scholarly journals ADVL1522: A phase 2 study of lorvotuzumab mertansine (IMGN901) in children with relapsed or refractory wilms tumor, rhabdomyosarcoma, neuroblastoma, pleuropulmonary blastoma, malignant peripheral nerve sheath tumor, or synovial sarcoma—A Children's Oncology Group study

Cancer ◽  
2020 ◽  
Vol 126 (24) ◽  
pp. 5303-5310
Author(s):  
James I. Geller ◽  
Joseph G. Pressey ◽  
Malcolm A. Smith ◽  
Rachel A. Kudgus ◽  
Mariana Cajaiba ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Wilms Tumor ◽  
Pleuropulmonary Blastoma ◽  
Nerve Sheath Tumor ◽  
Phase 2 ◽  
Oncology Group ◽  
Oncology Group Study ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Phase 2 Study

scholarly journals Wilms’ Tumor Gene 1 (WT1) Silencing Inhibits Proliferation of Malignant Peripheral Nerve Sheath Tumor sNF96.2 Cell Line

PLoS ONE ◽  
2014 ◽  
Vol 9 (12) ◽  
pp. e114333 ◽  
Author(s):  
Rosalba Parenti ◽  
Venera Cardile ◽  
Adriana Carol Eleonora Graziano ◽  
Carmela Parenti ◽  
Assunta Venuti ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Cell Line ◽  
Wilms Tumor ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Wilms Tumor Gene ◽  
Nerve Sheath ◽  
Tumor Gene

scholarly journals Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Replacing an Absent Kidney in a Child

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Samin Alavi ◽  
M. T. Arzanian ◽  
Yalda Nilipour
Keyword(s):  
Peripheral Nerve ◽  
Wilms Tumor ◽  
Abdominal Mass ◽  
Soft Tissue Sarcomas ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
First Case ◽  
Genitourinary Tract Anomalies

Malignant peripheral nerve sheath tumors (MPNSTs) are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. He underwent surgical resection of the tumor with a primary impression of Wilms tumor. To the authors’ knowledge, this is the first case of retroperitoneal malignant peripheral nerve sheath tumor and absent kidney. This case suggests the very rare probability of association of MPNSTs in children with genitourinary tract anomalies such as renal agenesis.

Malignant Peripheral Nerve Sheath Tumor Arising from a Solitary Neurofibroma in a Patient without Neurofibromatosis Type 1

2016 ◽  
Vol 78 (5) ◽  
pp. 516-521
Author(s):  
Fumihisa SAWADA ◽  
Eiichi MAKINO ◽  
Takenobu YAMAMOTO ◽  
Ryo TANAKA ◽  
Yutaka FUJIWARA ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Solitary Neurofibroma

scholarly journals RARE-20. MALIGNANT PERIPHERAL NERVE SHEATH TUMOR OF A CRANIAL NERVE IN AN INFANT WITH NEUROCUTANEOUS MELANOSIS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii446-iii446
Author(s):  
Lacey Carter ◽  
Naina Gross ◽  
Rene McNall-Knapp ◽  
,and Jo Elle Peterson
Keyword(s):  
Peripheral Nerve ◽  
Cranial Nerve ◽  
Leptomeningeal Disease ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Neurocutaneous Melanosis ◽  
Spindle Cell Neoplasm ◽  
Lower Back ◽  
Nerve Sheath ◽  
S 100

Abstract At one month of age, a female presented with a giant congenital nevus along lower back and thighs and hydrocephalus. A ventriculoperitoneal shunt was placed. An MRI was done at six months, initially reported as normal. At eleven months of age, five months after original MRI, patient presented with dysconjugate gaze and lethargy. MRI showed new 3.8 x 3.7 x 3.4 cm right cerebellopontine angle mass extending into Meckel’s cave and foramen ovale along with leptomeningeal disease extending from the mass along the entire length of the spinal cord. Retrospective review of prior MRI revealed subtle leptomeningeal enhancement concerning for neurocutaneous melanosis (NCM). Given the leptomeningeal disease, family elected for open biopsy and debulking of lesion instead of aggressive resection. Histologically, the mass showed hypercellular spindle cell neoplasm with mitotic activity and necrosis mixed with remnants of normal cranial nerve. GFAP was negative, excluding a glioma. HMB-45, MITF, panmelanoma, and Melan-A were negative, excluding melanoma. A negative myogenin stain ruled out ectomesenchymoma. S-100 protein and SOX-10 positivity with variable loss of staining for trimethylation of histone H3 K27 were indicative of malignant peripheral nerve sheath tumor (MPNST). Given the course of the mass, trigeminal nerve MPNST was presumed. Given the poor prognosis of intracranial MPNST and NCM, family elected to forgo treatment and was discharged with hospice. She died 25 days after surgery. Cranial nerve MPNST is rare. MPNST in patients with NCM has not previously been reported to our knowledge.

Chromosomal translocations inactivating CDKN2A support a single path for malignant peripheral nerve sheath tumor initiation

2021 ◽  
Author(s):  
Miriam Magallón-Lorenz ◽  
Juana Fernández-Rodríguez ◽  
Ernest Terribas ◽  
Edgar Creus-Batchiller ◽  
Cleofe Romagosa ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Chromosomal Translocations ◽  
Nerve Sheath Tumor ◽  
Tumor Initiation ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Single Path

Gastric Malignant Peripheral Nerve Sheath Tumor

2015 ◽  
Vol 23 (6) ◽  
pp. 505-508 ◽  
Author(s):  
Eun Young Kim ◽  
Sung Hak Lee ◽  
Han Mo Yoo ◽  
Kyo Young Song ◽  
Cho Hyun Park
Keyword(s):  
Peripheral Nerve ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath
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