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INTRODUCTION

Cancer of unknown primary site (CUPS) is defined as follows: biopsy-proven malignancy; primary site unapparent after history, physical exam, chest x-ray, abdominal and pelvic CT, complete blood count, chemistry survey, mammography (women), β-human chorionic gonadotropin (hCG) levels (men), α-fetoprotein (AFP) levels (men), and prostate-specific antigen (PSA) levels (men); and histologic evaluation not consistent with a primary tumor at the biopsy site. CUPS incidence is declining, probably because of better pathology diagnostic criteria; it accounts for about 3% of all cancers today, down from 10–15% 15 years ago. Most pts are over age 60. The tumors are often aneuploid. Cell lines derived from such tumors frequently have abnormalities in chromosome 1.

Clinical Presentation

Pts may present with fatigue, weight loss, pain, bleeding, abdominal swelling, subcutaneous masses, and lymphadenopathy. Once metastatic malignancy is confirmed, diagnostic efforts should be confined to evaluating the presence of potentially curable tumors, such as lymphoma, Hodgkin's disease, germ cell tumor, ovarian cancer, head and neck cancer, and primitive neuroectodermal tumor, or tumors for which therapy may be of significant palliative value such as breast cancer or prostate cancer. In general, efforts to evaluate the presence of these tumor types depend more on the pathologist than on expensive clinical diagnostic testing. Localizing symptoms, a history of carcinogen exposure, or a history of fulguration of skin lesion may direct some clinical testing; however, the careful light microscopic, ultrastructural, immunologic, karyotypic, and molecular biologic examination of adequate volumes of tumor tissue is the most important feature of the diagnostic workup in the absence of suspicious findings on history and physical exam (Table 82-1).

TABLE 82-1POSSIBLE PATHOLOGIC EVALUATION OF BIOPSY SPECIMENS FROM PATIENTS WITH METASTATIC CANCER OF UNKNOWN PRIMARY SITE

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