Rare Breast Malignancy Research Articles

Immediate Implant Breast Reconstruction with Acellular Dermal Matrix for Treatment of a Large Recurrent Malignant Phyllodes Tumor

Phyllodes tumors (PT) are rare fibroepithelial breast tumors representing less than 1% of all breast malignancies. These tumors are unpredictable and fast growing with a high local recurrence rate, making this disease challenging to treat. Previous literature focused on surgical resection, and breast reconstruction following a mastectomy in patients with PT is rarely addressed. We report a case of a recurrent malignant PT treated with a nipple-sparing mastectomy followed by immediate single-stage silicone implant breast reconstruction. While PT is a rare breast malignancy that presents challenges with both surgical resection and reconstruction, we demonstrate that nipple-sparing mastectomy with immediate implant breast reconstruction with AlloMax is curative and can offer an appealing cosmetic option. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Metaplastic breast carcinoma with osteogenic component

Metaplastic breast carcinomas (MBC) are rare primary breast malignancies characterized by the co-existence of carcinoma with non-epithelial cellular elements. They can be classified as monophasic spindle cell (sarcomatoid) carcinoma, biphasic carcinosarcoma, adenocarcinoma with divergent stromal differentiation (osseous, chondroid and rarely rhabdoid) as well as adenosquamous and pure squamous cell carcinomas. Nearly all reports portrait women in their forties or older, yet younger affected females in their twenties have been reported. The usual presentation included firm painless breast mass ranging between 1.4 and 14 cm in diameter, with no evidence of loco-regional or distal metastasis. Mammary osteosarcomas are aggressive tumors with a propensity for blood-borne rather than lymphatic spread. Metastatic disease expected to develop at a mean of 10.5 and 14.5 months from the initial diagnosis and demise followed within 20 months of the onset of metastasis. MBC remains a rare entity with poor response to both chemo-radiotherapy, histological diagnosis is challenging yet it is the main stay in outlining the management. The surgical option remains the only successful current treatment in the form of simple Mastectomy to achieve negative margins sparing the patients axillary node dissection.

Lessons Learned from an Unusual Case of Inflammatory Breast Cancer

Inflammatory breast cancer (IBC) is a rare breast malignancy that is associated with poor long-term outcomes despite aggressive surgical and chemotherapeutic interventions. We recently treated a 56-year-old woman with right-sided IBC and biopsy-proven cutaneous metastases to her back and left breast. She underwent chemotherapy, bilateral modified radical mastectomy, and radiation therapy. One year after diagnosis, she is currently disease-free based on positron-emission tomography (PET) imaging and repeat skin biopsies. To provide insight into the management of IBC, we present this interesting case with a reflection on important lessons to be learned.

Breast MRI as a Problem Solving Tool in Paget’s Disease of the Nipple

Paget’s disease of the nipple is a rare breast malignancy. It is usually, but not always, associated with an underlying ductal carcinoma in situ or invasive ductal carcinoma. Conventional breast imaging studies often fail to detect underlying breast malignancy when present. The following article will discuss the role of breast MRI as a problem solving tool in Paget’s disease of the nipple-areolar complex in patients with no abnormality on clinical examination and normal mammography. doi:10.4021/jcs80w

Double Feature: Carcinoma and Sarcoma Present in a Single Breast Tumor

Introduction. Primary breast sarcomas (PBSs) are rare nonepithelial breast tumors compromised of mesenchymal mammary tissue. Although its rare nature has made the best mode of PBS treatment difficult to determine, it seems better to treat it more like a sarcoma creating clear negative margins verses breast carcinoma utilizing lumpectomy, partial mastectomy, and total mastectomy. Case. A 47-year-old obese Caucasian postmenopausal female G2P2 presents with a breast lump demonstrating a histological sample with a biphasic pattern consistent with both ductal carcinoma containing typical malignant epithelial cells and sarcomatous differentiation of carcinosarcoma. Conclusion. Carcinosarcoma is a rare breast malignancy. Sarcomas of the breast tend to be negative for estrogen receptor and lack known risk factors. Current recommended treatment is to treat breast sarcomas like other soft tissue sarcomas by performing wide local excision instead of partial mastectomy. Antiestrogens and other chemotherapeutic agents typically used in breast epithelial malignancies are not recommended since these sarcomas tend to be negative with these receptors.

Phyllodes Tumors: Race-Related Differences

Phyllodes tumors (PT) are rare breast malignancies accounting for 0.5% to 1% of all breast tumors. PT have unpredictable behavior, with recurrence rates as high as 40%. A dearth of information exists about racial differences; elucidation of these differences is the objective of this study. A retrospective review of patients treated for PT at either Moffitt Cancer Center or University of Texas Health Science Center San Antonio from 1999 to 2010. Of the 124 patients, 71 (57%) were treated at Moffitt Cancer Center and 53 (42%) at University of Texas Health Science Center San Antonio. Mean age at diagnosis was 44 years (15 to 70 years). Thirty-three patients required mastectomy. Combining both cohorts, 42% of the patients were Caucasian, 43% were Hispanic, and 12% were black. Tumors were benign in 49% patients, borderline in 35%, and malignant in 16%, with a higher percentage of borderline and malignant tumors in Hispanic patients (p < 0.01). Hispanic patients tended to have larger tumors and higher mitotic rates (p = 0.01; p = 0.03). At a median follow-up time of 13 months, the local recurrence rate (6.4%) was associated with tumor size, tumor grade, mitotic rate, and close margin status (<2 mm) (p <0.01; p = 0.01; p = 0.01; p = 0.04). However, these findings did not translate into a survival difference by race. In this multi-institutional review of PT we found substantial pathologic differences by race with higher-grade tumors present more often in Hispanic patients. These differences did not substantially affect outcomes at short-term follow-up. Further investigation into additional molecular, biologic factors, geographic impact, and socioeconomic factors is needed to more clearly delineate this finding.

Sonographic Findings of Invasive Cribriform Carcinoma of the Breast

The purpose of this series was to evaluate the sonographic features of invasive cribriform carcinoma of the breast. Between 2004 and 2010, 3 patients had confirmed invasive cribriform carcinoma of the breast in our institution, and all of them underwent sonography. Sonograms showed masses with an oval (n = 2) or irregular (n = 1) shape, partially microlobulated (n = 2) or well-circumscribed (n = 1) margins, and a hypoechoic (n = 2) or an isoechoic (n = 1) internal echo texture. Sonographic assessments were classified as Breast Imaging Reporting and Data System category 4 in all 3 cases. Although invasive cribriform carcinoma is a rare breast malignancy, awareness of its sonographic features will be helpful for the diagnosis.

Secretory Carcinoma of the Breast

The purpose of this series was to evaluate the sonographic features of secretory carcinoma of the breast. Between 1994 and 2006, 9 patients had histologically confirmed secretory carcinoma of the breast in our institution, and 6 of them underwent breast sonography. We retrospectively evaluated the sonographic findings of the patients in correlation with other available images and reviewed the clinical records. Clinical manifestations were a palpable mass (n=3), a bloody nipple discharge (n=1), and screening-detected abnormalities (n=2). Breast sonograms showed masses with a round or oval (n=5) or tubular (n=1) shape, with relatively well-circumscribed (n=2) or partially microlobulated (n=4) margins, and with a hypoechoic (n=4) or an isoechoic (n=2) internal echo texture. Most lesions were single nodules (n=3) or groups of nodules (n=2) measuring 1 cm or smaller, except 1 mass measuring 3.5 cm with axillary lymph node metastasis. Two cases had associated ductectasia. Sonographic assessments were classified as Breast Imaging Reporting and Data System category 3 in 2 cases, category 4A in 3 cases, and category 4B in 1 case. Secretory carcinoma of the breast is frequently shown as a small benign-looking nodule or group of nodules or sometimes as an intraductal lesion with a low clinical stage on sonography. Although secretory carcinoma is a rare breast malignancy, awareness of its sonographic features will be helpful for the differential diagnosis.

Malignant Phyllodes Tumors: A Review of 752 Cases

Malignant phyllodes tumor (MPT) is a rare breast malignancy. Because of the scarcity of the disease, there are no evidence-based treatment or follow-up guidelines established. This study evaluated the survival of MPT patients to create recommendations for management. We identified 752 cases of malignant phyllodes tumors in the California Cancer Registry from the years 1988 to 2003. Relative survival was determined using Berkson-Gage life table analysis which was then compared with the relative survival of nonphyllodes breast cancer patients. For MPT patients, the relative annual survival at 1 year was 94 per cent and at 10 years was 99.6 per cent. Thus, after 10 years, these patients are no more likely to die than the general population. At 10 years, the relative cumulative survival of the MPT patients was 87.4 per cent, whereas the nonphyllodes breast cancer patients had only a 57.2 per cent relative cumulative survival. MPT patients with localized disease had a higher 10-year relative cumulative survival than those with regional disease (90.9% vs. 61.5%, P < 0.001). MPT has a good prognosis, particularly in patients with localized disease. After 10 years, MPT patients have no increased mortality relative to the general population. Clinicians should plan these patients' follow-up accordingly.

Metaplastic breast carcinoma: clinical–pathologic characteristics and HER2/neu expression

Metaplastic breast carcinomas (MBC) are rare primary breast malignancies characterized by the co-existence of carcinoma with non-epithelial cellular elements. They can be classified as monophasic spindle cell (sarcomatoid) carcinoma, biphasic carcinosarcoma, adenocarcinoma with divergent stromal differentiation (osseous, chondroid and rarely rhabdoid) as well as adenosquamous and pure squamous cell carcinomas. There is a paucity of information on clinically relevant pathologic features and clinical outcomes for these rare tumors. The aim of this study was to review the pathologic features and clinical outcomes of all cases of MBC seen at a single institution between 1971 and 2000. A computerized search of the Queen Elizabeth II Health Sciences Center (QEII HSC) surgical pathology files was performed for the years 1971-2000. Tumor blocks from identified cases were reviewed and immunohistochemistry was performed for estrogen and progesterone receptors (ER/PR), HER2/neu protein overexpression and cytokeratin profile. Clinical outcome information was obtained from hospital files and telephone contact with treating physicians. Twenty-six (26) cases were retrieved with only one case identified before 1990. All tumors were high grade with a median tumor size of 3.7 cm (range 1.4-9.5 cm). Thirteen cases had lymph node dissections available for evaluation, with 4 demonstrating nodal metastases. Five of 26 cases were ER positive within the adenocarcinomatous component, only two of which also expressed PR. Associated ductal carcinoma in situ (DCIS) was present in 11 cases. HER2/neu over-expression was seen in only one (1/26) adenosquamous carcinoma (3 + membranous staining of the malignant glandular component). At 23 months median follow-up, disease free survival (DFS) for the entire group was 53%. Although a rare breast cancer subtype, MBC is of considerable interest due to its pathological heterogeneity and differences in clinical behavior compared to typical breast carcinomas. Increasing pathologic recognition of MBC as a discrete entity is suggested by the number of MBC diagnoses in the last decade compared to previous years. The poor DFS associated with MBC suggests that further research exploring mechanisms of carcinogenesis and identifying clinically relevant prognostic factors is needed to direct optimum clinical care. Importantly, MBC variants appear to rarely overexpress the HER2/neu oncoprotein.

Risk Factors for Recurrence and Death After Primary Surgical Treatment of Malignant Phyllodes Tumors

Malignant phyllodes tumor (MPT) is a rare but aggressive breast malignancy. The aim of this study was to evaluate parameters that influence outcome in patients with MPT. Fifty women were diagnosed with MPT of the breast and treated between August 1971 and July 2000. All medical records were reviewed retrospectively. The Cox regression model was used for multivariate analysis. Tumors were classified as borderline (6%), low grade (32%), or high grade (62%). The median patient age was 46 years (range, 14-77 years). The median tumor diameter was 3.5 cm (range, 1.5-18 cm). Twenty-two patients had wide local excision (WLE), and 28 patients had mastectomy. The median follow-up was 91 months (range, 12-360 months). Local recurrence (LR) occurred in 16 patients (32%) an average of 26 months after surgery (median, 17 months; range, 3-72 months). Distant metastasis occurred in 13 patients (26%) at an average of 53.4 months (median, 36 months; range, 4-177 months). Sixteen (32%) patients have died of their disease. LR was significantly increased with stromal overgrowth (P < .0001), large tumor size (P = .0177), and surgical margins <1 cm (P = .0120), but not with WLE (P = .5099). Stromal overgrowth was the only independent variable predictive of systemic metastasis (P < .0001) and patient survival (P < .0001). Stromal overgrowth in MPT carries a grave prognosis. Close surgical margins and large tumor size, but not type of operation, significantly increased LR. Either WLE with adequate margins or mastectomy is an appropriate treatment for patients with MPT.

Adenoid Cystic Carcinoma of the Breast: Report of a Case

Adenoid cystic carcinoma of the breast is a rare breast malignancy. To our knowledge, only 140 cases have been reported in the English literature and no similar cases have been reported in Taiwan. A51-year-old woman presented with a left breast lump. During operation, the lesion was found to be a cystic mass with granulation growth. Frozen section of the excised tumor revealed infiltrating ductal carcinoma of the breast. She underwent Patey's type modified radical mastectomy. Pathological diagnosis was adenoid cystic carcinoma of the breast. She remains disease-free 25 months after operation, without adjuvant therapies.

Outcome and predictive factors of local recurrence and distant metastases following primary surgical treatment of high-grade malignant phyllodes tumours of the breast

Aim: High-grade malignant phyllodes tumour (MPT) is a rare but aggressive breast malignancy and forms approximately 25% of all phyllodes tumours. The aim of the study was to determine parameters that influence outcome in high-grade MPT. Methods: This study consisted of 48 women diagnosed with high-grade MPT. All patients were treated primarily with surgery by local excision (LE, margins <1 cm), wide local excision (WLE, margins ≥1 cm) or mastectomy. Cox's regression was used for multivariate analysis of the data. Results: The mean patient age was 47 (range 21–85) years and the average tumour size was 7.8 (range 1.5–20) cm. Ten patients were treated with LE, 14 with WLE and 24 with mastectomy. The median follow-up was 9 years (range 5 months–28 years). Local recurrence (LR) occurred in 19 patients (40%) at mean time of 28 (range 5–84) months after primary treatment. Distant metastasis (DM) occurred in 13 (27%) patients at average time of 25.6 (range 6–120) months. LR, subsequent metastatic spread and survival following treatment of MPT were related to tumour size and excision margins, but not to other clinical or histopathological characteristics. Conclusion: Tumour size and surgical margins were found to be the principal determinants of LR and DM. Complete surgical excision, by mastectomy if necessary, is important in the primary surgical treatment of high-grade MPT.

Invasive papillary carcinoma of the breast: mammographic appearance.

The mammographic findings in 18 patients with invasive papillary carcinoma were studied retrospectively. The mammograms of 10 patients showed a multinodular pattern, and seven patients had solitary nodules. One patient had an irregular, ill-defined mass in the retroareolar region. Two patients were found to have carcinoma in the contralateral breast, and two patients had intraductal carcinoma adjacent to the invasive papillary carcinoma. The varied mammographic features that may occur with this rare breast malignancy are discussed.