Pleomorphic xanthoastrocytoma in a case of tuberous sclerosis

Martin, Arvind G.; Singh, Mutum Samarendra; Idris, Badrisyah; Abdullah, Jafri Malin

doi:10.4103/0976-3147.133580

Cited by 6 publications

(1 citation statement)

References 8 publications

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“…These lesions were shown to be cases of PXT and fibrillary astrocytoma. [ 10 , 11 ] Therefore, the presence of a tumor should be taken into consideration in this case, given the progressive nature of the lesions in the MRI evaluation, although it is difficult to estimate whether the cystic mass in this case was a SEGA with atypical MRI presentation, a rare tumor associated with TSC, or a non-neoplastic lesion. Our findings imply that in patients with atypical brain MRI characteristics other than cortical or subcortical tubers, SENs or SEGA, further differential diagnosis should be considered.…”

Section: Discussionmentioning

confidence: 99%

Progressive cystic lesion in a middle-aged patient with tuberous sclerosis complex

Zhang

Jiang²,

Chen

et al. 2018

Medicine

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Rationale:Tuberous sclerosis complex (TSC) is an uncommon multiple systems disorder. The main characteristics of the disease in the central nervous system include cortical or subcortical tubers, subependymal nodules, and subependymal giant cell astrocytoma. However, progressive cystic lesions in the cerebral hemispheres have rarely been reported in previous studies of TSC.Patient concerns:We present the case of a 35-year-old man with TSC who was admitted to our hospital for a sudden attack of serious headache, vomiting, and left hemiplegia. Brain computerized tomography and magnetic resonance imaging (MRI) revealed multiple subependymal calcific nodes and multiple cystic lesions in the right frontal, temporal, and parietal lobes. The solid nodule in the lesion demonstrated contrast enhancement.Diagnoses:The patient was diagnosed with TSC, but the properties of the cystic lesion in the brain were unclear and a tumor was suspected.Interventions:Emergency operation was performed immediately. Pathological examination of the lesion revealed a vascular malformation, but no tumor cells.Outcomes:In the fourth year after the surgery, brain MRI revealed a relapse of the cystic mass and surgery was suggested again. However, the patient refused to undergo surgery again.Lessons:This case describes an atypical MRI presentation of TSC occurring in middle-age. This condition can cause a life-threatening condition and may recur after surgery. Our finding emphasizes the importance of neuroimaging surveillance in patients older than 25 years old and after lesion resection.

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Section: Discussionmentioning

confidence: 99%

Progressive cystic lesion in a middle-aged patient with tuberous sclerosis complex

Zhang

Jiang²,

Chen

et al. 2018

Medicine

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Novel characteristics of soluble fibrin: hypercoagulability and acceleration of blood sedimentation rate mediated by its generation of erythrocyte-linked fibers

et al. 2022

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Soluble fibrin (SF) in blood consists of monomers lacking both fibrinopeptides A with a minor population in multimeric clusters. It is a substantial component of isolated fibrinogen (fg), which spontaneously self-assembles into protofibrils progressing to fibers at sub-physiologic temperatures, a process enhanced by adsorption to hydrophobic and some metal surfaces. Comparisons of SF-rich (FR) and SF-depleted (FD) fg isolates disclosed distinct molecular imprints of each via an adsorption/desorption procedure using gold surfaced silica microplates. Accelerated plasminogen activator-induced lysis and decreased stiffness (G′) of thrombin-induced FR fg clots were revealed by thomboelastography. Erythrocyte sedimentation (ESR) in afibrinogenemic plasma (Hematocrit 25–33%) was accelerated by FR fg nearly threefold that of FD fg. Stained smears disclosed frequent rouleaux formations and fibers linking stacked erythrocytes in contrast to no rouleaux by FD fg. Rouleaux formations were more pronounced at 4 °C than at ambient temperatures and at fiber-membrane contacts displayed irregular, knobby membrane contours. One of several FR fg isolates also displayed incomplete fiber networks in cell-free areas. What is more, pre-mixing FR fg with each of three monoclonal IgG anti-fg antibodies at 1.5 mol/mol fg, that inhibited fibrin polymerization, prevented rouleaux formation save occasional 2–4 erythrocyte aggregates. We conclude that spontaneously generated SF fibers bound to erythrocytes forming intercellular links culminating in rouleaux formation and ensuing ESR acceleration which in clinical settings reflects hypercoagulability. Also, the results can explain the reported fg binding to erythrocytes via ligands such as CD47, stable in vivo RBC aggregates in capillaries, and red areas of pathologic thrombi.

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Giant cyst-like cortical tubers in an adult with tuberous sclerosis presenting as spastic tetraplegia

Esmat

2022

Annals of Medicine &Amp; Surgery

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Pleomorphic xanthoastrocytoma in a case of tuberous sclerosis

Abstract: Tuberous sclerosis is a known phakomatosis and the associated finding of a subependymal giant cell astrocytoma is common with this disorder. A case of tuberous sclerosis with a finding not previously reported, i.e. that of a pleomorphic xanthoastrocytoma, is presented here.

Cited by 6 publications

References 8 publications

Progressive cystic lesion in a middle-aged patient with tuberous sclerosis complex

Progressive cystic lesion in a middle-aged patient with tuberous sclerosis complex

Novel characteristics of soluble fibrin: hypercoagulability and acceleration of blood sedimentation rate mediated by its generation of erythrocyte-linked fibers

Giant cyst-like cortical tubers in an adult with tuberous sclerosis presenting as spastic tetraplegia

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