Serebellar Hemangioblastoma: Dört Olgu Sunumu ve Literatür Incelemesi

Hemanjiyoblastoma; histolojik kökeni henüz tam olarak tanımlanmamış, iyi huylu, yavaş büyüyen ve oldukça damarsal bir tümördür. Bu tümörler tüm kafatası içi neoplazmaların %1-2’sini oluşturur ki genellikle posterior fossada meydana gelirler. Hemanjiyoblastomalar normalde belirli aralıklarla düzensiz şekillerde meydana gelmektedirler ancak bu oran von hippel-lindau (VHL)’li vakaların yaklaşık %20-30’unda gözlenmektedirler. Burada Von Hippel-Lindau ile ilişkili olmayan dört serebellar hemanjiyoblastoma vakasının sunumu ve ilgili literatürün derlemesi yapıldı. Merkezi sinir sisteminde yer alan 4 hemanjiyoblastoma vakası hematoksilin-eosin, retikülin boya ve CD34, vimentin, NSE, S-100, CD99, CD56, GFAP, sitokeratin, epitelyal membran antijeni (EMA) ve CD10 antibiyotiklerini içeren bir panel ile incelemeleri yapıldı. Üç kadın bir erkek toplam 4 hastanın yaş ortalaması 54.75 (46 -60) idi. Hastalarda 2-3 cm çapında sistik nodüller vardı. Histopatolojik incelemeler sonucunda tümör kesitleri bizlere, büyük ve vaküollenmiş stromal Olgu Sunumu / Case Report 184 Cilt/Volume 40 Yıl/Year 2015 Cerebellar Hemangioblastoma hücreleri ve çok sayıda dallanma gösteren ve bir kıl kadar ince kan damarları olduğunu gösterdi. Tümörlerin bazıları atipikal çekirdek oluşumunu ortaya çıkardı. Vimentin tümörlerin hem stromal hücrelerinde hem de kan damarlarında yüksek seviyelerde pozitiflik gösterdi. Dört HB vakasında bazı stromal hücreler NSE ve CD99 için pozitifdi. Üç tümör S- 100 ve CD56 için pozitifti. İki tümör odaksal olarak glial fibriler asidik protein (GFAP) için pozitifti. Tümör stromal hücrelerinin negatif olmalarının aksin CD34 immün boyama, dallanan ve kompleks vasküler bağlantının varlığını gösterdi. Stromal hücreler sitokeratin, EMA ve CD10 gibi epitelyal işaretçiler için negatifti. Ki-67 indeksi tümör hücrelerinin %1’inden daha azdı. Nadir, histogenezisi tam olarak belli olmayan, iyi huylu tümör olan hemanjiyoblastoma; vakuollenmiş, lipid içeren hücreler ve iyi gelişmiş kapiller bağlantıların varlığı ile karakterize edilmektedir. HB’nin temel histolojik olarak ayrımlı teşhisi; metastatik açık hücre karsinomasıdır. İlaveten, sistik mural özelliklerinden dolayı serebellumun pilositik astrositomları hemanjiyoblastomlardan ayrılması gerekmektedir

Cerebellar Hemangioblastoma: Four Case Reports and Review of the Literature

Hemangioblastoma (HB) is a benign, slow-growing, highly vascular tumour of not well defined histological origin. These tumors make up about 1 to 2 percent of all intracranial neoplasms and occur primarily in the posterior fossa. Hemangioblastomas can occur sporadically but in about 20% to 30% cases, it is associated with von Hippel-Lindau (VHL) disease. Four cases of cerebellar haemangioblastoma, not associated with von Hippel-Lindau disease (sporadic haemangioblastomas), were presented and reviewed the relevant literature.Four hemangioblastomas of the central nervous system were examined with haematoxylin & eosin (H&E), reticulin stain and with a panel of antibodies including CD34, vimentin, NSE, S-100, CD99, CD56, GFAP, cytoceratin, epithelial membrane antigen (EMA), CD10. Of the 4 patients in this study 1 was male and 3 were female. Their ages ranged from 46 years to 60 years with a mean age of 54.75 years. All of them were as cystic nodules about 2-3 cm in diameter. In the histopathological examination, the tumors sections showed large and vacuolated stromal cells and numerous arborizing capillary-size blood vessels. Some tumors showed atypical nuclei. Vimentin was strongly positive both stromal cells and blood veessels in all tumors. In 4 cases of HB, some stromal cells were positive for NSE and CD99. Three tumors were positive for S-100 and CD56, two tumors were focally positive for glial fibrillary acidic protein (GFAP). CD34 immunostaining highlighted the arborizing and complex vascular network, whereas the tumor stromal cells were negative. The stromal cells were negative for epithelial markers such as cytokeratin, EMA and CD10. Ki-67 index was less than 1% of the tumor cells. Hemangioblastoma, a rare, benign tumors of uncertain histogenesis, is characterized histologically by the presence of vacuolated, lipid containing cells and a well developed, fine capillary network. The main histological differential diagnosis of HB is metastatic clear cell carcinoma. Additionally, because of the cystic mural features, pilocytic astrocytomas of the cerebellum must be separated from haemangioblastomas

Keywords:

hemangioblastoma, von Hippel-Lindau cantral nervous system, histopathology,

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