Malignant Peripheral Nerve Sheath Tumor Arising from an Adrenal Ganglioneuroma in a 6-Year-old Boy

Abstract

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.

Clinicopathological characteristics of neck ganglioneuroma

“…The Schwann cells showed positive staining for S-100 and negative staining for CgA, the opposite of the ganglion cells. The result is inconsistent with other reports (11)(12)(13) in which the ganglion cells in tumors showed positive immunohistochemical staining for S-100 and variable staining for Syn. The inconsistent findings by immunohistochemistry may be attributed to different tumor location, resource of primary antibody, treatment of the specimen, and method of the immunohistochemistry.…”

Morbidity After Ganglioneuroma Excision: Is Surgery Necessary?

“…However, most of these papers reported the histological diagnosis prior to the introduction of the International Neuroblastoma Pathology Classifi cation (the Shimada system) [10, 14, 15, 17 -19] , and it is possible that many of these tumours would now be diagnosed as iGNB (a form with potentially malignant characteristics) rather than GN. Only a few case reports of GN with malignant transformation were published after the introduction of the International Neuroblastoma Pathology Classifi cation [10,16,20] . In our study the resected tumours were reviewed by a paediatric pathologist according to the current guidelines and a change of diagnosis from GN to iGNB was made in 4 children (17 % ) in whom focal nests of neuropils with maturing ganglion cells were present in tumours showing predominant ganglioneuromatous diff erentiation, but all these patients survived without evidence of tumour relapse.…”

Adrenal Ganglioneuroma: A Rare Case Report and Literature Review

“…Laproscopical surgical resection is still the primary treatment for that carcinoma [1,16]. A rare case revealed adrenal ganglioneuromas maybe bloom into neuroblastoma in child, that reminds it is very meaningful to undergo postoperative regular examination and long-term follow-up [17].…”