A case of appendiceal ganglioneuroma in neurofibromatosis type 1

Background Neurofibromatosis type 1 is an autosomal dominant inherited disease associated with multiple skin neurofibromas or other neurogenic tumors, such as nodular plexiform neurinoma or cerebrospinal tumor. Gastrointestinal stromal tumors are often complicated in patients with neurofibromatosis type 1, although involvement of the appendix is rare, and there have been few reports of appendiceal ganglioneuroma. Case presentation The patient was a 29-year-old man diagnosed with neurofibromatosis type 1 based on physical findings and his family history. During the follow-up of neurofibromatosis, computed tomography was performed to detect neurological tumors, such as neurofibromas in the brain, spinal cord, and gastrointestinal tract. Computed tomography showed a markedly thickened appendix wall, and an appendiceal tumor was suspected. Laparoscopic appendectomy was performed, and a 50 × 35 mm appendiceal submucosal tumor was resected with a negative resection margin. At histopathological examination, the tumor was diagnosed as ganglioneuroma; it showed short spindle-shaped cells and ganglion cells diffusely infiltrated into the proper muscle layer and fibrous tissue that grew around nerve cells. The patient was discharged on the 5th postoperative day without postoperative complications and was doing well at 13 months following the operation. Conclusions Gastrointestinal stromal tumor and neurofibroma are the most common gastrointestinal tumors associated with neurofibromatosis type 1, but ganglioneuroma of the appendix is rare. Appendiceal neurogenic tumors should be considered in patients with neurofibromatosis type 1, and surgical resection is necessary because of the risk of malignancy.

Textbook outcome and survival of robotic versus laparoscopic total gastrectomy for gastric cancer: a propensity score matched cohort study

Textbook outcome is a composite quality measurement of short-term outcomes for evaluating complex surgical procedures. We compared textbook outcome and survival of robotic total gastrectomy (RTG) with those of laparoscopic total gastrectomy (LTG). We retrospectively reviewed 395 patients (RTG, n = 74; LTG, n = 321) who underwent curative total gastrectomy for gastric cancer via minimally invasive approaches from 2009 to 2018. We performed propensity score matched analysis to adjust for potential selection bias. Textbook outcome included a negative resection margin, no intraoperative complication, retrieved lymph nodes > 15, no severe complication, no reintervention, no unplanned intensive care unit admission, hospitalization ≤ 21 days, no readmission after discharge, and no postoperative mortality. Survival outcomes included 3-year overall and relapse-free survival rates. After matching, 74 patients in each group were selected. Textbook outcome was similar in the RTG and LTG groups (70.3% and 75.7%, respectively), although RTG required a longer operative time. The quality metric least often achieved was the presence of severe complications in both groups (77.0% in both groups). There were no differences in the 3-year overall survival rate (98.6% and 89.7%, respectively; log-rank P = 0.144) and relapse-free survival rate between the RTG and LTG groups (97.3% and 87.0%, respectively; log-rank P = 0.167). Textbook outcome and survival outcome of RTG were similar to those of LTG for gastric cancer.

Textbook outcome and survival of robotic versus laparoscopic total gastrectomy for gastric cancer: A propensity score matched cohort study

Introduction Textbook outcome is a composite quality measurement of short-term outcomes for evaluating complex surgical procedures. We compared textbook outcome and survival of robotic total gastrectomy(RTG) with those of laparoscopic total gastrectomy(LTG).Methods We retrospectively reviewed 395patients(RTG,n = 74;LTG,n = 321) who underwent curative total gastrectomy for gastric cancer via minimally invasive approaches from 2009 to 2018. We performed propensity score matched analysis to adjust for potential selection bias. Textbook outcome included a negative resection margin, no intraoperative complication, retrieved lymph nodes > 15, no severe complication, no reintervention, no unplanned intensive care unit admission, hospitalisation ≤ 21days, no readmission after discharge, and no postoperative mortality. Survival outcomes included 5-year overall and relapse-free survival rates.Results After matching, 74patients in each group were selected. Textbook outcome was similar in the RTG and LTG groups(70.3% and 75.7%,respectively), although RTG required a longer operative time. The quality metric least often achieved was the presence of severe complications in both groups(77.0% in both groups). There were no differences in the 5-year overall survival rate(90.7% and 85.9%,respectively;log-rank P = 0.144) and relapse-free survival rate between the RTG and LTG groups(89.5% and 85.2%,respectively;log-rank P = 0.167).Conclusion Textbook outcome and survival outcome of RTG were comparable to those of LTG for gastric cancer.

Liver Metastasectomy for Metastatic Breast Cancer Patients: A Single Institution Retrospective Analysis

The liver represents the first metastatic site in 5–12% of metastatic breast cancer (MBC) cases. In absence of reliable evidence, liver metastasectomy (LM) could represent a possible therapeutic option for selected MBC patients (patients) in clinical practice. A retrospective analysis including MBC patients who had undergone an LM after a multidisciplinary Tumor Board discussion at the Hepatobiliary Surgery Unit of Fondazione Policlinico Universitario “Agostino Gemelli” IRCCS in Rome, between January 1994 and December 2019 was conducted. The primary endpoint was overall survival (OS) after a MBC-LM; the secondary endpoint was the disease-free interval (DFI) after surgery. Forty-nine MBC patients underwent LM, but clinical data were only available for 22 patients. After a median follow-up of 71 months, median OS and DFI were 67 months (95% CI 45–103) and 15 months (95% CI 11–46), respectively. At univariate analysis, the presence of a negative resection margin (R0) was the only factor that statistically significantly influenced OS (78 months versus 16 months; HR 0.083, p < 0.0001) and DFI (16 months versus 5 months; HR 0.17, p = 0.0058). A LM for MBC might represent a therapeutic option for selected patients. The radical nature of the surgical procedure performed in a high-flow center and after a multidisciplinary discussion appears essential for this therapeutic option.

Outcome of multidisciplinary management of adrenocortical carcinoma: a retrospective study

Background: Adrenocortical Carcinoma (ACC) is a rare and highly malignant tumor with a high recurrence rate. Reoperation to attain negative resection margin is recommended for selected patients. However, there is limited data on the outcome of unresectable recurrent tumor treated with palliative radiotherapy (RT) and transhepatic arterial embolization (TAE).Method: A retrospective analysis of the Peking Union Medical College Hospital was conducted. Patients with pathologically confirmed ACC were included.Result: Ninety-two patients fulfilled the criteria. The 5-year, 3-year, 1-year overall survival (OS) rates were 27.2%, 44.6% and 71.7% respectively, and median OS was 32.1 months. Age < 50 years, ENSAT stage I/II, surgery and negative surgical margin were associated with improved OS (all P <0.05). A Ki67 index < 20% was a predictor of better disease-free interval (P =0.011). Of 51 patients with recurrent ACC, local treatment (reoperation, palliative RT and TAE) improved OS after recurrence from 14.5 months to 41.6 months. Local therapy and mitotane were both prognostic factors for improved OS after recurrence.Conclusion: multidisciplinary treatment is the major therapeutic approach for ACC. Resectable cases should receive complete resection to attain negative margin. Local treatment such as palliative RT and TAE may extend OS for unresectable recurrence.

The GALNT14 Genotype Predicts Postoperative Outcome of Pancreatic Ductal Adenocarcinoma

Pancreatic ductal adenocarcinoma (PDA) is notorious for its poor prognosis. The current mainstay of treatment for PDA is surgical resection followed by adjuvant chemotherapy. However, it is difficult to predict the post-operative outcome because of the lack of reliable markers. The single-nucleotide polymorphism (SNP) of N-acetylgalactosaminyltransferase14 (GALNT14) has been proven to predict the progression-free survival (PFS), overall survival (OS) and response to chemotherapy in various types of gastrointestinal (GI) cancers. However, its role in PDA has not been studied. This study aims to investigate whether the GALNT14 SNP genotype can be a prognostic marker for PDA. A cohort of one hundred and three PDA patients having received surgical resection were retrospectively enrolled. GALNT14 genotypes and the clinicopathological parameters were correlated with postoperative prognosis. The genotype analysis revealed that 19.4%, 60.2% and 20.4% of patients had the GALNT14 “TT”, “TG” and “GG” genotypes, respectively. The patients with the “GG” genotype had a mean OS time of 37.1 months (95% confidence interval [CI]: 18.2–56.1) and those with the “non-GG” genotype had a mean OS time of 16.1 months (95% CI: 13.1–19.2). Kaplan–Meier analysis showed that the “GG” genotype had a significantly better OS compared to the “non-GG” genotype (p = 0.005). However, there was no significant difference between the “GG” and “non-GG” genotypes in PFS (p = 0.172). The baseline characteristics between patients with the “GG” and “non-GG” genotypes were compared, and no significant difference was found. Univariate followed by multivariate Cox proportional hazard models demonstrated the GALNT14 “GG” genotype, negative resection margin, and locoregional disease as independent predictors for favorable OS (p = 0.003, p = 0.037, p = 0.021, respectively). Sensitivity analysis was performed in each subgroup to examine the relationship of GALNT14 with different clinicopathological variables and no heterogeneity was found. The GALNT14 “GG” genotype is associated with favorable survival outcome, especially OS, in patients with resected PDA and could serve as a prognostic marker.

Endoscopic Resection of a Pedunculated Cavernous Hemangioma of the Sigmoid Colon: A Case Report

Hemangiomas are common benign tumors that usually occur on the head and neck in children. However, colonic hemangiomas are rare in clinical practice. Approximately 80% of colonic hemangiomas are of the cavernous type, and morphologically, ≥80% of colonic hemangiomas are sessile and semi-pedunculated. Notably, pedunculated colonic hemangiomas are rare. A 69-year-old woman presented with hematochezia and underwent colonoscopy, which revealed a soft pedunculated submucosal tumor (SMT) measuring 1.5 cm in diameter, in the sigmoid colon. The surface of the SMT resembled the surrounding normal colonic mucosa with regard to color and appearance, with multiple red patches. Narrow-band imaging revealed a few telangiectasias on the surface of the SMT. The lesion could not be definitively diagnosed based on endoscopic findings. Therefore, for more accurate diagnosis, the SMT was removed by snare polypectomy with electrocautery after clipping the basal portion of the tumor stalk for prophylactic hemostasis. Histopathological examination of the specimen revealed a cavernous hemangioma with a negative resection margin. We report a case of a pedunculated cavernous hemangioma of the sigmoid colon removed by snare polypectomy with electrocautery after clipping the basal portion of the tumor stalk for prophylactic hemostasis.