Primary anastomosing hemangioma as a preoperative diagnostic mimicker of retroperitoneal cavernous hemangioma: A case report.

Anastomosing hemangioma (AH) is rare and a newly recognized variant of capillary hemangioma that is mostly found in the genitourinary tract. Additionally, AH is sometimes difficult to diagnose without pathological specimens. It is difficult to diagnose preoperatively due to the lack of specific clinical and radiologic appearance. The present report describes the imaging features from a radiological perspective and outlines the clinicopathologic features and treatment options. A 67-year-old woman was referred to Dokkyo Medical University Saitama Medical Center (Koshigaya, Japan) for a retroperitoneal tumor that was identified at a medical checkup 4 years prior. The patient had no symptoms, no abnormal physical signs and no past medical or specific family history. Routine blood tests were all within the normal ranges. A nonenhanced CT scan showed a circular, homogenous, well-circumscribed retroperitoneal tumor that was ~32×23 mm in size, between the abdominal aorta and the inferior vena cava, and just below the left renal vein. On a contrast-enhanced multidetector CT scan, the tumor showed heterogeneous septal enhancement in the arterial phase and persistent enhancement in the portal phase. The tumor was diagnosed as a benign neurogenic tumor or a retroperitoneal cavernous hemangioma at the time, and the patient was intended to be followed up at the outpatient clinic. However, it gradually increased to a maximum diameter of 35 mm over 4 years. Finally, it was completely resected by open laparotomy and pathologically diagnosed as AH. Retroperitoneal hemangioma is extremely rare in adulthood and has been confirmed in only 1-3% of all retroperitoneal tumors. To the best of our knowledge, only 6 cases of para-aortic AH have been reported. The incidence of this variant is very low. However, AH may be included in the differential diagnosis when a slowly progressing heterogeneous mass appears in the para-aortic region that exhibits a CT-enhanced pattern similar to a typical cavernous hemangioma.

Lymph node involvement is rare in mucinous cystic neoplasms of the pancreas: Role of minimally invasive surgery.

Mucinous cystic neoplasm (MCN) is a premalignant cystic tumor of the pancreas. Resection of MCN in the distal pancreas is a standard treatment; however, at present, there is no consensus on the necessity or extent of lymph node dissection, and minimally invasive pancreatectomy is commonly the preferred surgical technique. Thus, the present study aimed to assess the efficacy of minimally invasive surgery and the extent of lymph node metastasis as factors in determining an appropriate surgical treatment for MCN. The present study retrospectively analyzed 21 consecutive patients who underwent distal pancreatectomy (DP) for MCN under general anesthesia at Chiba University Hospital (Chiba, Japan) between April 2011 and July 2019. All 21 patients were female. DP with a splenectomy was performed in all the patients. A total of 14 patients underwent laparoscopic DP (LDP). No lymph node metastasis was found in any of the patients. The minimally invasive surgery group had lower operative blood loss and a shorter hospital stay than the open surgery group. There was no significant difference in the number of dissected lymph nodes between the open surgery group and the minimally invasive surgery group. Preoperative findings of malignancy in MCN included solid components on enhanced CT and endoscopic ultrasonography, high carbohydrate antigen 19-9 values and large tumor size. In conclusion, DP with spleen preservation, which is minimally invasive, may be preferentially considered as a surgical technique for MCN without malignant findings because lymph node metastases are rare in MCN and were not observed in the present study.

A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma.

Acquired hemophilia A (AHA) is a rare disease that results from factor VIII inhibitors causing abnormal coagulation, and certain cases may develop after highly invasive surgery. The present case study reports on a 68-year-old male patient who developed AHA after undergoing a subtotal stomach-preserving pancreatoduodenectomy for distal cholangiocarcinoma. The patient experienced complications after surgery, requiring reoperation on postoperative day (PD) 5 due to rupture of the Braun's enterostomy. On PD 6, angiography was performed after bleeding was detected in the jejunal limb, but hemostasis occurred spontaneously during the examination. Bleeding was observed again on PD 8 and direct surgical ligation was performed. On PD 14, bleeding recurred in the jejunal limb and angiography was performed to embolize the periphery of the second jejunal artery. During the procedure, the prothrombin time was normal, but only the activated partial thromboplastin time was prolonged. A close examination of the coagulation system revealed a decrease in factor VIII levels and the presence of factor VIII inhibitors, resulting in the diagnosis of AHA. Administration of steroids was initiated on PD 15 and, in addition to daily blood transfusions, activated prothrombin complex concentrate was administered to achieve hemostasis. The patient was discharged from the intensive care unit on PD 36 but later developed an intractable labial fistula due to suture failure at the gastrojejunostomy site. As the use of factor VIII inhibitors continued despite the administration of steroids, cyclophosphamide (CPA) pulse therapy was added at PD 58. However, CPA was ineffective and the administration of rituximab was initiated on PD 98. After 12 courses of rituximab, the patient tested negative for factor VIII inhibitors on PD 219. On PD 289, labial fistula closure was performed with continuous replacement of factor VIII and the patient was discharged on PD 342.

Primary hepatic neuroendocrine neoplasm presenting as a massive cystic liver tumor mimicking mucinous cystic neoplasm of the liver: A case report and literature review.

Neuroendocrine neoplasms (NENs) preferentially arise in the bronchopulmonary tree or the gastrointestinal tract. Notably, primary hepatic NENs are extremely rare. The present study describes a case of hepatic NEN presenting as a giant hepatic cystic lesion. A 42-year-old woman presented with a large liver tumor. Contrast-enhanced abdominal computed tomography revealed a cystic tumor (18 cm) in their left liver. The tumor exhibited liquid components and mural solid nodules with enhanced effects. The lesion was diagnosed as mucinous cystic carcinoma (MCC) preoperatively. The patient underwent a left hepatectomy, and the postoperative course was uneventful. The patient has been alive without recurrence for 36 months postoperatively. The pathological diagnosis was NEN G2. This patient had ectopic pancreatic tissue in the liver and thus the ectopic pancreatic origin of the tumor was suspected. The present study describes a case of resected cystic primary NEN of the liver that was difficult to differentiate from mucinous cystic neoplasms. As primary liver NENs are extremely rare, further studies are needed to establish their diagnosis and treatment.

Covering reinforced staples with polyethylene glycolic acid felt-covered fibrin sealant to prevent pancreatic fistula after distal pancreatomy: a retrospective comparative study.

BACKGROUND: In accordance with previous reports on the utility of polyethylene glycolic acid (PGA) felt and fibrin glue for postoperative pancreatic fistula (POPF), we usually perform distal pancreatectomy (DP) with a PGA felt reinforcement stapler when dissecting the pancreas and cover the stump with PGA felt and fibrin glue (the PPF method). In this study, we retrospectively analyzed our DP cases to compare the risk factors for POPF and the postoperative course of patients receiving the PPF method of treatment versus that of those receiving conventional treatment. METHODS: A total of 127 DP procedures performed in our department between January 2008 and June 2021 were retrospectively analysed. RESULTS: In the PPF method, grade B/C POPF rate tended to decrease, and POPF rate showed a significant decrease. The duration of drainage and the length of postoperative hospitalisation were also significantly shorter with the PPF method. The risk of grade B/C POPF significantly decreased with the PPF method if the pancreas was thick (> 13.5 mm) or the patients were obese. CONCLUSIONS: The PPF method is useful for POPF in DP and is particularly effective when a thick pancreas or obese patient is involved. Removing the drainage tube early in the PPF method may lead to early discharge.

LGR5 induces ß-catenin activation and augments tumour progression by activating STAT3 in human intrahepatic cholangiocarcinoma.

BACKGROUND & AIMS: LGR5 enhances Wnt-ß-catenin signalling; however, involvement of LGR5 or Wnt-ß-catenin signalling in ICC progression has not been reported. METHODS: Functions and regulations of LGR5-mediated ß-catenin activation in ICC progression were evaluated using surgical specimens collected from 61 ICC patients or 2 ICC cell lines. RESULTS: LGR5 expression was increased in some cases of ICC. It was positively correlated with ß-catenin activation, OLFM4 expression and STAT3 activation, and negatively correlated with GRIM19 expression in ICC, thereby enhancing cancer stem cell (CSC)-like property and EMT. High LGR5 expression was an independent factor for poor prognosis in ICC after operation. In vitro, Wnt inhibition by IWP-2 suppressed ß-catenin activation, OLFM4 expression and STAT3 activation. IWP-2 treatment decreased expression of EpCAM, CD133, vimentin and increased E-cadherin expression. The rate of mesenchymal cells was decreased and cell invasiveness was suppressed after IWP-2 treatment, suggesting that Wnt-ß-catenin signalling enhanced CSC-like property and EMT by activating STAT3. In addition, LGR5 knockdown inhibited ß-catenin activation, resulting in suppression of ß-catenin-induced STAT3 activation through inhibition of OLFM4-GRIM19 cascade. As these results, LGR5 knockdown suppressed CSC-like property and EMT. Therefore, LGR5 was a key regulator for ß-catenin activation, and ß-catenin was unable to be activated without LGR5. CONCLUSIONS: LGR5 is essential for ß-catenin activation induced by Wnt signalling. Activated ß-catenin further activates STAT3 and enhances CSC-like property and EMT, leading to aggressive tumour progression and poor prognosis in patients with ICC. Therefore, LGR5 is an excellent prognostic predictor and a promising therapeutic target for ICC.

Development and validation of a nomogram predicting postoperative pneumonia after major abdominal surgery.

PURPOSE: Postoperative pneumonia (POP) is a common complication that can adversely affect the outcomes after surgery. This study aimed to devise and validate a model for stratifying the probability of POP in patients undergoing abdominal surgery. METHODS: We included 1050 patients who underwent major abdominal surgery between 2012 and 2013. A nomogram was devised by evaluating the predictive factors for POP. RESULTS: Of the 1050 patients, 56 (5.3%) developed POP. Multivariable logistic regression analysis revealed that the independent predictive factors for POP were age, male sex, history of cerebrovascular disease, Brinkman Index (BI) ≥ 900, and upper midline incision. A nomogram was devised by employing these five significant predictive factors. The prediction model showed a relatively good discrimination performance, with a concordance index of 0.77. CONCLUSIONS: A nomogram based on age, male sex, history of cerebrovascular disease, BI ≥ 900, and upper midline incision may be useful for identifying patients with a high probability of developing POP after major abdominal surgery.

A case of successfully resected metachronous gastric and gallbladder metastases from pancreatic body cancer.

BACKGROUND: Pancreatic ductal adenocarcinoma (PDAC) readily metastasizes to the lymph nodes, liver, lung, and peritoneum; however, gastric and gallbladder metastases are rare. We report a case of metachronous gastric and gallbladder metastases from PDAC. CASE PRESENTATION: The patient is a 71-year-old man who underwent distal pancreatectomy for PDAC. Seventeen months after the surgery, a 30-mm nodule was detected at the lesser curvature of the stomach, which was diagnosed as recurrence of PDAC in the lymph nodes. He then received gemcitabine and S-1 combination chemotherapy for 6 months. Because tumor size remained approximately the same and tumor marker levels decreased, total gastrectomy and cholecystectomy were performed. Pathological examination showed well-differentiated tubular adenocarcinoma in the subserosa and muscularis propria of the stomach and gallbladder. The patient remains alive at 41 months after the second surgery with liver metastasis. CONCLUSION: We reported a rare case of metachronous gastric and gallbladder metastases from pancreatic body cancer.

Lower risk of postoperative delirium using laparoscopic approach for major abdominal surgery.

BACKGROUND: A laparoscopic approach is increasingly being used for major abdominal surgeries and is reportedly associated with a lower incidence of postoperative complications. However, the association of laparoscopic approach and postoperative delirium remains unclear. We aimed to retrospectively investigate risk factors for postoperative delirium after abdominal surgery particularly assessing the association between a laparoscopic approach and postoperative delirium. METHODS: We retrospectively studied 801 patients who underwent major abdominal surgery between April 2012 and December 2013. Among these, 181 (22.6%) patients underwent a laparoscopic and 620 (77.4%) patients underwent an open procedure. A Cox proportional hazard model analysis was used to identify risk factors for the development of postoperative delirium or overall survival within 180 days after surgery. Cumulative incidence of postoperative delirium was assessed using a propensity score-matching analysis. RESULTS: Postoperative delirium occurred in 56 patients (7.0%). A Multivariate Cox proportional hazard model analysis revealed that a laparoscopic approach reduced the risk of postoperative delirium [hazard ratio (HR) 0.30, 95% confidence interval (CI) 0.07-0.84, p = 0.019]. Postoperative delirium was associated with worse overall survival within 180 days (HR 4.91, 95% CI 1.96-12.22, p = 0.001) after adjusting for other confounders using the Cox proportional hazard model analysis. Patients who developed postoperative delirium showed worse outcomes including higher rate of morbidity except delirium (p < 0.001), longer hospitalization (p < 0.001), and post-discharge institutionalization (p < 0.001). After propensity score-matching, cumulative incidence rates of postoperative delirium were significantly lower in the laparoscopic group compared to the open group (30-day cumulative incidence rate, 1.7% vs. 7.8%, p = 0.006). CONCLUSIONS: The risk of postoperative delirium after major abdominal surgery is reduced using laparoscopic approach. Postoperative delirium should be prevented as it precipitates adverse postoperative events.

[Surgical Treatment for Liver Metastases of Gastric Cancer].

At present, there is no apparent consensus about the indications for hepatectomy for liver metastases of gastric cancer. In this study, we identified factors of poor prognosis to investigate the indications for hepatectomy in 24 cases of hepatectomy for liver metastases of gastric cancer at our hospital during a period from August 2001 to September 2017. The 1-, 3-, and 5- year survival rates in all 24 cases were 63%, 21%, and 17%, respectively. Single variable analysis revealed that significant factors of poor prognosis were 3 or more liver metastases, synchronous liver metastasis, and positive surgical margin. Multivariable analysis revealed that significant independent factors of poor prognosis were synchronous liver metastases(HR 4.71, 95%CI: 1.08-21.79, p=0.040)and positive surgical margin(HR 5.95, 95%CI: 1.56-25.81, p=0.009). These findings indicated that, in cases of metachronous tumors and negative surgical margin, favorable prognosis can be expected following surgical resection for liver metastases of gastric cancer.

Perioperative Factors Predicting Prolonged Postoperative Ileus After Major Abdominal Surgery.

BACKGROUND: Prolonged postoperative ileus (PPOI) is among the common complications adversely affecting postoperative outcomes. Predictors of PPOI after major abdominal surgery remain unclear, although various PPOI predictors have been reported in patients undergoing colorectal surgery. This study aimed to devise a model for stratifying the probability of PPOI in patients undergoing abdominal surgery. METHODS: Between 2012 and 2013, 841 patients underwent major abdominal surgery after excluding patients who underwent less-invasive abdominal surgery, ileus-associated surgery, and emergency surgery. Postoperative managements were generally based on enhanced recovery after surgery (ERAS) program. The definition of PPOI was based on nausea, no oral diet, flatus absence, abdominal distension, and radiographic findings. A nomogram was devised by evaluating predictive factors for PPOI. RESULTS: Of the 841 patients, 73 (8.8%) developed PPOI. Multivariable logistic regression analysis revealed smoking history (P = 0.025), colorectal surgery (P = 0.004), and an open surgical approach (P = 0.002) to all be independent predictive factors for PPOI. A nomogram was devised by employing these three significant predictive factors. The prediction model showed relatively good discrimination performance, the concordance index of which was 0.71 (95%CI 0.66-0.77). The probability of PPOI in patients with a smoking history who underwent open colorectal surgery was calculated to be 19.6%. CONCLUSIONS: Colorectal surgery, open abdominal surgery, and smoking history were found to be independent predictive factors for PPOI in patients who underwent major abdominal surgery. A nomogram based on these factors was shown to be useful for identifying patients with a high probability of developing PPOI.

[A Case of Advanced Gastric Cancer Responding to Neoadjuvant Chemotherapy with Docetaxel, Cisplatin, and 5-Fluorouracil, Leading to a Pathological Complete Response].

We report a 72-year-old woman who was initially diagnosed with locally advanced gastric cancer with involvement of the esophagus and pancreas. She received 3 courses of neoadjuvant chemotherapy(NAC)with docetaxel, cisplatin, and 5- fluorouracil(the DCF regimen)and achieved an excellent response. She underwent total gastrectomy with distal pancreatectomy, splenectomy, and D2 lymphadenectomy. Histological examination confirmed a pathological complete response. NAC chemotherapy can down stage/down size the disease and allow some patients to undergo curative radical surgery.

Renal neuroendocrine tumour and synchronous pancreas metastasis: histopathological diagnosis using prostatic acid phosphatase.

A woman aged 56 years developed 2 synchronous tumours: one, 1.2 cm in diameter at the head of the pancreas; and the other, 4.0 cm in diameter, at the left side of her horseshoe kidney. Preoperative differential diagnosis of these hypovascular lesions included pancreatic ductal carcinoma (PDC) with renal metastasis, PDC with renal angiomyolipoma, renal cell carcinoma with pancreatic metastasis or PDC and renal cell carcinoma. Following pancreaticoduodenectomy and left nephrectomy, both specimens were diagnosed as grade 2 neuroendocrine tumours (NETs). Immunohistochemistry revealed that both were positive for prostatic acid phosphatase (PAP), which is specific to hindgut-derived NET, including renal NET. Accordingly, the renal tumour was diagnosed as the primary lesion, and the pancreatic tumour as a metastasis. To the best of our knowledge, this is the first report of a renal NET with a synchronous pancreas metastasis. Immunohistochemical staining for PAP was a useful diagnostic marker for synchronous NETs in the kidney and pancreas.

Prognostic Factors for Surgically Resected N2 Non-small Cell Lung Cancer.

PURPOSE: Non-small cell lung cancers (NSCLCs) with pathologically documented ipsilateral mediastinal lymph node (LN) metastases (pN2) are a broad spectrum of diseases. We retrospectively analyzed prognostic factors for cases of pN2 NSCLC treated by surgical resection. METHODS: Clinicopathological data were reviewed for consecutive 121 patients who underwent anatomical pulmonary resection with mediastinal LN sampling or dissection for pN2 NSCLC over a 15-year period. RESULTS: The 5-year survival rate for all patients was 29.9%. Clinical N status, curability, surgical procedure and adjuvant chemotherapy were favorable prognostic factors in univariate analysis, with 5-year survival rates of 35.0% for cN0/1 vs. 17.7% for cN2/3 cases; 33.1% for R0 vs. 14.7% for R1/2 resection; 31.5% for lobectomy vs. 25.0% for bilobectomy and 15.6% for pneumonectomy; and 72.7% with adjuvant chemotherapy vs. 23.8% without adjuvant chemotherapy. Survival did not differ significantly based on gender, age, smoking status, clinical T status, tumor location, histology, skip metastasis, subcarinal LN metastasis, or number of involved N2 levels. In multivariate analysis, adjuvant chemotherapy, R0 resection, and lobectomy emerged as independent favorable prognostic factors. CONCLUSION: Complete resection using lobectomy and adjuvant chemotherapy are favorable prognostic factors in cases of pN2 NSCLC.

SP7 inhibits osteoblast differentiation at a late stage in mice.

RUNX2 and SP7 are essential transcription factors for osteoblast differentiation at an early stage. Although RUNX2 inhibits osteoblast differentiation at a late stage, the function of SP7 at the late stage of osteoblast differentiation is not fully elucidated. Thus, we pursued the function of SP7 in osteoblast differentiation. RUNX2 induced Sp7 expression in Runx2(-/-) calvarial cells. Adenoviral transfer of sh-Sp7 into primary osteoblasts reduced the expression of Alpl, Col1a1, and Bglap2 and mineralization, whereas that of Sp7 reduced Bglap2 expression and mineralization at a late stage of osteoblast differentiation. Sp7 transgenic mice under the control of 2.3 kb Col1a1 promoter showed osteopenia and woven-bone like structure in the cortical bone, which was thin and less mineralized, in a dose-dependent manner. Further, the number of processes in the osteoblasts and osteocytes was reduced. Although the osteoblast density was increased, the bone formation was reduced. The frequency of BrdU incorporation was increased in the osteoblastic cells, while the expression of Col1a1, Spp1, Ibsp, and Bglap2 was reduced. Further, the osteopenia in Sp7 or Runx2 transgenic mice was worsened in Sp7/Runx2 double transgenic mice and the expression of Col1a1 and Bglap2 was reduced. The expression of Sp7 and Runx2 was not increased in Runx2 and Sp7 transgenic mice, respectively. The expression of endogenous Sp7 was increased in Sp7 transgenic mice and Sp7-transduced cells; the introduction of Sp7 activated and sh-Sp7 inhibited Sp7 promoter; and ChIP assay showed the binding of endogenous SP7 in the proximal region of Sp7 promoter. These findings suggest that SP7 and RUNX2 inhibit osteoblast differentiation at a late stage in a manner independent of RUNX2 and SP7, respectively, and SP7 positively regulates its own promoter.

Rigid external distraction osteogenesis for a patient with maxillary hypoplasia and oligodontia.

OBJECTIVE: In this report, the orthodontic treatment combined with rigid external distraction osteogenesis in a 5.5-year-old girl with midfacial hypoplasia and oligodontia is described. PATIENT: The child presented with a reduced maxilla, protruding lower lip, skeletal Class III jaw relationship with a low mandibular plane angle, a short and flattened nose, anterior crossbite, and aplasia of 16 permanent teeth. The patient was treated with rigid external maxillary distraction osteogenesis, maxillary protraction headgear, and Class III elastics. Following treatment, the maxilla was displaced in a forward direction with new bone formation at the tuberosities and the mandible rotated backward in relation to the anterior cranial base. The anterior crossbite was corrected, and the skeletal jaw relationship changed from a Class III to a Class I skeletal pattern. The soft tissue facial profile showed that the nasal projection had been increased, the nasolabial angle increased, and the lower lip protrusion was reduced. Postoperative treatment results were acceptable. CONCLUSION: This report documents that early maxillary advancement with rigid external osteogenesis offers a promising treatment alternative for a very young patient with maxillary hypoplasia and oligodontia.