Microcephalic osteodysplastic primordial dwarfism Taybi-Linder type: report of four cases and review of the literature

Although lymph node metastasis is a major prognostic factor in gastric cancer, the optimal extent of lymph node dissection still remains a subject of debate. The influence of extended D2 lymphadenectomy on morbidity and long-term survival is controversial. Reports from many Japanese and some Western institutions show similar morbidity and mortality rates for both limited D1 and extended D2 resections. However, the four available randomised trials show a significant increase in operative morbidity and mortality after a D2 resection. The authors of these trials believe that distal pancreaticosplenectomy is responsible for this increased morbidity and mortality and not the lymphadenectomy itself. Retrospective and prospective non-randomised studies show superior stage (II/IIIA) specific survival rates after D2 resections. However, these studies did not eliminate stage migration and randomised trials failed to show any survival advantage in favour of the D2 resection. Current data suggest that D2 resection is beneficial to the subgroup of patients with N1 or N2 disease undergoing potentially curative resection. However, Western studies that support D2 resection, fail to show any survival advantage for D2 resection in N2 patients, reporting a benefit only to N0 or N1 patients. In contrast, Japanese series report a large number of N2 long-term survivors. The question as to the possible beneficial effect of extended lymphadenectomy in gastric cancer is difficult and complex. D2 resection increases the potentially curative resection rate, at least in N2 patients, achieves a better locoregional tumour control and provides the only chance for cure among N2 patients since adjuvant treatment in gastric carcinoma has not yet been proved effective. However, all randomised comparisons warn of an increased risk after D2 resection. By avoiding pancreaticosplenectomy, however, the morbidity can be within acceptable limits. D2 gastrectomy seems to be the most attractive procedure in the surgical management of gastric cancer.     

Clinicopathologic features of submucosal carcinoma of the stomach

A retrospective study of 155 patients with submucosal gastric carcinoma compared the clinicopathologic features with mucosal and muscularis proprial gastric carcinoma. Fifty-seven percent of the patients presented with gastrointestinal symptoms, whereas 36.1% had been detected by mass screening. The incidence of curative resection, lymph node metastasis, and complications were 96.1, 20.6, and 14.8%, respectively. Two patients died of sepsis and pulmonary infarction 30 days post-operatively. Five patients died of recurrent gastric cancer 1-5 years postresection. The overall 5-year survival rate was 90.2%. Recurrence patterns, histologic type, lymph node metastasis, lymphatic and venous infiltration, and growth pattern were similar to those of muscularis proprial carcinoma rather than mucosal carcinoma. Therefore, curative gastrectomy with extended lymphadenectomy (D2) may be feasible for submucosal carcinoma of the stomach.     

Lack of survival benefit of extended lymph node dissection for ductal adenocarcinoma of the head of the pancreas: retrospective multi-institutional analysis in Japan

It has not been established that extended lymph node resection is necessary for ductal adenocarcinoma of the head of the pancreas. According to the general rules for the study of pancreatic cancer, a multiinstitutional, retrospective clinical study was undertaken to investigate the efficiency of extended lymph node dissection for this malignancy. Altogether 501 patients underwent resection of the pancreas between 1991 and 1994 at 77 medical facilities; the surgical procedures, staging, lymph node dissection, curability, and survival rate were analyzed retrospectively. Eighteen of the patients died within 30 postoperative days, leaving 483 patients to be studied. The resection was curative microscopically in 94 patients, resulting in a 3-year survival of 29%. Macroscopically curative resection resulted in a 3-year survival of 14%; noncurative resection produced a 3-year survival of 6%. Although extended lymph node dissection was performed on 38 patients in stage I, 42 patients in stage II, 206 patients in stage III, and 1 patient in stage IV, there was no improvement in survival when the results were compared to those seen after standard or palliative lymph node dissection. The extent of lymph node dissection has not affected the prognosis for ductal adenocarcinoma of the head of the pancreas at any stage of the course of the disease. Excessive lymph node dissection in advanced cases does not necessarily lead to a favorable prognosis. The patients who undergo a radical operation with an adequate lymph node dissection have longer survivals.     

Prognostic significance of lymph node dissection in gastric cancer

The results for 162 patients who underwent curative gastrectomy for gastric cancer from January 1988 to June 1994 were analysed statistically with special reference to the effect of lymph node dissection. Median survival was 69.3 months and the overall cumulative 5-year survival rate was 50.2 (95 per cent confidence interval (c.i.) 41.6-58.1) per cent. By univariate analysis age, histology, depth of tumour invasion, node involvement, number of metastatic lymph nodes and type of lymphadenectomy were found to be significant factors related to survival time. Multivariate analysis with the Cox model and stratified for tumour node metastasis stage revealed that only the number of metastatic nodes (P = 0.04) and the extent of lymphadenectomy (P = 0.003) affected survival independently. With respect to D1 lymphadenectomy, the relative risk associated with D2 and D4 lymphadenectomy was respectively 0.61 (95 per cent c.i. 0.34-1.10) and 0.26 (95 per cent c.i. 0.12-0.60). The 5-year survival rate was 28 per cent for patients who had a D1 dissection, 63 per cent for those who had D2 and 68 per cent for those who had D4. These results suggest that extended lymphadenectomy (D2) and especially superextended lymphadenectomy (D4) can improve survival in patients with gastric cancer.     

Inadequate documentation and resection for gastric cancer in the United States: a preliminary report

Patients entered into Southwest Oncology Group gastric adjuvant protocol INT 0016 (SWOG 9008) after a "curative" gastric resection were assessed to determine practice patterns of more than 300 surgeons nationwide who performed "curative" gastric resections for 453 gastric cancer patients. The most common gastric resection performed was distal in 256 patients, proximal in 118, and total in 79. Extragastric organs resected were omentum (285), spleen (59), pancreas (18), and bowel (17). The extent of lymphadenectomy as staged by Japanese rules was 246 (54.2%) D0 resections, 173 (38.1%) D1 resections, 28 (6.2%) D2 resections, and 7 (1.5%) D3 resections. Staging of the cancer was poorly documented, with no statement made regarding the status of the primary cancer in 6 per cent, liver in 10 per cent, lymph nodes in 17 per cent, and omentum in 17 per cent. The greater the lymph node clearance, the greater the chance of resecting to a level of negative lymphatics, given that 45 per cent of nodes were involved when 10 or less were removed, whereas only 17 per cent were positive when more than 40 were cleared. The lack of adequate clearance of lymph nodes and poor documentation of tumor stage suggests that a more regimented surgical approach to this uncommon cancer is required.     

Surgical treatment of stomach cancer: analysis of our personal experience

The "curative" treatment of gastric carcinoma includes the complete removal of the tumour and of the nodes involved without any macroscopic residual of disease (RO). Out of 326 patients with gastric cancer observed, a series of 114 consecutive patients underwent surgical resection (total gastrectomy or subtotal distal gastrectomy) with D2 or D3 lymphadenectomy. Overall operative mortality was 5.3%. Since 1988 no postoperative death occurred. Overall morbidity was 15.8%, specific morbidity 10.5%, reduced after 1988 to 6.6%. No significant differences in operative mortality and need of blood transfusions were recorded between D2 and D3 lymphadenectomy. Overall 5-year survival was 32%. Univariate and multivariate analysis showed that only T and N stages are significant prognostic factors, whereas tumour location, total or subtotal gastrectomy in antral cancers, extent of lymphadenectomy (D2 vs D3) and histology were not significantly related to survival. Since most studies have clearly shown that T and N stages are the most important prognostic factors in gastric cancer, the present aim should be to plan the extent of surgical resection according to the T and N stages characteristics of the neoplasm.     

Importance of extended lymphadenectomy with lateral node dissection for advanced lower rectal cancer

A total of 448 patients with advanced lower rectal cancer who underwent curative wide lymphadenectomy with autonomic nerve preservation were reviewed with respect to surgical techniques, operative burdens, node status, survival rate, and mode of recurrence. Operative time and blood loss in patients who underwent lateral dissection were much greater than those encountered with conventional resection. According to the direction of lymphatic spread in patients with Dukes C disease, the incidence of upward spread was 94% and lateral spread 27%. The overall incidence of lateral metastasis was 14%. The overall 5-year survival was 70%. According to the Dukes classification, the 5-year survival rates were 92% for Dukes A, 79% for Dukes B, and 55% for Dukes C, whereas it was 43% in patients with lateral node metastasis. An analysis of the survival rate was carried out with regard to the number of node metastases, direction of lymphatic spread, and autonomic nerve preservation. The overall incidence of local recurrence was 9.3% and amounted to 16.0% in patients with Dukes C disease. The case of advanced lower rectal cancer was characterized by positive lymph nodes or circular lesions around the circumference (both diagnosed by endorectal ultrasonography). We recommend extended lymphadenectomy with lateral node dissection, as it preserves the autonomic nerve.     

Results of resection of gastric cancer with distant metastases

BACKGROUND/AIMS: The present study was carried out in order to examine the outcome of resection in cases of gastric cancer with distant metastases. METHODOLOGY: The survival rates of two hundred and eighty-one patients who had undergone resection for primary carcinomas of the stomach, and who had distant metastases according to the TNM classification, were studied. RESULTS: The 5-year survival rates for patients with metastasis to the peritoneum or group 3 nodes were 8.9% and 15.3% respectively and were significantly higher than the survival rates for patients with metastasis to the liver (0%), to group 4 nodes (2.2%) or to more than one site among the liver, lymph nodes and peritoneum (3.5%). Moreover, the 5-year survival rates for patients with metastasis to the peritoneum and N3 nodes increased significantly to 29.4% and 24.2%, respectively, when curative surgery was performed. CONCLUSIONS: The findings of the present study suggests that metastases to the adjacent peritoneum or group 3 nodes have a greater chance of being cured using radical surgery, and that gastrectomy with extended lymphadenectomy (D2-D3) may be used for advanced gastric cancer if there is no gross evidence of metastasis to the distant peritoneum, liver or group 4 nodes.     

Prognostic relevance of systematic lymph node dissection in gastric carcinoma. German Gastric Carcinoma Study Group

In a prospective multicentre study of 2394 patients with gastric carcinoma the prognostic relevance of systematic lymph node dissection was evaluated. Of 1654 patients undergoing resection, 558 had a standard lymph node dissection, defined as fewer than 26 nodes in the specimen, and 1096 underwent radical lymphadenectomy, i.e. 26 or more nodes in the specimen. Radical dissection significantly improved the survival rate in patients with Union Internacional Contra la Cancrum (UICC) stages II and IIIA tumours. Multivariate analysis identified radical dissection as an independent prognostic factor in the subgroups of patients with UICC tumour stages II and IIA. Radical dissection conferred no survival advantage in patients with pN2 tumours. There was no significant difference in morbidity and mortality rates between radical and standard lymph node dissection. Radical lymphadenectomy improves survival in patients with UICC gastric cancer stages II and IIIA, and should be the recommended treatment for such patients.     

Characteristics and clinical outcome of proximal-third gastric cancer

BACKGROUND: It is generally accepted that the prognosis of patients with proximal gastric cancer (PGC) is worse than that of patients with more distal gastric cancer. STUDY DESIGN: The aim of this study was to compare the clinical features and outcomes of PGC with those of middle- and distal-third gastric cancers. A total of 646 primary gastric cancers was analyzed as a retrospective study. RESULTS: Proximal gastric cancer occurred in 21.8% of the 646 cancers analyzed, and approximately 21% of PGCs had esophageal invasion. The 5-year survival rate for patients with PGC was significantly lower than that of patients with more distal tumors. When the PGC group was divided into patients with esophageal invasion and without esophageal invasion, patients with esophageal invasion had significantly worse outcomes. When corrected for depth of invasion, lesions with esophageal invasion had significantly worse outcomes than those of other sites in T2 curative cancers. Proximal gastric cancer with esophageal invasion was characterized by a larger tumor, deeper penetration, and a higher incidence of lymph node metastasis compared with tumors in other sites, and in multivariate analysis of all curative cases, these variables were independent prognostic factors for survival. The frequency of positive proximal margins of PGC was higher than those of other sites. CONCLUSIONS: The relatively poor prognosis associated with PGC is mainly from advanced tumor stages of esophageal invasion. Early detection is the most important strategy to improve the survival of patients with PGC. In addition, aggressive lymph node dissection and chemotherapy for esophageal invasion should be considered even if the tumor invasion is moderate (T2 tumor), and a tumor-free margin is important.     

Extended radical pancreatectomy for carcinoma of the head of the pancreas

BACKGROUND/AIMS: Pancreatic cancer has a poor prognosis, which is, in part due, to the unfortunately advanced stage, in which the tumor is diagnosed. Since 1973, we have utilized a unique method of extended radical pancreatectomy, using the translateral retroperitoneal approach (TRA) to facilitate combined portal resection. The advantages of this operation are described herein, for patients with carcinoma of the head of the pancreas. In addition, the problems associated with this operation are discussed. METHODOLOGY: Survival was calculated based on type of resection, degree of invasion of the retroperitoneal tissues, degree of lymph node involvement, and cancer stage. Extensive surgery has been performed for pancreatic carcinoma 216 patients. Of these, 14 patients had carcinoma of the head of the pancreas. There were 58 patients who underwent macroscopically curative resections. RESULTS: Only 39 patients were microscopically curative. Ten of the patients who underwent microscopically curative resections, survived for 5 years (34.0%). There were no statistically significant differences in survival based on tumor size. However, there was a significant difference in survival based on extent of invasion of the anterior capsule of the pancreas, extent of invasion of the retroperitoneal tissue, extent of lymph node involvement, cancer stage, and extent of invasion at the surgical margin of resection. CONCLUSION: The results suggest that extended radical pancreatectomy may be indicated for the treatment of cancer of the head of the pancreas.     

Stomach cancer. A 10-year material with emphasis on early cancer

Between 1982 and 1991, 112 patients were treated for gastric cancer at Harstad Hospital in Northern Norway. Early gastric cancer accounted for 20% of all the adenocarcinomas (110), which is high compared with figures from other western studies. The role of endoscopy for detecting early gastric cancer is discussed. The five year survival rate after radical surgery was found to be significantly higher for patients with early gastric cancer than for patients who underwent curative surgery for either infiltrative or regional cancer. For patients with a tumour that had invaded the regional lymph nodes the five year survival rate was only 10%. This result can be improved by more extended lymph node dissection. The perioperative mortality rate (8%) was usually a result of severe cancer cachexia or underlying cardial disease, and not anastomotic leakage. The results as regards the cure of early gastric cancer are comparable with those described in other studies.     

A case report of resected primary duodenal carcinoma associated with early gastric cancer and cumulative results at 21 institutions in Japan

A case of primary duodenal carcinoma simultaneously associated with an early gastric cancer is reported. A 72-year-old woman complaining of appetite loss and nausea was admitted in June 1988. Endoscopic examination showed an ulcerative lesion in the angle of the stomach and a Borrmann type 2 tumor in the bulb of the duodenum. Both lesions were revealed to be adenocarcinomas by histological examination of obtained biopsy specimens. Synchronous carcinoma was diagnosed and pancreatoduodenectomy and lymph node dissection were performed. The primary tumor of the duodenum was histologically a moderately differentiated adenocarcinoma, and the gastric cancer was a tumor limited to the mucosa. Metastasis was recognized in a regional lymph node (No. 14A). There has been no recurrence during the 4-year postoperative follow-up period. This result suggests that pancreatoduodenectomy with systematic regional lymph node dissection can greatly contribute to prolonging the survival of patients with advanced duodenal cancer. This case is very rare, in that curative operation was performed for a primary duodenal carcinoma simultaneously associated with an early gastric cancer.     

A rational treatment of stage I epithelial ovarian cancer

OBJECTIVE: To evaluate the incidence of lymph node metastasis and the role of lymphadenectomy in stage I epithelial ovarian cancer. METHODS: Forty patients with stage I epithelial ovarian cancer treated from 1985 to 1990, were divided into two groups and retrospectively analyzed. First group of 20 patients were treated by routine surgery and cis-platinum based chemotherapy. Second group of 20 patients were treated by routine surgery and cis-platinum based chemotherapy plus retroperitoneal lymphadenectomy, and on the basis of with normal ovary and uterus preserved in the younger stage I a patients. A comparison was made between the five-year survival rates of the two groups. RESULTS: Four patients in the second group were found to have retroperitoneal lymph node metastasis and should be staged as II c postoperatively. In three of the four patients aortic lymph node metastasis were diagnosed. The chances of metastasis to the pelvic and to the aortic lymph nodes were nearly equal. There is a significant difference in the 5-year survival rates between the two groups (85% vs 100%, P < 0.05). Ten patients with their ovaries and uteri preserved are living and well. CONCLUSION: It is suggested that to obtain accurate FIGO staging and to improve survival and its quality, retroperitoneal lymphadenectomy should be performed in all patients with stage I eipthelial ovarian cancer, and younger patients with stage I a cancer may preserve their gestational functions if desired.     

Chronologic changes in the clinicopathologic findings and survival of gastric cancer patients

PURPOSE: To determine the chronologic changes in the clinicopathologic features of gastric cancer patients. PATIENTS AND METHODS: The clinicopathologic findings of 1,795 patients with gastric cancer were examined retrospectively from hospital records obtained between 1969 and 1995. The patients were divided into three generations on the basis of chronologic order. The first generation included patients treated over the period 1969 to 1977; the second generation, 1978 to 1986; and the third generation, 1987 to 1995. RESULTS: The chronologic changes in the clinicopathologic findings for all gastric cancers included increases in the superficial type based on macroscopic appearance (P < .005), small-sized tumor (P < .025), superficial depth of invasion (P < .005), and earlier histologic stages (P < .005), in addition to a decrease in lymph node metastasis (P < .005). Overall, the postoperative survival rate has improved over time in gastric cancer patients, with 5-year survival rates of 36.0%, 53.3%, and 68.6% in the first, second, and third generations, respectively. In stages 1,2, and 3, the survival rate in the third generation was the highest of the three generations, whereas in stage 4, the survival rate did not differ between the three generations. Patients who underwent a D2 dissection showed a higher survival rate than those with D1 or D3 dissections, but there was no statistical difference in the survival of patients with D1, D2, and D3 dissections when stage 4 patients were excluded. CONCLUSION: The chronologic changes in gastric cancer patients over the past 27 years have included an increase in the incidence of earlier-staged gastric cancers, which has had a significant impact on the improved postoperative survival rate.     

Surgical treatment for advanced carcinoma of the gastric remnant

BACKGROUND/AIMS: Carcinoma of the gastric remnant has increased in recent years, but a therapeutic strategy for this disease has not been established. This retrospective study was performed to determine the most appropriate surgical procedure for carcinoma of the gastric remnant. METHODOLOGY: A total of 25 patients who underwent operation for advanced carcinoma of the gastric remnant that had developed after distal gastrectomy (13 for benign gastric diseases, B group; 12 for gastric carcinoma, M group) were studied. Clinicopathological features, as well as the status of lymph node metastasis, were investigated in the B and M groups. RESULTS: There were more patients with carcinoma invading other organs, stage IV disease, and with N2 or more lymph node metastasis (especially, with a high metastatic rate to lymph nodes along the splenic artery) in the M group than in the B group. Forty percent of patients in the M group were treated by left upper abdominal evisceration (LUAE), but only 8% in the B group. The survival rate (5-year, 46.0%) of the B group was significantly higher than that (5-year, 11.9%) of the M group. When we compared the survival rate of carcinoma of the gastric remnant with that of primary carcinoma of the upper third of the stomach, there was no difference between the two groups in the curative resection cases. CONCLUSIONS: Almost the same surgical strategy can be adopted for the B group as for primary gastric carcinoma. On the other hand, for the M group, a radical surgical procedure, LUAE, should be recommended.     

Our experience with lymphadenectomy in gastric cancer surgery

The Authors in this work study a comparison between 27 patients who underwent total gastrectomy with "regional" lymphadenectomy for gastric cancer during the period 1986-1991 (Group A), and 27 patients who underwent total or sub-total gastrectomy associated to D2 or D3 lymphadenectomy (Group B) according to the rules of the Japanese School (localization of the neoplasia and node involvement). No statistically relevant differences were shown in the overall long term survival, although in the group B there were both an high number of patients with stage III neoplasia and more invasive carcinomas. Extended lymphadenectomies, regional and D2 or D3, gave good results as far as long term survival was concerned in early stage cancers, but the same success was not achieved in advanced cancers especially in stage III. In order to improve the survival in advanced neoplasias since one year a D4 lymphadenectomy is performed in T2 or T3 and/or N2+ cases.     

Staging and management of lung cancer: sleeve resection

Sleeve lobectomy is a lung-saving procedure usually indicated for central tumors for which the alternative is a pneumonectomy. It preserves normal lung tissue and may enable pulmonary resection to be done in selected patients with inadequate cardiac or pulmonary reserve. One experience extends from January 1972 to December 1991, during which time 142 patients underwent a variety of sleeve resections for bronchogenic neoplasms. The majority of operations were upper-lobe sleeve resections (N = 110) and most procedures were considered complete and potentially curative (87%). There were three postoperative deaths (surgical mortality of 2.5%) and prolonged atelectasis was the most common major complication (N = 9). Follow-up was complete for the 139 survivors (mean follow-up time of 2,149 days) and overall survival was 46% at 5 years and 33% at 10 years. Five- and 10-year survivals for patients with stage I disease were 63% and 52%, respectively, while only 14% of patients with stage III disease survived 5 years. Local recurrences occurred in 23% of patients but when the resection had been complete, this incidence was 17% (21/124). These results indicate that sleeve resection is an adequate cancer operation for both compromised and uncompromised patients. Operative mortality, survival, and incidence of local recurrence are not different than what is seen after more conventional procedures.     

Systematic pelvic and paraaortic lymphadenectomy for advanced ovarian cancer: prognostic significance of node metastases

A retrospective study of 488 patients with untreated advanced ovarian cancer is presented. Systematic pelvic and paraaortic lymphadenectomy was performed in 248 cases (50.8%). Selective sampling and node biopsy was performed in 33 (6.7%) and 47 (9.6%) patients, respectively. Node metastases were found in 194 of 328 patients (59.1%). The incidence of metastatic nodes significantly increased with more advanced stages, with serous histology, and with a greater amount of residual tumor. Node status appeared to be related to pathology findings at second-look. A complete pathologic response was documented in 26 of 31 (83.8%) patients with negative nodes and in 38 of 59 (64.6%) with positive nodes at first surgery. Patients with negative nodes survived significantly longer (5-year survival, 46%; median, 60 months) than those who had node metastases (5-year survival, 25%; median, 36 months). Using multivariate analysis, lymph node status, together with the stage of disease and residual tumor, still had a significant impact on 5-year survival. Moreover, among patients with optimal cytoreduction, 5-year survival was 46% (median, 56 months) and 30% (median, 41 months) for patients who did and did not undergo lymphadenectomy, respectively (P = 0.05). Likewise, when suboptimal cytoreduction was considered, a median 5-year survival of 24 months was obtained in patients who underwent lymphadenectomy compared with 14 months in patients who did not (P < 0.005).     

Operative risk and prognostic factors of typical bronchial carcinoid tumors

BACKGROUND: This study estimated operative risk and examined factors determining long-term survival after resection of typical carcinoid tumors. METHODS: From 1976 to 1996, 139 consecutive patients (66 male and 73 female patients with a mean age of 47 +/- 15 years) underwent thoracotomy for typical carcinoid tumor. The tumors were centrally located in 102 patients (73.4%). RESULTS: Radical resection was performed in 106 patients (7 pneumonectomies, 13 bilobectomies, and 86 lobectomies) and conservative resection in 33 (3 segmentectomies, 3 wedge resections, 20 sleeve lobectomies, and 7 sleeve bronchectomies). There were no postoperative deaths. Complications occurred in 19 patients (13.7%). The morbidity rate was not increased after bronchoplastic procedures (chi 2 = 0.033, not significant). Staging was pT1 in 107 patients (77.0%) and pT2 in 32 (23.0%); 13 patients (9.4%) had nodal metastases. Seventeen patients have died (12.2%), during follow-up, but only three deaths were related to the disease. The overall survival rate at 5, 10, and 15 years was estimated to be 92.4%, 88.3%, and 76.4%, respectively; estimated disease-free survival was 100% at 5 years and 91.4% at 10 and 15 years. Estimated survival of patients with lymph node metastasis was 100% at 5, 10, and 15 years. Univariate analysis failed to demonstrate any prognostic significance for sex, tumor size (T1 versus T2), tumor location (central versus peripheral), and type of resection. CONCLUSIONS: These data confirm an excellent prognosis after complete resection of typical carcinoid tumors, including those with lymph node metastases. Parenchyma-saving resections should be preferred.