NCCN Clinical Practice Guidelines Occult primary.

Journal Article (Journal Article)

Occult primary tumors, or cancers of unknown primary (CUPs), are defined as histologically proven metastatic malignant tumors whose primary site cannot be identified during pretreatment evaluation. They have a wide variety of clinical presentations and a poor prognosis in most patients. Patients with occult primary tumors often present with general complaints, such as anorexia and weight loss. Clinical absence of primary tumor, early dissemination, aggressiveness, and unpredictability of metastatic pattern are characteristic of these tumors. Life expectancy is very short, with a median survival of 6 to 9 months. In most patients, occult primary tumors are refractory to systemic treatments, and chemotherapy is only palliative and does not significantly improve long-term survival. However, certain clinical presentations of these tumors are associated with a better prognosis. Special pathologic studies can identify subsets of patients with tumor types that are more responsive to chemotherapy. Treatment options should be individualized for this selected group of patients to achieve improved response and survival rates.

Full Text

Duke Authors

Cited Authors

  • Ettinger, DS; Agulnik, M; Cates, JMM; Cristea, M; Denlinger, CS; Eaton, KD; Fidias, PM; Gierada, D; Gockerman, JP; Handorf, CR; Iyer, R; Lenzi, R; Phay, J; Rashid, A; Saltz, L; Shulman, LN; Smerage, JB; Varadhachary, GR; Zager, JS; Zhen, WK; National Comprehensive Cancer Network,

Published Date

  • December 2011

Published In

Volume / Issue

  • 9 / 12

Start / End Page

  • 1358 - 1395

PubMed ID

  • 22157556

Electronic International Standard Serial Number (EISSN)

  • 1540-1413

Digital Object Identifier (DOI)

  • 10.6004/jnccn.2011.0117


  • eng

Conference Location

  • United States