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Childhood malignant schwannoma

MedGen UID:
83582
•Concept ID:
C0279987
•
Neoplastic Process
Synonyms: Childhood Malignant Neoplasm of Peripheral Nerve Sheath; childhood malignant neoplasm of peripheral nerve sheath; Childhood Malignant Neoplasm of the Peripheral Nerve Sheath; childhood malignant neoplasm of the peripheral nerve sheath; Childhood Malignant Neurilemmoma; childhood malignant neurilemmoma; Childhood Malignant Peripheral Nerve Sheath Neoplasm; childhood malignant peripheral nerve sheath neoplasm; Childhood Malignant Peripheral Nerve Sheath Tumor; childhood malignant peripheral nerve sheath tumor; childhood malignant peripheral nerve sheath tumour; Childhood Malignant Schwannoma; childhood malignant schwannoma; Childhood Malignant Tumor of Peripheral Nerve Sheath; childhood malignant tumor of peripheral nerve sheath; Childhood Malignant Tumor of the Peripheral Nerve Sheath; childhood malignant tumor of the peripheral nerve sheath; childhood malignant tumour of peripheral nerve sheath; childhood malignant tumour of the peripheral nerve sheath; Childhood MPNST; childhood MPNST; childhood neurofibrosarcoma; childhood neurogenic sarcoma; paediatric malignant neoplasm of peripheral nerve sheath; paediatric malignant neoplasm of the peripheral nerve sheath; paediatric malignant neurilemmoma; paediatric malignant peripheral nerve sheath neoplasm; paediatric malignant peripheral nerve sheath tumour; paediatric malignant schwannoma; paediatric malignant tumour of peripheral nerve sheath; paediatric malignant tumour of the peripheral nerve sheath; paediatric MPNST; Pediatric Malignant Neoplasm of Peripheral Nerve Sheath; pediatric malignant neoplasm of peripheral nerve sheath; Pediatric Malignant Neoplasm of the Peripheral Nerve Sheath; pediatric malignant neoplasm of the peripheral nerve sheath; Pediatric Malignant Neurilemmoma; pediatric malignant neurilemmoma; Pediatric Malignant Peripheral Nerve Sheath Neoplasm; pediatric malignant peripheral nerve sheath neoplasm; Pediatric Malignant Peripheral Nerve Sheath Tumor; pediatric malignant peripheral nerve sheath tumor; Pediatric Malignant Schwannoma; pediatric malignant schwannoma; Pediatric Malignant Tumor of Peripheral Nerve Sheath; pediatric malignant tumor of peripheral nerve sheath; Pediatric Malignant Tumor of the Peripheral Nerve Sheath; pediatric malignant tumor of the peripheral nerve sheath; Pediatric MPNST; pediatric MPNST
 
Monarch Initiative: MONDO:0004345

Definition

A malignant peripheral nerve sheath tumor occurring in children. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood malignant schwannoma

Professional guidelines

PubMed

Proportion of children with cancer that have an indication for genetic counseling and testing based on the cancer type irrespective of other features.
Nguyen TMK, Behnert A, Pietsch T, Vokuhl C, Kratz CP
Fam Cancer 2021 Oct;20(4):273-277. Epub 2021 Feb 26 doi: 10.1007/s10689-021-00234-4. PMID: 33634344Free PMC Article
Intraoperative electron irradiation in the management of pediatric malignancies.
Schomberg PJ, Gunderson LL, Moir CR, Gilchrist GS, Smithson WA
Cancer 1997 Jun 1;79(11):2251-6. PMID: 9179074

Recent clinical studies

Etiology

Cancer and Central Nervous System Tumor Surveillance in Pediatric Neurofibromatosis 2 and Related Disorders.
Evans DGR, Salvador H, Chang VY, Erez A, Voss SD, Druker H, Scott HS, Tabori U
Clin Cancer Res 2017 Jun 15;23(12):e54-e61. doi: 10.1158/1078-0432.CCR-17-0590. PMID: 28620005
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems.
Rodriguez FJ, Folpe AL, Giannini C, Perry A
Acta Neuropathol 2012 Mar;123(3):295-319. Epub 2012 Feb 12 doi: 10.1007/s00401-012-0954-z. PMID: 22327363Free PMC Article
Malignant transformation and new primary tumours after therapeutic radiation for benign disease: substantial risks in certain tumour prone syndromes.
Evans DG, Birch JM, Ramsden RT, Sharif S, Baser ME
J Med Genet 2006 Apr;43(4):289-94. Epub 2005 Sep 9 doi: 10.1136/jmg.2005.036319. PMID: 16155191Free PMC Article
Posterior mediastinal masses.
Saenz NC, Schnitzer JJ, Eraklis AE, Hendren WH, Grier HE, Macklis RM, Shamberger RC
J Pediatr Surg 1993 Feb;28(2):172-6. doi: 10.1016/s0022-3468(05)80268-4. PMID: 8437074
The value of adjuvant chemotherapy in the management of sarcomas in children.
Donaldson SS
Cancer 1985 May 1;55(9 Suppl):2184-97. doi: 10.1002/1097-0142(19850501)55:9+<2184::aid-cncr2820551422>3.0.co;2-n. PMID: 3884137

Diagnosis

Plexiform Cellular Schwannoma in Infancy and Childhood: A Clinicopathological Study of Seven Cases of an Underrecognized Nerve Sheath Tumor with a Tendency Toward Local Recurrence.
Sun M, Shao M, Liu J, Zhao L, Weng Lao I, Yu L, Wang J
Int J Surg Pathol 2022 May;30(3):265-272. Epub 2021 Oct 25 doi: 10.1177/10668969211052236. PMID: 34696615
The pathobiologic spectrum of Schwannomas.
Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM
Histol Histopathol 2003 Jul;18(3):925-34. doi: 10.14670/HH-18.925. PMID: 12792904
Posterior mediastinal masses.
Saenz NC, Schnitzer JJ, Eraklis AE, Hendren WH, Grier HE, Macklis RM, Shamberger RC
J Pediatr Surg 1993 Feb;28(2):172-6. doi: 10.1016/s0022-3468(05)80268-4. PMID: 8437074
The value of adjuvant chemotherapy in the management of sarcomas in children.
Donaldson SS
Cancer 1985 May 1;55(9 Suppl):2184-97. doi: 10.1002/1097-0142(19850501)55:9+<2184::aid-cncr2820551422>3.0.co;2-n. PMID: 3884137
Gastrointestinal neurofibromatosis.
Petersen JM, Ferguson DR
J Clin Gastroenterol 1984 Dec;6(6):529-34. doi: 10.1097/00004836-198412000-00008. PMID: 6439777

Therapy

Proportion of children with cancer that have an indication for genetic counseling and testing based on the cancer type irrespective of other features.
Nguyen TMK, Behnert A, Pietsch T, Vokuhl C, Kratz CP
Fam Cancer 2021 Oct;20(4):273-277. Epub 2021 Feb 26 doi: 10.1007/s10689-021-00234-4. PMID: 33634344Free PMC Article
Malignant transformation and new primary tumours after therapeutic radiation for benign disease: substantial risks in certain tumour prone syndromes.
Evans DG, Birch JM, Ramsden RT, Sharif S, Baser ME
J Med Genet 2006 Apr;43(4):289-94. Epub 2005 Sep 9 doi: 10.1136/jmg.2005.036319. PMID: 16155191Free PMC Article
Posterior mediastinal masses.
Saenz NC, Schnitzer JJ, Eraklis AE, Hendren WH, Grier HE, Macklis RM, Shamberger RC
J Pediatr Surg 1993 Feb;28(2):172-6. doi: 10.1016/s0022-3468(05)80268-4. PMID: 8437074
Therapy for childhood soft-tissue sarcomas other than rhabdomyosarcoma: a review of 62 cases treated at a single institution.
Horowitz ME, Pratt CB, Webber BL, Hustu HO, Etcubanas E, Miliauskas J, Rao BN, Fleming ID, Mahesh Kumar AP, Green AA
J Clin Oncol 1986 Apr;4(4):559-64. doi: 10.1200/JCO.1986.4.4.559. PMID: 3514805
The value of adjuvant chemotherapy in the management of sarcomas in children.
Donaldson SS
Cancer 1985 May 1;55(9 Suppl):2184-97. doi: 10.1002/1097-0142(19850501)55:9+<2184::aid-cncr2820551422>3.0.co;2-n. PMID: 3884137

Prognosis

Cancer and Central Nervous System Tumor Surveillance in Pediatric Neurofibromatosis 2 and Related Disorders.
Evans DGR, Salvador H, Chang VY, Erez A, Voss SD, Druker H, Scott HS, Tabori U
Clin Cancer Res 2017 Jun 15;23(12):e54-e61. doi: 10.1158/1078-0432.CCR-17-0590. PMID: 28620005
The pathobiologic spectrum of Schwannomas.
Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM
Histol Histopathol 2003 Jul;18(3):925-34. doi: 10.14670/HH-18.925. PMID: 12792904
Posterior mediastinal masses.
Saenz NC, Schnitzer JJ, Eraklis AE, Hendren WH, Grier HE, Macklis RM, Shamberger RC
J Pediatr Surg 1993 Feb;28(2):172-6. doi: 10.1016/s0022-3468(05)80268-4. PMID: 8437074
Mediastinal masses in childhood: a review from a paediatric pathologist's point of view.
Simpson I, Campbell PE
Prog Pediatr Surg 1991;27:92-126. doi: 10.1007/978-3-642-87767-4_7. PMID: 1907392
Neurofibromatosis in children.
Crawford AH
Acta Orthop Scand Suppl 1986;218:1-60. PMID: 3083645

Clinical prediction guides

Morphologic features in a series of 352 Spitz melanocytic proliferations help predict their oncogenic drivers.
Kervarrec T, Pissaloux D, Tirode F, Samimi M, Jacquemus J, Castillo C, de la Fouchardière A
Virchows Arch 2022 Feb;480(2):369-382. Epub 2021 Nov 11 doi: 10.1007/s00428-021-03227-x. PMID: 34761304
Cancer and Central Nervous System Tumor Surveillance in Pediatric Neurofibromatosis 2 and Related Disorders.
Evans DGR, Salvador H, Chang VY, Erez A, Voss SD, Druker H, Scott HS, Tabori U
Clin Cancer Res 2017 Jun 15;23(12):e54-e61. doi: 10.1158/1078-0432.CCR-17-0590. PMID: 28620005
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems.
Rodriguez FJ, Folpe AL, Giannini C, Perry A
Acta Neuropathol 2012 Mar;123(3):295-319. Epub 2012 Feb 12 doi: 10.1007/s00401-012-0954-z. PMID: 22327363Free PMC Article
Neurofibromatosis type 1 and childhood cancer.
Matsui I, Tanimura M, Kobayashi N, Sawada T, Nagahara N, Akatsuka J
Cancer 1993 Nov 1;72(9):2746-54. doi: 10.1002/1097-0142(19931101)72:9<2746::aid-cncr2820720936>3.0.co;2-w. PMID: 8402499
201Tl/99mTc-HMPAO SPECT imaging of treated childhood brain tumors.
O'Tuama LA, Janicek MJ, Barnes PD, Scott RM, Black PM, Sallan SE, Tarbell NJ, Kupsky WJ, Wagenaar D, Ulanski JS
Pediatr Neurol 1991 Jul-Aug;7(4):249-57. doi: 10.1016/0887-8994(91)90040-r. PMID: 1930415

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