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    Treatment Strategies for Dopamine Agonist-Resistant and Aggressive Prolactinomas: A Comprehensive Analysis of the Literature
    (GEORG THIEME VERLAG KG, 2021-01-01) Sari, Ramazan; Altinoz, Meric A.; Ozlu, Eylem Burcu Kahraman; Sav, Aydin; Danyeli, Ayca Ersen; Baskan, Ozdil; Er, Ozlem; Elmaci, Ilhan
    Despite most of the prolactinomas can be treated with endocrine therapy and/or surgery, a significant percentage of these tumors can be resistant to endocrine treatments and/or recur with prominent invasion into the surrounding anatomical structures. Hence, clinical, pathological, and molecular definitions of aggressive prolactinomas are important to guide for classical and novel treatment modalities. In this review, we aimed to define molecular endocrinological features of dopamine agonist-resistant and aggressive prolactinomas for designing future multimodality treatments. Besides surgery, temozolomide chemotherapy and radiotherapy, peptide receptor radionuclide therapy, estrogen pathway modulators, progesterone antagonists or agonists, mTOR/akt inhibitors, pasireotide, gefitinib/lapatinib, everolimus, and metformin are tested in preclinical models, anecdotal cases, and in small case series. Moreover, chorionic gonadotropin, gonadotropin releasing hormone, TGF beta and PRDM2 may seem like possible future targets for managing aggressive prolactinomas. Lastly, we discussed our management of a unique prolactinoma case by asking which tumors' proliferative index (Ki67) increased from 5-6\% to 26\% in two subsequent surgeries performed in a 2-year period, exerted massive invasive growth, and secreted huge levels of prolactin leading up to levels of 1 605 671ng/dl in blood.
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    Detection of KIAA1549-BRAF Fusion Transcripts in Formalin-Fixed Paraffin-Embedded Pediatric Low-Grade Gliomas
    (ELSEVIER SCIENCE INC, 2011-01-01) Tian, Yongji; Rich, Benjamin E.; Vena, Natalie; Craig, Justin M.; MacConaill, Laura E.; Rajaram, Veena; Goldman, Stewart; Taha, Hala; Mahmoud, Madeha; Ozek, Memet; Sav, Aydin; Longtine, Janina A.; Lindeman, Neal I.; Garraway, Levi A.; Ligon, Azra H.; Stiles, Charles D.; Santagata, Sandra; Chan, Jennifer A.; Kieran, Mark W.; Ligon, Keith L.
    Alterations of BRAF are the most common known genetic aberrations in pediatric gliomas. They frequently are found in pilocytic astrocytomas, where genomic duplications involving BRAF and the poorly characterized gene KIAA1549 create fusion proteins with constitutive B-Raf kinase activity. BRAF V600E point mutations are less common and generally occur in nonpilocytic tumors. The development of BRAF inhibitors as drugs has created an urgent need for robust clinical assays to identify activating lesions in BRAF. KIAA1549-BRAF fusion transcripts have been detected in frozen tissue, however, methods for FFPE tissue have not been reported. We developed a panel of FFPE-compatible quantitative RT-PCR assays for the most common KIAA1549-BRAF fusion transcripts. Application of these assays to a collection of 51 low-grade pediatric gliomas showed 97\% sensitivity and 91\% specificity compared with fluorescence in situ hybridization or array comparative genomic hybridization. In parallel, we assayed samples for the presence of the BRAF V600E mutation by PCR pyrosequencing. The data further support previous observations that these two alterations of the BRAF, KIAA1549 fusions and V600E point mutations, are associated primarily with pilocytic astrocytomas and nonpilocytic gliomas, respectively. These results show that fusion transcripts and mutations can be detected reliably in standard FFPE specimens and may be useful for incorporation into future studies of pediatric gliomas in basic science or clinical trials. (J Mal Diagn 2011, 13:669-677
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    Desmoplastic infantile ganglioglioma: Report of an unusual case with a cranial defect
    (MEDKNOW PUBLICATIONS \& MEDIA PVT LTD, 2014-01-01) Basaran, Recep; Cakir, Fatma Betul; Isik, Nejat; Sav, Aydin; Elmaci, Ilhan
    Desmoplastic infantile ganglioglioma (DIG) is a rare tumor that typically occurs in infants under the age of 24 months. These tumors commonly have a good prognosis after surgical resection despite their aggressive radiological appearances. Clinical signs are due to the large size of the tumor and include increased head circumference, bulging fontanel, sunset sign and seizures. We report an unusual DIG case who presented with parietal bulging associated with a bony defect. The patient was thought to have a leptomeningeal cystic formation, but on his cranial magnetic resonance imaging (MRI), we observed a centrally and homogeneously gadolinium-enhanced lesion fixed to the dura by its solid component. A surgical gross total resection was performed, and no residual tumor was observed on follow-up.
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    Purely extradural spinal nerve root hemangioblastomas
    (MEDKNOW PUBLICATIONS \& MEDIA PVT LTD, 2016-01-01) Aytar, Murat Hamit; Yener, Ulas; Eksi, Murat Sakir; Kaya, Behram; Ozgen, Serdar; Sav, Aydin; Alanay, Ahmet
    Spinal nerve root hemangioblastomas present mostly as intradural-extradurally. Purely extradural spinal nerve root hemangioblastoma is a very rare entity. In this study, we aimed to analyze epidemiological perspectives of purely extradural spinal nerve root hemangioblastomas presented in English medical literature in addition to our own exemplary case. PubMed/MEDLINE was searched using the terms ``hemangioblastoma,{''} ``extradural,{''} ``spinal,{''} and ``nerve root.{''} Demographical variables of age, gender, concomitant presence of von Hippel-Lindau (VHL) disease
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    Monostotic fibrous dysplasia involving occipital bone: a case report and review of literature
    (AFRICAN FIELD EPIDEMIOLOGY NETWORK-AFENET, 2014-01-01) Basaran, Recep; Kaksi, Mustafa; Gur, Erdal; Efendioglu, Mustafa; Balkuv, Ece; Sav, Aydin
    Fibrous dysplasia (FD) is a progressive systemic bone tumour of young and it can be seen on cranial bones. FD is divided into three types according to radiological features. The second most common subtype is polyostotic subtype. With this article, we aimed to review and present clinical features, radiological examination, differential diagnosis and treatment management of a case of solitary monostotic fibrous dysplasia of occipital bone. 15 years old female patient admitted to our hospital for a bump and in the back of his head that she noticed 1 month ago. Her physical and neurological examination was normal. On cranial CT examination we detected a bony defect. Her gadolinium enhanced cranial MRI revealed bony defect along with massive gadolinium enhancement in adjacent tissue. On histopathologic examination
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    Does ``cerebellar liponeurocytoma'' always reflect an expected site? An unusual case with a review of the literature
    (TERMEDIA PUBLISHING HOUSE LTD, 2014-01-01) Karabagli, Pinar; Sav, Aydin; Pamir, Necmettin
    A rare tumour, cerebellar liponeurocytoma, is classified into glioneuronal tumours under the 2000 World Health Organization (WHO) classification of tumours of the central nervous system. The current 2007 WHO classification, therefore, assigns grade II to the cerebellar liponeurocytoma. Tumours are predominantly localized in cerebellar hemispheres, and the second most common location is the vermis. To date, approximately 40 reported cases of cerebellar and 10 cases of supratentorial introventricular liponeurocytoma have been reported. In this report, an unusual case of cerebellar liponeurocytoma was presented with extracerebellar location. In the future tumour classification, it should be considered that liponeurocytomas are not restricted only to the cerebellum, but they are located in supratentorial areas as well.