Rak neznanega izvora Cancer of unknown primary

does primary still matter?

Abstract

Rak neznanega izvora (RNI) je opredeljen kot karcinom ali nediferencirana neoplazma, pri kateri z naborom standardnih diagnostičnih postopkov ni mogoče odkriti izvornega mesta bolezni. Tradicionalno RNI delimo v dve podskupini, pri čemer le približno 15 % primerov predstavlja prognostično ugodno skupino. Velika večina bolnikov spada v prognostično neugodno skupino in ima ob prvi prezentaciji obsežno breme bolezni. Možnosti zdravljenja so omejene, izidi bolnikov, zdravljenih z empirično kemoterapijo (KT) s platino ali taksani, pa so še vedno slabi, srednje celokupno preživetje je manj kot 10 mesecev. Za mnoge bolnike ostaja optimalna izbira najboljše možno podporno zdravljenje. Novi pristopi k obravnavi teh bolnikov se zdijo obetavni in so temeljit premik v paradigmi zdravljenja RNI; od zdravljenja, specifičnega za organ/tkivo, k zdravljenju, usmerjenemu na posameznega bolnika, ki temelji na genomskih spremembah njegovega tumorja. Prispevek povzema trenutne dokaze o uporabi vsakega od teh pristopov. Predstavljeno je tudi zdravljenje treh bolnikov z neugodnim RNI.

Abstract (Eng)

Cancer of unknown primary (CUP) is defined as a carcinoma or undifferentiated neoplasm in which a standardized diagnostic workup fails to identify the primary tumour responsible for metastatic seeding. Survival after diagnosis of CUP remains poor and has not improved significantly over time. Traditionally, CUP has been divided into two subsets, with only about 15% of cases in the favourable subset. The vast majority of patients are in the unfavourable subset and present with a high metastatic burden at first presentation. Their treatment options are limited and outcomes for patients treated with empiric chemotherapy consisting of platinumor taxane-based doublets remain poor, with median overall survival of less than 10 months. Best supportive care remains the best treatment option for many patients. Some novel approaches to the treatment of patients with CUP are promising and represent a fundamental shift in the paradigm of cancer treatment from organ/tissue-specific to individual patient-focused treatment based on tumour-specific genomic alterations. This paper summarizes the current evidence on the application of each of these approaches. The treatment of three clinical cases with unfavourable CUP using novel approaches is presented.