Primary Neuroectodermal Tumors of the Ovary

Kleinman, George; Young, Robert H.; Scully, Robert E.

doi:10.1097/00000478-199308000-00002

Cited by 188 publications

(142 citation statements)

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“…1,11 A majority of such PNETs show morphologic features of those primitive tumors of the central nervous system that are characteristically observed in children, 11 especially in the posterior fossa, and this is also true for the PNETs that develop in the ovary. 20 There remains, however, relatively scant information regarding the morphologic spectrum of these PNETs. 10 These considerations prompted this study.…”

Section: Discussionmentioning

confidence: 99%

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Primitive neuroectodermal tumors in patients with testicular germ cell tumors usually resemble pediatric-type central nervous system embryonal neoplasms and lack chromosome 22 rearrangements

et al. 2010

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Primitive neuroectodermal tumors (PNETs) are one of the most frequent types of 'non-germ cell' tumor in patients with testicular germ cell tumors and have a guarded prognosis when present in metastatic sites after cisplatin-based chemotherapy. Improved treatments, including targeted therapy, require understanding the biology of these neoplasms. We therefore analyzed the morphologic, immunohistochemical and molecular biologic features of 14 PNETs from 14 patients with concurrent or previous testicular germ cell tumors; 12 tumors were from metastatic sites and 2 were primary in the testis. Using standard light microscopic criteria for central nervous system and peripheral PNETs, we classified nine tumors as medulloepithelioma, three as medulloblastoma/supratentorial PNET, one as neuroblastic tumor with abundant neuropil and true rosettes and one as small cell embryonal tumor/PNET (Ewing sarcoma-like). Immunostains directed against INI1, CD57, S-100 protein, NeuN, WT1, neurofilament, CD99, GFAP, synaptophysin, chromogranin, AE1/AE3 cytokeratin, Fli-1 and collagen IV were performed for each case. INI1 was diffusely and strongly positive in all tumors whereas the other stains, except for cytoplasmic WT1 (which showed substantial reactivity in most tumors), were mostly focal to negative, including CD99 (eight negative, six focal) and Fli-1 (all negative). The most consistently reactive 'neuroendocrine' marker was CD57. Each case was also analyzed for chromosome 22 rearrangements using a FISH-based break-apart probe method. Only 1 tumor, classified as medulloepithelioma, was scored positive for chromosome 22 translocation (22% rearranged cells) and the remaining 13 were negative, including the one case that resembled peripheral PNET. We conclude that PNETs derived from testicular germ cell tumors mostly resemble central nervous system PNETs and generally lack the chromosome 22 translocation of peripheral PNETs. Future treatment strategies should take these findings into account.

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Section: Discussionmentioning

confidence: 99%

“…The same holds true for ovarian PNETs. In the series of Kleinman et al, 20 which included 12 cases of PNETs of the ovary among a collection of 25 primary ovarian neuroectodermal tumors, 4 were classified as medulloepitheliomas, 3 as neuroblastomas, 3 as ependymoblastomas and 2 as medulloblastomas.…”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumors in patients with testicular germ cell tumors usually resemble pediatric-type central nervous system embryonal neoplasms and lack chromosome 22 rearrangements

et al. 2010

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“…Ovarian PNETs have an aggressive clinical course and poor prognosis; 58,59 on the other hand, the prognosis of PNET in postpubertal testicular teratoma is less clear, 60 since, as discussed, these tumors are malignant in the absence of overgrowth of immature neuroepithelium. Nonetheless, since such elements seem to be chemoresistant, their overgrowth probably does have an ultimately negative prognostic impact.…”

Section: Immature Teratoma and Selected Monodermal Teratomasmentioning

confidence: 99%

“…60 ) Overgrowth of other types of neural elements has also been described in ovarian teratomas, resulting in different forms of 'monodermal' neuroectodermal tumors, including ovarian ependymoma (differentiated neuroectodermal tumor) ( Figure 8) and glioblastoma (anaplastic neuroectodermal tumor). 58 Ovarian ependymomas have a low-grade malignant course, and ovarian glioblastoma, like PNET, pursues an aggressive course. 58 Overgrowth of immature tubules, blastema, and stroma may result in nephroblastoma-like tumors.…”

Section: Immature Teratoma and Selected Monodermal Teratomasmentioning

confidence: 99%

“…58 Ovarian ependymomas have a low-grade malignant course, and ovarian glioblastoma, like PNET, pursues an aggressive course. 58 Overgrowth of immature tubules, blastema, and stroma may result in nephroblastoma-like tumors. These are uncommon and seen most frequently in the postchemotherapy setting of patients with metastatic testicular germ cell tumors.…”

Section: Immature Teratoma and Selected Monodermal Teratomasmentioning

confidence: 99%

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