Sclerosing Dermatofibrosarcoma Protuberans: A Possible Diagnosis When a Small Biopsy Shows Sclerotic Fibroma-Like Features.

ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a neoplasm of intermediate malignancy with high local recurrence rates. The sclerosing variant is characterized by the presence of sclerotic areas in more than 50% of tumors and is rarely reported. In this report, we describe a case of sclerosing DFSP with areas histopathologically resembling sclerotic fibroma, where the initial biopsy tissue presented a diagnostic challenge. A 77-year-old man presented with a 2-cm firm, erythematous nodule on the chest. A punch biopsy revealed plywood-like sclerosis and spindle cells with a vaguely storiform pattern. The tumor cells were positive for CD34. Sclerotic fibroma and DFSP were considered differential diagnoses. Subsequent excisional biopsy revealed that the tumor comprised 3 different histopathological areas: classic DFSP, sclerotic fibroma-like, and giant cell fibroblastoma-like. This report highlights the importance of reevaluating the clinical context and excision for further characterization.

Efficacy and Safety of Immune Checkpoint Inhibitor Combination Therapy for Dysphagia in Patients with Advanced Esophageal Cancer.

Background/Objectives: Recently, pembrolizumab plus 5-fluorouracil and cisplatin (FP), nivolumab plus FP, and nivolumab plus ipilimumab have become the first-line treatments for patients with advanced esophageal cancer. However, the treatment efficacy in primary tumors has not been reported. We assessed the outcomes of these treatments in advanced esophageal cancer, specifically focusing on esophageal dysphagia improvements and the primary tumor response. Methods: This retrospective study was conducted between October 2021 and November 2023. We investigated 23 patients with esophageal cancer and dysphagia who received an immune checkpoint inhibitor (ICI) plus FP or nivolumab plus ipilimumab. Results: The median progression-free survival (PFS) was 10.6 months (95% confidence interval [CI]: 9.0-12.5), and the median overall survival was not reached (95%CI: 13.0-NA). Improvement in dysphagia was observed in 19/23 (82.6%) patients, with a median time to improvement of 26 days (range: 15-77 days) and a median dysphagia PFS of 12.6 months (range: 8.1-NA months). Ten patients experienced immune-related adverse events (irAEs): seven had interstitial pneumonia, and three had thyroid dysfunction, pituitary dysfunction, and rash, respectively. Conclusions: Although there was a high frequency of irAEs, ICI for esophageal cancer achieved high response rates and prolonged survival. The observed improvement in dysphagia suggests the potential efficacy of the treatment against primary tumors.

Shorter Drainage Tube to the Pancreatic Stump Reduces Pancreatic Fistula After Distal Pancreatectomy.

BACKGROUND: We investigated the relationship between the length of a prophylactic closed-suction drainage tube and clinically relevant postoperative pancreatic fistula (CR-POPF) in distal pancreatectomy (DP). MATERIALS AND METHODS: The clinical data of 76 patients who underwent DP using a reinforced stapler for the division of the pancreas at Ehime University Hospital between December 2017 and May 2023 were retrospectively analyzed. Laparoscopic DP was performed in 41 patients (53.9%). Closed-suction drainage was performed using a 19 Fr ExuFlow Round Drain with a vacuum bulb. The drainage tube length was defined as the distance between the peripancreatic stump site and the abdominal wall insertion site using abdominal radiography. RESULTS: CR-POPF was observed in 12 patients (15.8%). Univariate analyses demonstrated that male sex (P=0.020), American Society of Anesthesiologists Physical Status (P=0.017), current smoking (P=0.005), and drainage tube length (P<0.001) were significantly associated with CR-POPF. The optimal cut-off value of drainage tube length for CR-POPF was 220 mm (area under the receiver operating characteristic curve=0.80). In multivariate analyses, drainage tube length (≥220 mm) was the sole independent predictor for CR-POPF (odds ratio, 6.59; P=0.023). According to computed tomography performed ∼1 week after surgery, the median volume of peripancreatic fluid collection was significantly higher in the long drainage tube group than in the short drainage tube group (P<0.001). CONCLUSION: A drainage tube inserted at a shorter distance to the pancreatic stump may reduce the incidence of CR-POPF after DP.

Sequential therapy for hereditary leiomyomatosis and renal cell cancer-associated renal cell carcinoma: a case report and report of a new family pedigree.

Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a rare autosomal-dominant disorder caused by a heterozygous germline mutation in the fumarate hydratase (FH) gene. HLRCC is clinically characterized by the development of three tumors: uterine leiomyomata, cutaneous leiomyomata, and renal cell carcinoma (RCC). HLRCC-associated RCC is aggressive and diagnosed at a much earlier age than sporadic RCC. It is essential for carriers of HLRCC to undergo annual renal screening by magnetic resonance imaging to detect early stage RCCs. Metastatic HLRCC-associated RCC must be treated by systemic therapy; however, it is unclear which medicines are most effective in treating this cancer owing to its low incidence rate. Immune checkpoint inhibitor (ICI) combinations or ICIs plus tyrosine kinase inhibitors are administered as systemic therapy for clear cell RCC. Here, we report a patient with HLRCC-associated RCC treated with sequential therapy, including ipilimumab plus nivolumab combination and cabozantinib, after diagnosis of HLRCC-associated RCC using FoundationOne Liquid CDx and single-site analysis. We also investigated familial FH mutations and describe a new family pedigree for HLRCC.

Semi-autonomous touch method merging robot's autonomous touch and user-operated touch for improving user experience in robot touch.

The demand for therapeutic robots to alleviate mental health problems is growing. Studies have shown that people's mental health improves when they are touched. Consequently, therapeutic robots are designed to alleviate stress through robot's autonomous touch. However, robot's autonomous touch can sometimes cause discomfort to recipients. This paper proposes a semi-autonomous touch method that merges robot's autonomous touch with user-operated touch to mitigate discomfort while maintaining the sensation of being touched by another person. We conducted an experiment involving 24 participants who were touched on the neck by robots under three conditions: robot's autonomous touch, user-operated touch, and the proposed semi-autonomous touch method condition. Additionally, the study investigated participants' impressions of the robot in each condition. The results showed that semi-autonomous touch condition mitigated discomfort more effectively compared with the robot's autonomous touch method condition. It also enhanced the feeling of being touched by another person entity and suppressed interaction boredom compared with the user-operated touch method condition. Participants reported higher trustworthiness and perceived friendliness in robots utilizing the semi-autonomous touch method compared to those with autonomous touch method condition. These findings indicate that robots featuring the proposed semi-autonomous touch method can provide a comforting experience, leveraging the therapeutic benefits of being touched by another person, and underscore their potential in mental health applications.

Impact of reconstructed portal vein morphology on postoperative nutritional status in pancreatoduodenectomy: a computational fluid dynamics study.

This study evaluated the impact of reconstructed portal vein/superior mesenteric vein (PV/SMV) morphology on the long-term nutritional status following pancreatoduodenectomy (PD) using computational fluid dynamics (CFD). Twenty-four patients who underwent PD with PV/SMV resection and reconstruction without tumor recurrence for over 9 months after the operation were enrolled in the study. Three-dimensional models were constructed from computed tomography images obtained 3-6 months postoperatively. The pressure (p) at the inlet and turbulence dissipation rate (ε) at the outlet were investigated in the models. Patients with values of either p or ε above the upper interquartile range were classified as the poor flow group. The prognostic nutritional index improvement rate was significantly lower at 9 postoperative months in the poor flow group than in the good flow group (P = 0.016). This finding indicates the utility of a CFD analysis for evaluating the reconstructed PV/SMV morphology.

Laparoscopic liver resection for a patient of hepatocellular carcinoma with von Willebrand disease: a case report.

BACKGROUND: The safety of laparoscopic hepatectomy for inherited coagulation disorders is unclear; however, the safety of open hepatectomy has been reported in several studies. Herein, we report the first case of a laparoscopic hepatectomy for a patient with von Willebrand Disease (VWD). CASE PRESENTATION: A 76-year-old male with a history of chronic hepatitis C and VWD type 2B was advised surgical resection of a 4 cm hepatocellular carcinoma in segment 7 of the liver. The patient was diagnosed with VWD in his 40 s due to gastrointestinal bleeding caused by gastric erosion. The von Willebrand factor (VWF) ristocetin cofactor activity was 30%, and VWF large multimer deficiency and increased ristocetin-induced platelet agglutination were observed. The preoperative platelet count was reduced to 3.5 × 104/µL; however, preoperative imaging findings had no evidence of liver cirrhosis, such as any collateral formations and splenomegaly. The indocyanine green retention rate at 15 min was 10%, and his Child-Pugh score was 5 (classification A). Perioperatively, VWF/factor VIII was administered in accordance with our institutional protocol. A laparoscopic partial hepatectomy of the right posterior segment was performed. The most bleeding during surgery occurred during the mobilization of the right lobe of the liver due to inflammatory adhesion between the retroperitoneum and the tumor. Bleeding during parenchymal transection was controlable. The duration of hepatic inflow occlusion was 65 min. The surgical duration was 349 min, and the estimated blood loss was 2150 ml. Four units of red blood cells and fresh frozen plasma were transfused at the initiation of parenchymal transection, and 10 units of platelets were transfused at the end of the parenchymal transection. On postoperative day 1, the transection surface drainage fluid became hemorrhagic, and emergency contrast-enhanced computed tomography showed extravasation in the greater omentum. Percutaneous transcatheter arterial embolization of the omental branch of the right gastroepiploic artery was performed. No further postoperative interventions were required. The patient was discharged on postoperative day 14. CONCLUSION: The indications for laparoscopic hepatectomy in patients with VWD should be carefully considered, and an open approach may still be the standard approach for patients with VWD.

Impact of tumor proximity to vessel on conversion in laparoscopic liver resection: A retrospective cohort study.

BACKGROUND: Although various difficulty scoring systems have been proposed for laparoscopic liver resection (LLR), details remain uncertain regarding distance between the tumor and vessels as a factor of difficulty. We aimed to examine the risk factors for conversion to open hepatectomy in LLR, including distance between tumor and vessels. METHODS: Between January 2012 and December 2022, 118 patients who underwent LLR were retrospectively enrolled and their perioperative characteristics were evaluated. RESULTS: A total of 10 cases (8.5%) were converted to open hepatectomy during LLR. The conversion group had lower platelet count, shorter distance between the tumor and a medium vessel (defined as diameter of 5-10 mm), and greater tumor depth compared with the pure LLR group. Receiver-operating characteristic curve analysis identified 10 mm as the optimal cutoff value of tumor proximity to a medium vessel (sensitivity, 80.0%, specificity, 78.7%, AUC 0.817) for predicting conversion. In multivariate analysis, lower platelet count (p = .028) and tumor proximity within 10 mm to a medium vessel (p = .001) were independent risk factors for conversion in LLR. CONCLUSIONS: Our study suggests tumor proximity within 10 mm to a medium vessel and lower platelet count as predictors of unfavorable intraoperative conversion in LLR.

Effect of postpartum depression and role of infant feeding practices on relative weight of child at 1 and 3 years of age.

BACKGROUND: Childhood obesity has increased and is considered one of the most serious public health challenges of the 21st century globally, and may be exacerbated by postpartum depression (PPD). The purpose of this study was to examine the association between PPD at 1st and 6th month postpartum, infant feeding practices, and body mass index (BMI) z-score of the child at one and three years of age. METHODS: This study used data from an ongoing prospective maternal-child birth cohort performed at the National Center for Child Health and Development (NCCHD) in suburban Tokyo, Japan with the period of recruitment from May 13, 2010 to November 28, 2013. Out of 2,309 total number of mothers, 1,279 mother-child dyads were assessed in the study. We performed multivariable linear regression analysis to examine the association between PPD and child's BMI z-score stratified by the child's age at 1 year and 3 years of age. RESULTS: The prevalence of PPD at 1 month postpartum (17%) was found to be higher than at 6 months (12%). In multivariable linear regression analysis we observed that children at 3 years who had mothers with PPD at 6 months had, on average, a BMI z-score 0.25 higher than children of mothers who did not have PPD at 6 months (ß coefficient 0.25, 95% CI [0.04 to 0.46], p value 0.02), holding all other covariates constant. Also, initiation of weaning food when child is at six months of age was associated with higher BMI z-score of the child at 3 years after adjusting for all covariates (ß coefficient = 0.18, 95% CI [0.03 to 0.34], p-value < 0.05). CONCLUSION: The significant association between PPD at 6 months and child's BMI z-score at 3 years of age, in conjunction with birth trends and high prevalence of PPD, can add to the body of evidence that there is need for multiple assessment across the first postpartum year to rule out PPD as early screening and early interventions may benefit both maternal health and child development outcomes. These findings can indicate the need for establishing support systems for care-giving activities for mothers with PPD.

Longitudinal hematological change in uncomplicated twin pregnancies: The white blood cell count decreases through pregnancy after the first trimester.

OBJECTIVE: Longitudinal hematological changes throughout twin pregnancies have not been reported. This study aimed to reveal longitudinal changes in hematological indices in twin pregnancies. MATERIALS AND METHODS: We conducted a retrospective chart review of hematological changes in uncomplicated twin pregnancies delivered at ≥37 weeks of gestation between 2010 and 2013 and randomly selected uncomplicated singletons during the same period. A complete blood count and hemogram were performed as blood examinations in the first trimester (9-13 weeks), late second trimester (22-27 weeks), mid-third trimester (33-35 weeks, only in twin pregnancies), and late third trimester (36-38 weeks). We evaluated inter-trimester differences in hematological indices and compared the values between twin and singleton pregnancies in each trimester. RESULTS: The final analysis group included 60 twin pregnancies and 63 singleton pregnancies. The white blood cell (WBC) count in twin pregnancies decreased throughout the pregnancy after the first trimester and became significantly lower than that in singletons in the late third trimester. The WBC count showed only a slight decrease in the third trimester in singleton pregnancies, whereas it showed a marked decrease throughout the pregnancy in twin pregnancies. The marked decrease in the total WBC count in twin pregnancies is mainly due to a decrease in neutrophils. The red blood cell count and hemoglobin and hematocrit values in twin pregnancies showed more marked decreases in the second trimester than in singletons. No decrease was observed after the second trimester of pregnancy. The platelet count decreased in the third trimester of twin pregnancies. CONCLUSION: We clarified the longitudinal hematological changes in twin pregnancies that showed augmentation of or differed from those of singleton pregnancies. It should be specifically mentioned that the WBC count markedly decreased through pregnancy after the first trimester, which is a characteristic change in twin pregnancies.

A Rare Presentation of Lymph Node Metastasis of VIPoma After Three Years of Resection: A Case Report.

Vasoactive intestinal peptide-producing tumor of the pancreas (VIPoma) is one of the rarer subtypes of neuroendocrine tumor (NET) of the pancreas. It usually represents intractable diarrhea, weight loss, and electrolyte abnormalities secondary to diarrhea. The most common site of metastasis of VIPoma is the liver. Furthermore, lymph node metastasis (LNM) is rare, and no metachronous LNM with a resectable situation has been reported before. A 60-year-old male patient (height: 181 cm, body weight: 74 kg) with a history of operated pancreatic VIPoma three years ago was referred to our department due to the detection of lymphadenomegaly which was suggestive of lymph node metastasis by routine follow-up computed tomography (CT). Preoperative CT showed a lymph node on the left side of the abdominal aorta and caudal side of the left renal vein with a size of 1 cm. Lymphadenectomy was performed without significant complications and blood loss. This is the first report of metachronous LNM in a patient with operated VIPoma. Although much rarer than solid organ metastasis of VIPoma, LNM in these patients can also be seen synchronously and metachronously. Close follow-up and vigilance are key to preventing recurrence-related morbidity and mortality in these patients.

Refractory postoperative pancreatic fistula following laparoscopic distal pancreatectomy for pancreatic cancer caused by incomplete pancreas divisum: a case report.

Pancreas divisum (PD) represents a prevalent congenital pancreatic variant, typically arising from the failure of fusion between the ventral and dorsal pancreatic ducts. This condition is frequently associated with recurrent pancreatitis. We herein present a case involving an incomplete PD diagnosis following the identification of a refractory postoperative pancreatic fistula (POPF) after laparoscopic distal pancreatectomy (DP) for pancreatic cancer. A 74-year-old female patient, who had undergone laparoscopic DP for pancreatic cancer, developed a POPF accompanied by intraabdominal bleeding, necessitating urgent intervention radiology to avert life-threatening complications. Following this, intraabdominal drainage was performed through an intraoperative drainage root. Subsequent fistulography and endoscopic retrograde pancreatography unveiled the presence of an incomplete PD for the first time. Consequently, a stent was placed in the Santorini duct. However, the volume of pancreatic juice from the intraabdominal drainage tube exhibited no reduction. Despite repeated attempts to access the pancreatic duct via a guidewire through the drainage tube, these endeavors proved futile. Paradoxically, the removal of the external drainage tube led to a recurrence of intraabdominal abscess formation. Consequently, reinsertion of the drainage tube became imperative. Consideration was given to draining the abscess under endoscopic ultrasonography and performing pancreatic duct drainage. However, due to the diminution of the abscess cavity through the external fistula drainage procedure, coupled with the absence of pancreatic duct dilation and its tortuous course, it was deemed a formidable challenge. the patient necessitated a lifestyle adaptation with a permanently placed percutaneous drainage tube.

Reduced chemokine C-C motif ligand 1 expression may negatively regulate colorectal cancer progression at liver metastatic sites.

Colorectal cancer (CRC) liver metastasis, albeit a stage-IV disease, is completely curable by surgical resection in selected patients. In addressing the molecular basics of this phenomenon, differentially expressed genes at primary and liver metastatic sites were screened by RNA sequencing with the use of paraffin-embedded surgical specimens. Chemokine C-C motif ligand 1 (CCL1), a chemotactic factor for a ligand of the chemokine C-C motif receptor 8 (CCR8), was isolated as one of the differentially expressed genes. Histological analysis revealed that the number of CCL1-positive cells, mainly tumour associated macrophages (TAMs) located in the stroma of CRC, decreased significantly at liver metastatic sites, while the expression level of CCR8 on CRC remained unchanged. To explore the biological significance of the CCL1-CCR8 axis in CRC, CCR8-positive CRC cell line Colo320DM was used to assess the effect of the CCL1-CCR8 axis on major signalling pathways, epithelial mesenchymal transition induction and cell motility. Upon stimulation of recombinant CCL1 (rCCL1), phosphorylation of AKT was observed in Colo320DM cells; on the other hand, the corresponding significant increase in MMP-2 levels demonstrated by RT-qPCR was nullified by siRNA (siCCR8). In the scratch test, rCCL1 treatment significantly increased the motility of Colo320DM cells, which was similarly nullified by siCCR8. Thus, the activation of the CCL1-CCR8 axis is a positive regulator of CRC tumour progression. Reduced CCL1 expression of TAMs at liver metastatic sites may partly explain the unique slow tumour progression of CRC, thus providing for a grace period for radical resection of metastatic lesions.

Prolonged warm ischemia time in the recipient is associated with post-transplant biliary stricture following living-donor liver transplantation.

PURPOSE: Post-transplant biliary stricture (PBS) is a common and important complication following orthotopic liver transplantation (LT). This study clarified the incidence of PBS and identified its risk factors. METHODS: We retrospectively reviewed the medical records of 67 patients who underwent living-donor LT (LDLT) at our institute between June 2010 and July 2022 and analyzed their clinical characteristics, prognosis, and risk factors for PBS. RESULTS: Of the 67 patients, 26 (38.8%) developed PBS during the observation period. Multivariate analyses revealed the following independent risk factors for PBS formation: increased red cell transfusion volume per body weight (> 0.2 U/kg; hazard ratio [HR], 3.8; P = 0.002), increased portal vein pressure (PVP) at the end of LT (> 16 mmHg; HR, 2.88; P = 0.032), postoperative biliary leakage (HR, 4.58; P = 0.014), and prolonged warm ischemia time (WIT) (> 48 min; HR, 4.53; P = 0.008). In patients with PBS, the cumulative incidence of becoming stent free was significantly higher in patients with a WIT ≤ 48 min than in those with a WIT > 48 min (P = 0.038). CONCLUSION: Prolonged WIT is associated with intractable PBS following LDLT.

Numerous multinucleated giant cells in cutaneous epithelioid angiosarcoma and pulmonary metastasis: A unique observation with potential diagnostic pitfalls.

The histopathologic diagnosis of poorly differentiated cutaneous angiosarcoma can be challenging. We report a case of cutaneous epithelioid angiosarcoma with numerous multinucleated giant cells (MGCs) developing pulmonary metastasis. A 79-year-old man presented with a red-purple plaque on the scalp. A skin biopsy revealed epithelioid cell proliferation, admixed with numerous MGCs, and background hemorrhage. Vascular spaces were focally present and lined by atypical endothelial cells, including MGCs. Immunohistochemically, tumor cells, including MGCs, were positive for CD31, D2-40, and ERG. The patient received radiation therapy and chemotherapy, after which a follow-up CT scan revealed symptomless pneumothorax and pulmonary metastases. The patient received palliative partial lung resection, and the specimen revealed histopathological and immunohistochemical features similar to the primary cutaneous lesion. Our report expands the morphologic spectrum of cutaneous epithelioid angiosarcoma. Cutaneous angiosarcoma is an aggressive neoplasm; thus, awareness of this rare manifestation is important.

Albumin-indocyanine green evaluation of future liver remnant predicts liver failure after anatomical hepatectomy for hepatocellular carcinoma: A dual-center retrospective study.

Aim: The albumin-indocyanine green evaluation (ALICE) score is a useful predictor of post-hepatectomy liver failure (PHLF); however, its usefulness in combination with future liver remnant (FLR), measured by 3-D volumetry, has not been investigated. This study aimed to investigate the relationship between the ALICE of the FLR (ALICE-FLR) score and severe PHLF. Methods: The clinical data of 215 patients who underwent anatomical hepatectomy for hepatocellular carcinoma without portal vein embolization at two institutes between January 2010 and December 2021 were analyzed retrospectively. PHLF occurrence and severity were determined according to the International Study Group of Liver Surgery's definition. Grades B and C PHLF were defined as severe PHLF. The ALICE-FLR, ALICE scores, and indocyanine green clearance of FLR (ICGK-FLR) were evaluated for severe PHLF prediction. Results: Severe PHLF was observed in 40 patients (18.6%). The areas under the curve (AUCs) for the ALICE-FLR, ALICE scores, ICGK-FLR, and FLR were 0.76, 0.64, 0.73, and 0.69, respectively. The AUC of the ALICE-FLR score was significantly higher than that of the ALICE score. The ALICE-FLR score was identified as an independent predictor of severe PHLF (the odds ratio for every 0.01 increment in the ALICE-FLR score was 1.24; 95% confidence interval, 1.070-1.453; p = 0.004). Among patients with severe PHLF, the ALICE-FLR score was significantly higher in the grade C than in the grade B PHLF group. Conclusion: The combination of liver function models, including indocyanine green, albumin, and FLR is considered compatible for predicting severe PHLF.

C-Reactive Protein-to-Albumin Ratio to Predict Tolerability of S-1 as an Adjuvant Chemotherapy in Pancreatic Cancer.

Adjuvant chemotherapy (AC) with S-1 after radical surgery for resectable pancreatic cancer (PC) has shown a significant survival advantage over surgery alone. Consequently, ensuring that patients receive a consistent, uninterrupted S-1 regimen is of paramount importance. This study aimed to investigate whether the C-reactive protein-to-albumin ratio (CAR) could predict S-1 AC completion in PC patients without dropout due to adverse events (AEs). We retrospectively enrolled 95 patients who underwent radical pancreatectomy and S-1 AC for PC between January 2010 and December 2022. A statistical analysis was conducted to explore the correlation of predictive markers with S-1 completion, defined as continuous oral administration for 6 months. Among the 95 enrolled patients, 66 (69.5%) completed S-1, and 29 (30.5%) failed. Receiver operating characteristic curve analysis revealed 0.05 as the optimal CAR threshold to predict S-1 completion. Univariate and multivariate analyses further validated that a CAR ≥ 0.05 was independently correlated with S-1 completion (p < 0.001 and p = 0.006, respectively). Furthermore, a significant association was established between a higher CAR at initiation of oral administration and acceptable recurrence-free and overall survival (p = 0.003 and p < 0.001, respectively). CAR ≥ 0.05 serves as a predictive marker for difficulty in completing S-1 treatment as AC for PC due to AEs.