Fibrolamellar Hepatocellular Carcinoma in an Infant and Literature Review

Cruz, Ofelia; Laguna, Astrid; Vancells, M; Krauel, Lucas; Medina, Marilyn; Mora, Jaume

doi:10.1097/mph.0b013e31818b0f82

Cited by 10 publications

(4 citation statements)

References 19 publications

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“…The youngest age reported in the literature is 7 months [48], but this case is such an exception that the diagnosis is strongly suspected. Likewise, the published images in another reported case of FLC in a 4-month-old infant do not strongly support the diagnosis of FLC [49]. However, FLC has been reported by several groups as early as 4 to 5 years of age [2, 50, 51].…”

Section: Clinical Findingsmentioning

confidence: 94%

Fibrolamellar Carcinoma: 2012 Update

Torbenson

2012

Scientifica

146

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Fibrolamellar carcinomas are a unique type of primary liver cancer. They occur most commonly in children and young adults. Their etiology remains a mystery, as they are not associated with chronic liver disease. Fibrolamellar carcinomas are not indolent tumors, but have an overall better prognosis than typical hepatocellular carcinomas, in part because of the younger age at presentation and the lack of cirrhosis. The most important prognostic feature is whether the tumor is resectable. Histologically, the tumor is made up of large cells that contain abundant mitochondria. The nuclei of the tumor cells have prominent nucleoli. The tumor cells induce the formation of extensive intratumoral fibrosis, which often grows in parallel, or lamellar bands. The tumor cells clearly show hepatocellular features but are also unique in showing both biliary and neuroendocrine differentiation. The uniqueness of fibrolamellar carcinoma extends to their molecular findings. While the genetic abnormalities that lead to fibrolamellar carcinomas are not yet known, studies have shown that they lack mutations in the genes most commonly mutated in typical hepatocellular carcinoma (TP53 and CTNNB1). In this paper, the clinical, pathological, and basic science literature on fibrolamellar carcinoma is comprehensively reviewed. Key areas of needed research are also discussed.

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Section: Clinical Findingsmentioning

confidence: 94%

Fibrolamellar Carcinoma: 2012 Update

Torbenson

2012

Scientifica

146

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“…It is more common in adolescents and young adults, in whom it accounts for 13-22 % of all HCC [ 90 ]. The overall age-adjusted incidence of FLC is 0.02 per 100,000.…”

Section: Clinical Features and Epidemiologymentioning

confidence: 98%

Gastrointestinal, Pancreatic and Hepatic Malignancies in Children

Towbin

Rowland

Parham

2014

Pediatric Malignancies: Pathology and Imaging

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The gastrointestinal tract, liver, and pancreas are uncommon sites for primary malignant neoplasms in the pediatric population. None of the individual malignant neoplasms of these sites represented more than 2 % of cancer diagnoses in children between 0 and 19 years of age in the SEER database [ 1 ]. This is in marked contrast to the frequency of malignant neoplasms of these sites in adulthood. Gastrointestinal TractLymphoma is the most common pediatric gastrointestinal tract malignancy, accounting for approximately 75 % of malignancies in most series. This is followed by neuroendocrine tumors (NET), adenocarcinoma, and sarcoma [ 2 -4 ]. Carcinomas and a variety of rare neoplasms can occur in the gastrointestinal tract but are not addressed in this chapter. Lymphoma is discussed in another chapter.

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“…However, young patients who develop HCC are less likely to have associated chronic liver disease (33%) compared to adults (70–90%) [1]. In children, inborn errors of metabolism, such as alpha-1-antitrypsin deficiency, hereditary tyrosinaemia, Gaucher's disease, congenital biliary atresia, urea cycle defects, and familial cholestatic syndrome caused by a bile salt export pump deficiency, may be the underlying cause [2]. Prognosis in the presence of metastatic disease is poor, although it may be somewhat better in children and younger adults compared to older adults, with long-term survival of 10–20% compared to less than 5%, respectively [1].…”

Section: Introductionmentioning

confidence: 99%