What is Primary Hepatic Leiomyosarcoma?Primary hepatic leiomyosarcoma is a rare type of cancer that arises from the smooth muscle cells of the liver, smooth muscle cells of liver blood vessels, smooth muscle cells of the bile ducts, and ligaments. It is usually accompanied by fever. Smooth muscles are found in the lining of various organs of the body, such as the stomach, bladder, uterus, blood vessels, liver, pancreas, and intestines. The mean age at the development or diagnosis of the disease is 58 years. It is equally common in both males and males. The prognosis of primary hepatic leiomyosarcoma is poor, but the results can be improved with the combined use of surgical removal of the tumour and chemotherapy.
Disease CausesThere is no exact well-known cause is found. Certain genetic mutations are considered responsible for the development of primary hepatic leiomyosarcoma. Present or previous viral hepatitis B infection, having HIV/AIDS, Hodgkin’s lymphoma, autosomal dominant polycystic disease, kidney transplantation, previous exposure to radiation therapy, or exposure to certain chemicals or drugs like dioxins, vinyl chloride, and pesticides are risk factors for the development of the tumour.
Disease EpidemiologyIt is the second most common cancer of the abdomen and accounts for less than 1% of liver cancer.
Signs and SymptomsThe patient may suffer from the following signs and symptoms. These are
- Persistent fever
- Dull pain in the upper part of the right side of the liver
- Mass or cyst in the upper right side of the abdomen
- Loss of appetite
- Constant feeling of tiredness
- Unintentional weight loss
- Jaundice (yellowish discolouration of the skin and eyes)
- Yellowish colour of the urine
- Pain in the back
DiagnosisThe following investigations help diagnose the disease and its extent. These are
- Complete blood count ( CBC)
- Blood coagulation profile (for example partial thromboplastin time PTT, international normalized ratio INR)
- Serum electrolyte levels
- Liver function tests (LFTs)
- Abdominal USG, CT scan, and MRI
- Immunohistochemical staining
TreatmentThe selection of suitable treatment depends upon multiple factors like the size, number, and location of the tumour. Other factors are the overall performance of the liver or liver function, the presence or absence of the spread of the tumour to other parts of the body, the presence or absence of Liver cirrhosis, its extent, and the risk involved in the case of surgery due to extent of the cirrhosis. On behalf of the above-mentioned factors there are the following treatment options;
- Resection; surgical removal of the tumour along with some healthy tissues from the surroundings of the tumour to stop the spread of cancer in the early stage of cancer.
- Radiofrequency Ablation; is a non-surgical technique to kill and stop the spread of cancer cells in cases where surgery is not a suitable option.
- Targeted immunotherapy
- Liver Transplantation: it is a surgical procedure through which a diseased liver is replaced by a healthy liver from a donor.