Survival Benefit for Individuals With Constitutional Mismatch Repair Deficiency Undergoing Surveillance

↓ Direkt till sidans innehåll
↓ Direkt till sidans sekundära innehåll (sidomenyn)

Search: WFRF:(Ling T G I) > (2020-2023) > Survival Benefit fo...

Details
MARC

Durno, C. (author)

Survival Benefit for Individuals With Constitutional Mismatch Repair Deficiency Undergoing Surveillance

Article/chapterEnglish2021

Publisher, publication year, extent ...

American Society of Clinical Oncology (ASCO),2021

Numbers

LIBRIS-ID:oai:gup.ub.gu.se/309357
https://gup.ub.gu.se/publication/309357URI
https://doi.org/10.1200/jco.20.02636DOI

Supplementary language notes

Language:English

Part of subdatabase

SwePubSwePub

Classification

Subject category:ref swepub-contenttype
Subject category:art swepub-publicationtype

Notes

PURPOSE Constitutional mismatch repair deficiency syndrome (CMMRD) is a lethal cancer predisposition syndrome characterized by early-onset synchronous and metachronous multiorgan tumors. We designed a surveillance protocol for early tumor detection in these individuals. PATIENTS AND METHODS Data were collected from patients with confirmed CMMRD who were registered in the International Replication Repair Deficiency Consortium. Tumor spectrum, efficacy of the surveillance protocol, and malignant transformation of low-grade lesions were examined for the entire cohort. Survival outcomes were analyzed for patients followed prospectively from the time of surveillance implementation. RESULTS A total of 193 malignant tumors in 110 patients were identified. Median age of first cancer diagnosis was 9.2 years (range: 1.7-39.5 years). For patients undergoing surveillance, all GI and other solid tumors, and 75% of brain cancers were detected asymptomatically. By contrast, only 16% of hematologic malignancies were detected asymptomatically (P < .001). Eighty-nine patients were followed prospectively and used for survival analysis. Five-year overall survival (OS) was 90% (95% CI, 78.6 to 100) and 50% (95% CI, 39.2 to 63.7) when cancer was detected asymptomatically and symptomatically, respectively (P = .001). Patient outcome measured by adherence to the surveillance protocol revealed 4-year OS of 79% (95% CI, 54.8 to 90.9) for patients undergoing full surveillance, 55% (95% CI, 28.5 to 74.5) for partial surveillance, and 15% (95% CI, 5.2 to 28.8) for those not under surveillance (P < .0001). Of the 64 low-grade tumors detected, the cumulative likelihood of transformation from low-to high-grade was 81% for GI cancers within 8 years and 100% for gliomas in 6 years. CONCLUSION Surveillance and early cancer detection are associated with improved OS for individuals with CMMRD.

Subject headings and genre

MEDICIN OCH HÄLSOVETENSKAP Klinisk medicin Cancer och onkologi hsv//swe
MEDICAL AND HEALTH SCIENCES Clinical Medicine Cancer and Oncology hsv//eng
european consortium care
tp53 mutation carriers
li-fraumeni syndrome
imaging surveillance
follow-up
hypermutation
recommendations
hereditary
mechanisms
guidelines
Oncology

Added entries (persons, corporate bodies, meetings, titles ...)

Ercan, A. B. (author)
Bianchi, V. (author)
Edwards, M. (author)
Aronson, M. (author)
Galati, M. (author)
Atenafu, E. G. (author)
Abebe-Campino, G. (author)
Al-Battashi, A. (author)
Alharbi, M. (author)
Azad, V. F. (author)
Baris, H. N. (author)
Basel, D. (author)
Bedgood, R. (author)
Bendel, A. (author)
Ben-Shachar, S. (author)
Blumenthal, D. T. (author)
Blundell, M. (author)
Bornhorst, M. (author)
Bronsema, A. (author)
Cairney, E. (author)
Rhode, S. (author)
Caspi, S. (author)
Chamdin, A. (author)
Chiaravalli, S. (author)
Constantini, S. (author)
Crooks, B. (author)
Das, A. (author)
Dvir, R. (author)
Farah, R. (author)
Foulkes, W. D. (author)
Frenkel, Z. (author)
Gallinger, B. (author)
Gardner, S. (author)
Gass, D. (author)
Ghalibafian, M. (author)
Gilpin, C. (author)
Goldberg, Y. (author)
Goudie, C. (author)
Hamid, S. A. (author)
Hampel, H. (author)
Hansford, J. R. (author)
Harlos, C. (author)
Hijiya, N. (author)
Hsu, S. (author)
Kamihara, J. (author)
Kebudi, R. (author)
Knipstein, J. (author)
Koschmann, C. (author)
Kratz, C. (author)
Larouche, V. (author)
Lassaletta, A. (author)
Lindhorst, S. (author)
Ling, S. C. (author)
Link, M. P. (author)
De Mola, R. L. (author)
Luiten, R. (author)
Lurye, M. (author)
Maciaszek, J. L. (author)
MagimairajanIssai, V. (author)
Maher, O. M. (author)
Massimino, M. (author)
McGee, R. B. (author)
Mushtaq, N. (author)
Mason, G. (author)
Newmark, M. (author)
Nicholas, G. (author)
Nichols, K. E. (author)
Nicolaides, T. (author)
Opocher, E. (author)
Osborn, M. (author)
Oshrine, B. (author)
Pearlman, R. (author)
Pettee, D. (author)
Rapp, J. (author)
Rashid, M. (author)
Reddy, A. (author)
Reichman, L. (author)
Remke, M. (author)
Robbins, G. (author)
Roy, S. (author)
Sabel, Magnus,1966Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics(Swepub:gu)xsabma (author)
Samuel, D. (author)
Scheers, I. (author)
Schneider, K. W. (author)
Sen, S. (author)
Stearns, D. (author)
Sumerauer, D. (author)
Swallow, C. (author)
Taylor, L. (author)
Thomas, G. (author)
Toledano, H. (author)
Tomboc, P. (author)
Van Damme, A. (author)
Winer, I. (author)
Yalon, M. (author)
Yen, L. Y. (author)
Zapotocky, M. (author)
Zelcer, S. (author)
Ziegler, D. S. (author)
Zimmermann, S. (author)
Hawkins, C. (author)
Malkin, D. (author)
Bouffet, E. (author)
Villani, A. (author)
Tabori, U. (author)
Göteborgs universitetInstitutionen för kliniska vetenskaper, Avdelningen för pediatrik (creator_code:org_t)

Related titles

In:Journal of Clinical Oncology: American Society of Clinical Oncology (ASCO)39:250732-183X1527-7755

Internet link

Find in a library

Journal of Clinical Oncology (Search for host publication in LIBRIS)

To the university's database

Find more in SwePub

By the author/editor: Durno, C.; Ercan, A. B.; Bianchi, V.; Edwards, M.; Aronson, M.; Galati, M.; show more...; Atenafu, E. G.; Abebe-Campino, G ...; Al-Battashi, A.; Alharbi, M.; Azad, V. F.; Baris, H. N.; Basel, D.; Bedgood, R.; Bendel, A.; Ben-Shachar, S.; Blumenthal, D. T ...; Blundell, M.; Bornhorst, M.; Bronsema, A.; Cairney, E.; Rhode, S.; Caspi, S.; Chamdin, A.; Chiaravalli, S.; Constantini, S.; Crooks, B.; Das, A.; Dvir, R.; Farah, R.; Foulkes, W. D.; Frenkel, Z.; Gallinger, B.; Gardner, S.; Gass, D.; Ghalibafian, M.; Gilpin, C.; Goldberg, Y.; Goudie, C.; Hamid, S. A.; Hampel, H.; Hansford, J. R.; Harlos, C.; Hijiya, N.; Hsu, S.; Kamihara, J.; Kebudi, R.; Knipstein, J.; Koschmann, C.; Kratz, C.; Larouche, V.; Lassaletta, A.; Lindhorst, S.; Ling, S. C.; Link, M. P.; De Mola, R. L.; Luiten, R.; Lurye, M.; Maciaszek, J. L.; MagimairajanIssa ...; Maher, O. M.; Massimino, M.; McGee, R. B.; Mushtaq, N.; Mason, G.; Newmark, M.; Nicholas, G.; Nichols, K. E.; Nicolaides, T.; Opocher, E.; Osborn, M.; Oshrine, B.; Pearlman, R.; Pettee, D.; Rapp, J.; Rashid, M.; Reddy, A.; Reichman, L.; Remke, M.; Robbins, G.; Roy, S.; Sabel, Magnus, 1 ...; Samuel, D.; Scheers, I.; Schneider, K. W.; Sen, S.; Stearns, D.; Sumerauer, D.; Swallow, C.; Taylor, L.; Thomas, G.; Toledano, H.; Tomboc, P.; Van Damme, A.; Winer, I.; Yalon, M.; Yen, L. Y.; Zapotocky, M.; Zelcer, S.; Ziegler, D. S.; Zimmermann, S.; Hawkins, C.; Malkin, D.; Bouffet, E.; Villani, A.; Tabori, U.; show less...

About the subject

MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Cancer and Oncol ...

Articles in the publication: Journal of Clini ...

By the university: University of Gothenburg

Search outside SwePub

Extend your search to:: Google; Google Book Search; Google Scholar

Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.

LIBRIS.kb.se