Cancer of Unknown Primary in AYA's: Clinicopathological Features, Prognostic Factors, Survival Outcomes

PLOS ONE (open access):
 Cancer of Unknown Primary in Adolescents and Young Adults: Clinicopathological Features, Prognostic Factors and Survival Outcomes
May 12, 2016
 

Methods on concordance of primary site

Molecular tumor profiling predicted a tissue of origin in 19 of 26 (73.0%) cases; most common being biliary tract 5 (26.3%), ovarian 4 (21.0%) and gastroesophageal 2 (10.5%) primaries.....

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.....
 

Introduction

Cancer in adolescents and young adults (AYAs) (15–39 years) is increasingly recognized as a distinct clinical and biological entity [1, 2]. AYAs with cancers have historically received less attention than either children or older adults with cancers. Managing cancer in these patients comes with several challenges owing to their unique clinical, psychological and socioeconomic demands [1–3]. Additionally, participation of AYAs in clinical trials has been inadequate for many reasons resulting in a relative lack of progress in this vulnerable group of cancer patients [4, 5]. Initiatives to overcome these impediments and research dedicated to cancers in AYAs have been encouraged by the National Cancer Institute to improve our understanding of cancers in this age-group and patient outcomes [6].
Cancer of unknown primary (CUP) is a heterogeneous group of cancers, for which despite detailed evaluation, the anatomical site of origin remains obscure [7, 8]. This is typically, a disease of older individuals, with a median age of 67 years. Management of these cancers requires a thorough physical examination, focused imaging and pathologic review. Treatment is site-specific therapy based on a putative primary when informed; otherwise, empiric platinum based chemotherapy is employed [7]. Nevertheless, survival outcomes in CUP patients remain suboptimal......