Inflammatory myofibroblastic tumor of the liver: A case report and review of literature

Abstract

Background: Inflammatory myofibroblastic tumors of the liver (IMTL) are extremely rare neoplasms and very little is known about their clinical presentation, pathogenesis, and biological behavior. Due to their absolute rarity, it is almost impossible to obtain a definite diagnosis without histological examination. Because of their intermediate biological behavior with the risk for local recurrence and metastases, surgical resection is recommend whenever IMTL is suspect.

Case summary: We herein present a case of an otherwise healthy 32-year-old woman who presented with intermittent fever, unclear anemia, malaise and right flank pain 4 mo postpartum. The liver mass in segment IVa/b was highly FDG avid in the positron emission tomography-computed tomography. Hepatic resection was performed achieving a negative resection margin and an immediate resolution of all clinical symptoms. Histological analysis diagnosed the rare finding of an inflammatory myofibroblastic tumor of the liver and revealed cytoplasmic anaplastic lymphoma kinase expression by immunohistochemistry. Twelve months follow-up magnetic resonance imaging showed no recurrence and no metastases in the fully recovered patient.

Conclusion: IMTLs are extremely rare and difficult to diagnose. Due to their intermediate biological behavior, surgical resection should be perform whenever feasible and patients should be followed-up in order to detect recurrence and metastasis as early as possible.

Keywords: Anaplastic lymphoma kinase-expression; Case report; Hepatic; Inflammatory; Inflammatory myofibroblastic tumor; Review.

Figure 1

Imaging features within the liver lesion in segment IV. A: The lesion was a first detected as an incidental finding in an unenhanced abdominal computed tomography to rule out kidney stones (asterisk); B: Conformed with an ultrasound examination (asterisk); C: In a following magnetic resonance imaging the lesion showed a homogeneous high signal in T2-weighted imaging (asterisk); D: After the application of intravenous hepatocyte specific contrast medium (gadoxetic acid, Primovist^®/Eovist^®, Bayer Healthcare Pharmaceuticals, Leverkusen, Germany) there was an early enhancement at the rim in the arterial phase (arrow); E: Followed by a strong enhancement in the venous phase (arrow); F: In the hepatobiliary phase after 20 min, the lesion appeared with a low intracellular uptake of the contrast medium compared with the adjacent liver tissue; G: In the diffusion-weighted imaging there was no clear diffusion restriction detection within the lesion (apparent diffusion coefficient); H: In an additional positron emission tomography-computed tomography examination the lesion showed an intensively increased tracer uptake; I: A follow-up magnetic resonance imaging examination after 3 mo confirmed a complete surgical resection (with multiple artifacts at the resection margin due to multiple clips) and ruled out new hepatic lesions.

Figure 2

Postoperative macroscopic pathology of the inflammatory myofibroblastic tumors.

Figure 3

Postoperative microscopic pathology of the inflammatory myofibroblastic tumors. A: Well demarcated firm vascularized tumor mass with spotty inflammatory infiltrate; B: Bland proliferation of spindle cells in broad fascicles at higher magnification. Scattered lymphocytes and plasma cell; C: Intense positivity of the spindel cells for anaplastic lymphoma kinase.