Malignant adenomyoepithelioma combined with adenoid cystic carcinoma of the breast: a case report and literature review
- Ying Yang†1,
- Yanmei Wang†2,
- Jinsong He1,
- Guoqing Pan1,
- Xiaoyu Tuo1,
- Aimei Jiang2 and
- Li Bian1Email author
© Yang et al.; licensee BioMed Central Ltd. 2014
Received: 16 April 2014
Accepted: 7 July 2014
Published: 23 July 2014
Malignant adenomyoepithelioma and adenoid cystic carcinoma are both rare malignant epithelial-myoepithelial tumors of the breast. We report a very rare case with a malignant adenomyoepithelioma combining with adenoid cystic carcinoma in a single mass.
A 61-year-old female presented with a palpable painless mass in the right breast. Mammography revealed a large irregular dense shadow without obvious internal calcification. A simple lumpectomy was performed, and a 1.6cm well-circumscribed pale-tan nodule was presented. Histologically, the nodule was composed of two obscure lobules. One exhibited typical histological image of adenoid cystic carcinoma, the other one showed the image of epithelial-myoepithelial carcinoma of salivary gland, and support the diagnosis of biphasic malignant adenomyoepithelioma. Transition between the two lobules was gradual. Immunohistochemically, CK18 and P63 highlighted the epithelial and myoepithelial cells respectively in both lesions. CD117 was positive in the epithelial cells of adenoid cystic carcinoma, but was totally negative in malignant adenomyoepithelioma.
This report is, to our knowledge, the first case that combines these two tumors in a single mass. In addition, we present a review of the literature. The histogenesis of these tumors is also discussed.
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KeywordsBreast Adenomyoepithelioma Adenoid cystic carcinoma
Malignant adenomyoepithelioma (AME) and adenoid cystic carcinoma (ACC) are both rare malignant epithelial-myoepithelial lesions of the breast. There are no more than 50 documented cases for the former , and the latter constitutes about 0.1% of all breast tumors . Difficulties are commonly encountered in diagnosis and differential diagnosis because of the rarity and similarity of these two tumors. Composed of epithelial and myoepithelial cells, these two salivary gland-like tumors of the breast are morphologically close. However, the relationship of them was seldom observed. There is only one report of adenoid cystic carcinoma arising within an adenomyoepithelioma so far .To the best of our knowledge, what we present herein is the first case that combines malignant adenomyoepithelioma and adenoid cystic carcinoma in a single mass. The aim of this report is to analyze the histological and immunohistochemical features of the two tumors by comparing each other, conclude the method of differential diagnosis, and most importantly, apply clue to the genesis of epithelial-myoepithelial tumors.
A modified radical mastectomy was performed according to the pathology results, the patient’s economic situation and with the patient’s consent. No lymph nodes involvement was found (0/21). The margins, basement membrane and nipple were all free of metastasis. Chemotherapy and radiation therapy were not indicated and the patient remained in a good condition throughout the 12-month follow-up period.
Reported cases of biphasic malignant adenomyoepithelioma
No. of cases
Histologic appearance of malignancy
Presence of metastasis
77 ~ 93
3.2 ~ 14.0
One epithelial-myoepithelial carcinoma with chondrosarcoma
One epithelial-myoepithelial carcinoma
1 lost to follow up
One epithelial-myoepithelial carcinoma/ACC like
Ahmed, et al. 
Nests and cords of both epithelial and myoepithelial tumor cells
Qureshi, et al. 
Epithelial glandular structures and myoepithelial spindle areas.
Hungermann, et al. 
Biphasic malignancy with well-developed tubular structures
Simpson, et al. 
Mixed osteogenic, spindle cell, and carcinomatous differentiation
Kiaer, et al. 
Peripherally proliferating myoepithelial cells and luminally localized eosinophilic cuboidal and columnar cells.
Pauwels, et al. 
A proliferation of tubules composed of epithelial cells surrounded by myoepithelial cells with high mitotic figures and focal atypia
Petrozza, et al. 
Focal epithelial-like component with high mitotic figures and partial myoepithelial-like component with atypia and high mitotic figures
Rasbridge, et al. 
39 ~ 76
1.3 ~ 4.5
Contain areas of invasive malignant cells or foci of cellular atypia and increased mitotic activity.
Trojani, et al. 
Bicellular pattern of epithelial and myoepithelial cells
Yang and Wang, et al.(current case)
Adenoid cystic carcinoma is another neoplasm in the spectrum of hyperplastic and neoplastic lesions characterized by dual differentiation into ductal and myoepithelial cells . There was only one report that adenoid cystic carcinoma arising in an adenomyoepithelioma in breast , which Hayes commented that “since these neoplasms are so closely related this is an arguable entity” . However, the difference does exist between the two entities. Adenoid cystic carcinoma has a characteristic cribriform architecture, the formation of true glandular spaces and pseudolumens, and the histologically distinct invagination of stroma. It lacks the papillary architecture which is frequently seen in adenomyoepithelioma. The myoepithelial cells tend to be smaller and more basaloid, and the arrangement of epithelial and myoepithelial cells is less irregular than that of adenomyoepithelioma [5, 18]. Immunohistologically, CD117 highlights the epithelial cells of adenoid cystic carcinoma [4, 19, 20] but is totally negative in malignant adenomyoepithelioma.
Comparison between the current case and the previous case
Type of AME
Proportion of AME to ACC
Relationship between AME and ACC
Van Dorpe et al. 
Juxta-areolar in right breast
A focus of ACC at the edge of AME penetrated the capsule
Yang et al. (current case)
Upper-outer quadrant of right breast
ACC as a lobule of the mass combined with malignant AME
Though ACC and AME are triple-negative breast cancers,which show a poor prognosis when paired with basal-like transcriptome, hormone receptor negativity status is not related to poor differentiation and a worse prognosis, as defined in invasive ductal carcinoma not otherwise specified (IDC NOS) [22–25]. In addition, compared with adenoid cystic carcinoma of salivary, although they have similar morphological and immunological phenotypes and even the same molecular genetic defect, the t(6;9)(q22–23;p23–24) translocation , the biological behavior of breast and salivary ACC is different. The former showed indolent clinical behavior, and the latter showed an aggressive course. Adenoid cystic carcinoma of breast presents as a localized disease with a low frequency of axillary lymph node involvement of less than 8%, and rare distant metastases of fewer than 20% [27–30]. Therefore, most clinicians recommend a breast-conserving surgical therapy with or without radiotherapy . However, some research performed grading according to the criteria accepted for salivary ACC and suggested that grade 3 (solid growth pattern) may have a higher propensity of recurrence and metastasis . This result has not been confirmed by long-term follow-up data. At the same time, AME of breast has better prognosis comparing with epithelial-myoepithelial carcinoma of salivary gland .Most of the adenomyoepitheliomas of breast behave as benign though rare cases have metastasized . The behavior of malignant adenomyoepithelioma seemed to be related to the grade of the malignant component and the tumor size [4, 5]. In our case, the component of high grade epithelial-myoepithelial carcinoma might indicate a high frequency of recurrence and metastases. To date, there are limited published data on the biological behavior and long-term clinical outcome of adenoid cystic carcinoma combining with malignant adenomyoepithelioma. As a result, it is necessary for the patient to be closely followed-up and periodically examined following treatment.
In this paper, we report the case of a rare biphasic malignant adenomyoepithelioma combining with adenoid cystic carcinoma in a single mass of a 61-year-old female. The histological characters of the two tumors were typical, and the transition from malignant AME to ACC was gradual. Immunohistochemistry was helpful in distinguishing these two types of malignant epithelial-myoepithelial tumors. Although most ACC has a more favorable clinical outcome, the component of high grade epithelial-myoepithelial carcinoma in this case might indicate a high frequency of recurrence and metastases.
Written informed consent was obtained from the patient and her family for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Adenoid cystic carcinoma
Emission computed tomography
Smooth muscle actin
Human epidermal growth factor receptor 2.
This work was supported by the National Natural Science Foundation of China (NO.81060189 to L.B), the young reserve talent of academic and technical of Yunnan (NO.2013HB086 to L.B.) and Doctor’s Foundation of the First Affiliated Hospital (NO.2013BS04 to L.B., NO.2013BS05 to Y.M.W.).
- Marian C, Boila A, Soanca D, Malau M, Podeanu DM, Resetkova E, Stolnicu S: Malignant transformation of adenomyoepithelioma of the breast by a monophasic population: a report of two cases and review of literature. APMIS. 2013, 121: 272-279. 10.1111/j.1600-0463.2012.02982.x.PubMedView Article
- Tavassoli FA, Eusebi V: AFIP atlas of tumor pathology (4th series): tumors of the mammary gland. 2005, Washington D.C: American registry of pathology
- Van Dorpe J, De Pauw A, Moerman P: Adenoid cystic carcinoma arising in an adenomyoepithelioma of the breast. Virchows Arch. 1998, 432: 119-122. 10.1007/s004280050144.PubMedView Article
- Lakhani SR, Hayes M, Eusebi V: Adenomyoepithelioma and adenomyoepithelioma with carcinoma. WHO classification of tumours of the breast. Edited by: Lakhani SR, Ellis LO, Schnitt SJ, Tan PH, van de Vijver MJ. 2012, Lyon: IARC, 122-123. 4
- Hayes MM: Adenomyoepithelioma of the breast: a review stressing its propensity for malignant transformation. J Clin Pathol. 2011, 64: 477-484. 10.1136/jcp.2010.087718.PubMedView Article
- Ahmed AA, Heller DS: Malignant adenomyoepithelioma of the breast with malignant proliferation of epithelial and myoepithelial elements: a case report and review of the literature. Arch Pathol Lab Med. 2000, 124: 632-636.PubMed
- Honda Y, Iyama K: Malignant adenomyoepithelioma of the breast combined with invasive lobular carcinoma. Pathol Int. 2009, 59: 179-184. 10.1111/j.1440-1827.2009.02347.x.PubMedView Article
- Han JS, Peng Y: Multicentric adenomyoepithelioma of the breast with atypia and associated ductal carcinoma in situ. Breast J. 2010, 16: 547-549. 10.1111/j.1524-4741.2010.00954.x.PubMedView Article
- Warrier S, Hwang S, Ghaly M, Matthews A: Adenomyoepithelioma with ductal carcinoma in situ: a case report and review of the literature. Case Rep Surg. 2013, 2013: 521417-PubMedPubMed Central
- Qureshi A, Kayani N, Gulzar R: Malignant adenomyoepithelioma of the breast: a case report with review of literature. BMJ Case Rep. 2009, 2009: pii: bcr01.2009.1442-
- Hungermann D, Buerger H, Oehlschlegel C, Herbst H, Boecker W: Adenomyoepithelial tumours and myoepithelial carcinomas of the breast–a spectrum of monophasic and biphasic tumours dominated by immature myoepithelial cells. BMC Cancer. 2005, 28: 92-View Article
- Simpson RH, Cope N, Skálová A, Michal M: Malignant adenomyoepithelioma of the breast with mixed osteogenic, spindle cell, and carcinomatous differentiation. Am J Surg Pathol. 1998, 22: 631-636. 10.1097/00000478-199805000-00015.PubMedView Article
- Kiaer H, Nielsen B, Paulsen S, Sørensen IM, Dyreborg U, Blichert-Toft M: Adenomyoepithelial adenosis and low-grade malignant adenomyoepithelioma of the breast. Virchows Arch A Pathol Anat Histopathol. 1984, 405: 55-67. 10.1007/BF00694925.PubMedView Article
- Pauwels C, De Potter C: Adenomyoepithelioma of the breast with features of malignancy. Histopathology. 1994, 24: 94-96. 10.1111/j.1365-2559.1994.tb01280.x.PubMedView Article
- Petrozza V, Pasciuti G, Pacchiarotti A, Tomao F, Zoratto F, Rossi L, Fontana A, Censi F, Sardella B, Di Cristofano C, Porta N, Della Rocca C: Breast adenomyoepithelioma: a case report with malignant proliferation of epithelial and myoepithelial elements. World J Surg Oncol. 2013, 11: 285-10.1186/1477-7819-11-285.PubMedPubMed CentralView Article
- Rasbridge SA, Millis RR: Adenomyoepithelioma of the breast with malignant features. Virchows Arch. 1998, 432: 123-130. 10.1007/s004280050145.PubMedView Article
- Trojani M, Guiu M, Trouette H, De Mascarel I, Cocquet M: Malignant adenomyoepithelioma of the breast: an immunohistochemical, cytophotometric, and ultrastructural study of a case with lung metastases. Am J Clin Pathol. 1992, 98: 598-602.PubMed
- Yoon JY, Chitale D: Adenomyoepithelioma of the breast: a brief diagnostic review. Arch Pathol Lab Med. 2013, 137: 725-729. 10.5858/arpa.2011-0404-RS.PubMedView Article
- Azoulay S, Laé M, Fréneaux P, Merle S, Al Ghuzlan A, Chnecker C, Rosty C, Klijanienko J, Sigal-Zafrani B, Salmon R, Fourquet A, Sastre-Garau X, Vincent-Salomon A: KIT is highly expressed in adenoid cystic carcinoma of the breast, a basal-like carcinoma associated with a favorable outcome. Mod Pathol. 2005, 18: 1623-1631.PubMed
- Mastropasqua MG, Maiorano E, Pruneri G, Orvieto E, Mazzarol G, Vento AR, Viale G: Immunoreactivity for c-kit and p63 as an adjunct in the diagnosis of adenoid cystic carcinoma of the breast. Mod Pathol. 2005, 18: 1277-1282. 10.1038/modpathol.3800423.PubMedView Article
- Boecker W, Buerger H: Evidence of progenitor cells of glandular and myoepithelial cell lineages in the human adult female breast epithelium: a new progenitor (adult stem) cell concept. Cell Prolif. 2003, 36 (Suppl 1): 73-84.PubMedView Article
- Weigelt B, Horlings HM, Kreike B, Hayes MM, Hauptmann M, Wessels LF, de Jong D, Van de Vijver MJ, Van’t Veer LJ, Peterse JL: Refinement of breast cancer classification by molecular characterization of histological special types. J Pathol. 2008, 216: 141-150. 10.1002/path.2407.PubMedView Article
- Trendell-Smith NJ, Peston D, Shousha S: Adenoid cystic carcinoma of the breast: a tumor commonly devoid of oestrogen receptors and related proteins. Histopathology. 1999, 35: 241-248. 10.1046/j.1365-2559.1999.00722.x.PubMedView Article
- Sanders ME, Kasami M, Means-Powell J, Page DL: Adenoid cystic carcinoma of the breast. Textbook of the uncommon cancer. Edited by: Raghavan D, Brecher ML, Johnson DH. 2006, New York: Wiley, 187-193. 3View Article
- Cakir A, Gonul II, U Luoglu O: A comprehensive morphological study for basal-like breast carcinomas with comparison to nonbasal-like carcinomas. Diagn Pathol. 2012, 7: 145-155. 10.1186/1746-1596-7-145.PubMedPubMed CentralView Article
- Persson M, Andrén Y, Mark J, Horlings HM, Persson F, Stenman G: Recurrent fusion of MYB and NFIB transcription factor genes in carcinomas of the breast and head and neck. Proc Natl Acad Sci U S A. 2009, 106: 18740-18744. 10.1073/pnas.0909114106.PubMedPubMed CentralView Article
- Vranic S, Bender R, Palazzo J, Gatalica Z: A review of adenoid cystic carcinoma of the breast with emphasis on its molecular and genetic characteristics. Hum Pathol. 2013, 44: 301-309. 10.1016/j.humpath.2012.01.002.PubMedView Article
- Ghabach B, Anderson WF, Curtis RE, Huycke MM, Lavigne JA, Dores GM: Adenoid cystic carcinoma of the breast in the United States (1977 to 2006): a population-based cohort study. Breast Cancer Res. 2010, 12: R54-10.1186/bcr2613.PubMedPubMed CentralView Article
- Thompson K, Grabowski J, Saltzstein SL, Sadler GR, Blair SL: Adenoid cystic breast carcinoma: is axillary staging necessary in all cases? results from the California cancer registry. Breast J. 2011, 17: 485-489. 10.1111/j.1524-4741.2011.01117.x.PubMedPubMed CentralView Article
- Coates JM, Martinez SR, Bold RJ, Chen SL: Adjuvant radiation therapy is associated with improved survival for adenoid cystic carcinoma of the breast. J Surg Oncol. 2010, 102: 342-347. 10.1002/jso.21638.PubMedView Article
- Khanfir K, Kallel A, Villette S, Belkacémi Y, Vautravers C, Nguyen T, Miller R, Li YX, Taghian AG, Boersma L, Poortmans P, Goldberg H, Vees H, Senkus E, Igdem S, Ozsahin M, Jeanneret Sozzi W: Management of adenoid cystic carcinoma of the breast: a rare cancer network study. Int J Radiat Oncol Biol Phys. 2012, 82: 2118-2124. 10.1016/j.ijrobp.2010.12.008.PubMedView Article
- Seifert G: Are adenomyoepithelioma of the breast and epithelial-myoepithelial carcinoma of the salivary glands identical tumours?. Virchows Arch. 1998, 433: 285-288. 10.1007/s004280050249.PubMedView Article
- Nadelman CM, Leslie KO, Fishbein MC: “Benign,” metastasizing adenomyoepithelioma of the breast: a report of 2 cases. Arch Pathol Lab Med. 2006, 130: 1349-1353.PubMed
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