Carcinoma of unknown primary is a type of cancer that is diagnosed by biopsy-proven malignancy and its anatomic site of origin remains unidentified. It is one of the 10 most frequently diagnosed cancers worldwide accounting for 3%-5% of all the cancers diagnosed. The word ‘unknown’of this type of cancer is now becoming obscure with the advent of new imaging, robust immunohistochemistry, and genomic and proteomic tools. The reason for CUP is unknown. One of the theories is that the primary tumor either regresses after seeding the metastasis or remains too small to be detected. 

The clinical evaluation of this type of tumors has two main goals: 

  • To search for the origin of the primary tumor based on pathologic evaluation, and
  • To determine the extent of the disease.
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It is very important to determine the anatomic site of origin of tumor to treat this type of tumors. Patients should undergo a directed diagnostic search for the primary tumor based on clinical and pathological data. The treatment encompasses systemic chemotherapy as the primary treatment modality in patients with disseminated disease. Careful integration of surgery, radiation therapy and periods of observation are important in the overall management of this condition. 

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