Fibrolamellar carcinoma of the liver--an unusual presentation.P Vaideeswar, MJ Pandit, JR Deshpande, A Sivaraman, IM Vora
Dept. of Pathology, Seth GS Medical College, Parel, Bombay, Maharashtra.
Non-bacterial thrombotic endocarditis was found at autopsy in a 17 year old male patient of fibrolamellar type of hepatocellular carcinoma with pericardial metastases. This had resulted in multiple embolic cerebral infarcts with long standing hemiplegia and later death due to acute left ventricular failure.
Keywords: Adolescent, Carcinoma, Hepatocellular, pathology,secondary,Case Report, Cerebral Infarction, pathology,Endocarditis, pathology,Endocardium, pathology,Heart Neoplasms, pathology,Human, Liver, pathology,Liver Neoplasms, pathology,Male, Mediastinal Neoplasms, pathology,secondary,Pericardium, pathology,Thrombosis, pathology,
Fibrolamellar carcinoma (FLC) is a variant of hepatocellular carcinoma (HCC) occurring in young individuals especially females. As compared to the usual type of HCC, it has a better prognosis.
Non-bacterial thrombotic endocarditis (NBTE) has been considered as an important cardiac valvular lesion capable of producing systemic embolization and occurs in association with a variety of malignant and non-neoplastic diseases,,,.
So far there have been no reports of NBTE in patients with FLC of liver. We are reporting a case of FLC associated with NBTE in a young hemiplegic.
A 17-year-old male with right-sided hemiplegia of 18 months duration presented with two days' history of abdominal pain followed by acute onset of breathlessness. The clinical impression was hemiplegia of unknown setiology with left ventricular failure and a hard irregular hepatomegaly. The patient died before any investigations could be performed.
On autopsy, the liver was found to be enlarged weighing 2.1 kg. The right lobe of the liver showed a large 10 x 8 cm well circumscribed mass with homogenous, grey white lobulated cut surface [Figure - 1]. Surrounding hepatic parenchyma was normal.
Microscopically, the tumour showed multiple nodules separated by broad interlacing band of fibrocollagenous tissue. The cells were large, polygonal with abundant granular eosinophilic cytoplasm and large hyperchromatic vesicular nuclei [Figure - 2]. Few of the cells showed ground glass cytoplasm. Staining for hepatitis B surface antigen (orcein stain) was negative.
There were two pericardial nodules, the gross and histologic features of which were similar to the primary liver tumour. Apart from the pericardial metastatic nodules, there was no evidence of any lymph node or organ metastasis.
The heart was minimally enlarged and weighed 250 gm. Both mitral and aortic valves showed the presence of large, bulky, firm and grey white vegetations [Figure - 3], producing marked narrowing of the orifices. The vegetations were composed mainly of fibrin and platelets along with few entrapped large cells resembling hepatic tumour cells [Figure - 4].
The brain showed multiple healed cystic infarcts involving the caudate nucleus, puamen, part of the insular cortex and occipital cortex in the left cerebral hemisphere. A diagnosis of fibrolamellar carcinoma of the liver and pericardial metastasis with associated non-bacterial thombotic endocarditis and multiple cystic cerebral infarcts was made.
Fibrolamellar carcinoma of the liver is an unusual variant of hepatocellular carcinoma occurring in young individuals. It is a slow growing tumour with a high resectability rate and lack of association with cirrhosis or any other underlying liver abnormality. The associated symptoms such as pain, malaise, hepatomegaly or epigastric mass are of a longer duration.
NBTE as defined by Rosen and Armstrong is the bland deposition of platelets and fibrin in one or more than one non-inflammed non-ulcerated valve cusp. It is most frequently reported with mucin secreting adenocarcinoma of the gastrointestinal tract and lung carcinoma and is attributed to an underlying hypercoaguable state. Its occurrence in malignant hepatic neoplasms is uncommon, though, in a series reported by Dicken and Chan, there was an increased incidence of NBTE in hepatic cholangiocarcinoma.
NBTE can complicate the natural history of cancer by producing vascular insufficiency and organ infarction and in some patients neurologic deficit is the only clinical evidence that the patient has NBTE, possibly due to an occult carcinoma.
In the present case, the patient had a long standing hemiplegia (18 months) with multiple old cystic cerebral infarcts. The underlying coagulopathy was induced by occult FLC, much before the appearance of clinical signs and symptoms related to heart and tumour. Later, the patient had abdominal and cardiac symptoms, produced by narrowing of mitral and aortic orifices by bulky vegetations.
The vegetations on the aortic valve showed mainly fibrin with few large cells similar to the hepatic tumour cells. Though Silver mentions this as rare possibility, it has not been reported in larger series of cases of NBTE with malignancies.
Distant metastases are uncommon in FLC. In our case, apart from the two pericardial metastatic nodules, there was no macroscopic or microscopic evidence of tumour elsewhere.
We are grateful to the Dean, Seth GS Medical College and King Edward Memorial Hospital, Parel, Mumbai for granting permission to publish this article.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]