Tuesday 7 January 2014

Primary liver cancer (PLCC)

    Primary liver cancer (PLCC) 
Primary liver cancer (PLCC) is a malignanttumour arising from either the hepatocytes or from the cholangiocytes.


CASE REPORT 
Mr S. A  was a 48-year old civil servant who presented at the gastroenterology clinic of the University of Ilorin Teaching Hospital (UITH),Ilorin on the 15th of November 2002 with right hypochondrial pain and swelling and weight loss of 5 weeks duration. The pain was aching in nature, non- radiating, with no known aggravating or relieving factors. There was no vomiting or change in bowel habit, nohaematochexia or passage of malaena stool.There  was  no history of  alcohol consumption or blood transfusion. No history of multiple sexual partners however there was a positive history of scarification marks.
On examination, he was pale, afebrile,anicteric with few palpable axillary lymph nodes. His abdomen was distendedl, moved with respiration and with tenderness over the right hypochondrial and epigastric regions. He had hepatomegaly of 16cm, the consistency was hard, and with irregular surface and edge. There was hepatic bruit but no friction rub was heard. There was ascites demonstrable by shifting dullness. A digital rectal examination done was unremarkable. Other systems were essentially normal. An abdomino-pelvic ultrasound scan done showed an enlarged liver with completely distorted parenchymalechoes.  There was a focus in the left lobeanteriorly measuring 40x44mm in size with increased brightly reflective but hazy echoes. The gallbladder, biliary tracts, pancreas, spleen and kidneys were normal, no para-aortic lymph node was seen. The stomach was distended, the urinary bladder and prostate were normal.
Other investigations done were  Prothrombintime (PT) with the following results
  
Test                       18sec
Control                  12sec
Normal range         10-14sec

HBsAg  was positive and the chest x-ray was within normal limits.
Liver function tests (LFT)
ALT     13iu/L  (0-15)               Total bilirubin < 20 iu/L  (1-20)
AST      28iu/L (0-20)                Protein            67g/L      (60-80)
ALP      33iu/L (21-91)             Albumin          42g/L     ( 35-50)

Fine Needle Aspiration Cytology (FNAC) of the liver done under ultrasound guide showed many enlarged and bizarre malignant cells, some with fatty nuclear vacuolations, the nuclei were hyperchromatic and coarse. The malignant cells were seen in a background of lymphocytes, polymorphs and erythrocytes.
A diagnosis of PLCC was made. He was managed conservatively with analgesics and was followed up on out-patient basis. He was seen a week later with complains of persisting hypochondrial pain although he was stable.
He re-presented about 13weeks later with further complains of nodular  swellings on the scalp (two in number) which had developed 10days prior to presentation. At the same time he was unable to close the right eye, and there was also deviation of the mouth to the left with associated pain in the left thigh. Physical examination revealed non-tender nodular scalp swellings with a VII cranial nerve palsy of the lower motor neurone type. The liver span was found to have increased to 21cm.
A repeat liver function test done at this time showed a derangement in liver function. A biopsy of the scalp swelling was done and it revealed malignant hyperchromatic cells with prominent nuclei and were found to bemetastatic deposits from the liver. An impression of PLCC with metastases to the brain and skull causing a VII cranial nerve palsy was made.

He was managed conservatively on outpatient basis with opioid  analgesics but was subsequently lost to follow-up.




DISCUSSION

There are basically two types of primary liver cancer, one is hepatocellular carcinoma (HCC); the other composed of bile duct epithelium is cholangiocarcinoma. HCC accounts for more than 90% of all primary cancers of the liver. Virtually all the remainder are cholangiocarcinoma; the mixed pattern is not common. The liver tumour may be single or multiple, large or small. The cells and their anatomical arrangement may be well differentiated or poorly differentiated. Symptoms of PLCC include malaise, anorexia, weight loss, abdominal discomfort and fever. In a patient with pre-existing chronic liver disease, when PLCC supervenes, the presentation may initially be as deterioration in the signs and symptoms of chronic liver disease (CLD)8.
       Complications which may occur in PLCC,include ascites, which may occur at sometime in about 50% of the patients; oesophagealvarices, which may bleed; endocrine effects, which may result in gynaecomastia and hypercalcaemia1. Metastases may occur to the lungs, bone, brain and right supraclavicularlymph nodes. Direct invasion of intra-abdominal structures may occur. Others areerythrocytosisdysfibrinogenaemia and thrombosis.1
The natural course of primary liver cancer is progressive enlargement of the primary mass until it encroaches on hepatic function or it metastasizes, generally first to the lungs and then to the other sites. Overall, death usually occurs within 6 months of diagnosis from (i) malignant cachexia (ii) gastrointestinal andoesophageal variceal bleeding (iii) liver failure with hepatic coma or (iv) rupture of thetumour into the peritoneum with fatalhaemorrhage.
At autopsy, almost one-half of the cases of PLC will show metastases, most often in the portal, pancreatic and para-aortic lymph nodes. The lung is the next most common site ofmetastases , with occasional metastases to the adrenal, bone, and myocardium8.
This patient presented with righthypochondrial pain and swelling, and  weightloss. These symptoms are similar to those of patients with chronic liver disease mentioned in the literature 8. The patient also presented with signs suggestive of liver malignancy such as tender hepatomegaly  which was hard in consistency with irregular surface and edge, and an hepatic bruit. These signs are also consistent with those of liver malignancy .8 On re-presentation weeks later, the patients disease had progressed .He had developed nodular scalp swellings with deviation of his mouth to the left and also pain in the left thigh. His hepatomegaly was found to have increased in size, an indication of progression of the disease. This scenario raised the possibility of metastases to the brain and skull. Metastases of PLCC is well documented in the literature.8,9,10
A review of the literatures showed howeverthat  metastases to the bone is rare 9, and thatcutaneous and bone metastases may reveal previously undiagnosed PLCC10.
                                   
                                           
Reference 
Department of Medicine,**Department of Pathology, University of Ilorin Teaching Hospital(UITH)

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