CD56 might be a useful diagnostic marker of müllerian-derived tumors: report of an uterine tumor resembling ovarian sex cord tumor (UTROSCT) suggesting polyphenotypic origin and review of the literature

Year 2013, , 132 - 141, 04.03.2014

Hatice Özer , Handan Aker Ahmet Mutlu Ali Yanık Tülay Koç Hande Keser

https://doi.org/10.12808/bcs.v2i3.31

Abstract

Abstract

We present the clinicopathologic and immunophenotypic features of a case of uterine tumor resembling ovarian sex cord tumor. The patient age was 38 years and presented with discomfort in the lower abdomen, vaginal bleeding, and dysmenorrhea. Pelvic ultrasound and computed tomography of the lower abdomen revealed a semisolid mass extending to umbilicus. Bilateral adnexal tenderness was found. The patient underwent operation with an ovarian tumor as a preoperative diagnosis. In the intraoperative consultation, there was a suspicion of a uterine tumor resembling ovarian sex cord stromal tumor. Oophorectomy was not performed because of her age. The well-circumscribed nodular tumor was 18 cm in diameter and composed of ovarian sex cord-like areas, bundles of myoid cells. Besides some cells exhibited clear cytoplasm. There were cystic spaces and infarct type of necrosis. All tumor cells showed strong immunoreactivity for CD56. In addition, CD10, desmin, actin, inhibin, WT-1, HBM45, estrogen, and progesterone receptor positivity were present but calretinin, CD99, CD34, CD117, S100, and pan-cytokeratin were negative. We think that this tumor as well as the other tumors of the female genital tract might be arisen from pluripotent mesenchymal cells which are derived from Müllerian ducts that could be differentiate into endometrial stromal, epithelial and smooth muscle. Our data support that this unusual uterine tumor is a polyphenotypic neoplasm with true sex cord differentiation.

Keywords: Uterine tumor, UTROSCT, immunohistochemistry, CD56, Müllerian system

Özet

Ovarian seks kord tümöre benzeyen  bir uterin tümör olgusunun klinikopatolojik ve immünofenotipik özelliklerini sunuyoruz. Olgunun yaşı 38 idive alt karın ağrısı, vajinal kanama ve dismenore ile başvurmuştu. Pelvik bölgenin ultrasonografi ve bilgisayarlı tomografisi göbeğe kadar uzanan semisolid bir kitle ortaya koymuştu. Bilateral adneksal hassasiyet vardı. Olgu ovarian tümör tanısı ile ameliyata alındı. İntraoperatif konsültasyonda ovarian seks kord tümöre benzeyen bir uterin tümör şüphesi vardı. Hastanın yaşından dolayı ooferektomi yapılmadı. İyi sınırlanmış nodüler tümörün çapı 18 cm idi ve myoid hücre demetleri içeren ovarian seks korda benzer alanlar gözlendi. Bunun yanında bazı hücrelerin stoplazmaları berraktı. Kistik boşluklar ve infarkta benzer nekroz alanları avrdı. Tüm tümör hücreleri CD56 için kuvvetli immünoreaktivite gösterdi. İlave olarak CD10, desmin aktin, inhibin, WT-1, HBM45, östrojen ve progesteron reseptör pozitifliği gözlendi ama kalretinin, CD99, CD34, CD117, S100 ve pansitokeratin negatif bulundu. Diğer kadın genital yol tümörleri gibi bu tümör de müllerian kanaldan köken alan pluripotent mezenşimal hücrelerden köken almış olabilir. Bulgularımız bu nadir görülen ovarian tümörün gerçek seks kord farklılaşması olan polifenotipik bir neoplazm olduğunu desteklemektedir.

Anahtar sözcükler: Uterin tümör, UTROSCT, immünohistokimya, CD56, Müllerian sistem

Keywords

Uterine tumor, UTROSCT, immunohistochemistry, CD56, Müllerian system

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