Intra-abdominal leiomyosarcomas in two sisters. Müllerian or somatic type tumors?
Dear Editor,
We read with interest the reports of intra-abdominal leiomyosarcomas by Koczkowska et al. [1]. The tumors occurred in two sisters in the mesentery, and both were leiomyosarcomas with almost identical histological and genetic features, indicating a possible autosomal recessive trait in the tumor development.
As both patients were female, it would be interesting to know whether these leiomyosarcomas were of Mullerian or somatic type. Although current data are still limited, several recent published findings indicate that extra-uterine leiomyomas and leiomyosarcomas occurring in the region of secondary Müllerian system [2] (peritoneum, retroperitoneum, extragenital pelvic tissue, inguinal region) can be subdivided into Müllerian (uterine type) and non-Müllerian (somatic) types, and that these types should represent distinctive groups of smooth muscle tumors with different morphological, clinical and genetic features [3-6]. Immunohistochemically, the Müllerian type leiomyomas and leiomyosarcomas express estrogen receptor, progesterone receptor, and WT1, in contrast with non-Müllerian somatic type tumors, which are negative for these antigens.
In case the tumors reported by Koczkowska et al. [1] are Müllerian type leiomyosarcomas, the significance of such a finding for carcinogenesis and an estimation of the risk of such leiomyosarcoma for male members of the family would be our questions to our colleagues in oncologic genetics.References
1. Koczkowska M, Lipska BS, Grzeszewska J, et al. Primary leiomyosarcoma of the mesentery in two sisters: clinical and molecular characteristics. Pol J Pathol 2013; 64: 59-63.
2. Lauchlan SC. The secondary Mullerian system. Obstet Gynecol Surv 1972; 27: 133-146.
3. Patil DT, Laskin WB, Fetsch JF, Miettinen M. 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.
4. Kelley TW, Borden EC, Goldblum JR. Estrogen and progesterone receptor expression in uterine and extrauterine leiomyosarcomas: an immunohistochemical study. Appl Immunohistochem Mol Morphol 2004; 12: 338-341.
5. Billings SD, Folpe AL, Weiss SW. Do leiomyomas of deep soft tissue exist? An analysis of highly differentiated smooth muscle tumors of deep soft tissue supporting two distinct subtypes. Am J Surg Pathol 2001; 25: 1134-1142.
6. Carvalho JC, Thomas DG, Lucas DR. Cluster analysis of immunohistochemical markers in leiomyosarcoma delineates specific anatomic and gender subgroups. Cancer 2009; 115: 4186-4195.
Michal Zamecnik, MD
Department of Pathology, Agel Labs. a.s.
Novy Jicin, Czech Republic
Medicyt s.r.o., Department of Pathology,
Trencin, Slovak Republic
Peter Kascak, MD, PhD
Department of Obstetrics and Gynecology,
Regional Hospital,
Trencin, Slovak Republic
Faculty of Health, Alexander Dubcek University,
Trencin, Slovak Republic
Address for correspondence:
Dr. M. Zamecnik
Medicyt, s.r.o., Agel Labs. a.s.
Legionarska 28
91171 Trencin
Slovak Republic
phone +421-907-156629
e-mail: zamecnikm@seznam.cz
Copyright: © 2013 Polish Association of Pathologists and the Polish Branch of the International Academy of Pathology This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (
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