Durable response of chemotherapy for cancer of unknown primary with unfavorable subset developed in retroperitoneal space

Distant metastases are the main cause of cancer-related deaths in patients with advanced tumors. A standard diagnostic workup usually contains the identification of the tissue-of-origin of metastatic tumors, although under certain circumstances, it remains elusive. This disease setting is defined as cancer of unknown primary (CUP). Accounting for approximately 3–5% of all cancer diagnoses, CUPs are characterized by an aggressive clinical behavior and represent a real therapeutic challenge. The lack of determination of a tissue of origin precludes CUP patients from specific evidence-based therapeutic options or access to clinical trial, which significantly impacts their life expectancy. In the era of precision medicine, it is essential to characterize CUP molecular features, including the expression profile of non-coding RNAs, to improve our understanding of CUP biology and identify novel therapeutic strategies. This review article sheds light on this enigmatic disease by summarizing the current knowledge on CUPs focusing on recent discoveries and emerging diagnostic strategies.

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Cancer of unknown primary

Medicine ◽

10.1097/md.0000000000006693 ◽

2017 ◽

Vol 96 (16) ◽

pp. e6693 ◽

Cited By ~ 1

Author(s):

Anne-Kirstine Dyrvig ◽

Knud Bonnet Yderstræde ◽

Oke Gerke ◽

Peter Bjødstrup Jensen ◽

Søren Hess ◽

...

Keyword(s):

Cancer Of Unknown Primary ◽

Unknown Primary

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Swedish National Multicenter Study on Head and Neck Cancer of Unknown Primary: Prognostic Factors and Impact of Treatment on Survival

International Archives of Otorhinolaryngology ◽

10.1055/s-0040-1712106 ◽

2020 ◽

Author(s):

Lars Axelsson ◽

Erik Holmberg ◽

Jan Nyman ◽

Anders Högmo ◽

Helena Sjödin ◽

...

Keyword(s):

Head And Neck Cancer ◽

Prognostic Factors ◽

Head And Neck ◽

Neck Dissection ◽

Neck Cancer ◽

Multicenter Study ◽

Performance Status ◽

Cancer Of Unknown Primary ◽

Unknown Primary ◽

N Stage

Abstract Introduction Head and neck cancer of unknown primary (HNCUP) is a rare condition whose prognostic factors that are significant for survival vary between studies. No randomized treatment study has been performed thus far, and the optimal treatment is not established. Objective The present study aimed to explore various prognostic factors and compare the two main treatments for HNCUP: neck dissection and (chemo) radiation vs primary (chemo) radiation. Methods A national multicenter study was performed with data from the Swedish Head and Neck Cancer Register (SweHNCR) and from the patients' medical records from 2008 to 2012. Results Two-hundred and sixty HNCUP patients were included. The tumors were HPV-positive in 80%. The overall 5-year survival rate of patients treated with curative intent was 71%. Age (p < 0.001), performance status (p= 0.036), and N stage (p= 0.046) were significant factors for overall survival according to the multivariable analysis. Treatment with neck dissection and (chemo) radiation (122 patients) gave an overall 5-year survival of 73%, and treatment with primary (chemo) radiation (87 patients) gave an overall 5-year survival of 71%, with no significant difference in overall or disease-free survival between the 2 groups. Conclusions Age, performance status, and N stage were significant prognostic factors. Treatment with neck dissection and (chemo) radiation and primary (chemo) radiation gave similar survival outcomes. A randomized treatment study that includes quality of life is needed to establish the optimal treatment.

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