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INTRODUCTION

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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

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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

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Work-up

Clinical evaluation

  • H&P, including pelvic, breast, and rectal exams

  • CBC

  • Comprehensive metabolic profile

  • Urinalysis

  • Occult blood in feces

  • Lactate dehydrogenase (LDH)

  • Serum human chorionic gonadotropin (HCG)

  • Alpha-fetoprotein (AFP)

  • Carcinoembryonic antigen (CEA)

  • CA 19-9, CA 27–29 (or CA 15-3), CA 125

  • Chest/abdominal/pelvic CT

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:

  • Electron microscopy

  • Cytogenetic analysis

  • Molecular profiling

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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

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Survival

  • 1-Year survival: 35–40%

  • 2-Year survival: 15–20%

  • 3-Year survival: 10–15%

  • 5-Year survival: 10%

  • 8-Year survival: < 10%

Survival varies by histology and clinical subsets

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