Authors : Shivani Malik1 , Arun Kumar Yadav2, Gaurav Dhakre3 , Mayank
Aggarwal4 , Roopali5 , Ashok Kumar Arya6
1,2,5,6 - affiliated to Department of Radiation oncology, Sarojini Naidu Medical College, Agra
3,4 - affiliated to Department of Neurosurgery,
ABSTRACT
Solitary fibrous tumors (SFT) are very rare, particularly those of the
central nervous system (CNS), and they have mesenchymal origin. Here, we
present the case of a 46-year-old man who, a year after suffering a head
injury, lost consciousness for an hour. He had therapy after developing
paralysis in his left arm and left foot after three months. A month and
a half earlier, he had a bad headache, which triggered an examination.
The right temporal frontal lesion on magnetic resonance imaging seems to
be a sizable, well-defined extra-axial solid lesion with lobulated
edges, predominantly isointense to the white matter with dispersed
patches of hypointensity hole on T1WI. The lesion is isointense to the
gray matter on T2WI, with sporadic hypointensities. Along the right
anterior eminence with the bulk medially, thick, smooth dura mater is
seen. A rise in the choline peak was seen in the MRI spectra. The right
frontal lobe’s white matter showed a little hyperintensity but no signs
of angioedema-related diffusion limitation. The pathological diagnostic
of the tumor was a grade 3 solitary fibrous tumor after it was
surgically excised during a right frontal craniotomy with nearly an
entire resection. S100, CD34, STAT Positivity for 6, CK, and KI67 were
found during an immunohistochemistry study, and it was done since SFT is
challenging to differentially detect through imaging. The patient had
extra adjuvant therapy in the form of whole-brain external beam
radiation (54 Gy/30#@1.8Gy/#@5#/week) due to the tumor’s grade 3
status. Additional research and observation are needed for the
prognosis.
Keywords: solitary fibrous tumor, central nervous system.
INTRODUCTION
Solitary fibrous tumors (SFT) contribute up to less than 2% of all soft
tissue masses. They are uncommon, collagen-rich, spindle cell tumors
belonging to mesenchymal origin. It was initially described by Wagner in
1870. Pleural SFT was initially described by Klempere and Rabin in 1931
[1]. The pericardium, peritoneum, lung, liver, upper respiratory
tract, mediastinum, thyroid, parotid gland, sinuses, orbits, and
systemic circulation are among the pleural and extrapleural locations
where instances have been described. [1, 7] As there is no real
connective tissue component in the CNS, extrapleural SFTs, particularly
CNS SFTs, are uncommon. One percent of all primary CNS tumors are this
kind. [3] Cerebellopontine angle, spinal dura mater, parasagittal
region, meninges, and ventral tegmental area SFTs have all been
documented. [1] The disease often affects individuals between the
ages of 20 and 70, and both men and women have equal chances of
contracting it. [4] SFTs must be distinguished from certain cancers,
including fibrous meningiomas and hemangiopericytomas, as well as myxoid
forms, including meningiomas, myxochordoid tumors, and myxoid peripheral
nerve sheath tumors. Rare mesenchymal tumors called hemangiopericytomas
(HPC) have SFT’s clinical, radiological, and pathological
characteristics [5].
NGFI-A binding protein (NAB2) and signal transducer and activator of
transcription 6 (STAT6) gene fusions are driver mutations of SFT [1,
6]. The World Health Organization (WHO) classified SFT and HPC as a
new combined entity in 2016 [2, 7] as a result of previous pathology
results showing that these tumor types share the same genetic defects.
Three levels of SFT/HPC, specifically levels I, II, and III, are
described by this classification. It should be underlined that while
there is a clear clinical and histological overlap, the difference
between the two is no longer clinically meaningful. The term
”perivascular hemangioma” was eliminated from the 2021 WHO
classification of CNS malignancies and replaced with SFT [8].
A 46-year-old guy who was the patient in this instance had CNS SFT.
CASE REPORT
A 46-year-old male patient presented to the hospital 1.5 months ago with
a severe headache for 2 days. He had a history of trauma to the right
side of his head from 1 year ago, following which he had lost
consciousness for 1 hour. Then he recovered spontaneously. Three months
later, he suffered from hemiparesis of the left hand and left foot, for
which he had been taking treatment for six months in the form of oral
medications. He did not have any significant past illnesses or family
history. A neurological examination was normal when the patient
presented to the radiation oncology department. The patient was
investigated, and a brain MRI was performed.
The contrast-enhanced MRI brain study suggested a large, well-defined
extra-axial solid region with lobulated margins in the right
fronto-temporal region, which appeared predominantly isointense to white
matter with patchy areas of hypointensity on T1WI. On T2WI, the lesion
is isointense to gray matter with patchy areas of hypointensity. Patchy
areas of diffusion restriction noted on DWI correspond to a low value on
ADC. In the post-contrast study, the lesion shows a tense homogenous
enhancement. On MRS, an increase in choline peak is seen, and a few
small cystic areas are also seen within the lesion. Smooth dural
thickening is seen along the right frontal convexity. Medially, the
lesion is causing a mass effect, as evidenced by the buckling of the
right fronto-temporal lobes, effacement of the sulci of the right
frontal and anterior temporal lobes, the ipsilateral sylvian cistern,
the body, atria, and temporal horn of the right lateral ventricle, the
third ventricle, the ipsilateral crural cistern, the ventral midline
shift of 6.5mm to the contralateral side, and subfalcine herniation.
T2/FLAIR hyperintensity was noticed in the white matter of the right
frontal lobe, showing no diffusion restriction suggestive of vasogenic
edema. Features were suggestive of atypical meningioma.