Tuberous Sclerosis Complex Research

BACKGROUND: Interferon alfa is widely used for metastatic renal-cell carcinoma but has limited efficacy and tolerability. Temsirolimus, a specific inhibitor of the mammalian target of rapamycin kinase, may benefit patients with this disease. METHODS: In this multicenter, phase 3 trial, we randomly assigned 626 patient…

• ERHAN GÖKMEN

PURPOSE: Focal cortical dysplasias (FCD) are localized regions of malformed cerebral cortex and are very frequently associated with epilepsy in both children and adults. A broad spectrum of histopathology has been included in the diagnosis of FCD. An ILAE task force proposes an international consensus classification s…

• ÇİĞDEM ÖZKARA

PURPOSE: By incorporating major developments in genetics, ophthalmology, dermatology, and neuroimaging, to revise the diagnostic criteria for neurofibromatosis type 1 (NF1) and to establish diagnostic criteria for Legius syndrome (LGSS). METHODS: We used a multistep process, beginning with a Delphi method involving gl…

BACKGROUND: Everolimus, a mammalian target of rapamycin (mTOR) inhibitor, has been used for various benign tumours associated with tuberous sclerosis complex. We assessed the efficacy and safety of two trough exposure concentrations of everolimus, 3-7 ng/mL (low exposure) and 9-15 ng/mL (high exposure), compared with…

• ZÜHAL YAPICI OBUZ

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• ALPER SOYLU

No abstract yet.

• FATİH ÖZALTIN

Among tumors classified as pilocytic astrocytoma (PA) in the Johns Hopkins Hospital Department of Pathology files, we identified 18 cases with a distinctive monomorphous pilomyxoid histological pattern and a higher recurrence rate than that of PA with classical histological features (classical PA). The majority of the…

Summary Objective To present the baseline data of the international TuberOus SC lerosis registry to increase disease Awareness ( TOSCA ) with emphasis on the characteristics of epilepsies associated with tuberous sclerosis complex ( TSC ). Methods Retrospective and prospective patients’ data on all aspects of TSC were…

• ZÜHAL YAPICI OBUZ

BACKGROUND: Most evidence for TSC-associated neuropsychiatric disorders (TAND) to date have come from small studies and case reports, and very little is known about TAND in adults. We explored baseline TAND data from the large-scale international TOSCA natural history study to compare childhood and adult patterns, des…

• ZÜHAL YAPICI OBUZ

A unique renal neoplasm characterized by eosinophilic cytoplasm and solid and cystic growth was recently reported in patients with tuberous sclerosis complex (TSC). We searched multiple institutional archives and consult files in an attempt to identify a sporadic counterpart. We identified 16 morphologically identical…

OBJECTIVE: The purpose of this pictorial essay is to identify different types of liver lesions that contain fat. Cross-sectional imaging findings of fat- or lipid-containing lesions can help in characterizing focal liver lesions. We searched our archive retrospectively and reviewed the literature for fat-containing li…

OBJECTIVE: The purpose of this pictorial essay is to identify different types of liver lesions that contain fat. Cross-sectional imaging findings of fat- or lipid-containing lesions can help in characterizing focal liver lesions. We searched our archive retrospectively and reviewed the literature for fat-containing li…

• DEVRİM AKINCI
• MUSTAFA NASUH ÖZMEN