1988
DOI: 10.1111/j.1365-2559.1988.tb01959.x
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Malignant nerve sheath tumour arising in a ganglioneuroma

Abstract: A malignant nerve sheath tumour arising within a thoracic ganglioneuroma is described. This is only the seventh such case described in the literature and the first at this site. The previously documented cases are reviewed.

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Cited by 32 publications
(14 citation statements)
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“…However, there have been at least seven documented cases of transformation of ganglioneuromas into malignant nerve sheath tumors. 3,5,7,8,11,14 Although malignant transformation involving the spindle cell component of a ganglioneuroma is a rare but proven event, to our knowledge, malignant transformation of the ganglion cell component into neuroblastoma has never been reported. The argument against this theory is that the neuronal component of a ganglioneuroma is presumably composed of mature, well-differentiated, postmitotic ganglion cells that should have lost the capacity to proliferate.…”
Section: Discussionmentioning
confidence: 98%
“…However, there have been at least seven documented cases of transformation of ganglioneuromas into malignant nerve sheath tumors. 3,5,7,8,11,14 Although malignant transformation involving the spindle cell component of a ganglioneuroma is a rare but proven event, to our knowledge, malignant transformation of the ganglion cell component into neuroblastoma has never been reported. The argument against this theory is that the neuronal component of a ganglioneuroma is presumably composed of mature, well-differentiated, postmitotic ganglion cells that should have lost the capacity to proliferate.…”
Section: Discussionmentioning
confidence: 98%
“…In three of these cases the PNSTs arose following radiotherapy to primary neuroblastomas, and could be considered to be radiation-induced neoplasms 10,13 . In four cases the transformation into malignant PNSTs appears to have been spontaneous 1,4,7,8 . It is likely that these tumours arose as a result of malignant transformation in the stromal cell component of the ganglioneuroma with Schwann cells the most probable cell of origin, as the mature post-mitotic ganglion cells should have lost the capacity to proliferate 11,13 .…”
Section: Discussionmentioning
confidence: 95%
“…Generally, ganglioneuromas appear secondary to maturation of a neuroblastoma or a ganglioneuroblastoma, although malignant degeneration of primary ganglioneuromas has also been described. 6,7 These tumors may demonstrate hormonal activity and secrete catecholamine as well as intestinal vasoactive peptide. They then cause diarrhea, distension of the abdomen, hot fl ushes, hypertension, and hypoglycemia.…”
Section: Discussionmentioning
confidence: 99%