Solitary fibrous tumor of the dura

Case contributed by Jose Luis Martinez Garza
Diagnosis certain

Presentation

Progressive holocranial headache of 1 year of evolution with late onset of stress incontinence, lower extremities weakness, and drowsiness.

Patient Data

Age: 55 years
Gender: Female

A non-contrast CT scan of the head shows a supratentorial extra-axial mass located in the anterior left parasagittal portion of the falx cerebri, with an irregular shape and ill-defined lobulated borders that are slightly hyperdense compared to adjacent brain parenchyma. It conditions mass effect over near structures and bone destruction of the inner table with demodulation of the outer table of the skull.

Enhanced brain MRI demonstrates an extra-axial mass in the anterior left parasagittal portion of the falx cerebri, with an irregular shape, well-defined lobulated borders, and no dural tail. On T1 and T2, weighted images behave with an isointense signal to grey matter, are hyperintense on FLAIR, have blooming artefacts on gradient echo, and have high cellularity confirmed with restricted diffusion on DWI and ADC maps. T1 + Gd has an avid heterogeneous enhancement with central zones of hypocaptive behavior compatible with necrosis. Lesion behavior is aggressive, with bone lysis of the frontal and anterior portions of the parietal bone of the skull. It conditions mass effect with a posterolateral displacement of adjacent brain parenchyma, the collapse of frontal horns of lateral ventricles, and subfalcine herniation on the left hemisphere. MRS demonstrated an elevation of lactate.

The patient went on to undergo surgical treatment with the following histopathological findings:

Histology

Macroscopic appearance: multiples fragments with tumoral adjacent bone tissue, dark brown alternated with clearer tones, irregular shape with a firm texture.

Microscopic description: >5 mitoses per field, multiple areas of necrosis.

Immunophenotype: SAT, EMA, AML, myogenin-positive.

Diagnosis: solitary fibrous tumor of the dura WHO Grade 3.

Case Discussion

This case represents typical findings of an intracranial solitary fibrous tumor with extensive progression. Due to the site of involvement in this case, differential diagnosis with lesions such as fibrous meningiomas may be challenging. Subtle differences such as lack of dural tail, brain edema, vascular flow void, higher ADC values, and MRS with lactate and lipids peaks should be remembered in their differentiation.

The patient was treated with surgical resection and frontal craniectomy with titanium mesh and, later on, was discharged after clinical improvement and was followed as an outpatient.

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