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Presented by Meredith Pittman, MD and prepared by Sarah Karram, MD
This case talks about a 25 year old woman with upper right quadrant pain. She takes oral contraceptives.
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Question 1 of 1
1. Question
Week (621): Case 2
25 year old woman with upper right quadrant pain. She takes oral contraceptives.
Correct
Answer: Fibrolamellar carcinoma
Fibrolamellar carcinoma (FLC) is a type of hepatocellular carcinoma that arises in younger patients who do not have chronic liver disease. For this reason, it is taught that fibrolamellar carcinoma has a better prognosis than classic hepatocellular carcinoma. Do not be fooled into thinking that FLC is an indolent neoplasm, though. While these patients may live longer because of their relative health, they often present with bulky tumors and lymph node metastases, and they can require multiple operations over time to control their disease. If the tumor is unresectable, the 5-year survival is dismal (~10%).
The histologic diagnosis of FLC depends on architecture and cytology. The cells of FLC grow in sheets or pseudoglands separated by thick, eosinophilic collagen bundles that are usually oriented in parallel to one another. The cells themselves are large and polygonal with very eosinophilic cytoplasm due to increased mitochondria. The nuclei have a single prominent, often red, nucleolus. Some tumors have cytoplasmic inclusions, either “pale bodies” made up of fibrinogen that resemble Hepatitis B inclusions, or PAS-positive hyaline globules. If stained, these tumors will react with Hep-Par1, canalicular pCEA, and usually CK7.Incorrect
Answer: Fibrolamellar carcinoma
Fibrolamellar carcinoma (FLC) is a type of hepatocellular carcinoma that arises in younger patients who do not have chronic liver disease. For this reason, it is taught that fibrolamellar carcinoma has a better prognosis than classic hepatocellular carcinoma. Do not be fooled into thinking that FLC is an indolent neoplasm, though. While these patients may live longer because of their relative health, they often present with bulky tumors and lymph node metastases, and they can require multiple operations over time to control their disease. If the tumor is unresectable, the 5-year survival is dismal (~10%).
The histologic diagnosis of FLC depends on architecture and cytology. The cells of FLC grow in sheets or pseudoglands separated by thick, eosinophilic collagen bundles that are usually oriented in parallel to one another. The cells themselves are large and polygonal with very eosinophilic cytoplasm due to increased mitochondria. The nuclei have a single prominent, often red, nucleolus. Some tumors have cytoplasmic inclusions, either “pale bodies” made up of fibrinogen that resemble Hepatitis B inclusions, or PAS-positive hyaline globules. If stained, these tumors will react with Hep-Par1, canalicular pCEA, and usually CK7.
