solitärer fibröser Tumor der Leber

Hepatic solitary fibrous tumors (hepatic SFTs) are rare mesenchymal tumors.

Epidemiology

Fewer than 60 cases have been reported in the literature. Female predominance (~70%). There is a mean age of 52 years at presentation, but a wide range of incidence (16-84 years).

Clinical presentation

If the patient is symptomatic at all, the presentation is usually mild and nonspecific, including abdominal pain, bloating, dyspepsia.

Hypoglycemia has been reported in some patients, presumably a paraneoplastic syndrome.

Pathology

Microscopic appearance

These tumors demonstrate high cellular proliferation of spindle cells arranged in a storiform pattern.

Immunophenotype
  • CD34: positive
  • vimentin: positive
  • Bcl-2: positive
  • SM-actin: negative

Radiographic features

A prospective diagnosis of hepatic SFT is not usually possible because imaging features are usually relatively nonspecific. Intratumoral necrosis has been reported in a few lesions. Benign tumors cannot currently be reliably be differentiated from malignant tumors.

CT
  • nonspecific enhancing mass
    • heterogeneous enhancement has been reported
MRI
  • T1: hypointense
  • T2: heterogeneous appearance
  • T1 C+ (Gd): heterogeneous enhancement, depending on the relative amounts of cellular and collagen components
Nuclear Medicine
  • FDG-PET: avid FDG uptake

Treatment and prognosis

Most recorded hepatic SFTs are benign, but it is possible for them to exhibit malignant behavior . Some think that malignant foci may exist in biopsy-benign tumors, and the risk of malignancy is estimated at ~10% .

Given the rarity of the tumor, no standard treatment exists. Partial hepatectomy has been performed for some lesions, given the concern for occult malignancy.

Differential diagnosis

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