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Presented by Ashley Cimino-Mathews, M.D. and prepared by Mohammed Lilo, M.D.
Case 1: A 15 year-old female with lymphadenopathy.
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Question 1 of 1
1. Question
Week 584: Case 1
A 15 year-old female with lymphadenopathyimages/lilo/Cim-1-A.jpg
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images/lilo/Cim-1-E.jpgCorrect
Answer: Metastatic hepatocellular carcinoma
Histology: The lymph node is largely replaced by nests and cords of large, atypical polygonal cells characterized by abundant eospinophilic and granular cytoplasm, central nuclei, and prominent cherry red nucleoli. The cells display occasional binucleation and intranuclear inclusions. Scattered nuclei are markedly enlarged with irregular nuclear contours and vesicular chromatin. The cells contain both intracytoplasmic and intraluminal yellow-pigmented secretions (bile pigment), as well as occasional large, pale pink-grey intracytoplasmic inclusions (“pale bodies”) that displace the nuclei. There are scattered mitotic figures and no necrosis.
Discussion: This patient had a history of fibrolamellar hepatocellular carcinoma (HCC), and this lesion represents a metastasis. Fibrolamellar represents a unique variant of HCC that typically occurs in younger patients without the concomitant presence of hepatic cirrhosis. Although fibrolamellar HCC occurs in younger patients including children and adolescents, it is not actually the most common type of HCC in children; the conventional (or typical) type of HCC is still the most common type of HCC seen in this population. Additionally, there has been great debate over the prognosis of fibrolamellar HCC. While it’s commonly reproted to have a better prognosis than the typical HCC, this better prognosis appears to be related to the lack of cirrhosis and thus lack of cirrhosis-associated morbidities rather than better biologic behavior of the tumor per se. Several studies in the pediatric population have shown that pediatric patients with fibrolamellar HCC do not have better survival than those with typical HCC.
Histologically, primary fibrolamellar HCCs are characterized by these large polygonal cells with prominent nucleoli and bands of fibrosis. About 50% of fibrolamellar HCCs also contain intracytoplasmic pale bodies, and some cases display pink hyaline inclusions. The presence of yellow bile pigment in this lesion aids in the histologic recognition of this tumor as HCC. The differential diagnosis of HCC in the metastatic setting includes other “pink tumors” such as adrenocortical carcinoma, melanoma, hepatoid adenocarcinomas, and any oncocytic neoplasm such as renal cell carcinoma or Hurthle cell carcinomas, etc. Certainly, clinical history and immunohistochemistry can aid in the differential.
Reference(s):
– Torbenson M. Review of the clinicopathologic features of fibrolamellar carcinoma. Adv Anat Pathol. 2007 May;14(3):217-23.
– Katzenstein HM, Krailo MD, Malogolowkin MH, et al. Fibrolamellar hepatocellular carcinoma in children and adolescents. Cancer. 2003 Apr 15;97(8):2006-12.
– Weeda VB, Murawski M, McCabe AJ, et al. Fibrolamellar variant of hepatocellular carcinoma does not have a better survival than conventional hepatocellular carcinoma–results and treatment recommendations from the Childhood Liver Tumour Strategy Group (SIOPEL) experience. Eur J Cancer. 2013 Aug;49(12):2698-704.Incorrect
Answer: Metastatic hepatocellular carcinoma
Histology: The lymph node is largely replaced by nests and cords of large, atypical polygonal cells characterized by abundant eospinophilic and granular cytoplasm, central nuclei, and prominent cherry red nucleoli. The cells display occasional binucleation and intranuclear inclusions. Scattered nuclei are markedly enlarged with irregular nuclear contours and vesicular chromatin. The cells contain both intracytoplasmic and intraluminal yellow-pigmented secretions (bile pigment), as well as occasional large, pale pink-grey intracytoplasmic inclusions (“pale bodies”) that displace the nuclei. There are scattered mitotic figures and no necrosis.
Discussion: This patient had a history of fibrolamellar hepatocellular carcinoma (HCC), and this lesion represents a metastasis. Fibrolamellar represents a unique variant of HCC that typically occurs in younger patients without the concomitant presence of hepatic cirrhosis. Although fibrolamellar HCC occurs in younger patients including children and adolescents, it is not actually the most common type of HCC in children; the conventional (or typical) type of HCC is still the most common type of HCC seen in this population. Additionally, there has been great debate over the prognosis of fibrolamellar HCC. While it’s commonly reproted to have a better prognosis than the typical HCC, this better prognosis appears to be related to the lack of cirrhosis and thus lack of cirrhosis-associated morbidities rather than better biologic behavior of the tumor per se. Several studies in the pediatric population have shown that pediatric patients with fibrolamellar HCC do not have better survival than those with typical HCC.
Histologically, primary fibrolamellar HCCs are characterized by these large polygonal cells with prominent nucleoli and bands of fibrosis. About 50% of fibrolamellar HCCs also contain intracytoplasmic pale bodies, and some cases display pink hyaline inclusions. The presence of yellow bile pigment in this lesion aids in the histologic recognition of this tumor as HCC. The differential diagnosis of HCC in the metastatic setting includes other “pink tumors” such as adrenocortical carcinoma, melanoma, hepatoid adenocarcinomas, and any oncocytic neoplasm such as renal cell carcinoma or Hurthle cell carcinomas, etc. Certainly, clinical history and immunohistochemistry can aid in the differential.
Reference(s):
– Torbenson M. Review of the clinicopathologic features of fibrolamellar carcinoma. Adv Anat Pathol. 2007 May;14(3):217-23.
– Katzenstein HM, Krailo MD, Malogolowkin MH, et al. Fibrolamellar hepatocellular carcinoma in children and adolescents. Cancer. 2003 Apr 15;97(8):2006-12.
– Weeda VB, Murawski M, McCabe AJ, et al. Fibrolamellar variant of hepatocellular carcinoma does not have a better survival than conventional hepatocellular carcinoma–results and treatment recommendations from the Childhood Liver Tumour Strategy Group (SIOPEL) experience. Eur J Cancer. 2013 Aug;49(12):2698-704.