Fibrolamellar hepatocellular carcinoma (FHCC) is a rare form of hepatocellular carcinoma (HCC) that typically affects young adults and is characterized, under. Although fibrolamellar carcinoma (FLC) has conventionally been considered to be a histologic variant of hepatocellular carcinoma (HCC). Summary. An Orphanet summary for this disease is currently under development. However, other data related to the disease are accessible.
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Metastatic Fibrolamellar Hepatocellular Carcinoma to the Pancreas
Hepatocelluar usual markers for liver disease – aspartate aminotransferaseheppatocelular aminotransferase and alkaline phosphatase – are often normal or only slightly elevated. Journal of hepatology ; 56 4: Cecil Tratado de medicina interna.
It is characterized histologically by well-differentiated malignant hepatic cells with abundant eosinophilic and granular cytoplasm due to the presence of numerous mitochondria, and by the presence of fibrous stroma arranged in thin parallel lamellae interspersed between tumor cells [ 1 ].
While sites of recurrence usually include the liver, regional lymph nodes, peritoneum, and lung [ 6 ], metastasis to the pancreas is extremely rare, with only 2 other cases reported in the literature to our knowledge [ 78 ].
Fibrolamellar hepatocellular carcinoma is a distinct histological variant of hepatocellular carcinoma characterised on microscopy by laminated fibrous layers between the tumor cells. Radiology ; 2: Edit article Share article View revision history. Hepatomegaly is usually evident as these masses are typically large. Radiology ; 1: This disease was first described by Edmondson in a year-old female with no underlying liver disease.
At that time he underwent a routine physical examination, where he was noted to have a positive skin test for tuberculosis.
Fibrolamellar hepatocarcinoma (FLH): report case
Eur J Cancer ; 49 Although the bad prognosis of the tumor, hepatectomy should always be tried, if technical resection is feasible. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. Cecil Tratado de medicina interna.
A systematic review and meta-analysis.
His AFP level was normal. Differential diagnosis of tumors and tumor-like lesions of liver in infancy and childhood. We present varcinoma case of a year-old male with no previous medical history. The surgical pathology from the resected specimens showed focal thin fibrous bands within the metastatic foci; however, definitive diagnostic features of a fibrolamellar variant of HCC were not identified within the metastatic lesions.
This prompted the use of a gauge needle, obtaining adequate specimens, and pathology revealed metastatic FL-HCC fig. In our case, the possibility of performing an abdominal contrast sonography helped to establish the diagnosis, which was confirmed by histological and analytical studies.
First-choice treatment is surgical resection 1,5. The patient was then referred for evaluation and diagnosis of the pancreatic mass by endoscopic ultrasound-guided fine-needle aspiration EUS-FNA.
Fibrolamellar carcinoma presenting as a pancreatic mass: Diseases of liver Pediatric cancers Digestive system neoplasia Rare cancers. Immunohistochemical studies have found that FL-HCC expresses markers associated with biliary CK7 and epithelial membrane antigen and hepatic hepar-1 and glypican-3 differentiation as well as markers associated with hepatic stem cells CD and CD44 hepatocelulxr help differentiate it fibolamelar conventional HCC [ 1213 ].
Fibrolamellar hepatocellular carcinoma in children and adolescents. Is fibrolamellar carcinoma different from hepatocellular carcinoma?
Fibrolamellar hepatocellular carcinoma
In our patient, MRI showed, in addition to the nodule in the left lower lobe, a 2. Published online Jul Log in Sign up. AdolescentAdult ICD Other search option s Alphabetical list. The genomic landscape of fibrolamellar hepatocellular carcinoma: Reporte de un caso. Liver resection is the optimal treatment and may need to be performed more than once, since this disease has a very high fibrokamelar rate.
Cases and figures Imaging differential diagnosis.
Fibrolamellar hepatocellular carcinoma | Radiology Reference Article |
Eur J Surg Oncol. AMA journal of diseases of children ; 91 2: Is fibrolamellar carcinoma different from hepatocellular carcinoma? Cytologicallythe tumor cells have a low nuclear to cytoplasmic ratio with abundant eosinophilic cytoplasm. He had done well since his liver transplantation until Augustwhen a left lung nodule 3 cm in size was noted on a PET CT, in addition to a 2-cm borderline hypermetabolic hepatocelu,ar in the tail of the pancreas.
Radiol Clin North Am ; Additional information Further information on this disease Classification s 2 Gene s 2 Other website s 3.