Olfaktör Nöroblastom: Olgu Sunumu

Year 2025, Volume: 4 Issue: 1, 22 - 27, 15.03.2025

Çayan Can Ergün , Sümeyye Ekmekci , Yeliz Pekçevik , Ülkü Küçük

https://doi.org/10.57221/izmirtip.1510203

Abstract

AMAÇ: Olfaktör nöroblastom (ONB) oldukça nadir izlenen tümörler olup sinonazal malign tümörlerin yaklaşık % 2’sini oluşturur. Burada nazal kavite yerleşimli ONB tanısı almış iki olgu klinik, radyolojik ve histopatolojik özellikleri ile sunulmuştur.
OLGU: Olgu 1: 33 yaşında erkek olgu, burun akıntısı ve burun tıkanıklığı şikayetleri ile hastanemize başvurdu. Muayenesinde sol nasal pasajda, hiperemik polipoid kitlesel lezyon görüldü. Paranazal sinüs bilgisayarlı tomografi (BT) incelemesinde; sol nazal kavitede, orta meatus yerleşimli, 25x10x25 mm boyutta, anteriorda vestibüle, süperiorda etmoid sinüse, medialde maksiler sinüs ostiumuna uzanan, yüksek dansiteli, polipoid kitlesel lezyon izlendi. Lezyondan alınan biyopsi materyalinin histopatolojik incelemesinde, tümörün küçük yuvarlak nükleuslu, dar sitoplazmalı hücrelerden oluştuğu ve zeminde yer yer fibriller bir matriksin bulunduğu görüldü. Tümör hücrelerinin, fokal odaklarda, lümen oluşturmayan rozet benzeri yapılanmalar gösterdiği dikkati çekti. Nükleer pleomorfizm ve nekroz görülmedi. Tümör hücreleri, immünohistokimyasal (İHK) olarak sinaptofizin, kromogranin ve CD56 ile diffüz pozitif reaksiyon gösterdi. S100 ile tümör adaları çevresindeki sustentaküler hücrelerde pozitivite saptandı. 10 büyük büyütme alanında 2 mitoz görüldü, Ki67 proliferasyon indeksi %2 idi. Olgu “ONB, Hyams derece II” olarak rapor edildi.
Olgu 2: 56 yaşında kadın olgu, burun kanaması şikayeti ile hastanemize başvurdu. Muayenesinde, sol nazal kavitede hiperemik polipoid lezyon tespit edildi. Kontrastlı BT’de sol nazal kaviteyi donduran, anteriorda vestibül ve posteriorda nazofarinkse uzanan hipervasküler kitlesel lezyon izlendi. Tanı için lezyondan alınan biyopside histopatolojik ve İHK özellikleri ilk olguya benzeyen tümör izlendi. Tümörde pleomorfizm, nekroz ve mitoz görülmedi. Ki67 proliferasyon indeksi %1-2 idi. Olgu “ONB, Hyams derece 1” olarak rapor edildi.
SONUÇ: Olfaktör nöroblastom, baş boyun bölgesinde oldukça nadir izlenen, prevalansı 4/10.000, geniş bir ayırıcı tanı yelpazesine sahip malign yuvarlak hücreli tümörlerdir. Kesin tanı için histopatolojik incelemeye ek yapılacak geniş İHK panel altın standarttır.

Keywords

Olfaktör Nöroblastom, Sinonasal bölge, Malign yuvarlak hücreli tümör

Olfactory Neuroblastoma; A Case Report

Abstract

Aim: Olfactory neuroblastoma (ONB) is a very rare tumor and constitutes approximately 2% of sinonasal malignant tumors. Here, two cases diagnosed with ONB localized in the nasal cavity are presented with their clinical, radiological and histopathological features.

Case: Case 1: A 33-year-old male patient was admitted to our hospital with complaints of nasal discharge and nasal congestion. On examination, a hyperemic polypoid mass lesion was observed in the left nasal passage. Paranasal sinus computed tomography (CT) examination revealed a high-density polypoid mass lesion located in the middle meatus in the left nasal cavity, measuring 25x10x25 mm in size, extending anteriorly to the vestibule, superiorly to the ethmoid sinus, and medially to the maxillary sinus ostium. Histopathological examination of the biopsy material taken from the lesion showed that the tumor consisted of cells with small round nuclei and narrow cytoplasm, and there was a fibrillar matrix in the background. It was noted that the tumor cells showed rosette-like structures in focal foci that did not form a lumen. No nuclear pleomorphism or necrosis was observed. The tumor cells showed diffuse positive reactions with synaptophysin, chromogranin and CD56 immunohistochemically (IHC). Positivity was detected in the sustentacular cells around the tumor islands with S100. Two mitoses were seen in 10 high-power fields, and the Ki67 proliferation index was 2%. The case was reported as “ONB, Hyams grade II”.
Case 2: A 56-year-old female patient applied to our hospital with a complaint of nosebleeds. During examination, a hyperemic polypoid lesion was detected in the left nasal cavity. Contrast-enhanced CT showed a hypervascular mass lesion that froze the left nasal cavity, extending anteriorly to the vestibule and posteriorly to the nasopharynx. In the biopsy taken from the lesion for diagnosis, a tumor with histopathological and IHC features similar to the first case was observed. Pleomorphism, necrosis and mitosis were not observed in the tumor. Ki67 proliferation index was 1-2%. The case was reported as “ONB, Hyams grade 1”.
CONCLUSION: Olfactory neuroblastoma is a malignant round cell tumor that is very rare in the head and neck region, with a prevalence of 4/10,000, and has a wide range of differential diagnoses. A broad IHC panel to be added to histopathological examination is the gold standard for definitive diagnosis.

Keywords

Olfactory Neuroblastoma, Sinonasal region, Malignant round cell tumor

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