scholarly journals Rare cancers and cancer of unknown primary: Here's what you should know!

2021 ◽  
Vol 30 (6) ◽  
Author(s):  
Saskia F. A. Duijts ◽  
Jan Maarten Zwan
Keyword(s):  
Cancer Of Unknown Primary ◽  
Unknown Primary ◽  
Rare Cancers

TCF-001 TRACK (Target Rare Cancer Knowledge): A national patient-centric precision oncology trial for rare cancers.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. TPS3143-TPS3143
Author(s):  
Vivek Subbiah ◽  
Roman Groisberg ◽  
Catherine Skefos ◽  
James M. Cleary ◽  
Ishwaria Mohan Subbiah ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Cancer Of Unknown Primary ◽  
Molecular Therapy ◽  
Tumor Board ◽  
Unknown Primary ◽  
Genomic Alterations ◽  
Rare Cancers ◽  
Rare Tumors ◽  
Patient Centric ◽  
Matched Treatment

TPS3143 Background: Many rare cancers are understudied, and affected patients often have few if any standard treatment options and limited access to clinical trials. Comprehensive genomic profiling (CGP) is a robust tool to efficiently identify the genomic alterations of cancer and can be applied to study rare tumors. The promise of this technology is to simultaneously enhance our understanding of diverse rare tumors and find matched therapies to benefit the individual patient in real-time. The COVID19 pandemic has challenged the “trial-centric” clinical trial infrastructure. Novel “patient-centric” trials are needed, especially for rare cancers. TRACK is a decentralized, patient advocacy-initiated trial that aims to establish whether patients with rare tumors and cancer of unknown primary can benefit from matched molecular therapy as dictated by their CGP results. Methods: This is a national open label, non-randomized, multi-center (includes community and academic centers) pragmatic study in rare tumors (<6 cases per 100,000 persons per year) and cancer of unknown primary. All participants will have tumor tissue and blood analyzed by CGP (FoundationOneCDx and FoundationOneLiquidCDx). A Virtual Molecular Tumor Board (VMTB) will convene to identify targeted treatment recommendations for participants with genomic alterations. Overall, 400 participants will be enrolled to achieve the goal of 100 patients matched to genomically informed treatment, utilizing remote consenting to ensure study access regardless of geographic location. The primary feasibility endpoint is the percent of participants who receive a molecularly matched treatment after recommendation from the VMTB. The primary efficacy endpoint is the progression-free survival (PFS) among participants who received the molecularly matched treatment. Secondary endpoints include the percent of participants with genomic alterations, overall response rate, response duration, overall survival, clinical benefit rate (SD > 6 months; PR; CR), and high-grade toxicities (as available) of matched targeted therapy. Exploratory endpoints include matching rates; understanding genomic correlates of response; concordance rates between CGP from tissue and blood, time-to-treatment failure, and the impact of the cancer treatment from the patient perspective (via ESAS-FS, a validated patient-reported outcomes (PRO) instrument). All eligible and enrolled patients, regardless of whether receiving matched treatment or not, will be followed for a minimum of one year in a uniform way such that the treatment efficacy and outcomes can be assessed in standard formats. The study is open with six patients enrolled at time of submission. Clinical trial information: NCT04504604.

scholarly journals Cancer of Unknown Primary: Challenges and Progress in Clinical Management

Cancers ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 451
Author(s):  
Noemi Laprovitera ◽  
Mattia Riefolo ◽  
Elisa Ambrosini ◽  
Christiane Klec ◽  
Martin Pichler ◽  
...  
Keyword(s):  
Current Knowledge ◽  
Distant Metastases ◽  
Cancer Of Unknown Primary ◽  
Unknown Primary ◽  
Diagnostic Strategies ◽  
Molecular Features ◽  
Tissue Of Origin ◽  
Novel Therapeutic Strategies ◽  
Aggressive Clinical Behavior

Distant metastases are the main cause of cancer-related deaths in patients with advanced tumors. A standard diagnostic workup usually contains the identification of the tissue-of-origin of metastatic tumors, although under certain circumstances, it remains elusive. This disease setting is defined as cancer of unknown primary (CUP). Accounting for approximately 3–5% of all cancer diagnoses, CUPs are characterized by an aggressive clinical behavior and represent a real therapeutic challenge. The lack of determination of a tissue of origin precludes CUP patients from specific evidence-based therapeutic options or access to clinical trial, which significantly impacts their life expectancy. In the era of precision medicine, it is essential to characterize CUP molecular features, including the expression profile of non-coding RNAs, to improve our understanding of CUP biology and identify novel therapeutic strategies. This review article sheds light on this enigmatic disease by summarizing the current knowledge on CUPs focusing on recent discoveries and emerging diagnostic strategies.

scholarly journals Cancer of unknown primary

Medicine ◽  
2017 ◽  
Vol 96 (16) ◽  
pp. e6693 ◽  
Author(s):  
Anne-Kirstine Dyrvig ◽  
Knud Bonnet Yderstræde ◽  
Oke Gerke ◽  
Peter Bjødstrup Jensen ◽  
Søren Hess ◽  
...  
Keyword(s):  
Cancer Of Unknown Primary ◽  
Unknown Primary

scholarly journals Swedish National Multicenter Study on Head and Neck Cancer of Unknown Primary: Prognostic Factors and Impact of Treatment on Survival

2020 ◽  
Author(s):  
Lars Axelsson ◽  
Erik Holmberg ◽  
Jan Nyman ◽  
Anders Högmo ◽  
Helena Sjödin ◽  
...  
Keyword(s):  
Head And Neck Cancer ◽  
Prognostic Factors ◽  
Head And Neck ◽  
Neck Dissection ◽  
Neck Cancer ◽  
Multicenter Study ◽  
Performance Status ◽  
Cancer Of Unknown Primary ◽  
Unknown Primary ◽  
N Stage

Abstract Introduction Head and neck cancer of unknown primary (HNCUP) is a rare condition whose prognostic factors that are significant for survival vary between studies. No randomized treatment study has been performed thus far, and the optimal treatment is not established. Objective The present study aimed to explore various prognostic factors and compare the two main treatments for HNCUP: neck dissection and (chemo) radiation vs primary (chemo) radiation. Methods A national multicenter study was performed with data from the Swedish Head and Neck Cancer Register (SweHNCR) and from the patients' medical records from 2008 to 2012. Results Two-hundred and sixty HNCUP patients were included. The tumors were HPV-positive in 80%. The overall 5-year survival rate of patients treated with curative intent was 71%. Age (p < 0.001), performance status (p= 0.036), and N stage (p= 0.046) were significant factors for overall survival according to the multivariable analysis. Treatment with neck dissection and (chemo) radiation (122 patients) gave an overall 5-year survival of 73%, and treatment with primary (chemo) radiation (87 patients) gave an overall 5-year survival of 71%, with no significant difference in overall or disease-free survival between the 2 groups. Conclusions Age, performance status, and N stage were significant prognostic factors. Treatment with neck dissection and (chemo) radiation and primary (chemo) radiation gave similar survival outcomes. A randomized treatment study that includes quality of life is needed to establish the optimal treatment.

scholarly journals Family history of cancer in first degree relatives and risk of cancer of unknown primary

2021 ◽  
Author(s):  
Alexander L. R. Grewcock ◽  
Karlijn E. P. E. Hermans ◽  
Matty P. Weijenberg ◽  
Piet A. Brandt ◽  
Caroline Loef ◽  
...  
Keyword(s):  
Family History ◽  
Cancer Of Unknown Primary ◽  
Unknown Primary ◽  
Family History Of Cancer ◽  
First Degree Relatives ◽  
History Of ◽  
Risk Of Cancer ◽  
History Of Cancer

scholarly journals Cancer of Unknown Primary Presenting as Bone‐Predominant or Lymph Node‐Only Disease: A Clinicopathologic Portrait

2021 ◽  
Author(s):  
Ryan W. Huey ◽  
Brandon G. Smaglo ◽  
Jeannelyn S. Estrella ◽  
Aurelio Matamoros ◽  
Michael J. Overman ◽  
...  
Keyword(s):  
Lymph Node ◽  
Cancer Of Unknown Primary ◽  
Unknown Primary

scholarly journals Cancer of Unknown Primary in the Molecular Era

2021 ◽  
Author(s):  
Shumei Kato ◽  
Ahmed Alsafar ◽  
Vighnesh Walavalkar ◽  
John Hainsworth ◽  
Razelle Kurzrock
Keyword(s):  
Cancer Of Unknown Primary ◽  
Unknown Primary

scholarly journals A Retrospective Analysis of Cancer of Unknown Primary in Ibaraki Prefectural Central Hospital

2013 ◽  
Vol 24 ◽  
pp. ix38
Author(s):  
F. Fujisawa ◽  
S. Meshihtsuka ◽  
S. Mitsuhashi ◽  
M. Hori ◽  
T. Kaburagi ◽  
...  
Keyword(s):  
Retrospective Analysis ◽  
Cancer Of Unknown Primary ◽  
Unknown Primary ◽  
Central Hospital ◽  
Prefectural Central Hospital
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