Trilateral Retinoblastoma

Trilateral retinoblastoma: ocular and pineal retinoblastomas

“…5 Knudson, et al, ~1 have advanced a "two-hit" hypothesis for retinoblastoma. The first hit in the genetic form of retinoblastoma is prezygotic and affects all subsequent somatic cells; a second hit results in tumor formation in the susceptible cells.…”

Trilateral retinoblastoma: ocular and pineal retinoblastomas

“…5 Knudson, et al, ~1 have advanced a "two-hit" hypothesis for retinoblastoma. The first hit in the genetic form of retinoblastoma is prezygotic and affects all subsequent somatic cells; a second hit results in tumor formation in the susceptible cells.…”

The correlation of neoplastic vulnerability with central neuroepithelial cytogeny and glioma differentiation

“…These features disappear soon after birth (48). The ontogenetic aspect of neoplastic differentiation in this instance may also be manifested by a different development, namely by the occasional association of heritable retinoblastomas, usually bilateral, with a subsequent pineoblastoma in the same patient (49)(50)(51)(52). The term 'trilateral retinoblastoma' that has been given to the association is perhaps too inclusive since not all the pineoblastomas presenting in this context have been shown to display retinoblastic differentiation.…”

Incomplete inhibition of the Rb tumor suppressor pathway in the context of inactivated p53 is sufficient for pancreatic islet tumorigenesis

“…There was, however, evident pathological consequences in another organ: the homozygous null b-Rb KO animals required euthanasia at 18-20 weeks of age due to acute cachexia from the effects of Rb-null pituitary tumors that developed with 100% penetrance, apparently resultant to the 'leaky' expression of the RIP2-Cre transgene in the pituitary (Gannon et al, 2000;and M Hebrok, personal communication). The pituitary has proved in other studies to be highly susceptible to tumorigenesis in the context of loss of Rb, as shown by the fact that Rb germline knockout heterozygote mice also develop pituitary tumors (Jacks et al, 1992) and by the high incidence of pituitary tumors in familial retinoblastoma mutations, clinically called trilateral retinoblastoma (Bader et al, 1980;Brownstein et al, 1984). Since RIP-Tag2 mice develop pancreatic islet tumors by 12 weeks of age, the slower pituitary tumorigenesis did not preclude the analysis of the faster-growing islet tumors, and so RIP-Tag2; b-Rb KO mice were generated, and the pancreatic islet tumor pathway evaluated.…”

Trilateral retinoblastoma: ocular and pineal retinoblastomas

“…5 Knudson, et al, ~1 have advanced a "two-hit" hypothesis for retinoblastoma. The first hit in the genetic form of retinoblastoma is prezygotic and affects all subsequent somatic cells; a second hit results in tumor formation in the susceptible cells.…”

The correlation of neoplastic vulnerability with central neuroepithelial cytogeny and glioma differentiation

“…These features disappear soon after birth (48). The ontogenetic aspect of neoplastic differentiation in this instance may also be manifested by a different development, namely by the occasional association of heritable retinoblastomas, usually bilateral, with a subsequent pineoblastoma in the same patient (49)(50)(51)(52). The term 'trilateral retinoblastoma' that has been given to the association is perhaps too inclusive since not all the pineoblastomas presenting in this context have been shown to display retinoblastic differentiation.…”

Incomplete inhibition of the Rb tumor suppressor pathway in the context of inactivated p53 is sufficient for pancreatic islet tumorigenesis

“…There was, however, evident pathological consequences in another organ: the homozygous null b-Rb KO animals required euthanasia at 18-20 weeks of age due to acute cachexia from the effects of Rb-null pituitary tumors that developed with 100% penetrance, apparently resultant to the 'leaky' expression of the RIP2-Cre transgene in the pituitary (Gannon et al, 2000;and M Hebrok, personal communication). The pituitary has proved in other studies to be highly susceptible to tumorigenesis in the context of loss of Rb, as shown by the fact that Rb germline knockout heterozygote mice also develop pituitary tumors (Jacks et al, 1992) and by the high incidence of pituitary tumors in familial retinoblastoma mutations, clinically called trilateral retinoblastoma (Bader et al, 1980;Brownstein et al, 1984). Since RIP-Tag2 mice develop pancreatic islet tumors by 12 weeks of age, the slower pituitary tumorigenesis did not preclude the analysis of the faster-growing islet tumors, and so RIP-Tag2; b-Rb KO mice were generated, and the pancreatic islet tumor pathway evaluated.…”

Trilateral retinoblastoma: ocular and pineal retinoblastomas

“…5 Knudson, et al, ~1 have advanced a "two-hit" hypothesis for retinoblastoma. The first hit in the genetic form of retinoblastoma is prezygotic and affects all subsequent somatic cells; a second hit results in tumor formation in the susceptible cells.…”

The correlation of neoplastic vulnerability with central neuroepithelial cytogeny and glioma differentiation

“…These features disappear soon after birth (48). The ontogenetic aspect of neoplastic differentiation in this instance may also be manifested by a different development, namely by the occasional association of heritable retinoblastomas, usually bilateral, with a subsequent pineoblastoma in the same patient (49)(50)(51)(52). The term 'trilateral retinoblastoma' that has been given to the association is perhaps too inclusive since not all the pineoblastomas presenting in this context have been shown to display retinoblastic differentiation.…”

Incomplete inhibition of the Rb tumor suppressor pathway in the context of inactivated p53 is sufficient for pancreatic islet tumorigenesis

“…There was, however, evident pathological consequences in another organ: the homozygous null b-Rb KO animals required euthanasia at 18-20 weeks of age due to acute cachexia from the effects of Rb-null pituitary tumors that developed with 100% penetrance, apparently resultant to the 'leaky' expression of the RIP2-Cre transgene in the pituitary (Gannon et al, 2000;and M Hebrok, personal communication). The pituitary has proved in other studies to be highly susceptible to tumorigenesis in the context of loss of Rb, as shown by the fact that Rb germline knockout heterozygote mice also develop pituitary tumors (Jacks et al, 1992) and by the high incidence of pituitary tumors in familial retinoblastoma mutations, clinically called trilateral retinoblastoma (Bader et al, 1980;Brownstein et al, 1984). Since RIP-Tag2 mice develop pancreatic islet tumors by 12 weeks of age, the slower pituitary tumorigenesis did not preclude the analysis of the faster-growing islet tumors, and so RIP-Tag2; b-Rb KO mice were generated, and the pancreatic islet tumor pathway evaluated.…”