Table 4. Diagnostic work-up for carcinoma of unknown primary site as a function of their histopathological and anatomic localisation.
| Step 1 | Step 2 | Step 3 |
|||
|---|---|---|---|---|---|
| Routine diagnostic work-up | Specific work-up depending on the histopathology | Specific work-up as a function of the localisation |
|||
| Localisation | Standards | Options | Recommendations | ||
| Neuroendocrine carcinoma | There are no standards | There are no options | There are no recommendations | ||
| Carcinoma of unknown primary site | Adenocarcinoma and undifferentiated carcinoma | Midline adenopathies | Testicular ultrasound (level of evidence: B2) Chest-abdominal CT scan (level of evidence: B2) | There are no options | There are no recommendations |
| Standards | (Standards, level of evidence: B2): | Mediastinal adenopathies | Testicular ultrasound and chest-abdominal CT scan to eliminate a germ cell tumour in men | There are no options | There are no recommendations |
| Pathological evaluation (level of evidence: B2) | For women | Cervical and/or supraclavicular adenopathies | Testicular ultrasound (in the presence of an associated midline adenopathy) Chest-abdominal CT scan) | There are no options | Panendoscopy and neck and face CT scan, serology for Epstein–Barr virus or detection of DNA by in situ hybridisation to eliminate diagnosis of a undifferentiated nasopharyngeal carcinoma (expert agreement) |
| Clinical history (level of evidence: B2) | Mammography | ||||
| Clinical examination (level of evidence: B2) | Ultrasound or pelvic CT scan | ||||
| Chest X-ray (level of evidence: B2) | For men | ||||
| No complete work-up (level of evidence: B2) | Serum PSA, αFP, βHCG assays | ||||
| Axillary adenopathy (women) | Breast ultrasounda (level of evidence: C) | There are no options | There are no recommendations | ||
| Breast MRIa (level of evidence: C) | |||||
| Inguinal adenopathy | There are no standards | There are no options | There are no recommendations | ||
| Liver metastases | Woman: αFP assay if undifferentiated carcinoma | Coloscopy | Coloscopy in the presence exclusive, resectable liver metastases (expert agreement) | ||
| Lung metastases | Women: βHCG assay | There are no options | There are no recommendations | ||
| Men: chest-abdominal CT scan and testicular ultrasound | |||||
| Bone metastases | There are no standards | There are no options | Bone scintigraphy and standard X-rays of painful zones | ||
| Brain metastases | There are no standards | There are no options | There are no recommendations | ||
| Single metastasis | There are no standards | Whole-body CT scan (expert agreement) Bone scintigraphy | There are no recommendations | ||
| Pleural effusion | There are no standards | Chest CT scan (expert agreement) | There are no recommendations | ||
| Peritoneal effusionb | There are no standards | Abdominal-pelvic CT scan in women | There are no recommendations | ||
| Squamous cell carcinoma | Cervical adenopathy | Panendoscopy (level of evidence: B2) | There are no options | There are no recommendations | |
| Head and neck CT scan (level of evidence: B2) | |||||
| Diagnostic bilateral amygdalectomy | |||||
| Supraclavicular or axillary adenopathy | There are no standards | Chest CT scan | There are no recommendations | ||
| No specific work-up for this histopathological type | Inguinal adenopathy | Clinical examination of the external genital organs | Pelvic CT scan or ultrasound | There are no recommendations | |
| Anuscopy and colposcopy | |||||
| Bone metastases | Complete clinical examination with a head and neck examination (expert agreement) | Panendoscopy | Bone scintigraphy and standard X-rays of painful zones (expert agreement) | ||
a
Examination to be performed after a ‘negative’ mammography.
b
Although this entity is not included in the classification of ‘carcinoma of unknown primary site’ because the primary tumour is known, the therapeutic implications from its diagnosis lead the working group to include this entity in the current SOR document.
CT=computed tomography; PSA=prostate antigen specific; αFP=α-foctoproteins; βHCG=β-human chorionic gonado trophin; MRI=magnetic resonance imaging.