Sadaf HAİYAT, Kafil AKHTAR, Durre ADEN, Mohammed NAİM

Tekrarlayan unilateral gonadoblastom: Nadir bir histopatolojik sunum

Gonadoblastom, malign transformasyon potansiyeli olan ve cinsel gelişim bozukluğu olan hastaları etkileyen nadir bir benign tümördür. Gonadoblastomun kesin prevalansı bilinmemektedir. Gonadoblastomaların dysgerminoma ile ilişkisi olguların %50-60'ında ve vakaların %10'unda yolk kesesi tümörü, embriyonal karsinom ve koriokarsinom gibi diğer malign germ hücreli tümörlerde görülür. Bu yazıda, doğumdan beri idrarın anormal geçişi ile başvuran 10 yaşındaki bir Hintli erkeğin nadir bir tekrarlayan tek taraflı gonadoblastoma vakasını sunduk. Fizik muayenede inmemiş sağ testis ve belirsiz dış genital bölgede fenotipik bir erkek vardı. Bilgisayarlı tomografi taraması, pelviste periferik olarak gelişen katı bileşen ve birkaç noktalama kalsifikasyon odakları ile karışık yoğunluktaki katı kistik kitleyi bildirdi. Histo-patolojik inceleme, yumurta hücresi tümör bileşeniyle birlikte malign seminomatöz, sertoli ve leydig hücresi tümörünü içeren heterojen bir tümör gösterdi. Serum alfa fetoprotein 1210 ng/ml düzeyine çıktı.

Anahtar Kelimeler:

Gonadoblastoma, Seminom, Yolk sak tümörü, Belirsiz genitalya

Recurrent Unilateral Gonadoblastoma - A rare histopathological presentation

Gonadoblastoma is a rare benign tumor that has the potential for malignant transformation and affects patients with disorders of sexual development. The exact prevalence of gonadoblastoma is not known. The association of gonadoblastomas with dysgerminoma is seen in 50-60% of cases and with other malignant germ cell tumours like yolk sac tumour, embryonal carcinoma and choriocarcinoma in 10% of cases. We herewith report a rare case of recurrent unilateral gonadoblastoma in a 10 year old Indian male who presented with abnormal passage of urine since birth. Physical examination showed a phenotypic male with undescended right testis and ambiguous external genitalia. Computed tomography scan reported mixed density solid cystic mass in pelvis with peripherally enhancing solid component and a few punctuate foci of calcification. Histo-pathological examination showed a heterogeneous tumor comprising of malignant seminomatous, sertoli and leydig cell tumor along with yolk cell tumor component. Serum alpha fetoprotein was raised to 1210 ng/ml.

Keywords:

Gonadoblastoma, Seminoma, Yolk sac Tumor, Ambiguous genitalia,

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