Clinicopathological Features and Type of Surgery for Lynch Syndrome: Changes during the Past Two Decades / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The Korean Hereditary Tumor Registry, the first and one of the largest registries of hereditary tumors in Korea, has registered about 500 families with hereditary cancer syndromes. This study evaluates the temporal changes in clinicopathologic features and surgical patterns of Lynch syndrome (LS) patients. MATERIALS AND METHODS: Data on 182 unrelated LS patients were collected retrospectively. The patients were divided into the period 1 group (registered in 1990-2004) and 2 (registered in 2005-2014). The clinical characteristics of the two groups were compared to identify changes over time. RESULTS: The period 1 group included 76 patients; the period 2 group, 106 patients. The mean ages at diagnosis were 45.1 years (range, 13 to 85 years) for group 1 and 49.7 years (range, 20 to 84 years) for group 2 (p=0.015). The TNM stage at diagnosis did not differ significantly-period 1 group: stage 0-I (n=18, 23.7%), II (n=37, 48.7%), III (n=19, 25.0%), and IV (n=2, 2.6%); period 2 group: stage 0-I (n=30, 28.3%), II (n=35, 33.0%), III (n=37, 34.9%), and IV (n=4, 3.8%). Extended resection was more frequently performed (55/76, 72.4%) in the period 1 group than period 2 (49/106, 46.2%) (p=0.001). CONCLUSION: Colorectal cancer in patients with LS registered at the Korean Hereditary Tumor Registry is still diagnosed at an advanced stage, more than two decades after registry's establishment. Segmental resection was more frequently performed in the past decade. A prompt nationwide effort to raise public awareness of hereditary colorectal cancer and to support hereditary cancer registries is required in Korea.

Histopathologic factors affecting tumor recurrence after hepatic resection in colorectal liver metastases

PURPOSE: Hepatic resection is a standard method of treatment for colorectal liver metastases (CRLM). However, the pathologic factors of metastatic lesions that affect tumor recurrence are less well defined in CRLM. The aim of this study was to evaluate the risk factors for recurrence of CRLM, focusing on histopathologic factors of metastatic lesions of the liver. METHODS: From January 2003 to December 2008, 117 patients underwent curative hepatic resection for CRLM were reviewed. Tumor size and number, differentiation, tumor budding, angio-invasion, dedifferentiation and tumor infiltrating inflammation of metastatic lesions were investigated. RESULTS: The mean number of hepatic tumors was 2 (range, 1-8). The mean size of the largest tumor was 2.9 cm (range, 0.3-18.5 cm) in diameter. The moderate differentiation of the hepatic tumor was the most common in 86.3% of the patients. Tumor budding, angio-invasion, and dedifferentiation were observed in 81%, 34%, and 12.8% of patients. Inflammation infiltrating tumor was detected in 6.8% of patients. Recurrence after hepatic resection appeared in 69 out of 117 cases (58.9%). Recurrence-free survival at 1, 2 and 5 years were 62.4%, 43.6%, and 34.3%. The multivariate analysis showed the number of metastases > or =3 (P = 0.007), the tumor infiltrating inflammation (P = 0.047), and presence of dedifferentiation (P = 0.020) to be independent risk factors for tumor recurrence. CONCLUSION: Histopathological factors, i.e., dedifferentiation and tumor infiltrating inflammation of the metastatic lesion, could be one of the risk factors of aggressive behavior as well as the number of metastases even after curative resection for CRLM.

Does T3 Subdivision Correlate with Nodal or Distant Metastasis in Colorectal Cancer?

PURPOSE: We analyzed the clinical data of T3 colorectal cancer patients to assess whether T3 subdivision correlates with node (N) or metastasis (M) staging and stage-independent factors. METHODS: Five hundred fifty-five patients who underwent surgery for primary colorectal cancer from January 2003 to December 2009 were analyzed for T3 subdivision. T3 subdivision was determined by the depth of invasion beyond the outer border of the proper muscle (T3a, 5 to 15 mm; T3d, >15 mm). We investigated the correlation between T3 subdivision and N, M staging and stage-independent prognostic factors including angiolymphatic invasion (ALI), venous invasion (VI) and perineural invasion (PNI). RESULTS: The tumors of the 555 patients were subclassified as T3a in 86 patients (15.5%), T3b in 209 patients (37.7%), T3c in 210 patients (37.8%) and T3d in 50 patients (9.0%). The nodal metastasis rates were 39.5% for T3a, 56.5% for T3b, 75.7% for T3c and 74.0% for T3d. The distant metastasis rates were 7.0% for T3a 9.1% for T3b, 27.1% for T3c and 40.0% for T3d. Both N and M staging correlated with T3 subdivision (Spearman's rho = 0.288, 0.276, respectively; P < 0.001). Other stage-independent prognostic factors correlated well with T3 subdivision (Spearman's rho = 0.250, P < 0.001 for ALI; rho = 0.146, P < 0.001 for VI; rho = 0.271, P < 0.001 for PNI). CONCLUSION: Subdivision of T3 colorectal cancer correlates with nodal and metastasis staging. Moreover, it correlates with other prognostic factors for colorectal cancer.

Comparison of surgical-site infection between open and laparoscopic appendectomy / 대한외과학회지

PURPOSE: An inflamed appendix can be removed either openly (open appendectomy [OA]) or laparoscopically (laparoscopic appendectomy [LA]). Surgical-site infection (SSI) is a representative healthcare-associated infection and can impose serious economic burdens on patients as well as affect morbidity and mortality rates. The aim of this study was to compare LA with OA in terms of SSI. METHODS: The medical records of 749 patients (420 males; mean age, 33 years) who underwent appendectomy (OA, 431; LA, 318) between September 1, 2008 and April 29, 2010 were retrospectively reviewed for demographic and pathologic characteristics, recovery of bowel movement, length of hospital stay, and postoperative complications. RESULTS: The frequency of purulent/gangrenous or perforated appendicitis was not significantly different between LA and OA groups (83% [263/318 cases] vs. 83% [359/431 cases], P = 0.183). The time to first flatus after surgery was not significantly different between the two groups (1.38 +/- 1.07 days for LA, 1.33 +/- 0.90 days for OA, P = 0.444), but the length of hospital stay was significantly shorter in LA group than in OA group (3.37 +/- 0.12 days vs. 3.83 +/- 0.12 days, P = 0.006). The frequency of overall SSI was not significantly different between the two groups (2.8% for LA, 4.6% for OA, P = 0.204), but that of superficial incisional SSI was significantly lower in LA group (0.6% vs. 3.9%, P = 0.016). CONCLUSION: The results of this study suggest that LA may lead to a shorter length of hospital stay and may have a lower risk of superficial incisional SSI than OA.

Prevalence of Gastric and Duodenal Polyps and Risk Factors for Duodenal Neoplasm in Korean Patients with Familial Adenomatous Polyposis

BACKGROUND/AIMS: The prevalence of gastric polyps, duodenal adenoma and duodenal cancer has been reported as being high among familial adenomatous polyposis (FAP) patients, but there have been no reports of this association in Korea. This study evaluated the prevalence of gastric and duodenal polyps and risk factors for duodenal neoplasm in FAP patients in Korea. METHODS: We reviewed both initial and follow-up endoscopic results from FAP patients. We also investigated the treatment modality of duodenal adenomas and analyzed the risk factors of duodenal neoplasms by logistic regression analysis. RESULTS: A total of 148 patients with FAP underwent esophagogastroduodenoscopy (EGD), and the fi ndings were as follows: gastric polyp 39.9% (fundic gland polyp 25.7% and gastric adenoma 14.2%), duodenal adenoma 15.5%, gastric cancer 2.7%, and duodenal cancer 0.7%. There were two cases of gastric cancer that developed from benign gastric polyps. There were progressions of duodenal adenomatosis during follow-up, and some degree of relapse occurred after endoscopic resection. Patients with gastric polyps showed a correlation with the occurrence of duodenal neoplasm (odds ratio, 2.814; p=0.024). CONCLUSIONS: In Korean FAP patients, gastric cancer was detected more frequently, but fundic gland polyps, duodenal adenoma and duodenal cancer were detected less frequently than in Western patients. FAP patients with gastric polyps should undergo regular EGD, particularly for the early detection of duodenal neoplasia.

Is neoadjuvant chemotherapy necessary for patients with initially resectable colorectal liver metastases in the era of effective chemotherapy?

BACKGROUNDS/AIMS: Hepatic resection has only guaranteed long-term survival in patients with colorectal liver metastasis (CRLM) even in the era of effective chemotherapy. The definite role of neoadjuvant chemotherapy (NCT) is to improve outcomes of unresectable CRLMs, but it its role has not been defined for initially resectable CRLMs (IR-CRLMs). METHODS: We reviewed the medical records of 226 patients, who had been diagnosed and treated for IR-CRLM between 2003 and 2008; the patients had the following pathologies: 10% had more than 4 nodules, 11% had tumors larger than 5 cm, and 61% had synchronous CRMLs. Among these patients, 20 patients (Group Y) were treated with NCT, and 206 (Group N) did not receive NCT according to their physician's preference. The median follow-up time was 34.1 months. RESULTS: The initial surgical plans were changed after NCT to further resection in 20% and to limited resection in 10% of 20 patients. Complication rates of Groups Y (30%) were indifferent from Group N (23%) (p=0.233), but intraoperative transfusions were more frequent in Group N (15%) than in Group Y (5%) (p=0.006). There was one case of hospital mortality (0.44%). Disease-free survival rates in Groups Y and N were 23% and 39%, respectively, and patient survival rates were 42% and 66% (p>0.05). By multivariate analysis, old age (> or =60 years), differentiation of primary tumor (poorly/mucinous), resection margin involvement, and no adjuvant chemotherapy were associated with poor patient survival; the number of CRLMs (> or =4) was associated with poor disease-free survival. CONCLUSIONS: NCT had neither a positive impact nor a negative impact on survival, even with intraoperative transfusion, as observed on operative outcomes for patients with IR-CRLM. Further study is required to elucidate the role of NCT for treatment of patient with IR-CRLMs.

Clinical Features of Colorectal Cancer Detected by the National Cancer Screening Program

PURPOSE: Since 2004, the National Cancer Screening Program of Korea has included colorectal cancer screening based on primary screening with the fecal occult blood test (FOBT). We report on the clinical features of colorectal cancer detected by the National Cancer Screening Program. METHODS: We retrospectively analyzed 577 patients who underwent elective surgery for colorectal cancer at the Seoul National University Hospital between January 2008 and December 2009. We compared the clinical features of colorectal cancers detected by the National Cancer Screening Program (NCSP group) with those of the control group in terms of age, gender, preoperative symptom, location of the tumor, surgical technique and tumor-node-metastasis (TNM) stage. RESULTS: Age, gender, location of the tumor and operation types were not different between the two groups. The proportion of asymptomatic patients was significantly higher in the NCSP group than it was in the control group (86.5% vs. 20.0%; P < 0.001). The proportion of less invasive lesions (T1 or T2) was significantly higher in the NCSP group (46.3% vs. 27.7%; P = 0.002). The pathologic stages of the colorectal cancers in the NCSP group were I, 40.3%; II, 17.9%; III, 40.3% and IV, 1.5% whereas in the control group, they were I, 20.8%; II, 32.9%; III, 34.9% and IV, 11.4%. The proportion of stage I cancer was significantly higher in the NCSP group than in the control group (40.3% vs. 20.8%; P = 0.006). CONCLUSION: Our study demonstrates the FOBT in the NCSP is effective in early detection of colorectal cancer.

Effect of Suboptimal Chemotherapy on Preoperative Chemoradiation in Rectal Cancer / 대한방사선종양학회지

PURPOSE: To examine the effect of suboptimal chemotherapy in patients undergoing preoperative chemoradiotherapy for the treatment of rectal cancer. MATERIALS AND METHODS: The medical records of 43 patients who received preoperative concurrent chemoradiotherapy, followed by radical surgery for the treatment of pathologically proven adenocarcinoma of the rectum from April 2003 to April 2006 were retrospectively reviewed. The delivered radiation dose ranged from 41.4 to 50.4 Gy. The standard group consisted of patients receiving two cycles of a 5-FU bolus injection for three days on the first and fifth week of radiotherapy or twice daily with capecitabine. The standard group included six patients for each regimen. The non-standard group consisted of patients receiving one cycle of 5-FU bolus injection for three days on the first week of radiotherapy. The non-standard group included 31 patients. Radical surgery was performed at a median of 58 days after the end of radiotherapy. A low anterior resection was performed in 36 patients, whereas an abdominoperineal resection was performed in 7 patients. RESULTS: No significant difference was observed between the groups with respect to pathologic responses ranging from grades 3 to 5 (83.3% vs. 67.7%, p=0.456), downstaging (75.0% vs. 67.7%, p=0.727), and a radial resection margin greater than 2 mm (66.7% vs. 83.9%, p=0.237). The sphincter-saving surgery rate in low-lying rectal cancers was lower in the non-standard group (100% vs. 75%, p=0.068). There was no grade 3 or higher toxicity observed in all patients. CONCLUSION: Considering that the sphincter-saving surgery rate in low-lying rectal cancer was marginally lower for patients treated with non-standard, suboptimal chemotherapy, and that toxicity higher than grade 2 was not observed in the both groups, suboptimal chemotherapy should be avoided in this setting.

Effects of Age and Sex on Anorectal Manometry

PURPOSE: This study was performed to evaluate the effects of age and sex on anorectal manometry. METHODS: Seventy-four consecutive patients who underwent surgery for sigmoid colon cancer and who had neither anal disorders nor colonic obstruction were included in this study. There were 45 men and 29 women, and the mean age was 58.6. Pressure measurements used both the rapid and the station pull-through (RPT and SPT) methods, and volume measurements used a balloon-tipped catheter. Three pressure indices (vector volume, maximal pressure, and mean pressure), three sphincter length indices (sphincter length, high pressure zone [HPZ] length, and maximal pressure position), and three volume indices (minimal sensory volume, maximal tolerance volume, and rectoanal inhibitory reflex) were analyzed. RESULTS: Squeezing pressures were higher in men than in women, especially in the RPT method, while resting pressures were not different. Sphincter length and HPZ length were not different between the sexes, but the maximal pressure position was farther from the anal verge in men. Rectal volume indices were not different between the sexes. The resting and squeezing pressures decreased linearly with aging in men, but not in women. The differences in squeezing pressures between men and women were evident in their forties and fifties, but decreased gradually with aging, with no differences being observed in their seventies. With aging, the minimal sensory volume increased in women, and the maximal tolerance volume increased in men. CONCLUSION: Anal canal pressures and volume indices are influenced by sex, age, and measurement method. Therefore, sex, age, and measurement method must be considered in the evaluation and application of anorectal manometry.

Effectiveness of Proactive Quitline Service and Predictors of Successful Smoking Cessation: Findings from a Preliminary Study of Quitline Service for Smoking Cessation in Korea

This study was to evaluate the effectiveness of the first proactive Quitline service for smoking cessation in Korea and determine the predictors of successful smoking cessation. Smoking participants were voluntarily recruited from 18 community health centers. The participants were proactively counseled for smoking cessation via 7 sessions conducted for 30 days from November 1, 2005 to January 31, 2006. Of the 649 smoking participants, 522 completed 30 days at the end of the study and were included in the final analysis. The continuous abstinence rate at 30 days of follow-up was found to be 38.3% (200/522), in the intention-to-treat analysis. Compared with non-quitters, quitters were mostly male, smoked or =20 yr, and were less dependent on nicotine. Based on the stepwise multiple logistic regression analysis, the significant predictors of successful smoking cessation were determined to be male sex, low cigarette consumption, and older age at smoking initiation. We investigated the short-term effectiveness of the Quitline service and determined the predictors of successful smoking cessation.

Cost of Colorectal Cancer Care in Korea: A Prospective Group Study with a 2-year Follow-up

PURPOSE: The incidence of cancer incidence and the rate of mortality are increasing in Korea. Specifically, colorectal cancer in men is one of the most sharply increasing malignancies. The objective of this study was to assess the direct costs for colorectal cancer patients and to identify the factors that influence cancer costs. METHODS: The direct costs of colorectal cancer were examined with a prospective group study at a hospital. The direct costs were assessed every 3 months over a 24-month period through patient interviews, medical records, and claims data. We identified the major factors associated with the cost of colorectal cancer by using a general linear model for the log-transformed data. RESULTS: The group was comprised of 100 patients with colon cancer and 120 patients with rectal cancer. The average costs per patient during the first and the second years after diagnosis were 16,280,000 won and 5,786,000 won respectively. Medical costs accounted for about 68% (11,090,000 won) of the first year's total cost and about 62% (3,602,000 won) of the second year's total cost. National Health Insurance (NHI) paid approximately 50% of the total medical cost. The total cost of colorectal cancer was clearly associated with the stage of the disease at first diagnosis, the cancer site, therapeutic modalities, and recurrence. CONCLUSIONS: These results indicate that colorectal cancer has a heavy financial impact on cancer patients. The total cost of colorectal cancer is clearly associated with the stage of the disease at first diagnosis. Increased efforts in terms of prevention and early detection may assist in reducing the costs.

Surgical Management of Colonoscopic Perforations

PURPOSE: The purpose of this study is to evaluate the clinical features that necessitate a temporary stoma for the treatment of colonoscopic perforations. RESULTS: Between January 2000 and July 2006, 30 patients were treated for colonoscopic perforation. Based on the perforation sites, we classified these patients into the following groups: proximal colon, sigmoid colon, and rectum; we then reviewed clinical data, including the time to operation and management. RESULTS: Seventeen patients had a perforation during the diagnostic colonoscopy. Of these patients, 14 patients had sigmoid colon perforation. Six underwent an operation within 10 hours after perforation. Of these six, four were managed by primary repair or resection with anastomosis, one sigmoid colon cancer patient by anterior resection, and one rectal cancer patient by low anterior resection with diverting ileostomy. Eight patients underwent more than 12 hours after perforation. Of these eight, three were managed by resection with anastomosis and diverting ileostomy and five by resection with end colostomy. Thirteen patients had a perforation during the therapeutic colonoscopy. Of these patients, 10 patients had a proximal colon perforation. Of these 10, 3 without fever or peritonitis symptom were managed by conservative management, 6 by primary repair or resection with anastomosis, and 1 transverse colon cancer patient by right hemicolectomy. Three patients had sigmoid colon perforation. Of these three, one was managed by primary repair, one by resection with anastomosis, and one sigmoid colon cancer patient by anterior resection. CONCLUSIONS: The mechanism of perforation, the site of the perforation, and the time to operation are associated with intraperitoneal contamination and have an influence on surgical treatment.

Treatment Patterns for Colorectal Cancer Patients at the National Cancer Center Korea in 2003

PURPOSE: In Korea, colorectal cancer (CRC) is one of the most sharply-increasing malignancies, and the National Colorectal Cancer Screening Program for persons over 50 years of age began in 2004. To determine the effectiveness of the program, comparative data regarding CRCs treated prior to 2004 must be analyzed. The present study assessed CRC status at diagnosis and treatment patterns in 2003. METHODS: In 2003, 503 patients were newly diagnosed with CRC and were treated at the Center for Colorectal Cancer, National Cancer Center (NCC). Clinical data were retrospectively reviewed. RESULTS: The 503 patients included 256 colon and 247 rectal cancer patients. Of the 256 colon cancer patients, 5 (2.0%) were diagnosed during screening colonoscopies and were successfully treated using an endoscopic mucosal resection (EMR), and 17 (6.6%) received only palliative chemotherapy because of distant metastases. Forty patients (15.6%) were treated with palliative surgery and chemotherapy, and 194 (75.8%) with curative surgery with or without adjuvant chemotherapy. Of the 247 rectal cancer patients, 9 (3.6%) were treated with an EMR, 20 (8.1%) with palliative chemotherapy with or without radiotherapy, 19 (7.7%) with palliative surgery and chemoradiotherapy, and 199 (80.6%) with curative surgery with or without chemoradiotherapy. Treatment with curative intent was possible in 199 of 256 (77.7%) colon cancer patients and in 208 of 247 (84.2%) rectal cancer patients. CONCLUSIONS: Only 12.1% of colon and 8.5% of rectal cancer patients were diagnosed early and treated without adjuvant therapies at the NCC in Korea in 2003.

Mucinous Adenocarcinoma of the Colon and Rectum

PURPOSE: This study was to evaluate and compare the clinical characteristics of a mucinous adenocarcinoma with those of a non-mucinous adenocarcinoma in colorectal cancer patients. METHODS: Data were retrospectively reviewed on 3,232 colorectal cancer patients, including 221 mucinous adenocarcinoma patients (6.1%), who received surgery between 1990 and 2003. RESULTS: The mean tumor size (6.5 cm) of the mucinous adenocarcinomas was bigger than that (5.2 cm) of the non-mucinous adenocarcinomas. The locations of the mucinous adenocarcinomas were 95 (48.2%) in the proximal colon, 35 (17.8%) in the distal colon, and 67 (34.0%) in the rectum whereas those of the non-mucinous adenocarcinomas were 559 (18.9%) in the proximal colon, 861 (29.2%) in the distal colon, and 1,533 (51.9%) in the rectum. Stage distribution was as follows: In mucinous adenocarcinomas, 7 stage A (3.3%), 84 stage B (39.3%), 76 stage C (35.5%), and 47 stage D (21.9%). In non-mucinous adenocarcinomas, 447 stage A (15.2%), 1,036 stage B (35.1%), 997 stage C (33.8%), and 469 stage D (15.9%). In the univariate analysis, the overall 5-year survival rate of patients with a mucinous adenocarcinoma was lower than that of patients with a non-mucinous adenocarcinoma (60% vs. 65%, P=0.016), but survival rates for each stage were not significantly different. The difference in recurrence rates was not statistically significant (33.3% vs. 24.2%, P=0.258). A multivariate analysis showed that the mucinous histologic type was not useful as an independent prognostic factor. CONCLUSIONS: Mucinous colorectal adenocarcinomas tend to be large, exist in a proximal location, have an advanced stage at diagnosis. The difference in survival rates for each stage was not statistically significant. A mucinous histologic type was not an independent prognostic factor.

Safety and Efficacy of Colonoscopic Tattooing of a Colorectal Neoplasm Prior to a Laparoscopic Resection

PURPOSE: Accurate tumor localization prior to a laparoscopic surgical resection is the critical. India ink tattoos properly placed in the colorectum are long lasting and have been reported to probably remain constantly in previous studies. The present study was done to review the safety and reliability of colonoscopic tattooing prior to a laparoscopic resection of a colorectal neoplasm. METHODS: Between May 2003 and August 2004, 20 patients underwent colonoscopic tattooing of a colorectal neoplasm prior to laparoscopic surgery. The clinical data were retrospectively reviewed. RESULTS: Among the 20 patients, 14 (70%) had tumors located in the sigmoid colon, 4 (20%) had tumors in the rectosigmoid junction, and 1 had a tumor (5%) in the upper rectum and descending colon. In six patients (30%) who had received an endoscopic mucosal resection (EMR), an additional surgical resection was required to remove the tumor completely, and those 6 patients needed another preoperative colonoscopy for India ink tattooing. The median time between tattooing and resection was 2 days (range: 1 to 18 days). Tattoos were visualized intraoperatively and localized the tumor accurately in 15 patients (75%). Seven patients underwent intraoperative colonoscopy; five didn't have tattoos that could be visualized intraoperatively, and two patients with visible tattoos needed intraoperative colonoscopy to confirm the site of tumor. Only one patient (5%) had mild fever with abdominal discomfort, which were relieved by hydration and administration of intravenous antibiotics for one day. CONCLUSIONS: A colorectal neoplasm can be localized with an acceptable reliability by using preoperative colonoscopic tattooing. India ink tattooing at the time of the EMR may reduce unnecessary colonoscopies if we doubt a complete resection has been achieved by using an EMR. The complications following colonoscopic tattooing were minimal.

A Case of Type 1 von Hippel-Lindau (VHL) Disease associated with VHL Germline Mutation / 대한내분비학회지

Von Hippel-Lindau (VHL) disease is an autosomal dominant neoplasia syndrome that result from a germline mutation in the VHL gene. Germline mutation in the VHL gene lead to the development of hemangioblastomas of the central nervous system and retina, cysts and clear cell carcinoma of the kidney, cyst adenomas of other organs, and pheochromocytoma. VHL is a tumor suppressor gene on the short arm of chromosome 3. VHL disease has been classified into two main clinical subtypes depending on the presence (type 2) or absence (type 1) of pheochromocytoma. Type 2 has been subdivided into three categories depending on the presence (type 2B) or absence (type 2A) of renal cell carcinoma, with type 2C being a rare subtype in which pheochromocytoma is the sole manifestation of VHL disease. Recently we experienced a family with VHL type 1 who carry C to T (Q73X) transition in codon 217 nonsense germline mutation in exon 1 of VHL gene. The authors report this case with literature review.

Comparison of Self-Expanding Metallic Stenting with Emergency Surgery as First-Management for Obstructing Primary Left-sided Colorectal Cancer

PURPOSE: Emergency surgery has been a conventional treatment for malignant large-bowel obstruction, but is associated with high morbidity and mortality. Recently, self-expanding metallic stents (SEMS) have provided a new modality as palliation or bridge therapy for patients with obstructing colorectal cancers. The purpose of the present study is to evaluate whether SEMS is useful in patients with malignant large bowel obstruction. METHODS: Between January 1999 and June 2004, 63 patients were treated for primary obstructing left-side colorectal cancer at the Department of Surgery, Seoul National University Hospital. 32 patients were managed firstly with SEMS (stent group), and 31 patients underwent an emergency operation (emergency surgery group). Clinical data were retrospectively reviewed. The results, including perioperative outcome and survival, were compared between the two groups. RESULTS: Clinicopathological parameters, including age, gender, ASA grade, tumor location and stage, were similar between the two groups. The rate of one-stage operations was significantly higher in the stent group (52% vs 13%; P=0.004). The stent group was associated with a lower rate of stoma formation (48% vs 87%; P=0.004), a shorter length of hospital stay (19 days vs 26 days; P=0.048), and fewer complications (7% vs 30%; P= 0.035). The overall and the disease-free survival rates were similar between the two groups (P=0.973, 0.126). The stent group was associated with fewer surgeries needed, shorter length of hospital stay, and fewer complications. CONCLUSIONS: Stenting with SEMS is useful in managing malignant large-bowel cancer with obstruction and may be better than traditional open surgery.

A Case Report of von Hippel-Lindau Disease Manifested in a Monozygous Twin / 대한내분비학회지

Von Hippel-Lindau (VHL) disease is an autosomal dominant disease, which forms hypervascular tumors in multiple organs, such as hemangioblastomas in the retina and central nervous system, renal cell carcinomas, pheochromocytomas and cysts in various organs. Recent advances in gene testing have made it possible to screen family members for VHL disease. We experienced a 28 year-old male, who was diagnosed with bilateral pheochromocytomas through a family screening test when his elder monozygous twin brother was diagnosed with a pheochromocytoma. He received no treatment until December, 2004, when he visited the Emergency room due to a headache. A hemangioma of the cerebellum was seen in the brain MR study, leading to the diagnosis of type 2A VHL disease. An abdominal CT scan revealed no lesions of the pancreas or kidney. There was no evidence of a hemangioma in the retinal scan. The subsequent gene testing showed a germline mutation in exon 3 codon 167 of the VHL gene. The mother of the patient was revealed to have the same mutation of the VHL gene, but the elder brother of the patient did not.

Hereditary Colorectal Cancer / 대한소화기학회지

Hereditary syndromes cause approximately 5 to 15% of overall colorectal cancer (CRC) cases. Hereditary CRC is conventionally divided into two major categories: hereditary non-polyposis colorectal cancer (HNPCC) and those related to polyposis syndromes including familial adenomatous polyposis (FAP), Peutz-Jegher syndrome (PJS), and juvenile polyposis (JP). The screening for the cancer and methods of treatment applied to patients with hereditary CRC are quite different from those applied to the general population. The genes responsible for these syndromes has recently identified, as a result, genetic testing has become the most important determining factor in clinical decisions. Germ-line mutation of the APC gene induces FAP, an autosomal dominant disorder, characterized by the development of hundreds to thousands of colonic adenomas. CRC appears in almost all affected individuals by the time they are 50 years of age. An affected individual should undergo colectomy by his/her late teens. Furthermore, according to the findings of genetic testing, at-risk family members also need endoscopic surveillance and surgery. Recently, a mutation on the MYH gene is increasingly being investigated in patients with multiple polyps, and autosomal recessive MYH polyposis is considered to be a new category of polyposis. More common than FAP, HNPCC is caused by germ-line mutations in DNA mismatch repair genes, mainly MLH1 and MSH2. Although there is no polyposis, polyps seem to be more villous and dysplastic and appear to grow rapidly into CRCs. The aggregate lifetime risk of CRC is about 80% for mutation carriers. The risk for other types of cancer, such as endometrial, ovarian, small bowel, and transitional cell cancer, is also increased. The Amsterdam criteria and Bethesda guidelines are the best-known tools for diagnosis and genetic testing, and colectomy followed by endoscopic follow-up is the standard treatment. PJS and JP are reported to be characterized by hamartomatous polyps throughout the GI tract and germ-line mutations in the STK11 gene (PJS) and the DPC4/BMPR1A gene (JP).

Biology of SNU Cell Lines / Journal of the Korean Cancer Association, 대한암학회지

SNU (Seoul National University) cell lines have been established from Korean cancer patients since 1982. Of these 109 cell lines have been characterized and reported, i.e., 17 colorectal carcinoma, 12 hepatocellular carcinoma, 11 gastric carcinoma, 12 uterine cervical carcinoma, 17 B-lymphoblastoid cell lines derived from cancer patients, 5 ovarian carcinoma, 3 malignant mixed Mllerian tumor, 6 laryngeal squamous cell carcinoma, 7 renal cell carcinoma, 9 brain tumor, 6 biliary tract, and 4 pancreatic carcinoma cell lines. These SNU cell lines have been distributed to biomedical researchers domestic and worldwide through the KCLB (Korean Cell Line Bank), and have proven to be of value in various scientific research fields. The characteristics of these cell lines have been reported in over 180 international journals by our laboratory and by many other researchers from 1987. In this paper, the cellular and molecular characteristics of SNU human cancer cell lines are summarized according to their genetic and epigenetic alterations and functional analysis.