We appreciate the interest in our paper [1] and raising the issues referring to the potential histogenesis of leiomyosarcoma (LMS). Indeed, LMS developing in the “secondary Müllerian system” area may be split into Müllerian and non Müllerian somatic types. Expression of the estrogen (ER) and progesterone receptor (PR), and Wilms tumor 1 protein (WT-1) may be helpful in this regard, since only the tumors from the former group present these markers.

At diagnosis, both our tumors did not express ER and PR. Similarly, there was no nuclear expression of Wilms tumor 1 protein (WT-1), suggesting somatic derivation of these tumors. As pointed out in the mentioned papers [1, 2], significance of the histogenetic derivation of LMS is not established. Expression of ER may have some diagnostic value in distinguishing uterine from extrauterine LMSs. Of course, as shown earlier [1, 3], the absence of these markers does not totally exclude Müllerian derivation of ER-/WT-1-negative LMSs. In addition, LMS showing ER expression may have more favorable clinical behaviour [1, 2], although this data is preliminary. Reassuming, at the present state of knowledge, significance of somatic vs. Müllerian phenotype in LMS is not finally proven. Likewise, estimation of the LMS risk in male members of the family reported in our case study would be highly speculative and not based on the scientific grounds.





Prof. Wojciech Biernat

Department of Pathology

Medical University of Gdańsk, Poland





Prof. Jacek Jassem

Department of Oncology and Radiotherapy

Medical University of Gdańsk, Poland

References

 1. Patil DT, Laskin WB, Fetsch JF, et al. Inguinal smooth muscle tumors in women-a dichotomous group consisting of Müllerian-type leiomyomas and soft tissue leiomyosarcomas: an analysis of 55 cases. Am J Surg Pathol 2011; 35: 315-324.

 2. Carvalho JC, Thomas DG, Lucas DR. Cluster analysis of immunohistochemical markers in leiomyosarcoma delineates specific anatomic and gender subgroups. Cancer 2009; 115: 4186-4195.

 3. Rao UN, Finkelstein SD, Jones MW. Comparative immunohistochemical and molecular analysis of uterine and extrauterine leiomyosarcomas. Mod Pathol 1999; 12: 1001-1009.