MyPACS.net: Radiology Teaching Files > Case 642452

never visited FIBROLAMELLAR HEPATOCELLULAR CARCINOMA
Contributed by: Children's Hospital Omaha, Radiologist, Omaha Childrens, Creighton University and UNMC, Nebraska, USA.
Patient: 17 year old
History: 17-year-old female with jaunice and right sided abdominal pain
Images:[small]larger

Fig. 1: Although usually see a solitary well circumscribed mass with variable enhancement, this patient also has nodules in right lobe of liver. The central scar is low attenuation without calcification . Calcification is usually seen in 40 to 45%
Diagnosis: FIBROLAMELLAR HEPATOCELLULAR CARCINOMA
Discussion:

Fibrolamellar hepatocellular carcinoma

- Occurs in adolescents and young adults.
- No underlying cirrhosis or known risk factors.
- The alpha-fetoprotein is negative
- Mean age 23 to 28 years with a range of 5 - 35 (mean 23) years; M:F = 1:1

Pathology: well-circumscribed strikingly desmoplastic tumor with calcifications and fibrous central scar

Histology: hepatocyte-like cells with granular eosinophilic cytoplasm growing in sheets/cords/trabeculae separated by broad bands of fibrous stroma arranged in parallel lamellae

CT:
- mass of low attenuation and varying degrees of enhancement
- Most commonly see a solitary well circumscribed mass with variable enhancement
- The central scar is low attenuation and calcification is usually seen in 40 to 45%.

Prognosis: 48% resectability rate; 32 months average survival time; 63% 5-year survival time. prognosis better than usual type of hepatocellular carcinoma

Differential Diagnosis: focal nodular hyperplasia (hyperintense central scar on T2WI)

References:

Siegel Marilyn, Pediatric Body CT, Lippincott ,Williams and Wilkins,1999.
Dahnert, Wolfgang. Radiology Review Manual 4th Edition. Williams and Wilkins, 1999

Submitted by: Paras Khandhar, Medical Student, Creighton University Medical Center
Phillip J. Silberberg, M.D, Radiology, Children's Hospital, Omaha, NE

Comments:
No comments posted.
Additional Details:

Case Number: 642452Last Updated: 11-07-2004
Anatomy: Gastrointestinal (GI)   Pathology: Neoplasm
Modality: CTExam Date: Access Level: Readable by all users
Keywords: fibrolamellar hepatocellular carcinoma

The reader is fully responsible for confirming the accuracy of this content.
Text and images may be copyrighted by the case author or institution.
You can help keep MyPACS tidy: if you notice a case which is not useful (e.g. a test case) or inaccurate, please send email to alert@mypacs.net.