Publications by authors named "Mika Shapira Rootman"

16 Publications

  • Page 1 of 1

Trametinib for orbital plexiform neurofibromas in young children with neurofibromatosis type 1.

Childs Nerv Syst 2021 Mar 22. Epub 2021 Mar 22.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Introduction: Plexiform neurofibromas (PNF) in neurofibromatosis type 1 (NF1) are usually diagnosed in childhood and can grow rapidly during this period. In 10% of patients, PNF involve the orbital-periorbital area and may cause visual problems including glaucoma, visual loss from amblyopia (deprivational, strabismic, or refractive), optic nerve compression, or keratopathy. Ptosis, proptosis, and facial disfigurement lead to social problems and decreased self-esteem. Complete surgical removal involves significant risks and mutilation, and regrowth after debulking is not uncommon. Inhibitors of the RAS/MAPK pathway have recently been investigated for their activity in PNF. We administered the oral MEK inhibitor trametinib to five young children with NF1 and PNF of the orbital area, with visual compromise and progressive tumor growth; and followed them clinically and by volumetric MRI.

Methods: Treatment was initiated at a mean age of 26.8 months (SD ± 12.8) and continued for a median 28 months (range 16-51). Doses were 0.025 mg/kg/day for children aged > 6 years and 0.032 mg/kg/day for those aged < 6 years.

Results: Volumetric MRI measurements showed a reduction of 2.9-33% at 1 year after treatment initiation, with maximal reductions of 44% and 49% in two patients, at 44 and 36 months, respectively. No change in visual function was recorded during treatment. One child reported decreased orbital pain after 2 weeks; and another, with involvement of the masseters, had increased ability to chew food. Toxicities were mostly to skin and nails, grades 1-2.

Conclusions: Trametinib can decrease tumor size in some young children with orbital PNF and may prevent progressive disfigurement.
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http://dx.doi.org/10.1007/s00381-021-05127-6DOI Listing
March 2021

Neuroimaging in Children with Ophthalmological Complaints: A Review.

J Neuroimaging 2021 Feb 22. Epub 2021 Feb 22.

Department of Radiology, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.

Pediatric patients are commonly referred to imaging following abnormal ophthalmological examinations. Common indications include papilledema, altered vision, strabismus, nystagmus, anisocoria, proptosis, coloboma, and leukocoria. Magnetic resonance imaging (MRI) of the brain and orbits (with or without contrast material administration) is typically the imaging modality of choice. However, a cranial CT scan is sometimes initially performed, particularly when MRI is not readily available. Familiarity with the various ophthalmological conditions may assist the radiologist in formulating differential diagnoses and proper MRI protocols afterward. Although MRI of the brain and orbits usually suffices, further refinements are sometimes warranted to enable suitable assessment and accurate diagnosis. For example, the assessment of children with sudden onset anisocoria associated with Horner syndrome will require imaging of the entire oculosympathetic pathway, including the brain, orbits, neck, and chest. Dedicated orbital scans should cover the area between the hard palate and approximately 1 cm above the orbits in the axial plane and extend from the lens to the midpons in the coronal plane. Fat-suppressed T2-weighted fast spin echo sequences should enable proper assessment of the globes, optic nerves, and perioptic subarachnoid spaces. Contrast material should be given judiciously, ideally according to clinical circumstances and precontrast scans. In this review, we discuss the major indications for imaging following abnormal ophthalmological examinations.
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http://dx.doi.org/10.1111/jon.12842DOI Listing
February 2021

The Modified Bosniak Classification for Intermediate-Risk Renal Cysts in Children.

Urology 2021 Mar 28;149:206-210. Epub 2020 Oct 28.

Pediatric Urology Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Objectives: To examine correlations of the modified Bosniak categories assigned by radiologists to histological results and inter-rater reliability, focusing on intermediate-risk lesions.

Materials And Methods: The data of pediatric patients who underwent surgery for intermediate-risk complex renal cyst at a tertiary medical center in 2006-2019 were collected retrospectively. Four pediatric radiologists from 2 different medical centers reviewed the available imaging scans, and assigned each to one of the four modified Bosniak classification categories. Binary cohorts of the Bosniak categories (I-II vs III-IV) were compared to the histological results. Diagnostic accuracy (benign- vs intermediate-risk lesion) was calculated for each radiologist and for each imaging modality. Krippendorff's α test was used to measure inter-rater reliability.

Results: The cohort included seven children, each with 1 complex cyst that was rated as intermediate-risk on pathological study. The median age was 1.5 years (IQR 1, 11.9). A correct classification was made in 41/56 imaging readings (sensitivity 73.2%). Applying Krippendorff's test to the binary Bosniak cohorts yielded poor inter-rater agreement (α = 0.08).

Conclusion: Implementation of the modified Bosniak classification in children caused a disconcerting underestimation of intermediate risk. There was a low inter-rater consistency for the categories intended to guide decisions regarding surgery or conservative management. The findings suggest that clinicians should be cautious using the modified Bosniak system for children.
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http://dx.doi.org/10.1016/j.urology.2020.10.022DOI Listing
March 2021

Not only appendicitis: rare appendix disorders manifesting as surgical emergencies in children.

Eur J Pediatr 2021 Feb 18;180(2):407-413. Epub 2020 Aug 18.

Department of Pediatric and Adolescent Surgery, Schneider Children's Medical Center of Israel affiliated with Sackler Faculty of Medicine, Tel Aviv University, Kaplan St 14, 4920235, Petah Tikva, Israel.

Acute appendicitis is the most common cause of acute abdominal pathology in children. However, other rare non-inflammatory non-neoplastic disorders involving the appendix may manifest as surgical emergencies. This study aimed to describe these atypical entities and present representative cases. The database of a tertiary children's medical center was reviewed for all the patients aged 0-18 years who underwent urgent appendectomy between June 2014 and December 2019, for rare disorders of the appendix unrelated to inflammatory or neoplastic processes. Of 1367 patients who underwent appendectomy, 1345 were operated urgently or emergently. Of these, six, all males, mean age 32.6 months (range 0.7-76), underwent appendectomy for rare surgical complications that involved the appendix. These included torsion of the appendix (2), a strangulated internal hernia through an appendicular ring (1) or through a mesoappendix gap (1), an incarcerated appendix in an acute hernia sac (1), and appendiceal intussusception (1). In all cases, the role of the appendix in the pathologic process was unexpected and came as a surprise to the surgeon. During a median follow-up of 4.2 months (range 1-8 months), one patient underwent relaparotomy for small bowel obstruction 4 weeks after the original procedure.Conclusion: The appendix in children can be the source of rare pathological disorders that present as surgical emergencies. Familiarity with these entities may aid in achieving accurate preoperative diagnosis and contribute to surgical team orientation on exploratory laparotomy. However, correct diagnosis is often only established during timely surgical intervention.
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http://dx.doi.org/10.1007/s00431-020-03784-4DOI Listing
February 2021

The great mimicker: Phenotypic overlap between constitutional mismatch repair deficiency and Tuberous Sclerosis complex.

Clin Genet 2020 02 4;97(2):296-304. Epub 2019 Dec 4.

The Rina Zaizov Hematology-Oncology Division, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Constitutional mismatch repair deficiency is a rare cancer predisposition syndrome caused by biallelic mutations in one of the four mismatch repair genes. Patients are predisposed to various tumors including hematological malignancies, brain tumors and colorectal carcinomas. Phenotypic overlap with Neurofibromatosis-1 is well known, with most patients presenting with café-au-lait macules. Other common features include axillary and/or inguinal freckling and intracranial MRI foci of high T2W/FLAIR signal intensity similar to the typical FASI seen in Neurofibromatosis-1. In this cohort of eight patients with constitutional mismatch repair deficiency we describe overlapping phenotypical features with Tuberous Sclerosis complex. In addition to "ash-leaf like" hypomelanotic macules (five patients), we detected intracranial tuber-like lesions (three patients), renal cysts (three patients) and renal angiomyolipomas (two patients). All our patients also had Neurofibromatosis-1 like features, mainly café-au-lait macules. This study suggests that features of Tuberous sclerosis especially when overlapping with those of Neurofibromatosis 1 or malignancies atypical for these syndromes should raise the possibility of constitutional mismatch repair deficiency. Correct diagnosis is essential for appropriate genetic counseling and pre-emptive cancer surveillance.
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http://dx.doi.org/10.1111/cge.13656DOI Listing
February 2020

Imaging features of primary hyperoxaluria.

Clin Imaging 2018 Nov - Dec;52:370-376. Epub 2018 Sep 15.

Imaging department, Schneider Children's Medical Center of Israel, 14 Kaplan street, Petach Tikva, Israel.

Primary hyperoxaluria (PH) is a group of autosomal recessive diseases that affect the metabolism of glyoxalate and oxalate. As a result of the enzymatic deficiency, there is overproduction and urinary excretion of oxalate with progressive renal damage and subsequent deposition of oxalate salts in various tissues. The definitive treatment in cases of end-stage kidney disease is a combined liver and kidney transplant. Imaging features are diverse and reflect the multiple organs that might be affected. These include nephrolithiasis and nephrocalcinosis, oxalate osteopathy, as well as other findings, such as splenomegaly and oxalate deposition in the heart. In this review article, we present various imaging findings that may appear in patients with PH.
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http://dx.doi.org/10.1016/j.clinimag.2018.09.009DOI Listing
January 2019

Four-dimensional Computed Tomography (4DCT) for Preoperative Localization of Parathyroid Adenomas.

Isr Med Assoc J 2017 Apr;19(4):216-220

Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel, affiliated with the Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.

Background: Four-dimensional parathyroid computed tomography (4DCT) is a relatively new parathyroid imaging technique that provides functional and highly detailed anatomic information about parathyroid tumors.

Objectives: To assess the accuracy of 4DCT for the preoperative localization of parathyroid adenomas (PTAs) in patients with biochemically confirmed primary hyperparathyroidism (PHPT) and a history of failed surgery or unsuccessful localization using 99mTc-sestamibi scanning and ultrasonography.

Methods: Between January 2013 and January 2015, 55 patients with PHPT underwent 4DCT at Hillel Yaffe Medical Center, Hadera, Israel. An initial unenhanced scan was followed by an IV contrast injection of nonionic contrast material (120 ml of at 4 ml/s). Scanning was repeated 25, 60, and 90 seconds after the initiation of IV contrast administration. An experienced radiologist blinded to the earlier imaging results reviewed the 4DCT for the presence and location (quadrant) of the suspected PTAs. At the time of the study, 28 patients had undergone surgical exploration following 4DCT and we compared their scans with the surgical findings.

Results: 4DCT accurately localized 96% (27/28) of abnormal glands, all of which were hypervascular and showed characteristic rapid enhancement on 4DCT that could be distinguished from Level II lymph nodes. Surgery found hypovascular cystic PTA in one patient who produced a negative 4DCT scan. All patients had solitary PTAs. The scan at 90 seconds provided no additional information and was abandoned during the study.

Conclusions: 4DCT accurately localized hypervascular parathyroid lesions and distinguished them from other tissues. A three-phase scanning protocol may suffice.
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April 2017

Preferential sites of metastatic relapse on MRI of initially localized ependymoma in children.

Clin Imaging 2017 Jul - Aug;44:12-15. Epub 2017 Mar 30.

Hematology-Oncology Division, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, 14 Kaplan Street, PO Box 559, Petach Tikvah 49202, Israel.

Objective: Relapse of ependymoma in childhood portends a grave prognosis. While the detection of local recurrence is usually simple, spotting leptomeningeal metastasis might be challenging. We aimed to evaluate possible "hotspots" where metastasis tend to appear.

Materials And Methods: Medical records and Magnetic Resonance (MR) studies of all patients diagnosed with brain ependymomas between the years 2000-2015 were reviewed.

Results: Leptomeningeal spread was detected among 42% of relapsed patients. The most common sites were spine and hypothalamic area (26% each).

Conclusion: A meticulous assessment of the brain and spine including a thorough evaluation of the hypothalamic area is recommended.
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http://dx.doi.org/10.1016/j.clinimag.2017.03.015DOI Listing
December 2017

Impact of Increased Visceral Fat Measured by CT on Colon Adenocarcinoma Stage.

J Gastrointest Cancer 2017 Dec;48(4):347-352

Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.

Purpose: Obesity is considered a risk factor for colon cancer. Worse outcome observed in colon cancer patients with obesity may have several mechanisms. However, the influence of obesity on colon cancer stage is yet to be clarified. The objective of this study was to determine the relationship between obesity and stage on presentation.

Methods: This is a retrospective analysis of patients' files in one university affiliated medical center. Included in this study were non-metastatic patients who underwent colectomy for adenocarcinoma of the colon in whom both the preoperative abdominal computed tomography and the postoperative pathology report were available for evaluation. Abdominal computed tomography measurement of retroperitoneal fat thickness was used as an indicator of obesity. Data was analyzed according to quartiles of fat thickness.

Results: Of 83 patients, 38 were males and 45 were females. Median age was 71 years (range 43-90 years). Increased retroperitoneal fat thickness was not associated with advanced T and N stages on presentation. Rather, patients in the first quartile presented with the worst T and N stages. The proportion of patients with T3 and T4 tumors was 95, 76.2, 66.7, and 66.7% in quartiles 1, 2, 3, and 4, respectively (p = 0.0327), while the proportion of patients with positive lymph node metastasis was 60, 23.8, 23.8, and 42.9% in quartiles 1, 2, 3, and 4, respectively (p = 0.0319).

Conclusions: The data presented here does not support the association of obesity with worse stage at presentation of colon cancer, and other possible mechanisms for worse outcome should be sought.
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http://dx.doi.org/10.1007/s12029-016-9891-8DOI Listing
December 2017

Role of Plain Abdominal Radiographs in the Evaluation of Patients with Non-Traumatic Abdominal Pain.

Isr Med Assoc J 2015 Nov;17(11):678-81

Background: Plain abdominal radiographs are still performed as a first imaging examination to evaluate abdominal pain in the emergency department (ED), despite uncertainty regarding their utility.

Objectives: To describe the frequency and outcomes of the use of plain abdominal radiographs in the diagnosis of patients presenting with acute non-traumatic abdominal pain in the ED of a medical center.

Methods: We retrospectively reviewed the records of patients presenting to the ED with acute abdominal pain during a 6 month period. Further imaging (computed tomography, ultrasonography), when performed, was compared with the abdominal radiography.

Results: Of 573 consecutive patients, 300 (52%) underwent abdominal radiography. Findings were normal in 88% (n = 264), non-specific in 7.3% (n = 22), and abnormal in 4.7% (n = 14). For those with normal results, no further imaging was ordered for 43% (114/264). Of the 57% (150/264) who had follow-up imaging, 65% (98/150) showed abnormal findings. In 9 (3%) of the 300 patients, abdominal radiography identified bowel perforations and obstructions, and treatment was provided without the need for further radiologic examination.

Conclusions: The use of plain abdominal radiography is still common despite the high rate of false positive results. Efforts are needed to decrease the indiscriminate use of radiography in patients presenting with abdominal symptoms.
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November 2015

Sonographic Diagnosis of Complicated Cholecystitis.

J Ultrasound Med 2015 Dec 30;34(12):2231-6. Epub 2015 Oct 30.

Department of Radiology (M.S.-R., N.R., A.N., A.-R.Z.) and Division of Surgery (A.M.), Hillel Yaffe Medical Center, Hadera, Israel; affiliated with the Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.

Objectives: Early detection of the complications of cholecystitis is important for clinical management, yet only a small percentage of patients have a correct diagnosis before surgery. The purpose of our study was to identify sonographic findings that are associated with complicated cholecystitis.

Methods: Sonographic, surgical, and pathologic reports were reviewed for 70 patients who underwent early cholecystectomies from January 2010 to August 2014. Sonograms were assessed for 16 independent variables. Statistical analyses were performed to evaluate associations between various sonographic features and complicated cholecystitis.

Results: Sonographic signs associated with complicated cholecystitis (P< .05) were a greater short-axis gallbladder diameter (mean, 4.4 versus 4.0 cm), a greater mean wall thickness (5.6 versus 4.2 mm), and the likelihood of wall striations, gallbladder echogenic content, pericholecystic free fluid, and local inflammatory fat changes. Specific sonographic signs, such as sloughed intraluminal membranes, were detected in a small percentage of cases (10%). None of the sonographic features evaluated in this study was found to be sensitive and specific enough to indicate complicated cholecystitis. In most cases, sonograms reflected severe inflammation, with multiple sonographic signs.

Conclusions: Although multiple sonographic signs are associated with complicated cholecystitis, none of them is sensitive and specific enough to definitively diagnose it. Sonograms usually reflect severe inflammation, with numerous sonographic signs. Thus, in the right clinical context, sonograms of severe cholecystitis should alert radiologists to the possibility of complications.
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http://dx.doi.org/10.7863/ultra.14.12072DOI Listing
December 2015

Giant colonic diverticulum: radiographic and MDCT characteristics.

Insights Imaging 2015 Dec 19;6(6):659-64. Epub 2015 Sep 19.

Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel.

Giant colonic diverticulum (GCD), defined as a diverticulum larger than 4 cm, is a rare entity that is generally a manifestation of colonic diverticular disease. Because of its rarity and its variable and non-specific presentation, the diagnosis of GCD depends mainly on imaging findings. Knowledge of the spectrum of radiographic and CT features of the GCD is important in making the correct diagnosis and potentially preventing complications. This review focuses on imaging findings characteristic of GCD as well as its complications and radiographic mimics. Teaching points • Giant colonic diverticulum is a rare complication of diverticulosis.• The most common symptom is abdominal pain presenting in approximately 70 % of patients.• Diagnosis is based on imaging findings with plain abdominal radiographs and MDCT.• Treatment consists of en bloc resection of the diverticulum and affected adjacent colon.
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http://dx.doi.org/10.1007/s13244-015-0433-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4656231PMC
December 2015

The diagnostic accuracy of pericolonic fat extension and attenuation for colorectal tumors.

Eur J Radiol 2015 Sep 3;84(9):1724-8. Epub 2015 Jun 3.

Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel; Affiliated with the Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.

Objective: To evaluate the utility of quantitative analysis of the extension and attenuation of pericolonic fat in the local staging of colorectal cancer (CRC) using multi detector computed tomography (MDCT).

Materials And Methods: This was a retrospective study of 110 patients who were operated due to pathologically proven CRC from January 2007 to January 2010, and who underwent preoperative MDCT of the abdomen and pelvis with administration of intravenous contrast material and image acquisition during the portal venous phase. The mean age was 69 years (range of 38-90 years). Pathological reports were reviewed for TNM staging. All MDCT studies were reviewed by two certified radiologists for maximal and minimal tumor diameter, extent of the infiltrated pericolonic fat (measured in mm), attenuation of the infiltrated pericolonic fat (measured in Hounsfield units), and attenuation of normally appearing fat next to the tumor. The sensitivity and specificity of these parameters in detecting patients with ≥ T3 CRC were calculated.

Results: The overall sensitivity, specificity, and accuracy of pericolonic fat infiltration in detecting patients with ≥T3 stage were 95% (95% CI, 89.0-98.7%), 20% (5.8-43.7%), and 81.9% (74.7-89%) respectively. The mean extent and attenuation of the infiltrated pericolonic fat, in addition to the maximal tumor diameter, were higher in the ≥T3 group (p<0.05). By defining threshold values to these parameters, the positive predictive value for detecting ≥T3 stage tumors approaches 100%.

Conclusion: Quantitative analysis of pericolonic fat increased the accuracy of MDCT in the detection of local tumor spread in CRC.
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http://dx.doi.org/10.1016/j.ejrad.2015.05.036DOI Listing
September 2015

Diagnosis of gallbladder perforation by ultrasound.

Clin Imaging 2015 Sep-Oct;39(5):827-9. Epub 2015 May 27.

Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel; Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel. Electronic address:

Purpose: The aim of this study was to identify possible pitfalls in the sonographic evaluation of perforated gallbladders.

Methods: This is a retrospective analysis of sonograms of 11 patients diagnosed by surgery or pathology with perforated gallbladder. Sonograms were evaluated for multiple sonographic features.

Results: Only three patients (27%) were diagnosed correctly with gallbladder perforation by ultrasonography prior to surgery. Gangrenous cholecystitis was reported in 10 cases (90%). Visualization of a wall defect was demonstrated in only five cases (45%) and was associated with a focal echogenic soft-tissue mass.

Conclusion: Missed perforations by ultrasound may partially result from technical inadequacies.
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http://dx.doi.org/10.1016/j.clinimag.2015.05.008DOI Listing
April 2016

The prevalence of pulmonary embolism among patients suffering from acute exacerbations of chronic obstructive pulmonary disease.

Emerg Radiol 2015 Jun 26;22(3):257-60. Epub 2014 Oct 26.

Department of Radiology, Hillel Yaffe Medical Center, P.O.B. 169, Hadera, 38100, Israel.

The clinical diagnosis of acute pulmonary embolism (PE) in patients with acute exacerbation of chronic obstructive pulmonary disease (COPD) is often difficult due to the similarity in the presenting symptoms of the two conditions. The purpose of this study was to determine the prevalence of PE in patients with acute exacerbation of COPD. Forty-nine consecutive patients admitted to our medical center for acute exacerbation of COPD were investigated for PE (whether or not clinically suspected), following a standardized algorithm based on D-dimer testing and computed tomography pulmonary angiography (CTPA). PE was ruled out by a D-dimer value <500 μg/L in 20 (41 %) patients and by negative CTPA in 40 (82 %). PE was confirmed in 9 patients. The prevalence of PE was 18 %. One patient with normal D-dimer had PE. Presenting symptoms and signs were similar between patients who did and did not have PE. PE was detected in 18 % of COPD patients who were hospitalized for an acute exacerbation. This finding supports the systematic evaluation of PE in hospitalized COPD exacerbated patients.
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http://dx.doi.org/10.1007/s10140-014-1280-7DOI Listing
June 2015

A patient presenting with an acute shortness of breath.

Eur J Intern Med 2015 Sep 16;26(7):e13-4. Epub 2014 Sep 16.

Department of Radiology, Hillel Yaffe Medical Center, Hadera, Israel. Electronic address:

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http://dx.doi.org/10.1016/j.ejim.2014.08.004DOI Listing
September 2015