Primary Leiomyosarcoma of the Breast Papilla: A Case Report

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Matthäeus Bürger
Edson Marchiori
Maria Celia R. Djahjah
Ana Helena Correia


Leiomyosarcoma, breast papilla, magnetic resonance imaging


Background: Breast leiomyosarcoma is a rare subtype of breast sarcoma, constituting 5–10% of the cases. Less than one-third of reported cases are located in the nipple papillary region, making it an extraordinarily rare malignancy, responsible for less than 0.1% of all malignant breast tumors. As no radiological criteria allow definitive diagnosis of papillary leiomyosarcoma, histopathological and immunohistochemical examination of suspicious lesions is required. The prognosis is generally optimistic compared with those for other breast sarcomas.
Case presentation: A 54-year-old previously healthy woman reported the appearance of a nodule in her right nipple in the preceding 9 months, associated with pruritus. Physical examination revealed that the lesion had a cystic consistency and measured approximately 3.0 cm. Imaging examinations showed no involvement of the adjacent breast parenchyma or axillary region and the lesion was classified as Breast Imaging-Reporting and Data System (BI-RADS) category IV. The lesion was excised, and the histopathological diagnosis was well-differentiated leiomyosarcoma of the mammary papilla, with immunohistochemical examination showing positivity for smooth-muscle tissue antigens. A staging CT examination was performed, showing no evidence of distant metastasis. After 2 years of follow-up, the patient shows no sign of tumor recurrence.
Conclusion: Although rare, leiomyosarcoma must be included in differential diagnosis of breast masses, especially those involving the periareolar region. Due to its good prognosis, reporting on this type of tumor is important to guide therapeutic planning, and to identify and track possible complications of its underdiagnosis.


1. Amberger M, Park T, Petersen B, Baltazar GA. Primary breast leiomyosarcoma with metastases to the lung in a young adult: Case report and literature review. Int J Surg Case Rep. 2018;47:34-7.
2. Felix C, Bispo M, Chagas C. Pancreatic Metastasis from Primary Leiomyosarcoma of the Breast. GE Port J Gastroenterol. 2019;26(3):223-5.
3. Wong LC, Huang PC, Luh SP, Huang CS. Primary leiomyosarcoma of the nipple-areola complex: report of a case and review of literature. J Zhejiang Univ Sci B. 2008;9(2):109-13.
4. Assis JOd, Pimentel ACdA, Chambo Filho A, Cintra LC, Scardini R. Leiomiossarcoma de mama: relato de caso. Revista Brasileira de Ginecologia e Obstetrícia. 2001;23(4):255-8.
5. Rane SU, Batra C, Saikia UN. Primary leiomyosarcoma of breast in an adolescent girl: a case report and review of the literature. Case Rep Pathol. 2012;2012:491984.
6. Testori A, Meroni S, Voulaz E, Alloisio M, De Sanctis R, et al. Primary breast leiomyosarcoma and synchronous homolateral lung cancer: a case report. J Thorac Dis. 2017;9(12):E1054-E9.
7. Oktay Y, Fikret A. Leiomyosarcoma of the breast. J Surg Case Rep. 2011;2011(7):1.
8. Alkabban FM, Ferguson T. Cancer, breast. StatPearls [Internet]: StatPearls Publishing; 2019.
9. Smith TB, Gilcrease MZ, Santiago L, Hunt KK, Yang WT. Imaging features of primary breast sarcoma. AJR Am J Roentgenol. 2012;198(4): W386-93.
10. Munitiz V, Rios A, Canovas J, Ferri B, Sola J, et al. Primitive leiomyosarcoma of the breast: case report and review of the literature. Breast. 2004;13(1):72-6.
11. Minami S, Matsuo S, Azuma T, Uga T, Hayashi T, et al. Parenchymal leiomyoma of the breast: a case report with special reference to magnetic resonance imaging findings and an update review of literature. Breast Cancer. 2011;18(3): 231-6.
12. Al-Benna S, Poggemann K, Steinau HU, Steinstraesser L. Diagnosis and management of primary breast sarcoma. Breast Cancer Res Treat. 2010;122(3):619-26.
13. Singh G, Sharma D, Goyal S. Primary Leiomyosarcoma of Breast Presenting with Metastasis: An Atypical Presentation with Dismal Prognosis. Indian J Med Paediatr Oncol. 2017;38(4):535-7.
14. Lim SZ, Ong KW, Tan BK, Selvarajan S, Tan PH. Sarcoma of the breast: an update on a rare entity. J Clin Pathol. 2016;69(5):373-81.
15. Pollard SG, Marks PV, Temple LN, Thompson HH. Breast sarcoma. A clinicopathologic review of 25 cases. Cancer. 1990;66(5):941-4.
16. Parisi G, Signor MA, Finato N, Ceschia T, Magli A. Breast leiomyosarcoma with myxoid aspects. Breast J. 2018;24(6):1086-