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Primary leiomyosarcoma of the skin: a comprehensive review on diagnosis and treatment

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Abstract

Sarcomas are a heterogeneous group of mesenchymal tumors which can affect bone and soft tissue. Leiomyosarcoma (LMS) is a rare subtype localized to the skin or subcutaneous tissue. Due to the heterogeneity of sarcomas, reviews and guidelines with an in-depth focus specifically on primary LMS of the skin are sparse. This article is intended to provide an up to date and systematic overview on diagnosis, treatment, and surveillance of this rare entity to provide a framework for decision making and management for dermato-oncologists. We discuss novel treatment options for advanced disease such as targeted therapy with kinase inhibitors and immune checkpoint blockade which may improve the prognosis even in advanced stages of LMS.

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References

  1. Zahm SH, Fraumeni JF Jr. The epidemiology of soft tissue sarcoma. Semin Oncol. 1997;24(5):504–14.

    PubMed  CAS  Google Scholar 

  2. Stout AP, Hill WT. Leiomyosarcoma of the superficial soft tissues. Cancer. 1958;11(4):844–54.

    Article  PubMed  CAS  Google Scholar 

  3. Cook TF, Fosko SW. Unusual cutaneous malignancies. Semin Cutan Med Surg. 1998;17(2):114–32.

    Article  PubMed  CAS  Google Scholar 

  4. Holst VA, Junkins-Hopkins JM, Elenitsas R. Cutaneous smooth muscle neoplasms: Clinical features, histologic findings, and treatment options. J Am Acad Dermatol. 2002;46(4):477–90. quiz, 491 – 474.

    Article  PubMed  Google Scholar 

  5. Wascher RA, Lee MY. Recurrent cutaneous leiomyosarcoma. Cancer. 1992;70(2):490–2.

    Article  PubMed  CAS  Google Scholar 

  6. Kohlmeyer J, Steimle-Grauer SA, Hein R. Cutaneous sarcomas. J Dtsch Dermatol Ges. 2017;15(6):630–48.

    PubMed  Google Scholar 

  7. Deneve JL, Messina JL, Bui MM, Marzban SS, Letson GD, Cheong D, et al. Cutaneous leiomyosarcoma: Treatment and outcomes with a standardized margin of resection. Cancer Control. 2013;20(4):307–12.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Fields JP, Helwig EB. Leiomyosarcoma of the skin and subcutaneous tissue. Cancer. 1981;47(1):156–69.

    Article  PubMed  CAS  Google Scholar 

  9. Phelan JT, Sherer W, Mesa P. Malignant smoothmuscle tumors (leiomyosarcomas) of soft-tissue origin. N Engl J Med. 1962;266:1027–30.

    Article  PubMed  CAS  Google Scholar 

  10. Dahl I, Angervall L. Cutaneous and subcutaneous leiomyosarcoma. A clinicopathologic study of 47 patients. Pathol Eur. 1974;9(4):307–15.

    PubMed  CAS  Google Scholar 

  11. Jensen ML, Jensen OM, Michalski W, Nielsen OS, Keller J. Intradermal and subcutaneous leiomyosarcoma: A clinicopathological and immunohistochemical study of 41 cases. J Cutan Pathol. 1996;23(5):458–63.

    Article  PubMed  CAS  Google Scholar 

  12. Farran Y, Padilla O, Chambers K, Philipovskiy A, Nahleh Z. Atypical presentation of radiation-associated breast angiosarcoma: A case report and review of literature. Am J Case Rep. 2017;18:1347–50.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Sabater-Marco V, Ferrando-Roca F, Morera-Faet A, Garcia-Garcia JA, Bosch SB, Lopez-Guerrero JA. Primary cutaneous leiomyosarcoma arising in a patient with li-fraumeni syndrome: A neoplasm with unusual histopathologic features and loss of heterozygosity at tp53 gene. Am J Dermatopathol. 2018;40(3):225–7.

    Article  PubMed  Google Scholar 

  14. Gao P, Seebacher NA, Hornicek F, Guo Z, Duan Z. Advances in sarcoma gene mutations and therapeutic targets. Cancer Treat Rev. 2018;62:98–109.

    Article  PubMed  CAS  Google Scholar 

  15. Aneiros-Fernandez J, Antonio Retamero J, Husein-Elahmed H, Ovalle F, Aneiros-Cachaza J. Primary cutaneous and subcutaneous leiomyosarcomas: Evolution and prognostic factors. Eur J Dermatol. 2016;26(1):9–12.

    PubMed  CAS  Google Scholar 

  16. Patt JC, Haines N. Soft tissue sarcomas in skin: Presentations and management. Semin Oncol. 2016;43(3):413–8.

    Article  PubMed  Google Scholar 

  17. Corcoran S, Hogan AM, Nemeth T, Bennani F, Sullivan FJ, Khan W, et al. Isolated cutaneous metastasis of uterine leiomyosarcoma: Case report and review of literature. Diagn Pathol. 2012;7:85.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Zarcone N, Ciacci A, Di Gregorio C, Rivasi F. [subcutaneous metastasis of uterine leiomyosarcoma]. Minerva Ginecol. 1988;40(8):489–91.

    PubMed  CAS  Google Scholar 

  19. Vandergriff T, Krathen RA, Orengo I. Cutaneous metastasis of leiomyosarcoma. Dermatol Surg. 2007;33(5):634–7.

    PubMed  CAS  Google Scholar 

  20. Soipi S, Vucic M, Ulamec M, Tomas D, Kruslin B, Spajic B. Leiomyosarcoma of the spermatic cord with scalp metastasis: Case report and literature review. Coll Antropol. 2014;38(2):763–6.

    PubMed  Google Scholar 

  21. Alessi E, Innocenti M, Sala F. Leiomyosarcoma metastatic to the back and scalp from a primary neoplasm in the uterus. Am J Dermatopathol. 1985;7(5):471–6.

    Article  PubMed  CAS  Google Scholar 

  22. Annest NM, Grekin SJ, Stone MS, Messingham MJ. Cutaneous leiomyosarcoma: A tumor of the head and neck. Dermatol Surg. 2007;33(5):628–33.

    PubMed  CAS  Google Scholar 

  23. Bernstein SC, Roenigk RK. Leiomyosarcoma of the skin. Treatment of 34 cases. Dermatol Surg. 1996;22(7):631–5.

    Article  PubMed  CAS  Google Scholar 

  24. Banuls J, Botella R, Sevila A, Aranda I, Roman P. Leiomyosarcoma of the upper lip. Int J Dermatol. 1994;33(1):48–9.

    Article  PubMed  CAS  Google Scholar 

  25. Piana M, Martinez Mansur R, Codone J, Elizalde F, Diez M, Reyes E, et al. [penile leiomyosarcoma: Case report and bibliographic review]. Arch Esp Urol. 2006;59(7):728–31.

    Article  PubMed  Google Scholar 

  26. Pow-Sang MR, Orihuela E. Leiomyosarcoma of the penis. J Urol. 1994;151(6):1643–5.

    Article  PubMed  CAS  Google Scholar 

  27. Rabinovich J. Leiomyosarcoma of the foreskin: A rare case of mesenchymal foreskin tumor. Urologe A. 2018;57(5):591–3.

    Article  PubMed  Google Scholar 

  28. John T, Portenier D, Auster B, Mehregan D, Drelichman A, Telmos A. (2006). Leiomyosarcoma of scrotum–case report and review of literature. Urology. 67(2):424 e413–424 e415.

    Article  Google Scholar 

  29. Lo Muzio L, Favia G, Farronato G, Piattelli A, Maiorano E. Primary gingival leiomyosarcoma. A clinicopathological study of 1 case with prolonged survival. J Clin Periodontol. 2002;29(2):182–7.

    Article  PubMed  Google Scholar 

  30. Kaltreider SA, Destro M, Lemke BN. Leiomyosarcoma of the orbit. A case report and review of the literature. Ophthalmic Plast Reconstr Surg. 1987;3(1):35–41.

    Article  PubMed  CAS  Google Scholar 

  31. Murback NDN, Takita LC, Castro BC, Hans Filho G. Cutaneous leiomyosarcoma on the face. An Bras Dermatol. 2018;93(2):262–4.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Kim NG, Kim JO, Park YJ, Kim JS, Lee YJ, Lee KS. Cutaneous leiomyosarcoma of the face. Arch Craniofac Surg. 2017;18(2):145–8.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Lonsdale RN, Widdison A. Leiomyosarcoma of the nipple. Histopathology. 1992;20(6):537–9.

    Article  PubMed  CAS  Google Scholar 

  34. Alessi E, Sala F. Leiomyosarcoma in ectopic areola. Am J Dermatopathol. 1992;14(2):165–9.

    Article  PubMed  CAS  Google Scholar 

  35. Hashimoto H, Daimaru Y, Tsuneyoshi M, Enjoji M. Leiomyosarcoma of the external soft tissues. A clinicopathologic, immunohistochemical, and electron microscopic study. Cancer. 1986;57(10):2077–88.

    Article  PubMed  CAS  Google Scholar 

  36. Winchester DS, Hocker TL, Brewer JD, Baum CL, Hochwalt PC, Arpey CJ, et al. Leiomyosarcoma of the skin: Clinical, histopathologic, and prognostic factors that influence outcomes. J Am Acad Dermatol. 2014;71(5):919–25.

    Article  PubMed  Google Scholar 

  37. Sleiwah A, Clinton A, Herbert K. (2018). Delayed diagnosis of dermal leiomyosarcoma mimicking keloid scar. BMJ Case Rep. 2018;https://doi.org/10.1136/bcr-2017-222616.

  38. Lin JY, Tsai RY. Subcutaneous leiomyosarcoma on the face. Dermatol Surg. 1999;25(6):489–91.

    Article  PubMed  CAS  Google Scholar 

  39. Massi D, Franchi A, Alos L, Cook M, Di Palma S, Enguita AB, et al. Primary cutaneous leiomyosarcoma: Clinicopathological analysis of 36 cases. Histopathology. 2010;56(2):251–62.

    Article  PubMed  Google Scholar 

  40. Luke JJ, Keohan ML. Advances in the systemic treatment of cutaneous sarcomas. Semin Oncol. 2012;39(2):173–83.

    Article  PubMed  CAS  Google Scholar 

  41. Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol. 1996;14(5):1679–89.

    Article  PubMed  CAS  Google Scholar 

  42. Harding-Jackson N, Sangueza M, Mackinnon A, Suster S, Plaza JA. Spindle cell atypical fibroxanthoma: Myofibroblastic differentiation represents a diagnostic pitfall in this variant of afx. Am J Dermatopathol. 2015;37(7):509–14. quiz 515 – 506.

    Article  PubMed  Google Scholar 

  43. Fernandez-Flores A, Monteagudo C. Immunoexpression of p53 in cutaneous and subcutaneous leiomyosarcomas. Ann Diagn Pathol. 2016;24:25–9.

    Article  PubMed  Google Scholar 

  44. Idriss MH, Elston DM. S100a6 expression in cutaneous smooth muscle neoplasms. APMIS. 2015;123(10):832–6.

    Article  PubMed  CAS  Google Scholar 

  45. Suster S, Rosen LB, Sanchez JL. Granular cell leiomyosarcoma of the skin. Am J Dermatopathol. 1988;10(3):234–9.

    Article  PubMed  CAS  Google Scholar 

  46. Suster S. Epithelioid leiomyosarcoma of the skin and subcutaneous tissue. Clinicopathologic, immunohistochemical, and ultrastructural study of five cases. Am J Surg Pathol. 1994;18(3):232–40.

    Article  PubMed  CAS  Google Scholar 

  47. Diaz-Cascajo C, Borghi S, Weyers W. Desmoplastic leiomyosarcoma of the skin. Am J Dermatopathol. 2000;22(3):251–5.

    Article  PubMed  CAS  Google Scholar 

  48. Kraft S, Fletcher CD. Atypical intradermal smooth muscle neoplasms: Clinicopathologic analysis of 84 cases and a reappraisal of cutaneous “leiomyosarcoma”. Am J Surg Pathol. 2011;35(4):599–607.

    Article  PubMed  Google Scholar 

  49. Coindre JM, Trojani M, Contesso G, David M, Rouesse J, Bui NB, et al. Reproducibility of a histopathologic grading system for adult soft tissue sarcoma. Cancer. 1986;58(2):306–9.

    Article  PubMed  CAS  Google Scholar 

  50. Group ESESNW. Soft tissue and visceral sarcomas: Esmo clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014;25(Suppl 3):iii102–112.

    Article  Google Scholar 

  51. Roberge D, Hickeson M, Charest M, Turcotte RE. Initial mcgill experience with fluorodeoxyglucose pet/ct staging of soft-tissue sarcoma. Curr Oncol. 2010;17(6):18–22.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  52. Roberge D, Vakilian S, Alabed YZ, Turcotte RE, Freeman CR, Hickeson M. Fdg pet/ct in initial staging of adult soft-tissue sarcoma. Sarcoma, 2012;https://doi.org/10.1155/2012/960194.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Tsutsumida A, Yoshida T, Yamamoto Y, Itoh T, Minakawa H, Sugihara T. Management of superficial leiomyosarcoma: A retrospective study of 10 cases. Plast Reconstr Surg. 2005;116(1):8–12.

    Article  PubMed  CAS  Google Scholar 

  54. Starling J IIIrd, & Coldiron BM. Mohs micrographic surgery for the treatment of cutaneous leiomyosarcoma. J Am Acad Dermatol. 2011;64(6):1119–22.

    Article  PubMed  Google Scholar 

  55. McKee MD, Liu DF, Brooks JJ, Gibbs JF, Driscoll DL, Kraybill WG. The prognostic significance of margin width for extremity and trunk sarcoma. J Surg Oncol. 2004;85(2):68–76.

    Article  PubMed  Google Scholar 

  56. Humphreys TR, Finkelstein DH, Lee JB. Superficial leiomyosarcoma treated with mohs micrographic surgery. Dermatol Surg. 2004;30(1):108–12.

    PubMed  Google Scholar 

  57. Hollmig ST, Sachdev R, Cockerell CJ, Posten W, Chiang M, Kim J. Spindle cell neoplasms encountered in dermatologic surgery: A review. Dermatol Surg. 2012;38(6):825–50.

    Article  PubMed  CAS  Google Scholar 

  58. Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113(3):573–81.

    Article  PubMed  Google Scholar 

  59. Mouridsen HT, Bastholt L, Somers R, Santoro A, Bramwell V, Mulder JH, et al. Adriamycin versus epirubicin in advanced soft tissue sarcomas. A randomized phase ii/phase iii study of the eortc soft tissue and bone sarcoma group. Eur J Cancer Clin Oncol. 1987;23(10):1477–83.

    Article  PubMed  CAS  Google Scholar 

  60. Tap WD, Jones RL, Van Tine BA, Chmielowski B, Elias AD, Adkins D, et al. Olaratumab and doxorubicin versus doxorubicin alone for treatment of soft-tissue sarcoma: An open-label phase 1b and randomised phase 2 trial. Lancet. 2016;388(10043):488–97.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  61. Le Cesne A, Blay JY, Judson I, Van Oosterom A, Verweij J, Radford J, et al. Phase ii study of et-743 in advanced soft tissue sarcomas: A european organisation for the research and treatment of cancer (eortc) soft tissue and bone sarcoma group trial. J Clin Oncol. 2005;23(3):576–84.

    Article  PubMed  CAS  Google Scholar 

  62. Garcia-Carbonero R, Supko JG, Manola J, Seiden MV, Harmon D, Ryan DP, et al. Phase ii and pharmacokinetic study of ecteinascidin 743 in patients with progressive sarcomas of soft tissues refractory to chemotherapy. J Clin Oncol. 2004;22(8):1480–90.

    Article  PubMed  CAS  Google Scholar 

  63. von Mehren M, Rankin C, Goldblum JR, Demetri GD, Bramwell V, Ryan CW, et al. Phase 2 southwest oncology group-directed intergroup trial (s0505) of sorafenib in advanced soft tissue sarcomas. Cancer. 2012;118(3):770–6.

    Article  CAS  Google Scholar 

  64. D’Angelo SP, Mahoney MR, Van Tine BA, Atkins J, Milhem MM, Jahagirdar BN, et al. Nivolumab with or without ipilimumab treatment for metastatic sarcoma (alliance a091401): Two open-label, non-comparative, randomised, phase 2 trials. Lancet Oncol. 2018;19(3):416–26.

    Article  PubMed  Google Scholar 

  65. D’Angelo SP, Shoushtari AN, Agaram NP, Kuk D, Qin LX, Carvajal RD, et al. Prevalence of tumor-infiltrating lymphocytes and pd-l1 expression in the soft tissue sarcoma microenvironment. Hum Pathol. 2015;46(3):357–65.

    Article  PubMed  CAS  Google Scholar 

  66. Maki RG, Jungbluth AA, Gnjatic S, Schwartz GK, D’Adamo DR, Keohan ML, et al.. A pilot study of anti-ctla4 antibody ipilimumab in patients with synovial sarcoma. Sarcoma. 2013;https://doi.org/10.1155/2013/168145.

    Article  PubMed  PubMed Central  Google Scholar 

  67. Tawbi HA, Burgess M, Bolejack V, Van Tine BA, Schuetze SM, Hu J, et al. Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (sarc028): A multicentre, two-cohort, single-arm, open-label, phase 2 trial. Lancet Oncol. 2017;18(11):1493–501.

    Article  PubMed  CAS  Google Scholar 

  68. Liao WC, Wang YC, Ma H. Cutaneous leiomyosarcoma: The clinical experience of taipei veterans general hospital revisited. Ann Plast Surg. 2017;78(3 Suppl 2):47-s51.

    Google Scholar 

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Author notes

  1. Martina Zacher and Markus V. Heppt have contributed equally to this work.

Authors and Affiliations

  1. Department of Dermatology and Allergy, University Hospital, LMU Munich, Frauenlobstr. 9–11, 80337, Munich, Germany

    Martina Zacher, Markus V. Heppt, Kinan M. Hayani, Michael J. Flaig & Carola Berking

  2. Department of Dermatology, Heidelberg University Hospital, University of Heidelberg, Heidelberg, Germany

    Titus J. Brinker

  3. Department of Translational Oncology, German Cancer Research Center, National Center for Tumor Diseases (NCT), Heidelberg, Germany

    Titus J. Brinker

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  1. Martina Zacher
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Correspondence to Carola Berking.

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Zacher, M., Heppt, M.V., Brinker, T.J. et al. Primary leiomyosarcoma of the skin: a comprehensive review on diagnosis and treatment. Med Oncol 35, 135 (2018). https://doi.org/10.1007/s12032-018-1196-2

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