24 yo F presents with 3 wk hx of crampy abd pain and nausea

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24 yo F presents with 3 wk hx of crampy abd pain
and nausea Pt is s/p uncomplicated SVD other than
anemia requiring transfusion
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MRI
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Bone scan
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• MRI Large mass replacing L hepatic lobe with
  extension into R hepatic lobe, most consistent
  with hepatic epithelioid hemangioendothelioma
  (low grade malignant vascular tumor of the
  liver). Enlarged lymph node in porta hepatis and
  high gastrohepatic ligament (suspicious for
  metastasis). Mass compresses the main PV with
  secondary abdominal ascites.
• Chest CT WNL
• Bone Scan Minimal inc in soft tissue
  radiotracer uptake within R and central abd in
  location of mass

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US
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Pelvic MRI
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Liver Biopsy

• Core needle FNA
• CK7, CK20, CA125, GCDFP, mucin .
• Interpretation Invasive moderately
  differentiated adenocarcinoma
• Ddx hepatic cholangiocarcinoma, metastatic
  adenocarcinoma
• CA-125 favors cholangiocarcinoma or
  adenocarcinoma of GYN origin

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CT
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CT
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Abd CT
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Adenocarcinoma of unknown origin

• Around 70 of unknown primary site tumors
• Often include liver, lungs, lymph nodes, bones
• Poor prognosis, with median survival of 4-6
  months
• Path grandular formation, positive for mucin

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Dx

• Through HP (pelvic prostate)
• CBC, chemistry, urinalysis, hemoccult
• CXR
• PSA or mammography
• Breast MRI for women with axillary lymph node
  involvement
• PET detects primary tumor, additional sites of
  metastasis
• Abd CT
• Possible colonoscopy

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Rx

• If no specific diagnosis, empiric chemo taxanes,
  topoisomerase I inhibitors, gemcitabine,
  vinorelbine
• Possible debulking