Chapter 7: Carcinoma of Unknown Primary

David R. Spigel; F. Anthony Greco

Epidemiology

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Epidemiology

Incidence:	Unknown^✫ (Estimated at 30,000–45,000 patients/year)	Stage at Presentation
Median age:	Varies by histology (usually sixth decade)	Local/regional:	<10%
Male to female ratio:	M ≅ F	≥2 sites:	>90%
^✫Due to patient heterogeneity and tumor registry misclassification Cancer Facts and Figures 2013, the America Cancer Society Greco FA, Hainsworth JD. In: DeVita VT, Jr, Hellman S, Rosenberg SA, eds. Cancer: Principles & Practice of Oncology, 8th ed. Lippincott; 2008:2363–2388 Hainsworth JD et al. J Clin Oncol 1991;9:1931–1938 Hainsworth JD, Greco FA. N Engl J Med 1993;329:257–263

Pathology

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Pathology

Adenocarcinoma (well differentiated or moderately differentiated)	60%
Poorly differentiated carcinoma/(± features of adenocarcinoma)	29%
Poorly differentiated malignant neoplasm	5%
Squamous carcinoma	5%
Neuroendocrine carcinoma	1%
Greco FA, Hainsworth JD. In: DeVita VT, Jr, Hellman S, Rosenberg SA, eds. Cancer: Principles & Practice of Oncology, 8th ed. Lippincott; 2008:2363–2388 Hainsworth JD, Greco FA. N Engl J Med 1993;329: 257–263

Work-up

Clinical evaluation

Where appropriate:

Positron emission tomography (PET)
Prostate specific antigen (PSA)
Mammography, bronchoscopy, and panendoscopy (particularly for squamous carcinomas)

Pathologic studies

Core needle or excisional biopsy preferred over fine-needle aspiration (FNA)/cytology (consider rebiopsy if insufficient material from initial biospy)
Immunohistochemistry (IHC) analyses for:
- PSA in men
- Estrogen receptor (ER) in women
- Progesterone receptor (PR) in women
- HER2/neu overexpression in women
- CD 117
- Consider gene signature profiling of tissue of origin

Where appropriate:

Focused Work-up

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Focused Work-up

Presentation	Men	Women
Head and neck or supraclavicular adenopathy	ENT exam Chest/abdominal CT PSA, testicular ultrasound	ENT exam Chest/abdominal CT Mammography, ER/PR Pathologic evaluation
Axillary adenopathy	Chest/abdominal CT PSA	Chest/abdominal CT Mammography, ER/PR (Consider ultrasound or MRI)
Mediastinal involvement	Chest/abdominal CT HCG/AFP PSA	Chest/abdominal CT HCG/AFP Mammography, ER/PR
Chest (effusion and/or nodules) involvement	Chest/abdominal CT PSA	Chest/abdominal/pelvic CT Mammography, ER/PR CA 125
Peritoneal involvement	Chest/abdominal/pelvic CT PSA	Chest/abdominal/pelvic CT Mammography, ER/PR CA 125
Retroperitoneal mass	Chest/abdominal/pelvic CT PSA HCG/AFP Testicular ultrasound	Chest/abdominal/pelvic CT Mammography, ER/PR CA 125
Inguinal adenopathy	Abdominal/pelvic CT PSA	Abdominal/pelvic CT Mammography, ER/PR CA 125
Hepatic involvement	Chest/abdominal/pelvic CT Colonoscopy AFP PSA	Chest/abdominal/pelvic CT Colonoscopy AFP Mammography, ER/PR
Skeletal involvement	Bone scan PSA	Bone scan Mammography, ER/PR
Brain involvement	Chest/abdominal CT	Chest/abdominal CT Mammography, ER/PR
Greco FA, Hainsworth JD. In: DeVita VT, Jr, Hellman S, Rosenberg SA, eds. Cancer: Principles & Practice of Oncology, 8th ed. Lippincott; 2008:2363–2388

Survival

Survival varies by histology and clinical subsets

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