shivani malik

and 5 more

A Case Report on an Intracranial Solitary Fibrous TumorAuthors : Shivani Malik , Arun Kumar Yadav, Gaurav Dhakre , Mayank Aggarwal , Roopali  ,  Ashok Kumar AryaABSTRACTSolitary 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.INTRODUCTIONSolitary 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 REPORTA 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.