2018 consensus statement by the Spanish Society of Pathology and the Spanish Society of Medical Oncology on the diagnosis and treatment of cancer of unknown primary

Abstract

Cancer of unknown primary (CUP) is defined as a heterogeneous group of tumours that present with metastasis, and in which attempts to identify the original site have failed. They differ from other primary tumours in their biological features and how they spread, which means that they can be considered a separate entity. There are several hypotheses regarding their origin, but the most plausible explanation for their aggressiveness and chemoresistance seems to involve chromosomal instability. Depending on the type of study done, CUP can account for 2–9% of all cancer patients, mostly 60–75 years old. This article reviews the main clinical, pathological, and molecular studies conducted to analyse and determine the origin of CUP. The main strategies for patient management and treatment, by both clinicians and pathologists, are also addressed.

Cancer of Unknown Primary: A Review on Clinical Guidelines in the Development and Targeted Management of Patients with the Unknown Primary Site

“…Site of metastasis and histology are two essential factors in the prognosis of CUP [10]. The incidence of CUP is highest in patients of age between 60-75 years [2]. In another study, the highest incidence ratio was at age 85-89 years, followed by a significant decrease by age ninety-plus (7-fold in men and 3-fold in women) [10].…”

“…Cancer of unknown primary (CUP) is a malignant widespread metastatic disease without an identifiable primary site after extensive clinical investigation [1]. Based on the biological features, clinical presentation, and atypical pattern of metastasis, the CUP can be considered as a separate disease from the other known primary tumors [1-2]. Hypotheses on the pathogenesis of CUP are:…”

“…3) Recently a possible explanation was proposed on the role of chromosomal instability contributing to aggressive disease presentation and chemoresistance [2].…”

Biomarkers in the diagnosis of pleural diseases: a 2018 update

“… 57 Subsequently, more tumor-specific markers are tested, such as TTF-1 and napsin A (nonsquamous lung cancer); GATA3, GCDFP15, mammaglobin and hormone receptors (breast); p40 and p63 (squamous-cell carcinomas); synaptophysin and chromogranin A (neuroendocrine tumors); CDX2 and cadherin 17 (gastrointestinal and pancreas); and PAX8 (ovary, kidney), among others. 58 …”

Immunotherapy Combined With Chemotherapy and Radiotherapy for a Patient With Cancer of Unknown Primary: A Case Report

“…Typically, CUP responds to chemotherapy (cisplatin and etoposide) with 70−80% overall response rates. 3 Ohta et al found that patients with CUP who received chemotherapy showed a relatively good prognosis. 4 However, chemotherapy has no impact on overall survival.…”