Mete GÜNGÖR, Berna SAVAŞ, Koray CEYHAN, Ebru EROL, Sibel PERÇİNEL, Nural ERDOĞAN

Primer over tümörü gibi prezentasyon gösteren dezmoplastik küçük yuvarlak hücreli tümör

Karında kitle nedeniyle hastaneye başvuran 27 yaşındaki bir kadın hastada klinik ve radyolojik olarak over karsinomundan şüphelenilmiştir. Bilgisayarlı tomografide karın ön duvarında tümör implantlarını yansıttığı düşünülen lokalize kalınlaşmalar gözlenmiştir. Ultrasonografide sağ adnekste pelvik orta hatta uzanan 13 cm uzun çaplı solid bir kitle saptanmıştır. Doppler ile yapılan incelemede kitlenin santralinde belirgin bir kanlanma izlenmiş ve bu paternin over tümörü ile uyumlu olabileceği düşünülmüştür. Hastada yaygın asit ve anürik klinik tablonun gelişmesi ve yapılan tedavilere cevap alınamaması üzerine bilateral salpingooforektomi, omentektomi, splenektomi ve apendektomi yapılmıştır. Makroskopik olarak bilateral over, omentum, dalak ve apendiks vermiformisin tümör ile yaygın olarak infiltre olduğu gözlenmiştir. Mikroskopik olarak sözü edilen bölgelerde dezmoplastik bir stroma içerisinde yuvarlak-oval, hiperkromatik nükleuslu, dar eozinofilik sitoplazmalı küçük monoton hücrelerden oluşan bir tümör izlenmiştir. İmmünhistokimyasal olarak tümör hücrelerinin desmin, vimentin, sinaptofizin, yüksek moleküler ağırlıklı-sitokeratin, epitelyal membran antijen ve CD56 ile reaktivite gösterdiği saptanmıştır. Klinik, radyolojik, makroskopik, mikroskopik ve karakteristik immünhistokimyasal bulgulara dayanarak olgu dezmoplastik küçük yuvarlak hücreli tümör tanısı (DKYHT) almıştır. Hasta kemoterapi tedavisi görmüş ve operasyondan çok kısa bir süre sonra eks olmuştur. DKYHT over tutulumu gösterebilir ve primer over tümörlerini taklit edebilir. Bu nedenle adneksial kitlesi olan genç hastalarda akılda tutulması gereken bir antitedir.

Desmoplastic small round cell tumor presenting as primary ovarian tumor

A 27-year-old woman was admitted to a hospital with a mass in the abdomen in which ovarian carcinoma was suspected clinically and radiologically. Computed tomography showed localized thickenings which were thought to represent tumor implants on the anterior abdominal wall. Ultrasonography revealed a solid mass of 13 cm in its maximum diameter in the right adnex which extended to the midline of the pelvic region. Doppler examination showed a conspicious vascularization in the centre of the tumor, the pattern of which was consistent with an ovarian tumor. Bilateral salpingo-oophorectomy, omentectomy, splenectomy, and appendectomy were performed due to the presence of widespread ascitis and anuric clinical picture of the patient and unresponsiveness to the treatment. Macroscopically, it was observed that bilateral ovaries, omentum, spleen, and appendix vermiformis were infiltrated by the tumor in a widespread manner. Microscopically, a tumor consisting of small monotonous cells having round to ovoid, hyperchromatic nuclei with scant eosinophilic cytoplasm in a desmoplastic stroma was observed in the aforementioned areas. Immunohistochemically, the tumor cells showed reactivity for desmin, vimentin, synaptophysin, high molecular weight-cytokeratin, epithelial membrane antigen, and CD56. On the basis of clinical, radiological, macroscopical, microscopical, and characteristic immunohistochemical findings, the case was diagnosed as desmoplastic small round cell tumor (DSRCT). The patient received chemotherapy but died soon after the operation. DSRCT can show ovarian involvement and can mimic primary ovarian tumors so this entity should be kept in mind in young patients who have adnexial mass.

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