Abstract Background/aims Long-term outcomes are understudied in patients with well-differentiated appendiceal neuroendocrine neoplasms (WD-ANENs). We aimed to evaluate the validity of currently applied criteria for completion prophylactic right hemicolectomy (pRHC) and determine its association with patient outcomes, including health-related quality of life (HRQoL). Methods...
Abstract Purpose To describe the case of a 9-year-old male patient with neurofibromatosis type 1 (NF1), partial empty sella (PES), transient central adrenal insufficiency (CAI) and growth hormone (GH) deficiency (GHD) treated with recombinant GH (rGH). Methods The diagnosis of GHD was established upon peak GH response <10 ng/mL following glucagon...
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Purpose: Pre-clinical studies show that anti-angiogenic therapy exacerbates tumor glycolysis and activates liver kinase B1 (LKB1)/AMP kinase (AMPK), a pathway involved in regulation of tumor metabolism. We investigated whether certain metabolism-related in situ biomarkers could predict benefit to regorafenib in the phase 2 randomized REGOMA trial. Experimental Design: Immunohistochemistry and digital pathology analysis were used to investigate expression in GBM sections of monocarboxylate transporter...
T-cell receptor β (TCRβ) analysis can be exploited to monitor changes in the clonotypic composition of TIL during ex vivo expansion in IL-2. Oligoclonal TIL are often outgrown by infrequent clonotypes with greater proliferative capacity, and this could impact on the antitumor reactivity of the expanded TIL.
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Next-generation sequencing of solid tumors has revealed variable signatures of immunogenicity across tumors, but underlying molecular characteristics driving such variation are not fully understood. While expression of endogenous retrovirus (ERV)-containing transcripts can provide a source of tumor-specific neoantigen in some cancer models, associations between ERV levels and immunogenicity across different types of metastatic cancer are not well established. We performed bioinformatics analysis...
Despite advances in the treatment of acute myeloid leukemia (AML), novel therapies are needed to induce deeper and more durable clinical response. Bispecific T cell Engager (BiTE®) molecules, which redirect patient T cells to lyse tumor cells, are a clinically-validated modality for hematologic malignancies. Due to broad AML expression and limited normal tissue expression, FLT3 is proposed to be an optimal BiTE® molecule target. Expression profiling of FLT3 was performed in primary AML patient samples...
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Abstract A 15-year-old female former gymnast with a history of pectus excavatum was reviewed due to unexplained paraparesis and urinary incontinence since age 10. Symptoms were commenced with intolerable upper back pain and development of a soft mass at the sacrum that remitted spontaneously. Brain and whole spine MRI imaging and blood and CSF testing were normal. The combination of skeletal, neurological, and bladder symptoms with normal lumbar MRI and abnormal urodynamic and...
Abstract Amniotic band syndrome (ABS) has been known since ancient times. Descriptions in modern medicine have occurred since the mid-nineteenth century. The association of the amniotic band syndrome with tethered cord is rare. It was generally thought to be incompatible with life. Of late, with better imaging, there have been case reports of amniotic bands causing cord tethering in neurosurgical literature, but its association with limited dorsal myeloschisis has hitherto not...
Abstract Object Traumatic atlanto-occipital dislocation (AOD) is a relatively uncommon traumatic cervical spine injury characterized by disruption and instability of the atlanto-occipital joint. At many centers, management of pediatric AOD includes occipitocervical arthrodesis, but whether external immobilization without surgery is a viable treatment option for some pediatric patients is unknown. To answer this question, we analyzed...
Abstract Purpose Many techniques were used for the treatment of hydrocephalus, and ventriculoperitoneal shunt surgery is a widely used procedure. Ventriculoperitoneal shunt surgery has been associated with several complications like obstruction of the tube, infection, cerebrospinal fluid loculation, intestinal obstruction, migration of the shunt, and perforation of the intestinal organs. Perforation of the bowel owing to protrusion...
Abstract Introduction Ventriculoperitoneal shunts (VPS) is commonly performed by pediatric neurosurgeons and there is no consensus in management of VPS infection as it relates to diagnosis and treatment. Objective We utilized an international practitioner-based survey to study the variability in VPS infection diagnostic and therapeutic measures. ...
Abstract Congenital encephalocele is a very rare entity, with herniation of normal brain or gliotic tissue through a defect in the skull. The objective is to present a newborn child diagnosed with transethmoidal encephaloceles at birth. She developed respiratory problems, feeding difficulties, and failure to thrive since the first days of life and so required early surgery at her 33th day of life, through an endoscopic nasal approach. Technical difficulties encountered, complications,...
Abstract Introduction Tuberous sclerosis complex (TSC) is a rare autosomal dominant disorder affecting multiple systems, due to inactivating mutations of TSC1 or TSC2 mTOR pathway genes. Neurological manifestations are observed in about 95% cases, representing the most frequent cause of morbidity and one of the most common causes of mortality. Background ...
Abstract Objective Hemifacial spasm (HFS) is usually caused by compression of the facial nerve at the root exit zone (REZ), and is extremely rare in adolescents and even rarer in aneurysm compression. Case report We describe symptomatic hemifacial spasm caused by a saccular aneurysm of the anterior inferior cerebellar artery (AICA) that was treated...
Abstract Purpose For young children and adolescents with Chiari malformation type I (CM-I), the determinants of extended length of hospital stay (LOS) after neurosurgical suboccipital decompression are obscure. Here, we investigate the impact of patient- and hospital-level risk factors on extended LOS following surgical decompression for CM-I in young children to adolescents. ...
Abstract Ventriculoperitoneal shunt placement for the treatment of hydrocephalus is one of the most common pediatric neurosurgical procedures. Complications, including infections, catheter obstruction, shunt breakdown, and hemorrhage, have been described in the literature. Occasionally, however, uncommon and devastating complications occur. We report a case of a 10-year-old female patient who at birth underwent surgical closure of lumbar myelomeningocele and placement of a CSF...
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