Primary Cutaneous Leiomyosarcoma: A Clinicopathological and Immunohistochemical Study of 7 Cases

Abstract

Primary cutaneous leiomyosarcomas are rare tumors, few series being reported in the current literature. A retrospective study of 7 cases was undertaken to understand the clinicopathological characteristics of these neoplasms and some of their molecular mutations. Histologically, a well-differentiated proliferation of cells of smooth muscle derivation was evident in all cases. The number of mitoses was considered the most important criterion of malignancy (more than 2 for 10 HPF). Smooth muscle actin, desmin, and vimentin were positive in all cases. Immunohistochemical analysis also revealed a positivity for p53 in 3 cases and no reaction for retinoblastoma protein. Research for Epstein-Barr virus was negative in all cases. Three patients developed local recurrences owing to incomplete surgical excision. Recurrent tumors were more atypical and located deeper. No distant metastases were observed. Our results emphasize that cutaneous leiomyosarcomas have an indolent biological course if treated by surgical excision with wide margins. Molecular abnormalities involving tumor suppressor genes are probably involved.

Keywords

cutaneous leiomyosarcoma histopathology molecular abnormalities

Get full access to this article

View all access options for this article.

References

1. Stout AP , Hill WT . Leiomyosarcoma of the superficial soft tissue. Cancer 11:844-854, 1958

2. Fields JP , Helwig EB . Leiomyosarcoma of the skin and subcutaneous tissue. Cancer 47:156-169, 1981

3. Wascher RA , Lee MYT . Recurrent cutaneous leiomyosarcoma. Cancer 70:490-492, 1992

4. Kaddu S , Beham A , Cerroni L , Humer-Fuchs U , Salmhofer W , Kerl H , Soyer HP . Cutaneous leiomyosarcoma. Am J Surg Pathol 21:979-987, 1997

5. 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 23:458-463, 1996

6. Swanson PE , Stanley MW , Scheithauer BW , Wick MR . Primary cutaneous leiomyosarcoma: A histological and immunohistochemical study of 9 cases, with ultrastructural correlation. J Cutan Pathol 15:129-141, 1988

7. Troiani M , Contesso G , Coindre JM , Rouesse J , Bui NB , de Mascarel A , Goussot JF , David M , Bonichon F , Lagarde C . Soft tissue sarcomas of adults: Study of pathologic prognostic variables and definition of a histopathologic grading system. Int J Cancer 33:37-42, 1984

8. Costa J , Wesley RA , Glatstein E , Rosenberg SA . The grading of soft tissue sarcomas: Results of a clinicohistopathologic correlation in a series of 163 cases. Cancer 53:530-541, 1984

9. Oliver GF , Reiman HM , Gonchoroff NJ , Muller SA , Umbert IJ . Cutaneous and subcutaneous leiomyosarcoma: A clinicopathological review of 14 cases with reference to antidesmin and nuclear DNA patterns studied by flow cytometry. Br J Dermatol 124:252-257, 1991

10.

10. Auroy S , Contesso G , Spatz A , Genin J , Margulis A , Lecesne A , Avril MF . Primary cutaneous leiomyosarcoma: 32 cases. Ann Dermatol Venereol 126:235-242, 1999

11.

11. Holst VA , Junkins-Hopkins JM , Elenitsas R . Cutaneous smooth muscle neoplasms: Clinical features, histologic findings and treatment options. J Am Acad Dermatol 46:477-490, 2002

12.

12. Porter CJ , Januszkiewicz JS . Cutaneous leiomyosarcoma. Plast Reconstr Surg 109:964-967, 2002

13.

13. Suster S . Epithelioid leiomyosarcoma of the skin and subcutaneous tissue. Clinicopathologic, immunohistochemical, and ultrastructural study of five cases. Am J Surg Pathol 18:232-240, 1994

14.

14. Miettinen M , Lehto VP , Bradley RA , Virtanen I . Expression of intermediate filaments in soft tissue sarcomas. Int J Cancer 20:541-546, 1982

15.

15. Dei Tos AP , Maestro R , Doglioni C , Piccinin S , Della Libera D , Boiocchi M , Fletcher DM . Tumor suppressor genes and related molecules in leiomyosarcoma. Am J Pathol 148:1037-1045, 1996

16.

16. Stratton MR , Williams S , Fisher C , Ball A , Westbury G , Gusterson BA , Fletcher CDM , Knight JC , Fung YK , Reeves BR , Cooper CS . Structural alterations of the RB 1 gene in human soft tissue tumors. Br J Cancer 60:202-205, 1989

17.

17. McClain KL , Leach CT , Jenson HB , Joshi VV , Pollock BH , Parmley RT , DiCarlo FJ , Chadwick EG , Murphy SB . Association of Epstein-Barr virus with leiomyosarcomas in young people with AIDS. N Engl J Med 332:12-18, 1995

18.

18. Kanikatis J . Leiomyomes, leiomyosarcomes cutanes et virus d'Epstein-Barr. Ann Dermatol Venereol 127:841-846, 2000