Pediatric Inflammatory Myofibroblastic Tumor of the Liver: A Rare Cause of Portal Hypertension

Abstract

Hepatic inflammatory pseudotumors or myofibroblastic tumors are benign neoplasms rarely seen in children. We report a case of a previously healthy 10-year-old girl with prolonged fever and abdominal pain who was found to have hepatosplenomegaly and pancytopenia. Imaging revealed a periportal mass along with thrombosis of portal vein and splenomegaly. Liver biopsy showed normal hepatic architecture with no evidence of cirrhosis. She underwent endoscopic banding of esophageal varices. Biopsy of the mass was suggestive of inflammatory myofibroblastic tumor without malignant changes. She has been successfully managed with nonsteroidal anti-inflammatory drug and pulse steroids with resolution of symptoms and decrease in size of the tumor with more than 2 years of follow-up.

Hepatic Inflammatory Myofibroblastic Tumor Detected in the Fetal Period That Caused an Oncologic Emergency

“…The etiology of IMT is not clearly understood, but it is possible that it is caused by inflammatory reactions such as infection, trauma, surgery, autoimmune diseases, vascular lesions, or chronic inflammation [ 4 ]. In particular, the inflammatory reaction in the digestive tract is reported to spread to the hepatic parenchyma to develop hepatic IMT [ 5 , 6 ].…”

“…The prognosis of hepatic IMT is generally good, since there have been reports of spontaneous regression [ 13 ] and improvement with nonsteroidal anti-inflammatory drugs or steroids for conservative treatment. Surgical resection is indicated when there are obstructions or exclusion symptoms that cause cholestasis or portal hypertension, or when the tumor does not shrink with conservative treatment [ 5 ]. In the current case, the pathological findings showed proliferation of spindle-shaped cells and infiltration of inflammatory cells such as plasma cells and lymphocytes, and, moreover, immunostaining analysis was positive for αSMA, muscle-specific actin, desmin and vimentin, and negative for anaplastic lymphoma kinase, S-100 and myogenin, which yielded the diagnosis of IMT.…”

Pancreatic inflammatory myofibroblastic of the pancreatic neck: a case report and literature review

“…In ammatory myo broblastic tumor(IMT)is an uncommon mesenchymal tumor of unknown pathogenesis that is malignant and aggressive potential, with a global incidence of less than 1% 1,2 . IMT most commonly occurs in children's or young adults' lungs, followed by the head and neck 3 , liver 4 , pancreas 5 , genitourinary tract 6 and thyroid 7 . The clinical presentation caused by pancreatic IMT varies depending on its anatomic location and the nal diagnosis of most lesions requires pathological examination.…”

Liver inflammatory pseudotumor in children: A case report

“…IPTs are benign neoplasms of uncertain etiology, affecting children as well as young adults. 1,2 IPTs of the liver continue to be rare entities, with the gross appearance of a malignant tumor. However, they have a benign course and histological appearance.…”

“…However, they have a benign course and histological appearance. 1,2 The major differential diagnoses of hepatic IPTs include lymphomas, hepatic abscesses, and other malignancies such as hepatocellular carcinoma and metastatic disease. Hepatic IPTs typically present with constitutional symptoms and abdominal pain; however, they may also be fully asymptomatic.…”