Management of adenoid cystic carcinoma of the breast: a Rare Cancer Network study.

Hôpital de Sion, CHCVs, Sion, Switzerland.
International journal of radiation oncology, biology, physics (Impact Factor: 4.26). 05/2011; 82(5):2118-24. DOI: 10.1016/j.ijrobp.2010.12.008
Source: PubMed


Mammary adenoid cystic carcinoma (ACC) is a rare breast cancer. The aim of this retrospective study was to assess prognostic factors and patterns of failure, as well as the role of radiation therapy (RT), in ACC.
Between January 1980 and December 2007, 61 women with breast ACC were treated at participating centers of the Rare Cancer Network. Surgery consisted of lumpectomy in 41 patients and mastectomy in 20 patients. There were 51(84%) stage pN0 and 10 stage cN0 (16%) patients. Postoperative RT was administered to 40 patients (35 after lumpectomy, 5 after mastectomy).
With a median follow-up of 79 months (range, 6-285), 5-year overall and disease-free survival rates were 94% (95% confidence interval [CI], 88%-100%) and 82% (95% CI, 71%-93%), respectively. The 5-year locoregional control (LRC) rate was 95% (95% CI, 89%-100%). Axillary lymph node dissection or sentinel node biopsy was performed in 84% of cases. All patients had stage pN0 disease. In univariate analysis, survival was not influenced by the type of surgery or the use of postoperative RT. The 5-year LRC rate was 100% in the mastectomy group versus 93% (95% CI, 83%-100%) in the breast-conserving surgery group, respectively (p = 0.16). For the breast-conserving surgery group, the use of RT significantly correlated with LRC (p = 0.03); the 5-year LRC rates were 95% (95% CI, 86%-100%) for the RT group versus 83% (95% CI, 54%-100%) for the group receiving no RT. No local failures occurred in patients with positive margins, all of whom received postoperative RT.
Breast-conserving surgery is the treatment of choice for patients with ACC breast cancer. Axillary lymph node dissection or sentinel node biopsy might not be recommended. Postoperative RT should be proposed in the case of breast-conserving surgery.

Download full-text


Available from: Kaouthar Khanfir, Jan 30, 2014
83 Reads
  • Source
    • "However, there is no randomized controlled trial comparing breast conserving surgery to mastectomy. Recent studies, however, have begun to report higher rates of lumpectomy (18), although, no information about surgical margins has been reported in the majority of patients. The role of radiotherapy following breast conservation surgery remains unclear. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Adenoid cystic carcinoma (ACC) is a rare malignant tumor of the breast that occurs in <0.1% of all patients diagnosed with breast cancer. The mean patient age at the time of diagnosis is 50-60 years. Typically, the tumor presents as a subareolar mass or as pain in the breast. While the radiological appearances of ACC are generally non-specific, the diagnosis can be made on fine-needle aspiration cytology. In the present study, a 58-year-old female patient was admitted to the Department of Radiation Oncology (Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey) with complaints of pain in the upper outer quadrant of the right breast. An excision biopsy of a lump in the upper outer quadrant revealed ACC, and perineural invasion was present. Subsequently, the patient underwent breast conservation surgery and sentinel lymph node dissection. Pathology from the second surgery depicted ACC in the form of microscopic foci around the initial surgical cavity, with two reactive sentinel lymph nodes and the closest negative margin at 2 mm. The patient was treated with radiotherapy following the surgery. No recurrence and metastasis were found after 20 months of follow-up. In conclusion, mammary ACC is a rare malignant neoplasm of the breast. Although surgery is the main treatment, the optimal adjuvant treatment of ACC of the breast has not yet been determined due to its low incidence.
    Oncology letters 05/2014; 7(5):1599-1601. DOI:10.3892/ol.2014.1945 · 1.55 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: The Rare Cancer Network (RCN), founded in 1993, performs research involving rare tumors that are not common enough to be the focus of prospective study. Over 55 studies have either been completed or are in progress. The aim of the paper is to present an overview of the 30 studies done through the RCN to date, organized by disease site. Five studies focus on breast pathology, including sarcoma, lymphoma, phyllodes tumor, adenoid cystic carcinoma, and ductal carcinoma in situ in young women. Three studies on prostate cancer address prostatic small cell carcinoma and adenocarcinoma of young and elderly patients. Six studies on head and neck cancers include orbital and intraocular lymphoma, mucosal melanoma, pediatric nasopharyngeal carcinoma, olfactory neuroblastoma, and mucosa-associated lymphoid tissue lymphoma of the salivary glands. There were 4 central nervous system studies on patients with cerebellar glioblastoma multiforme, atypical and malignant meningioma, spinal epidural lymphoma and myxopapillary ependymoma. Outside of these disease sites, there is a wide variety of other studies on tumors ranging from uterine leiomyosarcoma to giant cell tumors of the bone. The studies done by the RCN represent a wide range of rare pathologies that were previously only studied in small series or case reports. With further growth of the RCN and collaboration between members our ability to analyze rare tumors will increase and result in better understanding of their behavior and ultimately help direct research that may improve patient outcomes.
    Rare tumors 06/2012; 4(3-3):e35. DOI:10.4081/rt.2012.e35
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: The scientific literature to date lacks population-based studies on the demographics, clinical features, and survival of patients with adenoid cystic carcinoma (ACC) of different anatomic sites. The authors identified 5349 patients who had ACC of the major salivary glands (N = 1850), minor salivary glands (N = 2077), breast (N = 696), skin (N = 291), lung and bronchus (N = 203), female genital system (N = 132), and eye and orbit (N = 100) from the Surveillance, Epidemiology, and End Results (SEER) registry. Differences in demographics, clinical features, and survival of patients were assessed. ACC of the eye and orbit was associated with younger age at presentation (mean age,49.9 years). ACC of the skin or breast tended to present with less aggressive prognostic features, whereas ACC of the lung and bronchus or eye and orbit tended to present with more aggressive prognostic features. In a multivariate survival analysis of patients who presented with localized disease, patients with ACC of the breast (hazard ratio [HR], 0.40) or skin (HR, 0.40) had a significantly lower risk death than patients with ACC of the major salivary glands; whereas patients with ACC of the lung and bronchus (HR, 3.72) or the eye and orbit (HR, 3.67) had a significantly higher risk. For patients who presented with regional disease, the only clear prognostic difference in multivariate analysis was that patients with ACC of skin fared significantly better. The demographics and clinical features of patients with ACC differ by disease site. The current results indicated that site may be an important predictor of survival for patients who present with localized disease but is less important for patients who present with regional disease.
    Cancer 08/2012; 118(16):3945-53. DOI:10.1002/cncr.26740 · 4.89 Impact Factor
Show more