Malignant Cystosarcoma Phyllodes Research Articles

Extraskeletal myxoid chondrosarcoma: a case report high grade tumor in breast

Extraskeletal myxoid chondrosarcoma and breast sarcomas are rare tumors accounting for less than 3% of all soft tissue sarcoma and less than 1% of all malignant breast tumors. We report a case of aggressive histologic features of myxoid chondrosarcoma in breast. A 50-year-old female presented with a well-defined mass at left breast. Histological features reveal stromal cells proliferation with abundance epithelioid cells with high mitotic activity in variably sized nodular architectures in myxoid matrix. Immuno-histochemical studies show strongly positive for S-100 protein, scattered positive for EMA and focal immunoreactivity for cyto-keratins. CD 34 and Bcl-2 were negative, leading us to diagnose high-grade extraskeletal myxoid chondrosarcoma. High-grade extraskeletal myxoid chondrosarcoma is a rare primary tumor in breast which resembles malignant cystosarcoma phyllodes and metaplastic carcinoma.

Primary chondrosarcoma of the breast: a case presentation and review of the literature

Mammary sarcomas are uncommon tumors. When tumors like malignant cystosarcomaphyllodes and metaplastic carcinoma, where malignant cartilaginous areas may be present, are excluded, only nine cases have been reported to date.We report another case of primary chondrosarcoma of the breast here. A 24-year-old Mediterranean woman presented with a painful mass in the right breast and a physical examination revealed a palpable mass. An incisional biopsy was performed and primary chondrosarcoma was diagnosed based on histological examination. Our patient underwent a mastectomy. A preoperative clinical and cytological diagnosis of chondrosarcoma, even though possible in a few cases, is usually not attained due to its similarclinical behavior with other breast tumors.

Astonishingly rapid growth of malignant cystosarcoma phyllodes tumor in a pregnant woman— a case report

There are very few reports concerning the presence of malignant cystosarcoma phyllodes (CSP) in breasts of pregnant women. In the hereby described case, a 28-year-old woman presented in our department with huge (18 × 11 × 8 cm) tumor of left breast, 2 weeks after labor. The patient discovered a tumor in 34th week of pregnancy, 6 weeks before labor. Histopatholgic examination of excised tumor revealed the presence of malignant CSP tumor. Simple mastectomy was proposed to patient as a best treatment modality. However, the patient refused. She underwent excision of tumor bed (2-cm tumor-free margin was achieved). Despite insufficient treatment, she remains free of disease 20 months after the wide excision of breast malignancy. It is not known how pregnancy influences prognosis of patients with malignant CSP. Lack of such information prompted us to describe the clinical course of our patient.

Astonishingly rapid growth of malignant cystosarcoma phyllodes tumor in a pregnant womana case report

There are very few reports concerning the presence of malignant cystosarcoma phyllodes (CSP) in breasts of pregnant women. In the hereby described case, a 28-year-old woman presented in our department with huge (18 x 11 x 8 cm) tumor of left breast, 2 weeks after labor. The patient discovered a tumor in 34th week of pregnancy, 6 weeks before labor. Histopatholgic examination of excised tumor revealed the presence of malignant CSP tumor. Simple mastectomy was proposed to patient as a best treatment modality. However, the patient refused. She underwent excision of tumor bed (2-cm tumor-free margin was achieved). Despite insufficient treatment, she remains free of disease 20 months after the wide excision of breast malignancy. It is not known how pregnancy influences prognosis of patients with malignant CSP. Lack of such information prompted us to describe the clinical course of our patient.

Phyllodes Tumors

The purpose of this study is to formulate guidelines for proper surgical management ofphyllodes tumours. It is a disease often misdiagnosed by the pathologist and undertreated by surgeons. We have retrospectively studied 12 patients with histologically proven cystosarcoma phyllodes (CSP). There were 4 benign and 8 malignant lesions, with an incidence of 33.33% vs 66.66% respectively. The treatment was surgical in all patients. The extent of surgery ranged from wide local excision to Modified Radical Mastectomy (MRM). One patient with malignant CSP developed lung metastases (12.5%) and three had recurrences (37.5%). The exact recurrence rate can't be calculated because 5 patients lost follow-up. The results of this study indicates that local excision is safe for benign CSP and all patients with malignant CSP should be treated by mastectomy since there is a high recurrence rate with local excision (37.5 %).

Malignant Cystosarcoma Phyllodes of the Breast Treated with Oncoplastic Conservative Surgery

Malignant Cystosarcoma Phyllodes of the Breast Treated with Oncoplastic Conservative Surgery

Retrospective analysis of 25 women with malignant cystosarcoma phyllodes--treatment results.

Mastectomy without axillary dissection should be the standard treatment in patients with malignant form of cystosarcoma phyllodes. The role of postoperative radiotherapy and chemotherapy remains to be fully established. We evaluate treatment results in a group of patients with cystosarcoma phyllodes (CP) treated at our Institute. In this report we analyze treatment outcome in 25 patients with malignant cystosarcoma phyllodes treated at Masaryk Memorial Cancer Institute between 1970 and 1995. Mean tumor size was 10 cm in diameter. All patients underwent surgery. Subsequently, 17 patients (68%) received radiotherapy on the breast or chest wall. Median follow-up was 139.5 months. Local recurrence was observed in 16% of all patients and all patients with local recurrence died. Time to local relapse after surgery was 4-11 months. Distant metastases occurred in 5 patients. All patients with local recurrence had distant metastases. Dissemination occurred 3-19 months after local recurrence. Five-year survival of all patients was 80%. A specific protocol for the treatment of cystosarcoma phyllodes is missing, probably due to rarity of the disease. The treatment of local recurrent disease remains unsuccessful in most CP patients. We recommend postoperative irradiation on the chest wall in patients with malignant form of CP, because adjuvant radiotherapy decreased the incidence of local relapse in our group of patients.

Cystosarcoma Phyllodes Metastatic to the Mandible: Report of a Rare Case and Literature Review

Cystosarcoma phyllodes is a rare breast tumor with variable malignant potential. Metastasis has been reported in a small percentage of cases. We describe the case of a 52-year-old woman who developed a large facial tumor 1 year after she had undergone a mastectomy for a rapidly enlarging breast neoplasm. The facial lesion was found to be a malignant cystosarcoma phyllodes metastatic to the mandible, and the patient died shortly after diagnosis. To our knowledge, this patient represents only the third reported case of a phyllodes tumor metastatic to the mandible.

Surgical Management of Primary Breast Sarcoma

Primary sarcoma constitutes less than one per cent of breast malignancies. A retrospective review of this disease at our institution was undertaken to assess the effect of different treatment modalities on outcome. Over a 24-year period 28 patients were identified. Follow-up ranged from one to 228 months. Partial mastectomy was done in seven patients, whereas ten underwent total mastectomy and nine had modified radical mastectomy. Two refused surgery. All margins of resection were negative. In total ten axillary lymph node dissections were done with no positive nodes identified. Pathologic analysis of tumors revealed a variety of sarcomas including high-grade malignant cystosarcoma phyllodes in 13. Recurrence of disease occurred in two women, both with malignant cystosarcoma phyllodes. One was a local recurrence in a patient who had undergone partial mastectomy. This was successfully treated with a total mastectomy. The second recurrence involved a distant metastasis in a patient treated with modified radical mastectomy that eventually led to her death. For the entire group the disease-free survival was 75 per cent at 10 years whereas overall survival was 87.5 per cent. In conclusion an adequate margin of resection is the single most important determinant of long-term survival. Axillary lymph node dissection is not necessary for the treatment of these tumors.

P53 and Ki-67 expression as prognostic factors in cystosarcoma phyllodes.

We have reviewed the histopathological, clinical outcome and immunohistochemical status in 21 women with cystosarcoma phyllodes (CSP) tumors of the breast. We assessed 12 tumors as histopathologically benign and 9 tumors as malignant. The median patient ages in benign and malignant CSP tumors were 39.6 and 45.4 years of age, respectively. The stromal cellularity, stromal cellular atypism, high mitotic activity, atypic mitoses, stromal overgrowth, infiltrative tumor contour, and heterologous stromal elements were significant features of the malignant CSP tumors. Benign CSP tumors were predominantly of fibroadenomatous architecture with cellular stroma (mild or moderate) and some distortion and elongation of glandular elements. Five malignant CSP tumors were stained positively with p53, and 6 malignant CSP tumors were stained immunohistochemically with Ki-67. All benign CSP tumors were negatively stained for p53 and Ki-67. The patients with benign CSP tumors were treated with local excision ( n=11) and with subcutaneous mastectomy ( n=1). Malignant CSP tumors were treated with wide local excision ( n=1), partial mastectomy ( n=1), simple mastectomy ( n=2), and modified radical mastectomy ( n=5). Two patients with a high mitotic rate and high values of p53 and Ki-67 received additional radiotherapy and chemotherapy. One case had liver metastasis. This tumor had high mitotic figures, stromal overgrowth, severe stromal cellularity, and 20% Ki-67 and mild p53 positivity. We suggest that p53 and Ki-67 can play an important role in predicting prognosis and yielding additional therapy besides conventional prognostic factors in the treatment of the CSP patients.

Primary treatment of cystosarcoma phyllodes of the breast.

Cystosarcoma phyllodes is a rare sarcoma of the breast. Although surgical removal is the mainstay of treatment, the extent of surgery required (excision vs. mastectomy) and the need for additional local therapy, such as radiotherapy, are unclear. The current study evaluated the rate of local and distant failure, as well as potential prognostic factors, to better define appropriate treatment strategies. One hundred one patients treated primarily for cystosarcoma phyllodes of the breast were evaluated. These tumors were classified histologically into benign (58%), indeterminate (12%), and malignant (30%) based on well defined criteria. Stromal overgrowth (29%) was considered separately. Surgery was comprised of local excision with breast conservation (47%) or mastectomy (53%). Microscopic surgical margins were negative in 99% of cases. Six patients received adjuvant radiotherapy. Overall survival for the 101 patients was 88%, 79%, and 62% at 5, 10, and 15 years, respectively. For patients with nonmalignant (benign or indeterminate) and malignant cystosarcoma phyllodes, the overall survival was 91% and 82%, respectively, at 5 years, and 79% and 42%, respectively, at 10 years. Similar rates were observed based on the presence or absence of stromal overgrowth. Local recurrence occurred in 4 patients, with an actuarial 10-year rate of 8%. Eight patients developed distant metastases, with an actuarial 10-year rate of 13%. Multivariate analysis using Cox proportional hazards regression revealed stromal overgrowth to be the only independent predictor of distant failure. Local failure in this group of largely margin negative patients with cystosarcoma phyllodes of the breast was low, showing that breast-conserving surgery with appropriate margins is the preferred primary therapy. The current study data do not support the use of adjuvant radiotherapy for patients with adequately resected disease. Patients with stromal overgrowth, particularly when the tumor size was > 5 cm, were found to have a high rate of distant failure; such patients merit consideration of a trial that examines the efficacy of systemic therapy.

In vivo chemosensitivity of human malignant cystosarcoma phyllodes xenografts.

Malignant cystosarcoma phyllodes (MCSP) is a rare breast tumor. Chemotherapeutic regimens for treatment of MCSP have not been established. We previously established an MCSP xenograft line MC-3-JCK. In this study, we established a new MCSP xenograft line, MC-10-JCK, by serial transplantation in nude mice. We studied the chemosensitivity of these two MCSP tumor xenografts to anticancer drugs in vivo. We also examined the expression of multidrug resistance-related proteins such as p-glycoprotein (Pgp) and multidrug resistance-associated protein (MRP) by immunohistochemical analysis. These two xenografts were sensitive to doxorubicin, vincristine and cyclophosphamide in vivo. Immunohistochemically, clinical specimens and xenografts were negative for Pgp and MRP expression. These results are consistent with the chemosensitivity of human MCSP to lipophilic anticancer compounds.

An analysis of 78 breast sarcoma patients without distant metastases at presentation

Purpose: A retrospective review of a single cancer center experience was undertaken to identify clinical or treatment prognostic factors for these unusual tumors, to allow for a recommendation regarding management. Methods and Materials: The charts of 76 women and 2 men with breast sarcoma and without distant metastases at presentation registered from 1958 to 1990 were reviewed. Pathology was centrally reviewed in 54 cases. Histology, tumor size, grade, nodal status, age, menopausal status, history of benign breast disease, extent of surgery, resection margins, and radiation dose were each examined as potential prognostic factors by univariate analysis. To allow an analysis of radiation dose, total dose was normalized to a daily fraction size of 2 Gy. Results: The median age at diagnosis was 50.5 years (13–82 years). The pathologic diagnosis was found to be malignant cystosarcoma phyllodes in 32 patients, with the remainder being stromal sarcoma (14), angiosarcoma (8), fibrosarcoma (7), carcinosarcoma (5), liposarcoma (4), other (8). Eighteen patients had grade I or II tumors, 43 had grade III or IV, and 18 were not evaluable. The 5- and 10-year actuarial rates for all 78 patients were 57% and 48% for cause-specific survival (CSS), and 47% and 42% for the relapse-free rates (RFR), respectively. The local relapse-free rate (LRFR) was 75% at both 5 and 10 years. The 5-year CSS for grade I or II tumors was 84% versus 55% for grade III or IV tumors ( p = 0.01). Conservative surgery versus mastectomy did not lead to statistically significant different outcomes for CSS, RFR, or LRFR. The comparison of positive versus negative margins showed a 5-year LRFR of 33% versus 80% ( p = 0.009). Pairwise comparisons of the 5-year CSS of 91% for > 48 Gy versus either 50% for ≤ 48 Gy or 50% for no radiation showed p-values of 0.03 and 0.06, respectively. Conclusion: The authors propose that if negative surgical margins can be achieved, breast sarcoma should be managed by conservative surgery with postoperative irradiation to a microscopic tumoricidal dose (50 Gy) to the whole beast, and at least 60 Gy to the tumor bed. The decision to treat should be preceded by a preoperative multidisciplinary assessment. It is also recommended that an axillary lymph node dissection is not indicated, with the possible exception of patients with carcinosarcoma.

Immediate TRAM Flap Reconstruction of the Breast after Mastectomy for Cancer: A Thai Experience

: From January 1990 to June 1995, immediate transverse rectus abdominis myocutaneous (TRAM) flap reconstruction of the breast after mastectomy for cancer was performed in 40 patients with the objective of improving the quality of life. The 40 cases included one malignant cystosarcoma phyllodes (MCP), two intraductal carcinomas, four mucinous carcinomas (T1–2 NO), one lobular carcinoma (T2N1), and 32 invasive ductal carcinomas (T1–2 NO = 19, T3NO = 1, T1–2N1 = 9, T3N1 = 3). Modified radical mastectomy (MRM) was performed in all but one patient (MCP) who had subcutaneous mastectomy. A single pedicle TRAM flap was used for breast reconstruction in 36 cases and double pedicles in the remaining four cases. Since mid-1991, the mastectomy incision has been modified from elliptical incision to new skin-sparing incisions of various patterns depending on the tumor sites (n= 30). This change permitted a better cosmetic result and lessened the paresthetic area of the breast. Major complications were found in only 6 cases (20% skin flap necrosis in 1, major fat necrosis with discharge in 3, wound infection with foreign body in 1, and abdominal wound dehiscence in 1 case). The average postoperative hospital stay was 9 days (5–23 days). Adjuvant chemotherapy was given in 22 cases. All but two cases received the treatment within 1 month postoperatively (PO). Subsequent nipple reconstruction was accomplished in 18 cases using local skin flap in 16 and nipple sharing in 2 patients. There were two deaths from distant metastasis (one also had local chest wall recurrence). In the other cases no evidence of disease was found. The follow-up periods ranged from 6 to 72 months. From our experience so far, we have found that, in early breast cancer patients, MRM with a skin-sparing incision and immediate TRAM flap reconstruction does not compromise the oncological sound, but improves the cosmetic result, provided that the patients receive a full course of treatment as planned.

Fine needle aspiration cytology of malignant cystosarcoma phyllodes a case report with special reference to pitfall at cytology

Fine needle aspiration cytology of malignant cystosarcoma phyllodes a case report with special reference to pitfall at cytology

Malignant Cystosarcoma Phyllodes: A Review of the Clinical Experience at King Faisal Specialist Hospital and Research Centre

Twelve females with cystosarcoma phyllodes of the breast were referred to King Faisal Specialist Hospital and Research Centre (KFSH&RC) between 1980 and 1990, representing 0.8% of breast cancer patients seen during this period. Median age was 45 years (range 16 to 65 years). Seven patients (58%) were premenopausal. All patients presented with breast mass, which measured >10 cm in 58% of them. The median duration of symptoms was 15 months (range two to 174). Neither axillary lymph nodes nor distant metastases were seen at presentation. Surgery was the cornerstone of primary treatment (wide local excision in three and mastectomy in nine). Two patients received adjuvant chemotherapy and locoregional irradiation. All patients had malignant tumors histologically. Of the four tumors assayed for hormonal receptors, one was positive for estrogen and progesterone receptors. At a median follow-up of 17 months (range two to 77), four patients had relapsed; one died at 19 months and the projected five year survival is 83%. This limited data supports the observation that malignant cystosarcoma phyllodes is a distinctive clinicopathological entity of female breast cancer with a different natural history from carcinoma of the breast. Our survival results are similar to reported experience from the literature.

The descriptive epidemiology of malignant cystosarcoma phyllodes tumors of the breast

Malignant cystosarcoma phyllodes tumors of the breast are rare fibroepithelial tumors. The epidemiologic features of these malignancies in a defined population have not previously been described. Incidence data were collected from 1972-1989 by the Cancer Surveillance Program, the population-based cancer registry for Los Angeles County. The average annual age-adjusted incidence rate of malignant cystosarcoma phyllodes is 2.1 per 1 million women. Latina whites have a higher risk of this cancer than other racial-ethnic groups. The age-incidence curve shows that risk of cystosarcoma phyllodes peaks in the 45-49-year-old age group, but the age patterns vary by race-ethnicity, with Asian and Latina patients significantly younger, on average, than non-Latina white patients. The incidence rates of malignant cystosarcoma phyllodes were substantially higher in the 1980s than in the 1970s, particularly among Latina whites and Asians. For Latina whites, birthplace is a significant predictor of risk. Women born in Mexico and Central and South America are at threefold to fourfold greater risk of malignant cystosarcoma phyllodes tumors than Latina whites born in the United States. The epidemiology of malignant cystosarcoma phyllodes tumors of the breast is strikingly different from that of the more common histologic types of breast cancer. The high risk in Latina immigrants may offer an important clue as to the cause of this rare tumor.

Cystosarcoma Phyllodes

Cystosarcoma phyllodes (CSP) is an unusual fibroepithelial tumor of the breast. Characterized microscopically by stromal overgrowth and hypercellularity, CSP may originate in fibroadenomas or arise de novo. The distinction between benign and malignant CSP is based on a variety of histologic criteria; however, there is no reliable pathologic indicator of clinical behavior. Wide local excision of the tumor is the basis of the surgical treatment of CSP. Like other sarcomas, CSP has a tendency to recur locally. Metastatic disease usually is seen in association with malignant CSP. There is no known effective systemic therapy for this disease.

下顎に転移した乳腺悪性葉状嚢胞肉腫の１例

下顎に転移した乳腺悪性葉状嚢胞肉腫の１例

Metastatic Malignant Cystosarcoma Phyllodes of the Breast Occurring as Small Bowel Obstruction.

Metastatic Malignant Cystosarcoma Phyllodes of the Breast Occurring as Small Bowel Obstruction.