Omnipresent, omnipotent - extra-uterine leiomyosarcoma with varied presentation - case series of a dozen!!
Keywords:
extra uterine, leiomyosarcoma, immunohistochemistry, sarcomaAbstract
Background: Leiomyosarcoma (LMS), accounting for 7-10% of all soft tissue tumors, is derived from smooth muscles and arises most commonly from uterus, retroperitoneum, extremities, but can potentially involve any site of the body. Through this case series of 12 extra-uterine LMSs, we highlight the wide variety of clinical presentations of this tumor.
Methods: Retrospective analysis of clinical details, histopathological examination (HPE) and immunohistochemistry (IHC) of LMSs diagnosed in the extra uterine sites from 2012-2016 in our tertiary care institute.
Result: The mean age of presentation was 55.8 years, with male: female ratio 2:1. The site distribution was: stomach (3), non visceral soft tissue-thigh (2), breast (2), cutaneous (2), ileum(1), esophagus(1), and larynx (1).
Conclusion: The variable presentations of LMS at extra-uterine sites, makes it a challenging diagnosis for the unsuspecting clinicians and histopathologists. Thus, any soft tissue tumor in similar clinical scenarios may turn out to be LMS.
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DOI:10.21276/APALM.1588
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References
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2. B Davies CE, Davies AM, Kindblom LG, James SL. Soft tissue tumors with muscle differentiation. Semin Musculoskelet Radiol 2010;14:245-56.
3. Gladdy RA, Qin LX, Moraco N, Agaram NP, Brennan MF, and Singer S. Predictors of survival and recurrence in primary leiomyosarcoma. Annals of Surgical Oncology 2013;20:1851–7.
4. Ren B, Yu YP, Jing L, Liu L, Michalopoulos GK, et al. Gene expression analysis of human soft tissue leiomyosarcomas. Hum Pathol 2003;34:549-58.
5. Toro JR, Travis LB, Wu HJ, Zhu K, Fletcher CDM, Devesa SS. Incidence patterns of soft tissue sarcomas,regardless of primary site, in the surveillance, epidemiology and end results program, 1978–2001: an analysis of 26,758 cases. International Journal of Cancer 2006;119:2922–30.
6. Aggarwal G, Sharma S, Zheng M, et al. Primary leiomyosarcomas of the gastrointestinal tract in the post-gastrointestinal stromal tumor era. Ann Diagn Pathol 2012;16:532–40.
7. Katz SC, DeMatteo RP. Gastrointestinal stromal tumors and leiomyosarcomas. J Surg Oncol 2008;97:350–9.
8. Svarvar C, Böhling T, Berlin O, Gustafson P, Follerås G, et al. Clinical course of nonvisceral soft tissue leiomyosarcoma in 225 patients from the Scandinavian Sarcoma Group. Cancer 2007;109:282-91.
9. Paal E, Miettinen M. Retroperitoneal leiomyomas: a clinicopathologic and immunohistochemical study of 56 cases with a comparison to retroperitoneal leiomyosarcomas. Am J Surg Pathol 2001;25:1355-63.
10. Lo Muzio L, Favia G, Mignogna MD, Piattelli A, Maiorano E. Primary intraoral leiomyosarcoma of the tongue: an immunohistochemical study and review of the literature. Oral Oncol 2000;36:519-24.
11. Abbas A, Ikram M, Yagoob N. Leiomyosarcoma of the larynx: A case report. Ear Nose Throat J 2005;84:435-6.
12. Bernstein SC, Roenigk RK. Leiomyosarcoma of the skin: treatment of 34 cases. Dermatol Surg. 1996;22:631-5.
13. Miller JS, Zhou M, Brimo F, Guo CC, Epstein JI. Primary leiomyosarcoma of the kidney: a clinicopathologic study of 27 cases. Am J Surg Pathol 2010;34: 238-42.
14. Kaur S, Tyagi R, Selhi P, Singh A, Puri H, Sood N. Subcutaneous Axillary and Scalp Metastases from Non-Gynecological Retroperitoneal Leiomyosarcoma: An Unusual Presentation After Surgical Resection. Rare Tumors 2015 ;7:5970. doi: 10.4081/rt.2015.5970.PMID: 26788272
15. Zahm SH, Fraumeni JF, Jr. The epidemiology of soft tissue sarcoma. Semin Oncol 1997;24:504–14.
16. Paal E, Miettinen M. Retroperitoneal leiomyomas: a clinicopathologic and immunohistochemical study of 56 cases with a comparison to retroperitoneal leiomyosarcomas. Am J Surg Pathol 2001;25:1355-63.
17. Marioni G, Bertino G, Mariuzzi L, Bergamin-Bracale A, Lombardo M, Beltrami C. Laryngeal leiomyo- sarcoma. J Laryngol Otol 2000;114:398-401.
18. Ragazzini P, Gamberi G, Pazzaglia L, Serra M, Magagnoli G, et al. Amplification of CDK, MDM, SAS and GLI genes in leiomyosarcoma, alveolar and embryonal rhabdomyosarcoma. Histol Histopathol 2004;19:401-11.
19. Italiano A, Toulmonde M, Stoeckle E et al. Clinical outcome of leiomyosarcomas of vascular origin: comparison with leiomyosarcomas of other origin. Annals of Oncology 2010;21:1915–21.
20. Skubitz KM and Skubitz APN. Differential gene expression in leiomyosarcoma. Cancer 2003;98:1029–38.
21. Yang J , Du X, Chen K et al.Genetic aberrations in soft tissue leiomyosarcoma. Cancer Letters 2009;275:1–8.
22. Farid M, Ong WS, Tan MH, et al. The influence of primary site on outcomes in leiomyosarcoma: a review of clinicopathologic differences between uterine and extrauterine disease. Am J Clin Oncol 2013;36:368–74.
23. Beck AH, Lee CH, Witten DM, et al. Discovery of molecular subtypes in leiomyosarcoma through integrative molecular profiling. Oncogene 2010;29: 845-54.
24. Italiano A, Lagarde P, Brulard C, Terrier P, Laë M, et al. Genetic profiling identifies two classes of soft-tissue leiomyosarcomas with distinct clinical characteristics. Clin Cancer Res 2013;19:1190-6.
25. Miyajima K, Oda Y, Oshiro Y, Tamiya S, Kinukawa N, et al. Clinicopathological prognostic factors in soft tissue leiomyosarcoma: a multivariate analysis. Histopathology 2002;40:353-9.
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27-02-2018
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