Malignant peripheral nerve sheath tumor with multilineage divergent differentiation including a neuroblastic component

2021 ◽  
Author(s):  
Lucas F. Abrahao‐Machado ◽  
Carlos E. Bacchi ◽  
Cristielle P. Freitas ◽  
Jason L. Hornick
Keyword(s):  
Peripheral Nerve ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Divergent Differentiation

Malignant Peripheral Nerve Sheath Tumor With Divergent Differentiation

2003 ◽  
Vol 127 (3) ◽  
pp. e147-e150 ◽  
Author(s):  
Li Huang ◽  
Carmen Espinoza ◽  
Ronald Welsh
Keyword(s):  
African American ◽  
Peripheral Nerve ◽  
Pulmonary Embolus ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Spindle Cells ◽  
Divergent Differentiation ◽  
Rare Cells ◽  
Tumor Death

Abstract Right posterior thigh malignant peripheral nerve sheath tumor with malignant glandular and rhabdomyoblastic components was diagnosed in a 24-year-old African American man. Malignant glands and rhabdomyoblastic cells were admixed with the spindle cells. Immunohistochemistry demonstrated spindle cells positive for S100 and vimentin; the glandular component was positive for chromogranin, and rare cells were positive for CK20 but negative for CK7. Rhabdomyoblastic cells were positive for muscle-specific actin and desmin. A single pulmonary metastasis occurred 5 months after surgical resection of the tumor. Death occurred 1 month later and was probably due to a pulmonary embolus.

scholarly journals Malignant peripheral nerve sheath tumor with divergent differentiation

2009 ◽  
Vol 52 (1) ◽  
pp. 74 ◽  
Author(s):  
TN Suresh ◽  
ML Harendra Kumar ◽  
C.S.B.R Prasad ◽  
R Kalyani ◽  
K Borappa
Keyword(s):  
Peripheral Nerve ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Divergent Differentiation

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
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