This report describes the youngest patient to develop a malignant peripheral nerve sheath tumor arising from a ganglioneuroma (MPNST ex Ganglioneuroma). The patient, a 6-year-old boy, was never irradiated and had no history or stigmata of neurofibromatosis. The report also includes a review of the previously published related cases, and an analysis of the immunohistochemistry and electron microscopy data available to date on the subject.
CoffinCM, DehnerLP. Peripheral neurogenic tumors of the soft tissues in children and adolescents: a clinicopathologic study of 139 cases. Pediatr Pathol1989;9:387–407.
2.
RicciAJr, ParhamDM, WoodruffJM, GreenA, CallihanT, ErlandsonRA. Malignant peripheral nerve sheath tumor arising from ganglioneuromas. Am J Surg Pathol1984;18:19–29.
3.
KellerSM, PapazoglouS, McKeeverP, BakerA, RothJA. Late occurrence of malignancy in a ganglioneuroma 19 years following radiation therapy to a neuroblastoma. J Surg Oncol1984;25:227–231.
4.
ChandrasomaP, ShibataD, RadinR, PinterBrown L, KossM.Malignant peripheral nerve sheath tumor arising in an adrenal ganglioneuroma in an adult male homosexual. Cancer1986;57:2022–2025.
5.
FletcherCD, FernandoIN, BraimbridgeMV, McKeePH, LyallJR. Malignant nerve sheath tumor arising in a ganglioneuroma. Histopathology1988;12:445–448.
6.
BanksE, MoonahmY, BrodheckerC, GoheenM.A malignant peripheral nerve sheath tumor in association with a paratesticular ganglioneuroma. Cancer1989;64:1738–1742.
7.
DamianiS, ManettoV, CarilloG, Di BlasiA, NappiO, EusebiV.Malignant peripheral nerve sheath tumor arising in a “de novo” ganglioneuroma. A case report. Tumori1991; 77:90–93.
8.
GhaliVS, GoldJE, VincentRA, CosgroveJM. Malignant peripheral nerve sheath tumor arising spontaneously from retroperitoneal ganglioneuroma: a case report, review of the literature, and immunohistochemical study. Hum Pathol1992;23:72–75.
9.
DragoG, PasquierB, PasquierD, Malignant peripheral nerve sheath tumor arising in a “de novo” ganglioneuroma: a case report and review of the literature. Med Pediatr Oncol1997;28:216–222.
10.
GrippariJL, NeveuxY, ArborioM, Tumor of the peripheral nerve sheath with rhabdomyoblastic differentiation arising in a ganglioneuroma: neurolemmoma? A fortuitously detected case. Ann Pathol1996;16:128–132.
11.
NavarroO, Nunez-SantosE, DanemanA, FariaP, DaltroP.Malignant peripheral nerve-sheath tumor arising in a previously irradiated neuroblastoma: report of 2 cases and a review of the literature. Pediatr Radiol2000;30:176–180.
LackEE. Pathology of Adrenal and Extra-Adrenal Paraganglia. Major Problems in Pathology, vol. 29. Philadelphia: WB Saunders, 1994, 358–360.
14.
HiroseT, HasegawaT, KudoE, SekiK, SanoT, HizawaK.Malignant peripheral nerve sheath tumors: an immunohistochemical study in relation to ultrastructural features. Hum Pathol1992;.:865–870.
15.
KatsetosCD, KarkavelasG, FrankfurterA, The stromal Schwann cell during maturation of peripheral neuroblastomas: immunohistochemical observations with antibodies to the neuronal class III β-tubulin isotype (βIII), and S-100 protein. Clin Neuropathol1994;13:171–180.
16.
KatsetosCD, KarkavelasG, HermanMM, Class III β-tubulin isotype in the adrenal medulla. I. Localization in the developing human adrenal medulla. Anat Rec1998;250:335–343.
17.
KatsetosCD, HermanMM, MorkSJ (2003) Class III β-tubulin in human development and cancer. Cell Motil Cytoskeleton55: 77–96
18.
KatsetosCD, SavoryJ, HermanMM, Neuronal cytoskeletal lesions induced in the CNS by intraventricular and intravenous aluminium maltol in rabbits. Neuropathol Appl Neurobiol1990;16:511–528.
19.
MolenaarWM, MuntingheFL. Expression of neural cell adhesion molecules and neurofilament protein isoforms in Ewing's sarcoma of bone and soft tissue sarcomas other than rhabdomyosarcoma. Hum Pathol1999;30:1207–1212.
20.
Veeranna., AminND, AhnNG, Mitogen-activated protein kinases (Erk1,2) phosphorylate Lys-Ser-Pro (KSP) repeats in neurofilament proteins NF-H and NF-M. J Neurosci1998;18:4008–4021.
21.
WayJ, HellmichMR, JaffeH, A high-molecular-weight squid neurofilament protein contains a lamin-like rod domain and a tail domain with Lys-Ser-Pro repeats. Proc Natl Acad Sci USA1992;89:6963–6967.
22.
SchillingK, DuvernoyC, KeckS, PilgrimC.Detection and partial characterization of a developmentally regulated nuclear antigen in neural cells in vitro and in vivo. J Histochem Cytochem1989;37:241–247.
23.
AmbrosIM, ZellnerA, RoaldB, Role of ploidy, chromosome 1p, and Schwann cells in the maturation of neuroblastoma. N Engl J Med1996;.:1505–1511.
24.
MoraJ, CheungNK, JuanG, Neuroblastic and schwannian stromal cells of neuroblastoma are derived from a tumoral progenitor cell. Cancer Res2001;61:6892–6898.
25.
ScheithauerBW, WoodruffJM, ErlandsonRA. MPNST ex ganglioneuroma In: RosaiJ, SobuiLH, eds. Atlas of Tumor Pathology. Tumors of the Peripheral Nervous System. Third series, Fascicle 24. Washington, DC: Armed Forces Institute of Pathology, 1999; 350–356.
