Malignant peripheral nerve sheath tumor arising in a “de novo” ganglioneuroma: A case report and review of the literature

Abstract

A case of a “de novo” ganglioneuroma showing a large area of malignant peripheral nerve sheath tumor (MPNST) is described. The tumor arose in an 11.5‐year‐old girl with neither stigmata nor family history of von Recklinghausen's neurofibromatosis. In addition, the patient had no previous history of a neuroblastoma or radiation therapy. This report provides new evidence that, although rare, the spontaneous development of an MPNST in a benign ganglioneuroma can occur. Immunohistochemical and electron microscopy studies supported the finding that the spindle cell component was of nerve sheath origin. Med. Pediatr. Oncol. 28:216–222 © 1997 Wiley‐Liss, Inc.

Practical Issues for Retroperitoneal Sarcoma

“…Malignant transformation into MPNST has been reported. 90,91 Schwannoma, a peripheral nerve sheath tumor consisting of well-differentiated Schwann cells, is usually encapsulated, and its cut surfaces have a pink, white, or yellow appearance. 66 Retroperitoneal tumors are large and may have areas of cystic degeneration Fig 9. -Axial contrast-enhanced computed tomography demonstrates a heterogeneous nonadipocytic, largely necrotic upper retroperitoneal mass along the aorta (stent graft in place) and pulmonary metastases.…”

Ganglioneuroma of the uterine cervix—a case report

“…Surgical excision is usually sufficient treatment, but metastasis has been reported in ganglioneuromas, representing maturation of neuroblastoma [13]. Rarely, ganglioneuroma may reveal malignant transformation, most commonly malignant peripheral nerve sheath tumor, considering de novo ganglioneuroma or that derived from maturation in a neuroblastoma [14,15]. Thus, regular follow-up is necessary.…”

Laparoscopic excision of retroperitoneal tumors: Report of three cases

“…19 Ganglioneuroma is a welldifferentiated tumor, most commonly seen in children older than 10 years. 20 Interestingly, Moschovi et al 21 and Kulkarni et al 22 described the malignant transformation of dormant ganglioneuroma, and Drago et al 23 reported the spontaneous development of a malignant peripheral nerve-sheath tumor in benign ganglioneuroma. Thus, careful monitoring may be necessary after the resection of benign retroperitoneal tumors.…”