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Malignant nerve sheath tumor

Hannah Gilder, Ross C Puffer, Robert J Spinner, Aditya Raghunathan, Mohamad Bydon
It is important to differentiate low grade malignant peripheral nerve sheath tumors (MPNSTs) from benign nerve sheath tumors as MPNSTs may require a more aggressive treatment strategy during and after initial resection. Loss of expression of the trimethyl Histone H3 at the Lys27 position (H3K27-me3) has recently been described in MPNSTs, and may help distinguish this tumor from pathologic mimics. A 43-year-old woman presented with symptoms of radiculopathy and a history of pelvic radiation for cervical cancer 7 years prior...
June 14, 2018: World Neurosurgery
Muhammad Firdaus, Arwinder Singh Gill, Dewi Aisiyah Mukarramah, Rini Andriani, Lenny Sari, Dian Cahyanti, Ahmad Faried
Background: Malignant peripheral nerve sheath tumors (MPNSTs) constitute a group of soft tissue neoplasm with neuroectodermal origin. Most cases are small at presentation and only some have been described reaching giant dimensions. Case Description: We report two cases that were diagnosed and treated as giant MPNST of the scalp. Both patients had extensive lesion on the head with intracranial infiltration. Microsurgical resection was indicated and a vascularized free flap was used to cover the defect...
2018: Surgical Neurology International
Christopher Weiyang Liu, David Flamer
The perineural administration of alcohol or phenol results in protein denaturation and, consequently, neurolysis. This can produce long-lasting analgesia, with a duration of 3-6 months. Although neurolytic blocks for the brachial plexus have been described in multiple review articles and textbooks, they are rarely performed. As such, little is known about the efficacy and adverse effect profile of this commonly described treatment. In this article, we report the outcomes of a patient who underwent a left brachial plexus neurolytic block for the management of upper limb pain arising from a malignant peripheral nerve sheath tumor...
June 11, 2018: A&A practice
Diana W Bartenstein, Taylor M Coe, Samantha C Gordon, Alison M Friedmann, Maryanne M Senna, Cassandra M Kelleher, Cristina R Antonescu, Rosalynn M Nazarian, Elena B Hawryluk
A 14-month-old boy presented with a slow-growing, asymptomatic back plaque, which was biopsied and found to have S100 positivity, sparse CD34 staining, and no significant mitotic activity, nuclear pleomorphism, or necrosis; genetic workup found LMNA-NTRK1 gene fusion, overall consistent with lipofibromatosis-like neural tumor (LPF-NT). LPF-NT is rare, with 14 cases previously reported, and our patient is the first report of this diagnosis in infancy. This case report and literature review includes comparison of similar diagnoses including lipofibromatosis, low-grade malignant peripheral nerve sheath tumor, infantile fibrosarcoma, and dermatofibrosarcoma protuberans and serves to aid detection of LPF-NT presenting in pediatric patients by highlighting similarities and differences that should prompt consideration...
March 2018: JAAD Case Reports
Probst Monika, Koerdt Steffen, Ritschl Lucas Maximilian, Bissinger Oliver, Liesche Friederike, Gempt Jens, Meyer Bernhard, Burian Egon, Lummel Nina, Kolk Andreas
OBJECTIVE: Malignant peripheral nerve sheath tumors (MPNST) are infiltrating, aggressive tumors belonging to the group of soft tissue sarcomas. This report refers to three patients with a tumorous swelling in the entire inferior alveolar nerve (IAN) with similar disease courses suspect for a MPNST, which is particularly rare in the trigeminal nerve. METHODS: Diagnostic tools, surgical proceedings and reconstructive procedures were highlighted. Three male patients (58-68 years), who suffered from numbness, pain and mild swelling in the sensation area served by the mental nerve presented at the department of oral and maxillofacial surgery and underwent diagnostic workup including CT, MRI, F18-PET-CT, as well as a biopsy of the clinical visible tumor mass with histopathological and molecular pathological analysis...
June 5, 2018: World Neurosurgery
Nwokoro Chingbundu Collins, Emmanuel Ezekiel Ayodeji, Fatungase Oluwabunmi Motunrayo, Salami Babatunde Abayomi, Ogundele Ibukunolu Olufemi, Amosu Lukmon Olusesan
Background: Neurofibromatosis is a genetically inherited disorder of the nervous system (brain and spinal cord) which mainly affects the development of nerve (neural) cell tissues, causing tumors (neurofibromas) to develop on nerves. It is the most common single gene disorder of the nervous system and inheritance is through autosomal dominance. They are usually classified into types 1 and 2, the type 1 is the commoner type and also known as superficial neurofibroma. Plexiform neurofibromas are the next most common type of tumor in individuals with type 1 neurofibroma...
March 2018: Malawi Medical Journal: the Journal of Medical Association of Malawi
Yin P Hung, Christopher D M Fletcher, Jason L Hornick
AIMS: Infantile fibrosarcoma is characterized by intersecting fascicles of spindle cells and ETV6-NTRK3 gene fusion in most cases. Given histologic overlap with other spindle-cell tumors, the diagnosis can be challenging and often requires molecular confirmation. A recently developed pan-TRK antibody shows promise for identifying tumors with NTRK fusions. The purpose of this study was to evaluate the potential diagnostic utility of pan-TRK immunohistochemistry for infantile fibrosarcoma...
June 4, 2018: Histopathology
Vickie Y Jo, Adrián Mariño-Enríquez, Christopher D M Fletcher, Jason L Hornick
Biphenotypic sinonasal sarcoma (BSNS) is a distinctive, anatomically restricted, low-grade spindle cell sarcoma that shows considerable histologic overlap with other cellular spindle cell neoplasms. This tumor type shows both myogenic and neural differentiation, which can be demonstrated by immunohistochemistry; however, the available diagnostic markers are relatively nonspecific. BSNS is characterized by PAX3 rearrangements, with MAML3 as the most common fusion partner. Our aim was to determine whether immunohistochemistry using a monoclonal PAX3 antibody could distinguish BSNS from potential histologic mimics, as well as to evaluate a widely available polyclonal PAX8 antibody, which is known to cross-react with other paired box transcription factor family members...
May 31, 2018: American Journal of Surgical Pathology
Rashmi Kumari, Martin R Silic, Yava L Jones-Hall, Alexandra Nin-Velez, Jer-Yen Yang, Suresh K Mittal, GuangJun Zhang
Malignant peripheral nerve sheath tumors (MPNSTs) are a type of sarcoma with poor prognosis due to their complex genetic changes, invasive growth, and insensitivity to chemo- and radiotherapies. One of the most frequently lost chromosome arms in human MPNSTs is chromosome 9p. However, the cancer driver genes located on it remain largely unknown, except the tumor suppressor gene, p16 (INK4)/CDKN2A . Previously, we identified RECK as a tumor suppressor gene candidate on chromosome 9p using zebrafish-human comparative oncogenomics...
May 4, 2018: Oncotarget
Hsiang-Chih Lu, Vanessa Eulo, Anthony J Apicelli, Melike Pekmezci, Yu Tao, Jingqin Luo, Angela C Hirbe, Sonika Dahiya
Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are aggressive soft tissue sarcomas that can occur sporadically or in the setting of the Neurofibromatosis type 1 (NF1) cancer predisposition syndrome. These tumors carry a dismal overall survival. Previous work in our lab had identified ATRX chromatin remodeler ( ATRX ), previously termed, Alpha Thalassemia/Mental Retardation Syndrome X Linked as a gene mutated in a subset of MPNSTs. Given the great need for novel biomarkers and therapeutic targets for MPNSTs, we sought to determine the expression of ATRX in a larger subset of sporadic and NF1 associated MPNSTs (NF1-MPNSTs)...
May 1, 2018: Oncotarget
Meritxell Carrió, Bernat Gel, Ernest Terribas, Adriana Carolina Zucchiatti, Teresa Moliné, Inma Rosas, Álex Teulé, Santiago Ramón Y Cajal, Juan Carlos López-Gutiérrez, Ignacio Blanco, Elisabeth Castellanos, Conxi Lázaro, Anat Stemmer-Rachamimov, Cleofé Romagosa, Eduard Serra
Plexiform neurofibromas (PNFs) are benign peripheral nerve sheath tumors involving large nerves present in 30-50% Neurofibromatosis type 1 (NF1) patients. Atypical neurofibromas (ANF) are distinct nodular lesions with atypical features on histology that arise from PNFs. The risk and timeline of malignant transformation in ANF is difficult to assess. A recent NIH workshop has stratified ANFs and separated a subgroup with multiple atypical features and higher risk of malignant transformation termed atypical neurofibromatous neoplasms with uncertain biological potential (ANNUBP)...
May 17, 2018: Human Mutation
Jiazhong Ren, Guoren Yang, Jing Zhou, Zheng Fu
RATIONALE: Neurofibromatosis type one (NF1) is characterized by cutaneous and nervous lesions, and the tendency to form plexiform neurofibromas (PNFs). PNFs may undergo malignant transformation into a malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs often carry an significant morbidity and mortality. PATIENT CONCERNS: A 17-year-old man with gradually increased multiple subcutaneous soft lesions. He also presented with numerous lentigines and multiple café-au-lait macules on his body...
May 2018: Medicine (Baltimore)
Giancarlo A Garcia, Andrew E Choy, Anton N Hasso, Don S Minckler
Purpose of the Study: Orbital meningiomas are typically benign tumors, most commonly originating from the dura of the sphenoid wing or the optic nerve sheath. Procedures: We describe an unusual case of a malignant meningioma originating from the frontal lobe that ultimately produced orbital and distant metastases. Results and Conclusions: Orbital invasion by the meningioma was preceded by multiple incomplete resections, which may have facilitated access to the orbit...
April 2018: Ocular Oncology and Pathology
Masanori Teshima, Hirotaka Shinomiya, Naoki Otsuki, Hidehito Kimura, Masaaki Taniguchi, Kazunobu Hashikawa, Eiji Kohmura, Ken-Ichi Nibu
Objective  Nasal and paranasal malignant tumors invading the skull base are rare and poorly studied. We evaluated postoperative complications in patients undergoing salvage surgery for such tumors. Design  Retrospective study. Setting  Kobe University Hospital. Participants  Among 48 patients who underwent surgery for tumors involving the skull base between 1993 and 2015, 21 patients had squamous cell carcinoma, 13 had olfactory neuroblastoma, 5 had adenocarcinoma, 2 had sarcoma, 2 had adenoid cystic carcinoma, and 1 each had malignant melanoma, poorly differentiated carcinoma, undifferentiated carcinoma, myoepithelial carcinoma, and malignant peripheral nerve sheath tumor...
June 2018: Journal of Neurological Surgery. Part B, Skull Base
Jody F Longo, Shannon M Weber, Brittany P Turner-Ivey, Steven L Carroll
The diagnosis of a neurofibroma or a malignant peripheral nerve sheath tumor (MPNST) often raises the question of whether the patient has the genetic disorder neurofibromatosis type 1 (NF1) as well as how this will impact the patient's outcome, what their risk is for developing additional neoplasms and whether treatment options differ for NF1-associated and sporadic peripheral nerve sheath tumors. Establishing a diagnosis of NF1 is challenging as this disorder has numerous neoplastic and non-neoplastic manifestations which are variably present in individual patients...
May 4, 2018: Advances in Anatomic Pathology
Martin R Silic, GuangJun Zhang
Bioelectricity, endogenous electrical signaling mediated by ion channels and pumps located on the cell membrane, plays important roles in signaling processes of excitable neuronal and muscular cells and many other biological processes, such as embryonic developmental patterning. However, there is a need for in vivo electrical activity monitoring in vertebrate embryogenesis. The advances of genetically encoded fluorescent voltage indicators (GEVIs) have made it possible to provide a solution for this challenge...
April 25, 2018: Journal of Visualized Experiments: JoVE
Olimpia Alessandra Buoninfante, Bas Pilzecker, Muhammad Assad Aslam, Ioannis Zavrakidis, Rianne van der Wiel, Marieke van de Ven, Paul C M van den Berk, Heinz Jacobs
DNA damage tolerance (DDT) enables replication to continue in the presence of a damaged template and constitutes a key step in DNA interstrand crosslink repair. In this way DDT minimizes replication stress inflicted by a wide range of endogenous and exogenous agents, and provides a critical first line defense against alkylating and platinating chemotherapeutics. Effective DDT strongly depends on damage-induced, site-specific PCNA-ubiquitination at Lysine (K) 164 by the E2/E3 complex (RAD6/18). A survey of The Cancer Genome Atlas (TCGA) revealed a high frequency of tumors presents RAD6/RAD18 bi-allelic inactivating deletions...
April 10, 2018: Oncotarget
Jacqueline D Peacock, Matthew G Pridgeon, Elizabeth A Tovar, Curt J Essenburg, Megan J Bowman, Zachary B Madaj, Julie Koeman, Elissa A Boguslawski, Jamie Grit, Rebecca D Dodd, Vadim Khachaturov, Diana M Cardona, Mark Chen, David G Kirsch, Flavio Maina, Rosanna Dono, Mary E Winn, Carrie R Graveel, Matthew R Steensma
Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are highly resistant sarcomas that occur in up to 13% of individuals with Neurofibromatosis Type 1 (NF1). Genomic analysis of longitudinally collected tumor samples in a case of MPNST disease progression revealed early hemizygous microdeletions in NF1 and TP53, with progressive amplifications of MET, HGF, and EGFR. To examine the role of MET in MPNST progression, we developed mice with enhanced MET expression and Nf1 ablation (Nf1fl/KO;lox-stop-loxMETtg/+;Plp-creERTtg/+; referred to as NF1 MET)...
May 2, 2018: Cancer Research
Ibrahim M Hanif, Nilesh H Pawar, Wing Yan Mok, Melvin Chua
Schwannomas are nerve sheath tumors that occur in Schwann cells. They are usually benign, but malignant transformation can occur. Symptomatology depends on the involvement of the surrounding tissues or the mass effect of the tumor. We describe a case of a 28-year-old man who initially presented with right iliac fossa pain associated with radiating pain over the anterior and lateral aspect of his right knee. Following subsequent investigations, we found a retroperitoneal schwannoma of the right lateral femoral cutaneous nerve...
February 21, 2018: Curēus
Shivani Ahlawat, Laura M Fayad
OBJECTIVE: To determine the utility of "target sign" on diffusion weighted imaging (DWI) and apparent diffusion coefficient (ADC) mapping for peripheral nerve sheath tumor (PNST) characterization. MATERIALS AND METHODS: This IRB-approved, HIPAA-compliant study retrospectively reviewed the MR imaging (comprised of T2- FS, DWI (b-values 50, 400, 800 s/mm2 and ADC mapping), and static contrast-enhanced (CE) T1-W imaging) of 42 patients (mean age: 40 years (range 8-68 years), 48% (20/42) females) with 15 malignant PNSTs (MPNSTs) and 33 benign PNSTs (BPNSTs)...
May 2018: European Journal of Radiology
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