpapillary ductal adenocarcinoma (p = 0.002). Patients with ampullary adenocarcinoma treated by local resection had a 5-year survival rate of 40.9%. These data justify continued use of a selective radical approach in the resection of most periampullary tumors with local resection for small tumors in high-risk patients.     

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.     

Adenome der Ampulla Vateri und des Duodenums Darstellung von Diagnostik und Therapie durch endoskopisch interventionelle und chirurgische Methoden

Background. Adenoma of the ampulla of Vater and the duodenum are rare tumors. The leading symptoms are unspecific; sometimes they are due to the disturbances of the passage or erosion of the tumor. Early and correct diagnosis and the appropriate therapy are of decisive importance because these tumors are precancerous. Patients and methods. We report the cases of seven patients with periampullary adenoma of the duodenum, in one case with incorporation of the the distal bile duct. The diagnostic steps and the surgical treatment are reported and discussed. In achieving a definitive diagnosis, endoscopic retrograde cholangiopancreatography is of paramount importance. Three patients subsequently underwent partial pancreatoduodenectomy (Whipple's procedure); in three patients transduodenal local tumor excision was performed. Postoperative course was without complications in each case and no operative mortality occurred. One patient was treated by argon-coagulation. Results. The histopathological examination showed a tubular villous adenoma in six cases and in one case a villous adenoma (ranging from low to high grade dysplasia). Because there is evidence of an adenoma-carcinoma-sequence, radical surgical treatment is recommended, even in adenoma with low grade dysplasia, in spite of the absence of malignancy.     

原发性十二指肠恶性肿瘤的诊断与治疗:附82例报告

目的　探讨原发性十二指肠恶性肿瘤的临床表现、病理学特征及诊治手段。方法　对10年间收治的经病理证实的 82例原发性十二指肠恶性肿瘤患者的临床资料进行回顾性分析。结果　发病部位 :乳头区 64例 ,降段 11例 ,其他部位 7例。临床主要表现为上腹疼痛 (5 7例 ) ,黄疸 (5 3例 )和消化道出血 (4 1例 )。病理学类型以腺癌多见 (72例 ) ,胃肠道间质瘤 (5例 ) ,恶性淋巴瘤 (3例 )次之。 3 6例行肿瘤根治性切除术 ,3 1例行姑息性手术 ,15例未进行外科治疗。本组病例随访患者 5a生存率 2 .4%。结论　原发性十二指肠恶性肿瘤临床以上腹痛、黄疸、消化道出血为主要表现 ,但缺乏特征性。病理类型以腺癌为主 ,好发于十二指肠乳头区及降段。CT和B超及内镜检查是诊断检查原发性十二指肠恶性肿瘤的主要手段 ,手术切除是主要治疗方法。原发性十二指肠恶性肿瘤的预后甚差。     

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.     

原发性十二指肠恶性肿瘤的诊断和治疗：附54例报告

目的：探讨改善原发性十二指肠恶性肿瘤的诊断和治疗方法。方法：回顾性分析6年间收治原发性十二指肠恶性肿瘤54例的临床资料。结果：肿瘤位于球部6例（11.1%）、降部44例（81.5%），其中乳头部38例（占降部的86.4%，总数的70.4%），水平部2例（3.7%），升部2例（3.7%）。腺癌50例（92.6%）。主要症状有黄疸、上腹部痛、体重下降伴纳差、腹胀、消化道梗阻等。上腹部压痛72.0%，无阳性体征20.0%，腹块8.0%。术前合并胆囊病变率37.0%。术前经十二指肠镜、ERCP确诊率为94.4%及77.8%。术前确诊为原发性十二指肠癌15例（27.8%）,胰十二指肠切除38例，节段性十二指肠切除1例，姑息性手术9例，失去手术时机6例。手术切除率88.9%，根治性切除率72.2%。行胰十二指肠切除3年和5年生存率分别为40.6%和21.9%;姑息性手术3~24个月死亡，未行手术者半年内死亡。结论：十二指肠恶性肿瘤以降部乳头最多见，以腺癌为主，进展期症状复杂，腹部体症特异性表现低，易合并胆囊病变，术前确诊率低。十二指肠镜和ERCP是诊断的首选方法。胰十二指肠切除可延长生存期。     

Adenocarcinoma of the small intestine

Sixty-five patients with adenocarcinoma of the small intestine were encountered over a 31 year period. The duodenum was the most common location, with a decreasing frequency distally. Associated malignancies were present in a fourth of the patients. Presenting signs and symptoms were vague and related to either obstruction or bleeding. Barium contrast examination and endoscopy for duodenal tumors were the primary diagnostic modalities. Curative treatment was wide resection of bowel and mesentery for jejunal and ileal tumors and pancreaticoduodenectomy for duodenal tumors. Favorable prognosticators included jejunal location, absence of nodal metastases, and a well-differentiated grade. Stage for stage, the prognosis of patients with adenocarcinoma of the small intestine parallels that of patients with adenocarcinoma of the colon. With greater awareness of this tumor, it is possible that earlier detection will lead to improved overall survival.     

Villous adenoma of the duodenum: A case report and review of the literature

A case of villous adenoma of the duodenum, with focal in situ carcinomatous changes, has been described with a review of forty-two other case reports from the world literature. Occult bleeding, resulting in anemia, and vague obstructive symptoms appear to be the most common presenting findings. The average age was 56.4 years, which was seven years younger than the average age for villous tumors of the colon. Adequate radiologic studies should establish the diagnosis preoperatively. These tumors obtain relatively large size before causing significant symptoms. Approximately one third showed carcinomatous changes, and approximately one half of these were in situ changes. Local segmental resection for duodenal villous tumors is desirable when possible. However, in areas where this is not feasible, local mucosal excision is acceptable for benign tumors and for those with in situ carcinoma. If invasive carcinoma is found in the excised specimen, pancreatoduodenectomy is recommended. Insufficient evidence is available to adequately evaluate survival for malignant villous tumors of the duodenum, but the available data suggest that the survival after treatment of malignant villous tumors is comparable to other malignant lesions originating in the duodenum.     

Treatment of polyps and villous tumors of the large intestine

Polyps and villous tumors were found in 1525 of 10624 patients (14.3%) aged from 40 to 60. In 82% of the cases they were in combination with ulcer disease of the stomach and duodenum, with inflammatory diseases of the gastro-intestinal tract, diverticulitis, tumors of the colon. 1519 polypectomies and dissection of villous tumors both via the fibroscope, rectoscope and by transanal access were made. Complications were obtained in 5 patients, recurrent disease was noted in 3 patients (0.9%).     

Surgical treatment of villous tumors of the rectum and sigmoid. Apropos of 79 cases

The authors analyse a series of 79 villous tumors of the rectum and sigmoid. These tumors are observed more often in men at an average age of 65. They distinguish benign tumors (44 cases), premalignant (21 cases) and malignant tumors (14 cases). The histology of the whole piece has a greater value than a preoperative biopsy. There are many clinical symptoms and the forms with electrolyte depletion are not rare (6 cases). Tumors with wide implantation need a surgical treatment. It will be conservative in benign cases, but the excision will be wide in cases of invasive malignancy. The indication are based upon the localisation of the tumor on the rectum. Mortality of wide excision is quite important (10) because of the bad general feature of the patients. Recurrences must be detected early by regular endoscopic follow-up.     

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.     

Villous tumors of the duodenum.

Five cases of villous tumors of the duodenum are reported, all of which involve the ampulla of Vater. Three of the five lesions contained either infiltrating carcinoma or carcinoma in situ. Although preoperative endoscopic biopsy was performed on all tumors no malignancy was identified. Frozen sections done at the time of operation on the three patients with carcinoma also failed to identify malignancy. One patient underwent pancreaticoduodenectomy and four patients had local excision of the tumor. Three of the patients treated with local excision developed recurrence and two subsequently had pancreaticoduodenectomy. Because of the difficulty in making an accurate diagnosis and the chance of recurrence when local excision is employed, strong consideration should be given to pancreaticoduodenectomy as the initial form of treatment of these lesions.     

Primary adenocarcinoma of the bladder: favorable prognostic significance of deoxyribonucleic acid diploidy measured by flow cytometry

Flow cytometric nuclear deoxyribonucleic acid ploidy analysis was done successfully on 38 specimens of primary bladder adenocarcinoma treated between 1954 and 1985. Of the specimens 10 (26%) were deoxyribonucleic acid diploid, 8 (21%) were tetraploid and 20 (53%) were aneuploid. Distribution of ploidy patterns between the 14 histological low grade and 24 high grade tumors was similar. Of 38 tumors 35 (92%) showed muscle invasion. One tumor arose in a previously exstrophied bladder, 10 were of urachal origin and 27 arose in an anatomically normal bladder. Of the urachal origin tumors 80% were deoxyribonucleic acid aneuploid. At 5 and 10 years after diagnosis 80 and 70%, respectively, of the patients with diploid tumors were free of disease. By contrast, at 5 and 10 years after treatment only 20 and 12%, respectively, of the patients with nondiploid tumors have not had disease progression (p less than 0.001 log-rank test). None of the 6 patients with diploid, high grade, high stage, muscle invasive tumors had subsequent progression. In contrast, 16 of 17 patients (94%) with high grade, high stage, nondiploid tumors had either local or distant tumor recurrence (p less than 0.0005). Nuclear deoxyribonucleic acid ploidy pattern appears to be the most significant prognostic information currently available to stratify expected prognosis for patients with muscle invasive adenocarcinoma of the bladder. This test probably should be a standard tool in the clinical management of patients with this rare bladder malignancy.     

原发性十二指肠肿瘤的诊断与治疗(附22例报告)

目的探讨原发性十二指肠肿瘤的诊断和选择适当的手术方法。方法对２２例原发性十二指肠肿瘤的临床资料进行分析。结果十二指肠气钡造影１３例,１０例（７６９％）诊断肿瘤;十二指肠镜检查１４例,发现肿瘤１３例（９３％）。肿瘤位于十二指肠降部者１１例,占５０％。良性肿瘤８例,恶性肿瘤１４例。恶性肿瘤中行胰十二指肠切除６例,有２例生存已达６年。良性肿瘤７例行肿瘤及局部肠管切除,已有２例生存１２年。结论内窥镜及气钡双重造影检查是诊断本病的主要方法,手术是其重要的治疗手段。     

Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases

The classification of appendiceal mucinous tumors is controversial and terminology used for them inconsistent, particularly when they lack overtly malignant features but are associated with extra-appendiceal spread. We reviewed 107 appendiceal mucinous neoplasms and classified them as low-grade appendiceal mucinous neoplasm (LAMN) (n = 88), mucinous adenocarcinomas (MACAs) (n = 16), or discordant (n = 3) based on architectural and cytologic features. LAMNs were characterized by a villous or flat proliferation of mucinous epithelium with low-grade atypia. Thirty-nine tumors were confined to the appendix, but 49 had extra-appendiceal tumor spread, including 39 with peritoneal tumor characterized by mucin pools harboring low-grade mucinous epithelium, usually dissecting in a hyalinized stroma. Eight of the 16 MACAs lacked destructive invasion of the appendiceal wall and eight showed an infiltrative pattern of invasion. Extra-appendiceal tumor spread was present in 12 MACAs (four peritoneum, seven peritoneum and ovaries; one ovaries only). In MACAs with an infiltrative pattern, peritoneal tumor consisted of glands and single cells in a desmoplastic stroma. The peritoneal tumor in the remaining cases consisted of mucin pools that contained mucinous epithelium with high-grade atypia and, in some cases, increased cellularity compared with that seen in peritoneal spread in cases of LAMN. Three cases were classified as discordant because the appendiceal tumors were LAMNs but the peritoneal tumors were high-grade. Follow-up was available for 49 LAMNs, 15 MACAs, and 2 discordant cases. None of the patients with LAMNs confined to the appendix experienced recurrence (median follow-up 6 years). LAMNs with extra-appendiceal spread were associated with 3-, 5-, and 10-year survival rates of 100%, 86%, and 45%, respectively. Patients with MACA had 3- and 5-year survival rates of 90% and 44%, respectively (p = 0.04). The bulk of peritoneal disease correlated with prognosis among patients with MACA (p = 0.04) and, to a lesser degree, among patients with LAMNs (p = 0.07). We conclude that: 1) appendiceal mucinous neoplasms can be classified as either low-grade mucinous neoplasms or mucinous adenocarcinoma based on architectural and cytologic features; 2) tumors that can be confidently placed in the low-grade group (which requires rigorous pathologic evaluation of the appendix) and are confined to the appendix are clinically benign in our experience to date; 3) low-grade tumors confined to the appendix are morphologically identical to those with extra-appendiceal spread (except for the usual identification of breach of the wall in the latter cases) and the same designation is appropriate for the appendiceal neoplasia in each situation; 4) the long-term outlook for patients with low-grade tumors and peritoneal spread is guarded with just over half dying of disease after 10 years; 5) appendiceal mucinous tumors with destructive invasion of the appendiceal wall, complex epithelial proliferations, or high-grade nuclear atypia generally pursue an aggressive clinical course and should be classified as mucinous adenocarcinomas; 6) peritoneal tumor can be classified as involvement by LAMN or MACA, and this distinction is of prognostic significance; 7) bulky peritoneal tumor worsens prognosis; and 8) LAMNs associated with high-grade peritoneal tumor behave as adenocarcinoma.