This article appears in the following Annals of Oncology issue: ESMO Clinical Recommendations [View the issue table of contents]
ESMO clinical recommendations |
Cancers of unknown primary site: ESMO Clinical Recommendations for diagnosis, treatment and follow-up
1 Department of Medical Oncology, University of Ioannina, Ioannina Ipiros, Greece
2 Medical Oncology Service, Vall d'Hebron University Hospital, Barcelona, Spain
* Correspondence to: ESMO Guidelines Working Group, ESMO Head Office, Via L. Taddei 4, CH-6962 Viganello-Lugano, Switzerland; E-mail: clinicalrecommendations{at}esmo.org
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incidence |
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 Top incidence diagnosisstaging and risk assessmenttreatmentresponse evaluationfollow-upnotereferences |
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Cancers of unknown primary site (CUPs) represent a heterogeneous group of metastatic tumors for which a work-up as listed below fails to identify the site of origin at the time of diagnosis. CUPs accounts for 3–5% of all malignancies.
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diagnosis |
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Topincidencediagnosisstaging and risk assessmenttreatmentresponse evaluationfollow-upnotereferences |
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CUPs require pathology evaluation and are categorized by pathology into:
- well- and moderately differentiated adenocarcinomas;
- poorly differentiated carcinomas;
- squamous cell carcinomas;
- undifferentiated neoplasms;
- carcinomas with neuroendocrine differentiation.
Immunohistochemistry should be applied in poorly differentiated cases to exclude chemosensitive and potentially curable tumors (i.e. lymphomas and germ cell tumors).
If diagnosis is adenocarcinoma, immunostaining for prostate-specific antigen (PSA) in male patients and for estrogen and progesterone receptors in females with axillary node metastases is advisable to rule out hormone-sensitive tumors amenable to specific therapy. Staining for keratins CK7 and CK20 may provide indications towards a possible primary site.
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staging and risk assessment |
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Topincidencediagnosisstaging and risk assessmenttreatmentresponse evaluationfollow-upnotereferences |
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Appropriate staging and diagnostic work-up can help to identify a minority of CUP patients who can expect to benefit from directed therapy. The following recommendations epitomize standard and optional assessments suggested.
- Thorough physical examination (including head and neck, rectal, pelvic and breast examination), basic blood and biochemistry survey, urinalysis, fecal occult blood test and CT scan of thorax, abdomen and pelvis constitute a minimal basic work-up.
- Endoscopies should be sign- or symptom-guided. Serum assessment of
-fetoprotein (aFP), β-human chorionic gonadotropin (bHCG) and PSA is suggested in male patients to exclude potentially curable extragonadal germ cell tumor and amenable to hormone treatment prostate cancer.
- A mammogram should be performed in women with an adenocarcinoma.
- Whole body 2-deoxy-2-[18F]fluoro-D-glucose positron emission tomography (CT/FDG-PET) may contribute to the management of patients with CUP tumors and especially those with cervical adenopathies and single metastasis.
- Subsets of chemosensitive and potentially curable tumors, such as patients with predominantly nodal metastases of poorly differentiated carcinomas and females with peritoneal carcinomatosis of a serous histologic type adenocarcinoma must not be missed.
Diagnostic and staging guidelines for patients with an anticipatory CUP diagnosis are summarized in Table 1.
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treatment |
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Topincidencediagnosisstaging and risk assessmenttreatmentresponse evaluationfollow-upnotereferences |
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Therapy should be tailored on an individual basis by recognition of well-defined clinicopathologic subsets that differ in prognosis as described in Table 2 [III, B].
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response evaluation |
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Topincidencediagnosisstaging and risk assessmenttreatmentresponse evaluationfollow-upnotereferences |
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Response evaluation is recommended after two or three chemotherapy cycles by individually adequate tests.
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follow-up |
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Topincidencediagnosisstaging and risk assessmenttreatmentresponse evaluationfollow-upnotereferences |
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There is no evidence that follow-up of asymptomatic patients is needed. Specific examinations as clinically indicated.
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note |
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Topincidencediagnosisstaging and risk assessmenttreatmentresponse evaluationfollow-upnotereferences |
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Levels of evidence [I–V] and grades of recommendation [A–D] as used by the American Society of Clinical Oncology are given in square brackets. Statements without grading were considered justified standard clinical practice by the expert authors and the ESMO faculty.
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footnotes |
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Approved by the ESMO Guidelines Working Group: April 2002, last update June 2008. This publication supercedes the previously published version—Ann Oncol 2008; 19 (Suppl 2): ii106–ii107.
Conflict of interest: the authors have reported no conflicts of interest.
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references |
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Topincidencediagnosisstaging and risk assessmenttreatmentresponse evaluationfollow-upnotereferences |
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1. Pavlidis N, Briasoulis E, Hainsworth J, Greco FA. Diagnostic and therapeutic management of cancer of an unknown primary. Eur J Cancer (2003) 39:1990–2005.[CrossRef][Web of Science][Medline]
2. Abbruzzese JL, Abbruzzese MC, Lenzi R, et al. Analysis of a diagnostic strategy for patients with suspected tumors of unknown origin. J Clin Oncol (1995) 13:2094–2103.
3. Culine S, Kramar A, Saghatchian M, et al. Development and validation of a prognostic model to predict the length of survival in patients with carcinomas of an unknown primary site. J Clin Oncol (2002) 20:4679–4683.
4. Seve P, Billotey C, Broussolle C, et al. The role of 2-deoxy-2-[F-18]fluoro-D-glucose positron emission tomography in disseminated carcinoma of unknown primary site. Cancer (2007) 109:292–299.[CrossRef][Web of Science][Medline]
5. Bugat R, Bataillard A, Lesimple T, et al. Summary of the standards, options and recommendations for the management of patients with carcinoma of unknown primary site. Br J Cancer (2003) 89(Suppl 1):S59–S66.[CrossRef][Web of Science][Medline]
6. Pavlidis N. Forty years experience of treating cancer of unknown primary. Acta Oncol (2007) 46:592–601.[CrossRef][Web of Science][Medline]
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