Rev Med Chil. 2009 Mar;137(3):394-400. Epub 2009 Jun 15.

[Fibrolamellar liver carcinoma: report of two cases and review of the literature].

[Article in Spanish]

Butte JM, Waugh E, Meneses M, Pruzzo R, Carvallo C, Redondo F, Suárez C, Parada H, Amaral H, de La Fuente H.

Source

Instituto Oncológico Fundación Arturo López Pérez, Santiago,Chile.

Abstract

Fibrolamellar hepatocellular carcinoma (FLC) is a rare histologic variant of hepatocellular carcinoma that appears most commonly in teenagers and young adults. The diagnosis is often made incidentally and surgical resection is the only curative treatment. Here we report two cases of incidental FLC involving a 19 year-old male, initially diagnosed with screening abdominal ultrasound, and a 14 year-old female that presented with abdominal pain. Diagnostic workup consisted of abdominal PET/CT and MR1 Imaging studies and tissue diagnosis was confirmed with percutaneous liver biopsy. Both patients were treated with radical liver resection/tumor excision. However, tumor recurrence was observed in both during short-term follow-up. The male patient was treated successfully with surgical treatment however the female patient succumbed top regression of disease.

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Radiographics. 1999 Mar-Apr;19(2):453-71.

Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation.

McLarney JK, Rucker PT, Bender GN, Goodman ZD, Kashitani N, Ros PR.

Source

Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington DC 20306, USA.

Abstract

Fibrolamellar carcinoma is a malignant hepatocellular tumor with distinct clinical and pathologic differences from hepatocellular carcinoma. It differs from hepatocellular carcinoma in demographics, condition of the affected liver, tumor markers, and prognosis. Fibrolamellar carcinoma characteristically manifests as a large hepatic mass in adolescents or young adults (without gender predominance). Cirrhosis; elevated alpha-fetoprotein levels; and typical risk factors for hepatocellular carcinoma such as viral hepatitis, alcohol abuse, and metabolic disease are typically absent. Fibrolamellar carcinoma is characterized pathologically by cords of tumor cells surrounded by abundant collagenous fibrous tissue arranged in a parallel or lamellar distribution. Fibrotic lamellae often coalesce to form a central scar. Fibrolamellar carcinoma characteristically appears on radiologic images as a lobulated heterogeneous mass with a central scar in an otherwise normal liver. Radiologic evidence of cirrhosis, vascular invasion, or multifocal disease--findings typical of hepatocellular carcinoma--is uncommon in fibrolamellar carcinoma. Imaging features of fibrolamellar carcinoma overlap with those of other scar-producing lesions including focal nodular hyperplasia (FNH), hepatocellular adenoma and carcinoma, hemangioma, metastases, and cholangiocarcinoma. FNH, in particular, may simulate fibrolamellar carcinoma, since both have similar demographic and clinical characteristics. Because some believe that radiologic diagnosis of FNH is possible, it is important to understand the imaging appearance of fibrolamellar carcinoma to avoid misdiagnosing this malignant tumor as a FNH.

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CASE: HEPATOBLASTOMA in adolescence female- left hepatectomy^-www.drkeyurbhatt.in*

15 yrs female with vague pain in epigastric region
O/E : large 15 x 12 cm mass palpable arising from liver left lobe, hard, nontender.
NO Anorexia, No wt loss.
AFP, CEA,CA 19.9, CA 125 Were normal

CT / MR Abdomen angio:
s/o malignant liver mass from segment 3, hanging in nature with doubtful lesion in seg 4b as well.

SURGERY: Left hepatectomy with excision of seg 4b lesion, DURATION: 4.5 hrs. No blood transfusion required..and pt was shifted to ward post operatively..

was discharged on POD 5, With normal diet, and without any undue complication..

HPE : IHC S/o Hepatoblastoma, node negative, resection margins free of tumor..
pt subjected to adjuvent chemotherapy to totally eradicate the disease..

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CASE: Corrosive esophagial stricture - esophago colo plasty^-www.drkeyurbhatt.in*

14 yrs girl with accidental ingestion of corrosive poison before 3 yrs..was kept on dilatation for initial years and feeding gastrostomy... grade 4-5 dysphagial with undilatable esophagus and long stricture for last one year..

SURGERY: Esophago colo plasty (retrosternal condute, based on LCA)

dye study on POD 5 demonstrated no leak.

was started on oral liquids to semisolids..and discharged on POD 7. After one month feeding gastrostomy was removed as patient gained 2 kg wt with taking normal diet orally..and back to school on day 20.

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CASE: Infactive bowel gangrene- RA^-www.drkeyurbhatt.in*

Middle aged male with severe pain in abdomen for 2 days with nausea and vomiting

and abdominal distension.

Known DM case for last 5 yrs on OHA.

NO H/O HTN.

or surgery in past...or other medical disease.

presented with shock and hypotension not responding to fluid challenge..and started with inotrops..and low urine output

CECT: S/O intestinal obstruction...with terminal ileal ishcemia?

SURGERY: Exploratory laparotomy and resection of Gangrenous bowel. and jejuno ileal anastomosis..was done and discharged in time..without any undue complications.

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