Retinoblastoma and Neuroblastoma Predisposition and Surveillance.
Abstract
Retinoblastoma (RB) is the most common intraocular malignancy in childhood. Approximately 40% of retinoblastomas are hereditary and due to germline mutations in the RB1 gene. Children with hereditary RB are also at risk for developing a midline intracranial tumor, most commonly pineoblastoma. We recommend intensive ocular screening for patients with germline RB1 mutations for retinoblastoma as well as neuroimaging for pineoblastoma surveillance. There is an approximately 20% risk of developing second primary cancers among individuals with hereditary RB, higher among those who received radiotherapy...
Source: Clinical Cancer Research - July 1, 2017 Category: Cancer & Oncology Authors: Kamihara J, Bourdeaut F, Foulkes WD, Molenaar JJ, Mossé YP, Nakagawara A, Parareda A, Scollon SR, Schneider KW, Skalet AH, States LJ, Walsh MF, Diller LR, Brodeur GM Tags: Clin Cancer Res Source Type: research
PTEN, DICER1, FH, and Their Associated Tumor Susceptibility Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood.
Abstract
PTEN hamartoma tumor syndrome (PHTS), DICER1 syndrome, and hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome are pleiotropic tumor predisposition syndromes that include benign and malignant neoplasms affecting adults and children. PHTS includes several disorders with shared and distinct clinical features. These are associated with elevated lifetime risk of breast, thyroid, endometrial, colorectal, and renal cancers as well as melanoma. Thyroid cancer represents the predominant cancer risk under age 20 years. DICER1 syndrome includes risk for pleuropulmonary blastoma, cystic nephroma...
Source: Clinical Cancer Research - June 15, 2017 Category: Cancer & Oncology Authors: Schultz KAP, Rednam SP, Kamihara J, Doros L, Achatz MI, Wasserman JD, Diller LR, Brugières L, Druker H, Schneider KA, McGee RB, Foulkes WD Tags: Clin Cancer Res Source Type: research
Second primary malignancies in retinoblastoma patients treated with intra-arterial chemotherapy: the first 10 years.
CONCLUSIONS: In our 10-year experience, we have found that SPM development in patients with germline retinoblastoma treated with OAC alone is comparable to previously published rates. In the first 10 years, OAC did not increase the known incidence of SPMs. This cohort will continue to be followed to establish the rate of development with extended follow-up.
PMID: 28600304 [PubMed - as supplied by publisher] (Source: The British Journal of Ophthalmology)
Source: The British Journal of Ophthalmology - June 9, 2017 Category: Opthalmology Authors: Habib LA, Francis JH, Fabius AW, Gobin PY, Dunkel IJ, Abramson DH Tags: Br J Ophthalmol Source Type: research
Pediatric imaging in DICER1 syndrome
ConclusionThe spectrum of DICER1-related tumors and the young age at presentation suggest early surveillance of at-risk patients is critical, while minimizing exposure to ionizing radiation. (Source: Pediatric Radiology)
Source: Pediatric Radiology - May 4, 2017 Category: Radiology Source Type: research
Histopathologic review of pineal parenchymal tumors identifies novel morphologic subtypes and prognostic factors for outcome
Conclusions.
PPTIDs can be classified into 1 of 2 novel morphologic subtypes that are associated with distinct clinical outcomes. Tumor grade, neuraxis spread, and extent of resection also influence outcome for patients with PPT. (Source: Neuro-Oncology)
Source: Neuro-Oncology - December 26, 2016 Category: Cancer & Oncology Authors: Raleigh, D. R., Solomon, D. A., Lloyd, S. A., Lazar, A., Garcia, M. A., Sneed, P. K., Clarke, J. L., McDermott, M. W., Berger, M. S., Tihan, T., Haas-Kogan, D. A. Tags: Basic and Translational Investigations Source Type: research
Patterns of care and survival outcomes in patients with pineal parenchymal tumor of intermediate differentiation: An individual patient data analysis.
CONCLUSION: PPTIDs are associated with a moderate outcome with a median progression free survival of 5.17years and median overall survival of 14years. Patients with a sub total resection should be treated with adjuvant radiotherapy as addition of radiation is associated with better survival outcomes.
PMID: 27865543 [PubMed - as supplied by publisher] (Source: Radiotherapy and Oncology : journal of the European Society for Therapeutic Radiology and Oncology)
Source: Radiotherapy and Oncology : journal of the European Society for Therapeutic Radiology and Oncology - November 15, 2016 Category: Radiology Authors: Mallick S, Benson R, Rath GK Tags: Radiother Oncol Source Type: research
Patterns of care and survival outcomes in patients with pineal parenchymal tumor of intermediate differentiation: An individual patient data analysis
Pineal parenchymal tumor constitutes less than 1% of all CNS tumors. Pineal parenchymal tumor of intermediate differentiation is a rare tumor arising from the pineal parenchyma lying between the spectrum of Pineocytoma and Pineoblastoma. (Source: Radiotherapy and Oncology)
Source: Radiotherapy and Oncology - November 15, 2016 Category: Radiology Authors: Supriya Mallick, Rony Benson, G.K. Rath Tags: Original article Source Type: research
Ntox-02. development of cystic malacia after high dose cranial irradiation of pediatric cns embryonal tumors in long-term follow-up
CONCLUSION:We attribute the high incidence of post-RT CM in our long-term follow-up study to the cranial irradiation for embryonal tumors, especially supratentorial brain tumors, and recommend regular, long-term follow-up of brain tumor patients treated with cranial irradiation. (Source: Neuro-Oncology)
Source: Neuro-Oncology - November 6, 2016 Category: Cancer & Oncology Authors: Yamasaki, F., Kolakshyapati, M., Takayasu, T., Nosaka, R., Sugiyama, K., Kurisu, K. Tags: NEUROTOXICITY OF THERAPY Source Type: research
P18.03 Third ventricle tumors: surgical treatment
Conclusions:Acceptable results of surgical treatment third ventricle tumors are in direct dependence from the tumor histological structure. The most unfavorable factor is low degree of tumor differentiation. (Source: Neuro-Oncology)
Source: Neuro-Oncology - September 20, 2016 Category: Cancer & Oncology Authors: Havryliv, T. S. Tags: P18 New developments in surgery Source Type: research
MRI-based assessment of the pineal gland in a large population of children aged 0 –5 years and comparison with pineoblastoma: part I, the solid gland
Conclusion < /h3 > < p class= " a-plus-plus " > This study establishes norms for solid pineal gland size in non-retinoblastoma children aged 0 –5 years. Knowledge of the size of the normal pineal gland is helpful for detection of pineal gland abnormalities, particularly pineoblastoma. < /p > < /span > (Source: Neuroradiology)
Source: Neuroradiology - June 30, 2016 Category: Radiology Source Type: research
Cat Scratch Disease: Expanded Spectrum
Conclusion: Optic nerve granulomas are a rare presentation of cat scratch disease and could potentially masquerade as retinoblastoma.Ocul Oncol Pathol 2016;2:246-250 (Source: Ocular Oncology and Pathology)
Source: Ocular Oncology and Pathology - June 30, 2016 Category: Opthalmology Source Type: research
Pnr-31 * inhibiting cell cycle checkpoints improves the efficacy of chemotherapy in pineoblastoma
(Source: Neuro-Oncology)
Source: Neuro-Oncology - June 11, 2016 Category: Cancer & Oncology Authors: Endersby, R., Kuchibhotla, M., Schoep, T., George, C., Hii, H., Strowger, B., Gande, S., Kees, U., Gottardo, N. Tags: CNS PNET AND RARE TUMOURS Source Type: research
Pnr-30 * effectivity of two different radiation-avoiding treatment regimens for young children with pineoblastoma--results of the hit2000 trial
(Source: Neuro-Oncology)
Source: Neuro-Oncology - June 11, 2016 Category: Cancer & Oncology Authors: Juhnke, B.-O., Mynarek, M., von Bueren, A. O., Friedrich, C., Gerber, N. U., Kortmann, R.-D., Pietsch, T., Warmuth-Metz, M., von Hoff, K., Rutkowski, S. Tags: CNS PNET AND RARE TUMOURS Source Type: research
Pnr-09 * evaluation of age-dependent treatment strategies for children and young adults with pineoblastoma: analysis of pooled siop-e and head start data
(Source: Neuro-Oncology)
Source: Neuro-Oncology - June 11, 2016 Category: Cancer & Oncology Authors: Mynarek, M., Pizer, B., Dufour, C., van Vuurden, D., Garami, M., Massimino, M., Fangusaro, J., Davidson, T., Gil-da-Costa, M. J., Sterba, J., Benesch, M., Gerber, N., Juhnke, B. O., Kwiecien, R., Pietsch, T., Kool, M., Clifford, S., Ellison, D. W., Gianga Tags: CNS PNET AND RARE TUMOURS Source Type: research
Isolation and characterization of tumorspheres from a recurrent pineoblastoma patient: Feasibility of a patient-derived xenograft.
Abstract
The existence of tumorspheres (TSs) might confer treatment resistance to pineoblastoma (PB). The existence of PB TSs with cellular immortalization potential has not yet been reported. We developed a procedure for isolating TSs from recurrent PB (rPB) and tested whether their properties made them suitable for use as a patient-derived xenograft (PDX). Immunocytochemical staining, RT-PCR and quantitative real-time PCR showed that, among stemness proteins, CD133, musashi and podoplanin were expressed at elevated levels in rPB TSs, but nestin was not. rPB TSs cultured under neuro-glial differentiation ...
Source: International Journal of Oncology - May 31, 2016 Category: Cancer & Oncology Authors: Kwak J, Shim JK, Kim DS, Lee JH, Choi J, Park J, Shin KJ, Kim SH, Kim P, Huh YM, Kim EH, Chang JH, Kim SH, Kang SG Tags: Int J Oncol Source Type: research
