SESSION TITLE: Cancer Cases
SESSION TYPE: Case Reports
PRESENTED ON: Sunday, March 23, 2014 at 09:00 AM - 10:00 AM
INTRODUCTION: Carcinoma with hepatoid features is a subtype of hepatoid adenocarcinoma (HAC), a rare, aggressive extrahepatic tumor morphologically mimicking classical hepatocellular carcinoma (HCC) but with different immunohistochemical features. The first HAC was described in 1985 as an alpha-fetoprotein (AFP) producing gastric carcinoma with features of hepatic differentiation (1). We present a case of a primary lung carcinoma with hepatoid features, the first reported without accompanying elevation in AFP.
CASE PRESENTATION: A 54 year-old male with polysubstance abuse presented with cough, shortness of breath, and hemoptysis for one month with a 35 pound weight loss. A chest CT revealed a 4x5cm right paratracheal mass with heterogeneous contrast enhancement. The mass was biopsied via bronchoscopy with a histopathological diagnosis of carcinoma with hepatoid features. The tumor was positive for Hep-Par, CK7 and had diffuse cytoplasmic positivity for CEA. In HCC, Hep-Par, CK7, and CEA staining would be positive, negative, and have a canalicular pattern, respectively. Further studies showed no molecular rearrangements and a serum AFP of 2 (normal). A brain MRI showed metastatic disease but a CT abdomen/pelvis was negative for disease. The patient was diagnosed with Stage IV Carcinoma with Hepatoid Features likely of lung primary based on clinical-radiographic correlation.
DISCUSSION: Hepatoid adenocarcinoma has been described as originating from multiple organs, including stomach (63%), ovaries (10%), lung (5%), and gallbladder, pancreas and uterus (all 4%). HAC often present in advanced stages as they grow in nodular, expansive fashion and have a proclivity to invade vessels. Production of AFP is one of the most characteristic phenotypes of HACs, though HAC without AFP production has also been reported. In a review of the 261 known cases of HAC originating from any organ system, the most striking finding in most cases was the ‘hepatoid’ appearance of the tumor tissue followed by the highly increased AFP levels (all tumors from the lung had grossly elevated AFP levels) (2). HAC, irrespective of AFP production, has a poorer prognosis than AFP-producing carcinomas without hepatoid features. Also, non-lung HACs that produce AFP are not associated with a significant increase in patient morbidity compared to non-lung HACs that do not produce AFP (3).
CONCLUSIONS: This case highlights a rare tumor with an uncommon phenotypic pattern, which to our knowledge is the first of its type to be reported in the literature. The rarity of the tumor and uncommon laboratory presentation makes this a difficult diagnosis.
Reference #1: Ishikura et al. An AFP-producing gastric carcinoma with features of hepatic differentiation. Cancer. 1985;56(4):840-8.
Reference #2: Metzgeroth et al. Hepatoid Adenocarcinoma - Review of the Literature Illustrated by a Rare Case Originating in the Peritoneal Cavity. Onkologie. 2010;33:263-9.
Reference #3: Nagai et al. Hepatoid Adenocarcinoma of the Stomach. Cancer. 1993;72(6):1827-35.
DISCLOSURE: The following authors have nothing to disclose: Kate Grossman, Mary Beth Beasley, Sidney Braman
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