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PLoS One. 2016 May 12;11(5):e0154985. doi: 10.1371/journal.pone.0154985. eCollection 2016.

Cancer of Unknown Primary in Adolescents and Young Adults: Clinicopathological Features, Prognostic Factors and Survival Outcomes.

Author information

1
Department of Gastrointestinal Medical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, United States of America.
2
Division of Cancer Medicine, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, United States of America.
3
Department of Biostatistics, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, United States of America.
4
Diagnostic Radiology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, United States of America.
5
Department of Pathology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, United States of America.

Abstract

BACKGROUND:

Cancer in adolescents and young adults (AYAs) (15-39 years) is increasingly recognized as a distinct clinical and biological entity. Cancer of unknown primary (CUP), a disease traditionally presenting in older adults with a median age of 65 years, poses several challenges when diagnosed in AYA patients. This study describes clinicopathological features, outcomes and challenges in caring for AYA-CUP patients.

METHODS:

A retrospective review of 47 AYAs diagnosed with CUP at MD Anderson Cancer Center (6/2006-6/2013) was performed. Patients with favorable CUP subsets treated as per site-specific recommendations were excluded. Demographics, imaging, pathology and treatment data was collected using a prospectively maintained CUP database. Kaplan-Meier product limit method and log-rank test were used to estimate and compare overall survival. The cox-proportional model was used for multivariate analyses.

RESULTS:

Median age was 35 years (range 19-39). All patients underwent comprehensive workup. Adenocarcinoma was the predominant histology (70%). A median of 9 immunostains (range 2-29) were performed. The most common putative primary was biliary tract based on clinicopathological parameters as well as gene profiling. Patients presented with a median of 2 metastatic sites [lymph node (60%), lung (47%), liver (38%) and bone (34%)]. Most commonly used systemic chemotherapies included gemcitabine, fluorouracil, taxanes and platinum agents. Median overall survival for the entire cohort was 10.0 (95% confidence interval (CI): 6.7-15.4) months. On multivariate analyses, elevated lactate dehydrogenase (Hazard ratio (HR) 3.66; 95%CI 1.52-8.82; P = 0.004), ≥3 metastatic sites (HR 5.34; 95%CI 1.19-23.9; P = 0.029), and tissue of origin not tested (HR 3.4; 95%CI 1.44-8.06; P = 0.005) were associated with poor overall survival. Culine's CUP prognostic model (lactate dehydrogenase, performance status, liver metastases) was validated in this cohort (median overall survival: good-risk 25.2 months vs. poor-risk 6.1 months).

CONCLUSIONS:

AYA-CUP is associated with a poor prognosis. In the current "-omics" era collaborative research efforts towards understanding tumor biology and therapeutic targets in AYA-CUP is an unmet need, necessary for improving outcomes in young CUP patients.

PMID:
27171493
PMCID:
PMC4865168
DOI:
10.1371/journal.pone.0154985
[Indexed for MEDLINE]
Free PMC Article

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