Adenocarcinoma of unknown primary site

Visão geral 
Teoria 
Diagnóstico 
Tratamento 
ACOMPANHAMENTO 
Recursos 

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Última revisão: 20 Aug 2025

Última atualização: 30 Jul 2025

Adenocarcinoma of unknown primary site is a common clinical entity, comprising 2% to 4% of all incident cancers worldwide.

Untreated, prognosis is poor, with a median survival between 3 and 4 months.

A key goal in the diagnostic workup is to identify subsets of patients with a more favorable clinicopathologic subtype and likely better outcome.

Systemic chemotherapy is the mainstay of treatment for the overwhelming majority of patients.

Rare subgroups may be candidates for targeted or immune therapies based on results of next-generation sequencing or other biomarkers predictive of response.

Supportive therapies control pain and other symptoms.

Definição

Adenocarcinoma of unknown primary site (ACUP) is the major subgroup in the common clinical entity of cancer of unknown primary site (CUP). It is defined as metastatic adenocarcinoma for which the primary site is undetectable at presentation, despite thorough physical exam, and laboratory and radiographic investigation.[1]

História e exame físico

Principais fatores diagnósticos

pain (e.g., abdomen, chest, bone)
family history of cancer
constitutional symptoms

Detalhes completos

Outros fatores diagnósticos

jaundice
symptoms of postobstructive pneumonia
hemoptysis
ascites
cervical chain adenopathy
personal history of previous cancer
history of smoking
palpable mass
neuropathic pain or weakness
headaches
seizures
delirium
history of heavy alcohol consumption
hepatomegaly

Detalhes completos

Fatores de risco

age >60 years

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

complete blood count
comprehensive metabolic panel
CT chest/abdomen/pelvis
MRI
biopsy (pathologic assessment)
immunohistochemistry (IHC) tests

Detalhes completos

Investigações a serem consideradas

fecal occult blood test
lactate dehydrogenase
urinalysis
18F-fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT
breast imaging (mammography, MRI, ultrasound)
transvaginal ultrasound
paracentesis
endoscopy
direct laryngoscopy with or without esophagoscopy and bronchoscopy
serum tumor markers
genetic biomarker testing

Detalhes completos

Novos exames

tissue of origin testing

Algoritmo de tratamento

Inicial

identification of primary site pending

AGUDA

unfavorable subtype: multiple metastases and/or without a likely primary site

favorable subtype: women with isolated axillary lymphadenopathy (likely breast primary)

favorable subtype: women with peritoneal carcinomatosis of a serous papillary adenocarcinoma (likely ovarian primary)

favorable subtype: poorly differentiated carcinoma with neuroendocrine features (likely small cell lung cancer primary)

favorable subtype: well-differentiated neuroendocrine tumors (likely neuroendocrine primary)

favorable subtype: adenocarcinoma with colorectal immunohistochemistry (likely colorectal primary)

favorable subtype: poorly differentiated carcinoma of the mediastinum or retroperitoneum in males <40 years (likely testicular primary)

favorable subtype: blastic bone metastases with immunohistochemistry/serum prostate-specific antigen (likely prostate primary)

favorable subtype: single metastatic lesion

favorable subtype: oligometastatic disease

Colaboradores

Autores

Michael S. Lee, MD

Associate Professor

Department of Gastrointestinal Medical Oncology

Division of Cancer Medicine

University of Texas MD Anderson Cancer Center

Houston

Declarações

MSL has consulted for Pfizer, Bayer, Delcath, and Imvax. His institution has received research funding from Amgen, Exelixis, Bristol-Myers Squibb, Pfizer, Rafael Pharmaceuticals, EMD Serono, Genentech/Roche, Merck, Arcus, and Shanghai EpiMab Biotherapeutics. MSL is the author of a number of papers cited in this topic.

Agradecimentos

Dr Michael S. Lee would like to gratefully acknowledge Dr Ross C. Donehower, Dr David Cosgrove, and Dr Hatim Hussain, previous contributors to this topic.

Declarações

RCD, DC, and HH declare that they have no competing interests.

Revisores

Nikhil Khushalani, MD

Assistant Professor

Department of Medicine

Roswell Park Cancer Institute

Buffalo

Declarações

NK declares that he has no competing interests.

Zelig Tochner, MD

Associate Professor

Radiation Oncology

Children's Hospital of Philadelphia

Philadelphia

Declarações

ZT declares that he has no competing interests.

Justin Stebbing, MA, MRCP, MRCPath, PhD

Consultant Medical Oncologist/Senior Lecturer

Department of Medical Oncology

Imperial College/Imperial Healthcare NHS Trust

Charing Cross Hospital

London

Declarações

JS declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: occult primary [internet publication].Texto completo

Krämer A, Bochtler T, Pauli C, et al. Cancer of unknown primary: ESMO clinical practice guideline for diagnosis, treatment and follow-up. Ann Oncol. 2023 Mar;34(3):228-46.Texto completo Resumo

National Institute for Health and Care Excellence. Metastatic malignant disease of unknown primary origin in adults: diagnosis and management. Apr 2023 [internet publication].Texto completo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Squamous or neuroendocrine carcinoma of unknown primary
Mais Diagnósticos diferenciais
Diretrizes
- NCCN clinical practice guidelines in oncology: head and neck cancers
- NCCN clinical practice guidelines in oncology: occult primary
Mais Diretrizes
Conectar-se ou assinar para acessar todo o BMJ Best Practice

O uso deste conteúdo está sujeito ao nosso aviso legal

Adenocarcinoma of unknown primary site

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Novos exames

Algoritmo de tratamento

identification of primary site pending

unfavorable subtype: multiple metastases and/or without a likely primary site

favorable subtype: women with isolated axillary lymphadenopathy (likely breast primary)

favorable subtype: women with peritoneal carcinomatosis of a serous papillary adenocarcinoma (likely ovarian primary)

favorable subtype: poorly differentiated carcinoma with neuroendocrine features (likely small cell lung cancer primary)

favorable subtype: well-differentiated neuroendocrine tumors (likely neuroendocrine primary)

favorable subtype: adenocarcinoma with colorectal immunohistochemistry (likely colorectal primary)

favorable subtype: poorly differentiated carcinoma of the mediastinum or retroperitoneum in males <40 years (likely testicular primary)

favorable subtype: blastic bone metastases with immunohistochemistry/serum prostate-specific antigen (likely prostate primary)

favorable subtype: single metastatic lesion

favorable subtype: oligometastatic disease

Colaboradores

Autores

Michael S. Lee, MD

Declarações

Agradecimentos

Declarações

Revisores

Nikhil Khushalani, MD

Declarações

Zelig Tochner, MD

Declarações

Justin Stebbing, MA, MRCP, MRCPath, PhD

Declarações

Peer reviewer acknowledgements

Disclosures

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice