Rare Diseases Symptoms Automatic Extraction

A retrospective and comparative study of inflammatory myofibroblastic tumor of the liver.

[cholangiocarcinoma]

Inflammatory myofibroblastic tumor of the liver (IMTL) is a very rare benign disease with a good prognosis.To determine the clinical, radiological, and pathological characteristics of IMTL. The diagnosis and treatment strategies were discussed.A total of eleven patients with pathologically confirmed IMTL receiving treatment over a 15-year period were reviewed retrospectively. The analysis included demographics information and pertinent clinical data. Results were compared that obtained from patients with hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (IHCC) and, metastatic liver cancer (MLC) receiving surgical resection.In comparison to HCC, IHCC, and MLC, IMTL has an earlier onset (P<0.001). IMTL patients had significantly lower AST (P=0.003) and higher ALP (P=0.034) than HCC patients, and higher GGT (P=0.010) than MLC patients. Increased serum alpha-fetoprotein (AFP) level was detected in only one patient. Serum AFP was significantly lower in patients with IMTL (P=0.000) than in those with HCC but not IHCC (P=0.558) or MLC (P=0.514). In contrast to elevated serum CA19-9 in patients with HCC/IHCC/MLC, the serum CA19-9 in IMTL cases was generally normal (vs. HCC P=0.008; vs. IHCC P=0.000; vs. MLC P=0.022). In 9 IMTL patients, the tumor appeared as a hypoechogenic solid mass on the ultrasonography. In contrast, most patients with HCC, IHCC, or MLC showed hybrid echo. In contrast CT and MRI, the lesion of IMTL and MLC appeared as peripheral enhancement.Lab tests, imaging features, and patient history are helpful in differential diagnosis of IMTL from HCC/IHCC/MLC. Surgical resection is curative for IMTL.