Histologically benign metastasizing tenosynovial giant cell tumor mimicking metastatic malignancy: A case report and review of literature.

Diffuse-type tenosynovial giant cell tumor (D-TGCT), otherwise known as pigmented villonodular synovitis, is a locally aggressive tumor which can show multiple recurrences but is rarely associated with metastasis. A handful of studies have elucidated the imaging features and clinical course in metastatic D-TGCT with malignant transformation on histology. However, only 5 cases of metastatic D-TGCT with benign histological features have been reported in the literature, with the clinical course and prognosis reported in only 1 case. Therefore, relatively little is known about the implications of histologically benign metastasis on the role of imaging, management, and clinical outcomes. We report a case of a 51-year-old female with recurrent D-TGCT localized to the knee that metastasized to the lymph nodes and soft tissue 3 years after above-the-knee amputation and 16 years after initial diagnosis of localized D-TGCT, despite benign histologic features on lymph node excision. This case highlights the necessity of timely MRI imaging to prevent delayed diagnosis, the role of histological findings on treatment response, and clinical outcomes associated with metastasized D-TGCT.

Decision Making for Selection of Transarterial Locoregional Therapy of Metastatic Neuroendocrine Tumors.

Transarterial locoregional therapies (LRTs) are indispensable components of the modern interventional oncologic therapy of liver-dominant metastatic neuroendocrine tumors (NETs). The scope of available LRTs and their nuanced differences mandates a thorough understanding of their relative applicability and effectiveness in certain clinical circumstances to prescribe appropriate, patient-specific, image-guided therapy. This article aims to provide an overview of transarterial LRT options for liver-dominant metastatic NETs and therapy selection by reviewing procedure types, their advantages and disadvantages, and comparative efficacy in common case scenarios.

An Important Mimic of Inguinal Hernia.

Spermatic cord malignancy is a rare and challenging diagnosis, often misdiagnosed as an inguinal hernia or cord lipoma. In these images, we show a 61-year-old male for whom laparoscopic repair of clinically diagnosed hernia was attempted. Laparoscopy revealed closed internal ring without hernia. Imaging showed large paratesticular mass; radical inguinal excision of testicle, cord, and mass was performed. Pathology showed mixed low-grade and high-grade sarcoma. Liposarcoma should be considered in cases of unusual inguinal mass; appropriate imaging can guide surgical approach and optimize outcomes.

A comparative evaluation of EUS-guided biliary drainage and percutaneous drainage in patients with distal malignant biliary obstruction and failed ERCP.

BACKGROUND AND AIM: Endoscopic ultrasound-guided biliary drainage (EGBD) may be a safe, alternative technique to percutaneous transhepatic biliary drainage (PTBD) in patients who fail ERCP. However, it is currently unknown how both techniques compare in terms of efficacy, safety, and cost. The aims of this study were to compare efficacy, safety, and cost of EGBD to that of PTBD. METHODS: Jaundiced patients with distal malignant biliary obstruction who underwent EGBD or PTBD after failed ERCP were included. Technical success, clinical success, and adverse events between the two groups were compared. RESULTS: A total of 73 patients with failed ERCP subsequently underwent EGBD (n = 22) or PTBD (n = 51). Although technical success was higher in the PTBD group (100 vs. 86.4 %, p = 0.007), clinical success was equivalent (92.2 vs. 86.4 %, p = 0.40). PTBD was associated with higher adverse event rate (index procedure: 39.2 vs. 18.2 %; all procedures including reinterventions: 80.4 vs. 15.7 %). Stent patency and survival were equivalent between both groups. Total charges were more than two times higher in the PTBD group (p = 0.004) mainly due to significantly higher rate of reinterventions (80.4 vs. 15.7 %, p < 0.001). CONCLUSION: EGBD and PTBD are comparably effective techniques for treatment of distal malignant biliary obstruction after failed ERCP. However, EGBD is associated with decreased adverse events rate and is significantly less costly due to the need for fewer reinterventions. Our results suggest that EGBD should be the technique of choice for treatment of these patients at institutions with experienced interventional endosonographers.

PCR Analysis of IgH and TCR-γ Gene Rearrangements as a Confirmatory Diagnostic Tool for Lymphoproliferative Disorders.

This study investigates PCR analysis of immunoglobulin heavy chain (IgH) and T cell receptor (TCR) gene rearrangements on paraffin-embedded tissue sections and bone marrow aspirates of patients suspected to have lymphoproliferative disorders but with inconclusive diagnosis in histopathological examination. 130 samples of patients with inconclusive immunohistochemistry results were evaluated for clonal rearrangement of IgH and TCR genes. Based on histopathology examination, the patients were divided into three groups: the first group without any definite diagnosis of lymphoproliferative disorders (60 cases, 46.2 %), the second group suspected to have a lymphoproliferative disorder but in favor of benign disorders (19 cases, 14.6 %) and the third group suspect to lymphoproliferative disorders but relatively in favor of malignant disorders (51 cases, 39.2 %). After DNA extraction and quality control, semi-nested PCR was performed using consensus primers for amplification of TCR-γ and CDR-3 regions of IgH genes. PCR products were analyzed after heteroduplex analysis using polyacrylamide gel electrophoresis, and were subject to silver staining. Totally, in over half of the cases (55.4 %), a monoclonal pattern was found in IgH or TCR-γ genes rearrangements. Monoclonal IgH gene rearrangement was detected in 48.1 % of patients, whereas monoclonal TCR-γ gene rearrangement was found in 33.6 % of them, which was not statistically significant (P = 0.008). Only in 32 patients (24.6 %) were the results of TCR-γ and IgH gene rearrangements consistent with respect to the presence (2.3 %) or absence (22.3 %) of monoclonality. Finally, PCR analysis of TCR-γ and IgH gene rearrangements led to definite diagnosis in 105 patients (80.8 %), and only 25 cases (19.2 %) remained inconclusive. Our results emphasize the usefulness of gene rearrangement study in cases without a definite diagnosis in immunohistochemistry studies. Multiple PCR analysis results when combined with patient's clinical course and immunohistochemistry can lead to early diagnosis and subsequent therapy.

A novel viscous dissecting gel for endoscopic submucosal dissection: a prospective survival study in a porcine model.

BACKGROUND AND STUDY AIMS: Endoscopic submucosal dissection (ESD) is a technically challenging procedure. A novel gel can facilitate ESD due to its submucosal dissecting properties. This prospective porcine survival study evaluated clinical and histologic parameters of hybrid ESD using the gel. PATIENTS AND METHODS: Gastric submucosal lesions were created in six pigs and hybrid ESD was performed. Healing was assessed weekly until necropsy at Day 28. RESULTS: En bloc resection was achieved in all lesions (mean size 40.7 mm). The mean total procedure time was 13.5 minutes and the mean resection time was 5.5 minutes. The mean total histologic injury score was 4. At necropsy, four ulcers had healed completely and two were < 6 mm in size. CONCLUSION: Hybrid ESD of large gastric lesions in a porcine model can be facilitated by the novel gel, dramatically reducing procedure and resection times by eliminating the need for time-consuming submucosal dissection. The novel gel is safe and easy to use, and has the potential to simplify ESD. Further prospective human studies are needed to validate these findings.

Multicenter experience with performance of ERCP in patients with an indwelling duodenal stent.

Endoscopic retrograde cholangiopancreatography (ERCP) in patients with a preexisting duodenal stent covering the papilla is particularly challenging. The aim of this study was to describe a multicenter experience of performing ERCP in patients with biliary obstruction in whom the papilla was obscured by a preexisting duodenal stent. A total of 38 patients with preexisting duodenal stents obscuring the papilla underwent ERCP. Endoscopic biliary cannulation was successful in 13 patients (34.2 %). In 12 of these 13 patients (92.3 %), endoscopic therapy was performed during the same procedure and achieved clinical success with relief of jaundice in all cases (100 %). The most commonly utilized procedure in patients in whom ERCP failed was EUS-guided biliary drainage (EGBD; n = 13 /22, 59.1 %), followed by percutaneous transhepatic biliary drainage (n = 9 /22, 40.9 %). Three patients in whom ERCP failed either did not consent to further intervention or were transferred to other centers. Thus, ERCP was technically challenging in our cohort of patients with preexisting duodenal stents, but was nonetheless successful in about one third of cases. Overall, when performed by experts, endoscopic biliary drainage (via ERCP or EGBD) can be successfully achieved in the majority of patients with indwelling duodenal stents.

A prospective study of tubular dysfunction in pediatric patients with Beta thalassemia major receiving deferasirox.

BACKGROUND: Beta thalassemia major is a lifelong transfusion-dependent disorder. Transfusion-dependent thalassemia patients are prone to develop renal dysfunction due to iron overload, chronic anemia, and/or chelation therapy. METHODS: In this prospective study, thalassemia patients who fitted inclusion and exclusion criteria received Deferasirox 20 mg/kg/day. A complete biochemistry analysis of serum and 24-hour-urine specimens was performed before and after treatment. Estimated glomerular filtration rate (eGFR), Fractional excretion of sodium (FENA), potassium (FEK), uric acid (FEUA), and the maximum ratio of tubular reabsorption of phosphorus to eGFR (TmP/GFR) at baseline and after treatment was calculated and compared. RESULTS: A total of 30 patients with mean age of 4.9 ± 3.2 years were recruited. The mean serum creatinine increased significantly after 6 months of treatment (0.54 ± 0.08 vs. 0.67 ± 0.16, P < .001) while eGFR was decreased (104.36 ± 19.62 vs. 86.00 ± 16.92, P < .001). Mean potassium level in serum was increased after treatment, while serum calcium, magnesium, and uric acid levels decreased significantly (P > .05). A significant increase was confirmed for mean urinary ß2-microglobulin (ß2-MG), protein, uric acid, calcium, and magnesium (P > .05). CONCLUSION: Our findings highlighted tubular nephropathy induced by Deferasirox in patients with beta thalassemia, and confirmed the necessity for diligent monitoring of renal function in thalassemia patients receiving Deferasirox.

EUS-guided biliary drainage by using a standardized approach for malignant biliary obstruction: rendezvous versus direct transluminal techniques (with videos).

BACKGROUND: EUS-guided biliary drainage (EGBD) can be performed via direct transluminal or rendezvous techniques. It is unknown how both techniques compare in terms of efficacy and adverse events. OBJECTIVE: To describe outcomes of EGBD performed by using a standardized approach and compare outcomes of rendezvous and transluminal techniques. DESIGN: Retrospective analysis of prospectively collected data. SETTING: Two tertiary-care centers. PATIENTS: Consecutive jaundiced patients with distal malignant biliary obstruction who underwent EGBD after failed ERCP between July 2006 and December 2012 were included. INTERVENTION: EGBD by using a standardized algorithm. MAIN OUTCOME MEASUREMENTS: Technical success, clinical success, and adverse events. RESULTS: During the study period, 35 patients underwent EGBD (rendezvous n = 13, transluminal n = 20). Technical success was achieved in 33 patients (94%), and clinical success was attained in 32 of 33 patients (97.0%). The mean postprocedure bilirubin level was 1.38 mg/dL in the rendezvous group and 1.33 mg/dL in the transluminal group (P = .88). Similarly, length of hospital stay was not different between groups (P = .23). There was no significant difference in adverse event rate between rendezvous and transluminal groups (15.4% vs 10%; P = .64). Long-term outcomes were comparable between groups, with 1 stent migration in the rendezvous group at 62 days and 1 stent occlusion in the transluminal group at 42 days after EGBD. LIMITATIONS: Retrospective analysis, small number of patients, and selection bias. CONCLUSION: EGBD is safe and effective when the described standardized approach is used. Stent occlusion is not common during long-term follow-up. Both rendezvous and direct transluminal techniques seem to be equally effective and safe. The latter approach is a reasonable alternative to rendezvous EGBD.

A novel ballooned-tip percutaneous endoscopic gastrojejunostomy tube: a pilot study.

BACKGROUND: The tip of currently available percutaneous endoscopic gastrojejunostomy (PEGJ) tubes frequently migrates back into the stomach. OBJECTIVE: To study the safety of a novel, ballooned-tip, PEGJ tube and assess the risk of retrograde migration into the stomach within 3 weeks of placement. DESIGN: Prospective clinical study (NCT01551095). SETTING: Tertiary-care center. PATIENTS: Seven patients who required post-pyloric feeding were included. INTERVENTION: Placement of PEGJ feeding tubes. MAIN OUTCOME MEASUREMENTS: Position of the PEGJ, abdominal radiograph findings, adverse events. RESULTS: Seven patients underwent placement of self-propelled PEGJ tubes during the study period. Technical success was achieved in all patients (100%). All procedures were rated as technically simple, and jejunostomy tubes were placed in <5 minutes during all procedures. Abdominal radiographs showed that the jejunostomy tubes were in the jejunum in all 7 patients at both 1 and 3 weeks after tube placement. LIMITATIONS: Small number of patients and short follow-up. CONCLUSION: Ballooned-tip PEGJ feeding tubes were safe and easy to place. The presence of the balloon prevented migration into the stomach. Ballooned-tip PEGJ tubes have the potential to eliminate the need for hospital readmission and repeat endoscopies for retrograde tube migration, and this may result in large systemic cost savings.

Differential expression of RBM5 and KRAS in pancreatic ductal adenocarcinoma and their association with clinicopathological features.

RNA binding motif 5 (RBM5) is a tumor suppressor gene that regulates cell proliferation, differentiation and apoptosis through pre-mRNA splicing of related genes. This study aimed to detect RBM5 and KRAS expression in pancreatic ductal adenocarcinoma and their association with clinicopathological features. Detection of RBM5 and KRAS expression by quantitative reverse transcription-polymerase chain reaction (qRT-PCR) and western blotting was performed at mRNA and protein levels, respectively, in pancreatic cancer and non-tumor tissues. In addition, the association of RBM5 and KRAS expression with clinicopathological parameters and tumor recurrence was analyzed. The expression of RBM5 was significantly downregulated in pancreatic cancer tissues compared to peritumoral tissues at the mRNA and protein levels. Contrastingly, KRAS was significantly overexpressed in pancreatic cancerous tissues compared to peritumoral tissues. Analysis revealed that RBM5 expression was negatively correlated with KRAS expression in pancreatic cancer. Furthermore, reduced RBM5 expression has a close association with lymph node metastasis, distant metastasis, Union for International Cancer Control (UICC) stage and nerve and venous invasion, while overexpression of KRAS proteins was significantly correlated with tumor size, lymph node metastasis, UICC stage and nerve and venous invasion of pancreatic cancer. Significant RBM5 underexpression and KRAS overexpression were observed in pancreatic cancer compared to non-tumor tissues. There is a close association of differential RBM5 and KRAS with poor clinicopathological features, suggesting their potential roles in the progression and metastasis of pancreatic cancer.

Early hemodynamic changes after mitral valve replacement in patients with severe and mild pulmonary artery hypertension.

PURPOSE: to assess the early hemodynamic changes after elective mitral valve replacement (MVR) in patients with severe and mild pulmonary arterial hypertension (PAH). METHODS: a total of 45 consecutive patients, who were candidate for elective MVR, were enrolled in this prospective observational study. Patients were divided into two groups based on the absence (group A, 20 patients) or presence (group B, 25 patients) of severe pulmonary artery hypertension (PAH) defined as systolic pulmonary artery pressure ≥50 mmHg measuring by catheterization. MVR was performed using standard cardiopulmonary bypass (CBD) technique. The hemodynamic and arterial blood gas assessments were carried out at baseline before the induction of general anesthesia, in the operating room immediately after MVR, and then continued after stabilization of hemodynamic status with 2 hr interval up to 24 hours. RESULTS: The mean CPB and aortic cross-clamp times were similar in two groups (95.3 ± 49.5 and 61.8 ± 36.3 minutes in group A and 103.1 ± 34.7and 61.9 ± 20.0 minutes in group B). In group A, the mean PAP showed an increase immediately after the operation (from 40.4 ± 7.3 to 43.10 ± 6.2 mmHg) and then decreased significantly to 32.5 ± 3.9 mmHg (P <0.05). In group B, the mean PAP showed no significant reduction immediately after MVR, but it decreased significantly below the range of severe PAP over the first 24 hours. CONCLUSION: MVR is safe and effective even in patients with severe PAH. The anesthetic technique and postoperative cares can be useful in improving the outcome in such patients.

Endoscopic findings in a mass screening program for gastric cancer in a high risk region - Guilan province of Iran.

BACKGROUND AND OBJECTIVES: Gastric cancer is a leading cause of cancer-related deaths in both sexes in Iran. This study was designed to assess upper GI endoscopic findings among people>50 years targeted in a mass screening program in a hot-point region. METHODS: Based on the pilot results in Guilan Cancer Registry study(GCRS), one of the high point regions for GC - Lashtenesha - was selected. The target population was called mainly using two methods: in rural regions, by house-house direct referral and in urban areas using public media. Upper GI endoscopy was performed by trained endoscopists. All participants underwent biopsies for rapid urea test (RUT) from the antrum and also further biopsies from five defined points of stomach for detection of precancerous lesions. In cases of visible gross lesions, more diagnostic biopsies were taken and submitted for histopathologic evaluation. RESULTS: Of 1,394 initial participants, finally 1,382 persons (702 women, 680 men) with a mean age of 61.7 ± 9.0 years (range:50-87 years) underwent upper GI endoscopy. H.pylori infection based on the RUT was positive in 66.6%. Gastric adenocarcinoma and squamous cell carcinoma of esophagus were detected in seven (0.5%) and one(0.07%) persons, respectively. A remarkable proportion of studied participants were found to have esophageal hiatal hernia(38.4%). Asymptomatic gastric masses found in 1.1% (15) of cases which were mostly located in antrum (33.3%), cardia (20.0%) and prepyloric area (20.0%). Gastric and duodenal ulcers were found in 5.9% (82) and 6.9% (96) of the screened population. CONCLUSION: Upper endoscopy screening is an effective technique for early detection of GC especially in high risk populations. Further studies are required to evaluate cost effectiveness, cost benefit and mortality and morbidity of this method among high and moderate risk population before recommending this method for GC surveillance program at the national level.

A 10-year study of esophageal cancer in Guilan province, Iran: the Guilan Cancer Registry Study (GCRS).

BACKGROUND: Northern Iran counts as one of the highest prevalence regions for esophageal cancer (EC) worldwide. This study was designed to assess the epidemiologic aspects of EC in north central and northwest Iran over a 10 year period. MATERIALS AND METHODS: The Guilan cancer registry study (GCRS) is a population- based cancer registry study featuring retrospective (1996-2003) and prospective (2004-2005) phases. A detailed questionnaire based on WHO standards for cancer registratration was applied to gather the required information. Two trained physicians coded information using ICD-O-3 in close coordination with an expert pathologist. RESULTS: A total of 19,936 cases of malignancy (mean age 55.4±18.0 years, range: 1-98 years) were registered, including 1,147 cases (670 males, 447 female; mean age: 64.0±11.5 years) of EC. In 1996 the male/female ratio among patients with EC was 1.25 which increased to 1.53 in 2005. The lower third of the esophagus still remained the most common site of tumors. The average age-standardized rate (ASR) was 6.9 and 4.1 per 105 men and women, respectively. In 1996, the ASRs were 7.2 and 5.2 per 105 men and women which decreased to 6.9 and 4.1 per 105 in 2004-2005. Squamous cell carcinoma (SCC) was the most prevalent histological subtype of EC accounting over 80% of cases. CONCLUSIONS: However the prevalence of adenocarcinoma (ADC) showed an increase to 18.4%. Guilan province may be considered a relatively low incidence region for EC.

Successful management of acute necrotizing mediastinitis with trans-cervical drainage.

Acute necrotizing mediastinitis (ANM) is a lethal disease which without antibiotic therapy and surgical intervention can lead to about 40% mortality. With the development of imaging technology, spiral computed tomography (CT) scanning and shortening of the time of diagnosis and surgery, the prognosis of these patients is excellently improved. This study describes the clinical presentation, management and outcome of 4 patients (mean age: 35 years) with ANM. All patients were operated on by a trans-cervical approach, and only one patient was operated on by a trans-thoracic one. After surgery, patients were transferred to the intensive care unit and underwent daily washing and debridement with antibiotic treatments. Odontogenic infection (2 cases), pharyngeal perforation and cervical esophageal perforation were the causes of the ANM. Infection of cervical space (perivisceral spaces) and superior Mediastinum were found in all patients, and Infection below the carina was found in two. All patients were discharged with a good, general condition after an average of 24 days. Early diagnosis of ANM with clinical presentation and on-time CT scanning, early drainage and careful post operation care are very important in the management of patients with ANM and can improve the outcome of trans-cervical drainage to an acceptable technique.