Phyllodes Tumor of the Breast
To better identify prognostic factors for local control and survival, as well as the role of different therapeutic options, for phyllodes tumors, a rare fibroepithelial neoplasm of the breast.
Data from 443 women treated between 1971 and 2003 were collected from the Rare Cancer Network. The median age was 40 years (range, 12-87 years). Tumors were benign in 284 cases (64%), borderline in 80 cases (18%), and malignant in 79 cases (18%). Surgery consisted of breast-conserving surgery (BCS) in 377 cases (85%) and total mastectomy (TM) in 66 cases (15%). Thirty-nine patients (9%) received adjuvant radiotherapy (RT).
After a median follow-up of 106 months, local recurrence (LR) and distant metastases rates were 19% and 3.4%, respectively. In the malignant and borderline group (n = 159), RT significantly decreased LR (p = 0.02), and TM had better results than BCS (p = 0.0019). Multivariate analysis revealed benign histology, negative margins, and no residual disease (no RD) after initial treatment and RT delivery as independent favorable prognostic factors for local control; benign histology and low number of mitosis for disease-free survival; and pathologic tumor size < or = 3 cm and no tumor necrosis for overall survival. In the malignant and borderline subgroup multivariate analysis TM was the only favorable independent prognostic factor for disease-free survival.
This study showed that phyllodes tumor patients with no RD after treatment have better local control. Benign tumors have a good prognosis after surgery alone. In borderline and malignant tumors, TM had better results than BCS. Thus, in these forms adjuvant RT should be considered according to histologic criteria.
Available from: Alessandra Franzetti Pellanda
- "Some RCN series are the largest worldwide or the second largest in their field, like for example those on phyllodes tumors of the breast (443 patients), solitary plasmocytoma (258 patients), pediatric nasopharyngeal tumors (165 patients), mandibular osteosarcoma (111 patients), primary bone lymphoma (116 patients), and several other disease sites. See Table 3 for a summary of the five largest studies published.4-8 Of all completed studies, the most frequent tumor sites or tissues were the CNS/eye (8), the soft tissue/bone (8), the male genito-urinary system (7), the head and neck (6), the breast (6), the female genito-urinary system (4) and the remainder in other various sites, as shown in Figure 2. Of interest, there were 8 published papers on extra-nodal NHL, the majority of these were amongst the largest in the literature. "
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ABSTRACT: Approximately, twenty years ago, the Rare Cancer Network (RCN) was formed in Lausanne, Switzerland, to support the study of rare malignancies. The RCN has grown over the years and now includes 130 investigators from twenty-four nations on six continents. The network held its first international symposium in Nice, France, on March 21-22, 2014. The proceedings of that meeting are presented in two companion papers. This manuscript reviews the history of the growth of the RCN and contains the abstracts of fourteen oral presentations made at the meeting of prior RCN studies. From 1993 to 2014, 74 RCN studies have been initiated, of which 54 were completed, 10 are in progress or under analysis, and 9 were stopped due to poor accrual. Forty-four peer reviewed publications have been written on behalf of the RCN.
Rare tumors 07/2014; 6(3):5462. DOI:10.4081/rt.2014.5462
Available from: Anthony P Conley
- "The case reported herein has several clinical features typical of metastatic malignant phyllodes tumor. Previous published reports have reported a median age at diagnosis of 50 years, the time to development of metastatic lesions between 12 and 24 months after surgery , and a predominance of metastasis to the lungs [7,9,10]. Moreover, this patient presented with well-characterized risk factors for the development of metastatic disease, including the presence of stromal overgrowth, mastectomy at initial surgery, larger tumor size, and high mitotic index . "
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ABSTRACT: Phyllodes tumors are uncommon breast tumors that account for less than 0.5% of all breast malignancies. After metastases develop, the prognosis is poor, with very few patients living more than 1 year. The biology of this unusual cancer is not understood and, consequently, no potential targets for treatments are currently available. There has been an exponential increase in the number of commercially available tumor profiling services. Herein, we report a case of metastatic malignant phyllodes tumor for which a comprehensive molecular analysis was performed by using Clinical Laboratory Improvement Amendments (CLIA)-certified labs, providing new insights into the potential opportunities for molecularly targeted therapies for this extremely rare disease.
Next-generation sequencing was performed by using the FoundationOnetm platform (Foundation Medicine, Cambridge, MA). Whole-genome array-based comparative genomic hybridization (array CGH) was performed by using the DNAarraytm (CombiMatrix Diagnostics, Irvine, CA). Immunohistochemical and morphoproteomics analysis were performed at Consultative proteomics(R),The University of Texas, UT Health Medical School, Houston,TX (Robert E Brown Lab); Clarient Diagnostics, Aliso Viejo, CA; and Caris Life Sciences Target one, Irving, TX, USA.
Next-generation sequencing showed 3 aberrant genes: activating mutation Q61L on NRAS; inactivating mutations Q504* and K740* on RB1; and TP53 loss. Whole-genome array-based comparative genomic hybridization (array CGH) revealed amplifications of chromosome (chr.) 1 (CKS1B gene), chr. 8 (MYC gene), and chr. 9 (CDKN2A gene) Deletions of chr. 17 (TP53), chr. 10 (GATA3), chr. 11 (FGF4 and CCND1 genes), and chr.22 (PDGFbeta). Immunohistochemical analysis for relevant markers showed a positive staining for transducing-like enhancer of split (TLE) 3; secreted protein acidic and rich in cysteine (SPARC) was expressed at 2-3+ in the cytoplasm of the tumors cells, whereas mammalian target of rapamycin (mTOR) was expressed up to 2+ in the nuclei of the tumor cells.
We describe for the first time an NRAS mutation with concomitant activation of PIK3/Akt/mTOR in phyllodes tumor. We also found markers for sensitivity to taxane-based therapies, especially albumin-bound paclitaxel. Exploring the biology of rare malignancies by CLIA certified labs may be reasonable strategy for the development of targeted treatments.
Orphanet Journal of Rare Diseases 07/2013; 8(1):112. DOI:10.1186/1750-1172-8-112 · 3.36 Impact Factor
Available from: Samer Sawalhi
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ABSTRACT: The challenging issue for the breast surgeons is local recurrence of phyllodes tumor. The histological criteria to predict local recurrence has been a controversial issue. The objective of this study was to determine pathological parameters and surgical margins that influence outcome of local recurrence and distant metastasis in phyllodes tumor (PT).
Retrospective review between January 2003 to August 2008 at King Hussein Cancer Center-Jordan.
Forty-two female patients diagnosed as having PT were classified to benign, borderline and malignant. The medical records were reviewed in relation to the surgical management, recurrence, follow-up, the histological features of the tumor and grading of tumors based on the following histological parameters: mitotic count, stromal cellularity, stromal overgrowth, cellular pleomorphism, nuclear grade, tumor necrosis, tumor margin, and surgical margin status. All patients underwent wide local excision of the tumor or mastectomy.
Forty-two patients with PT (16 benign, 9 borderline, 17 malignant PT) were followed up for 30 months. The mean age was 39.8 years, and the average tumor size was 6.6 cm. The recurrence rate of PT in our study was 21% at a mean time of 11 months. Nine patients had local recurrence; 2 benign, 6 malignant and 1 borderline. Cellular pleomorphism had correlation with recurrence rate (P=.045). We had six patients (14%) with distant metastasis. All had malignant PT. Metastasis in PT has a relationship with histological grade (P=.02).
We conclude that patients with moderate and severe cellular pleomorphism had higher local recurrence, while metastatic PT occur more in patients with high nuclear grade.
Annals of Saudi medicine 03/2013; 33(2):162-8. DOI:10.5144/0256-4947.2013.162 · 0.49 Impact Factor
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