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A 30-year-old woman presented with breast masses. One of the breast masses was clinically suggestive of cystosarcoma phyllodes. Excision of the breast masses revealed bilateral cystosarcoma phyllodes. A few weeks later another lump appeared in the left groin, and this on excision was found to be cystosarcoma phyllodes at an aberrant site. We consider it a unique clinical presentation of cystosarcoma phyllodes.
A series of 17 females having cystosarcoma phyllodes underwent surgical treatment. The subsequent clinical course was observed periodically. Twelve patients had originally histologically benign tumors. Six patients had a recurrence, and of these, malignant characteristics developed in four instances. Five patients had originally histologically malignant tumors. Recurrences were encountered in four. Five patients died from a generalized spread of the tumor. Three of these patients had originally histologically benign tumors, in which malignant features of the recurrent tumor subsequently developed. The surgical treatment ranged from local excision to simple mastectomy with additional roentgenotherapy in three. Neither procedure prevented a recurrence from the original tumor, whether benign or malignant. Poor correlation exists between biologic behavior and histologic appearance of cystosarcoma phyllodes, independent of the surgical procedure. Wide local excision in histologically benign and mastectomy in histologically malignant tumors are advised, but no final decision could be reached. It is proposed to restrict ther term cystosarcoma phyllodes to histologically malignant tumors and use the term fibroadenoma phyllodes in instances of histologically benign tumors.
Cystosarcoma phyllodes is a very rare tumor which maybe difficult to diagnose clinically. The epidemiology and pathobiology are different from those of breast carcinoma. Risk factors, multicentricity, bilaterality, as associated with breast carcinoma, are not observed in patients with cystosarcoma phyllodes. Although the term "sarcoma" indicates a malignant tumor, only 10%-30% of cystosarcomas are histologically diagnosed as malignant; clinical diagnosis of malignancy does not exceed 10%. Axillary node involvement is rare, but hematogenous spread of cystosarcoma occurs into lung, pleura, bone, and liver. Clinically, cystosarcoma is a large (usually 3-5 cm in diameter) painless tumor with sudden growth acceleration especially during pregnancy. Cystosarcoma is usually circumscribed, containing firm and soft areas. The differential diagnosis has to include fibroadenoma, fibrocystic disease, mastitis, abscess, and medullary carcinoma. Neither clinical, mammographic or sonographic signs exist to predict a benign or malignant tumor. Therapy of cystosarcoma is not uniformly agreed upon. Radical, modified-radical, and simple mastectomy and tumorectomy are typical treatments; therapeutic results are the same for each treatment modality. For histologically diagnosed malignant cystosarcoma, the relative 5-year survival rate is about 80%. Clinically, malignant metastatic cystosarcoma is incurable; radiotherapy, endocrine treatment, and polychemotherapy are all ineffective. Because of the specific tumor pathobiology of cystosarcoma and its rarity, evaluation of treatment modalities and comparison of survival rates are difficult.
PURPOSE: 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. PATIENTS AND METHODS: 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. RESULTS: 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%. CONCLUSION: 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.
Predicting the biologic behavior of cystosarcoma phyllodes of the breast by histopathologic examination has been difficult. Flow cytometric DNA and cell cycle analysis was performed for 30 patients with cystosarcoma phyllodes of the breast to determine its potential prognostic utility. Traditional histopathologic parameters and the patients' clinical outcome with a minimum follow-up period of at least five years were also analyzed. Of all the variables examined, DNA ploidy, proliferative index, number of mitoses, and tumor margin were significantly associated with an adverse clinical course. Multivariate regression analysis of the authors' data showed that DNA content was a significant predictor of the patients' clinical outcome. The authors conclude that the DNA ploidy study is a useful, objective adjunct to the clinicopathologic assessment of cystosarcoma phyllodes of the breast.
A liposarcoma arising within a cystosarcoma phyllodes in a 17-year-old black female was treated by total mastectomy without the need of radiation and/or chemotherapy. The patient is alive and well with no evidence of recurrence or metastasis at 20 months recent follow-up. The clinicopathological features of malignant cystosarcoma phyllodes-liposarcoma are discussed.
An extremely rare case of coexistent carcinoma arising in a cystosarcoma phyllodes of the breast is reported. A 41-year-old Japanese woman was referred with a lump in the right breast. A tumor measuring 5.6 X 3.6 X 3.2 cm was removed by local excision. Histological examination revealed foci of papillo-tubular carcinoma within the benign cystosarcoma phyllodes. Radical mastectomy was performed and the axillary lymph nodes showed no metastasis. While 22 cases of coexistent carcinoma and cystosarcoma phyllodes in the same breast have been reported, in only nine of those cases, the carcinomas including this case have developed within the cystosarcoma phyllodes.
A 59-year-old patient, who had had recurring giant fibroadenomas and cystosarcoma phyllodes over a 36-year period, presented with cystosarcoma phyllodes and adenocarcinoma of the breast. Only seven other well documented instances of the coexistence of these lesions have been reported. The prognosis and treatment of adenocarcinoma was not affected by coexisting cystosarcoma phyllodes.
The fine structure of the stomal component of cystosarcoma phyllodes was compared with that of intracanalicular fibroademona, leiomyoma and leiomyosarcoma. The stromal cells of fibroadenoma was intermediate between fibroblasts and smooth muscle cells and may be designated as myofibroblasts. The stromal cells of cystosarcoma phyllodes have fulfilled the ultrastructural criteria for smooth muscle cells. Therefore, cystosarcoma phyllodes should be regarded as smooth muscle tumors or those of myoepithelial origin.
Cystosarcoma phyllodes of the breast appears to encompass a diverse group of tumors with variable clinical behaviors. Although the tumor can behave in a malignant fashion, metastases to the head and neck region are distinctly uncommon. A case is presented of a solitary metastasis to the mandible appearing 1 year after mastectomy and in the absence of widespread disease. The metastatic potential of this neoplasm is discussed and involvement of the head and neck region is reviewed.
Cystosarcoma phyllodes is a rare predominantly benign tumour usually affecting the female breast. In a retrospective review of giant breast masses seen over a period of 20 years, we encountered two cases of this tumour, one benign and the other malignant, the latter exhibiting the very unusual feature of axially lymph node metastases. We present these two cases and briefly review the literature.
Cystosarcoma phyllodes is a rare neoplasm of the breast. The tumor contains both stromal and epithelial elements. Local excision or simple mastectomy is usually curative. Most recurrences are local and can be controlled with excision. We treated a 23-year-old woman in whom disease had metastasized to the lungs with six courses of doxorubicin hydrochloride and cisplatin at three-week intervals and achieved complete remission.
BACKGROUND: We report here an extremely rare case of cystosarcoma phyllodes of the seminal vesicle. METHODS: A 65-year-old man presented with urinary hesitancy, frequency and constipation. Clinical examinations including two needle biopsies were performed, and the patient had undergone open surgery. RESULTS: The final pathological diagnosis was cystosarcoma phyllodes of the seminal vesicle. Seven months after the operation, a chest X-ray showed lung metastasis, and the patient died 11 months after the operation. CONCLUSION: To our knowledge, only one case of cystosarcoma phyllodes of the seminal vesicle has been previously reported.
Cystosarcoma phyllodes is a rare breast tumor and only five cases have been previously reported in males. A case of cystosarcoma phyllodes in a man treated with polyestradiolphosphate for prostatic carcinoma and by radiation because of breast tenderness is presented. The tumor showed a malignant stroma histologically and the epithelial component was similar to a ductal carcinoma in situ. Electron microscopy revealed several cell types. The possible relationship to radiation and estrogen therapy is discussed.
Electron microscopic studies were conducted on 11 cases of cystosarcoma phyllodes (6 benign, 3 malignant, 2 recurrent), 1 stromal sarcoma, and 15 cases of fibroadenomas. Particular emphasis was placed on examination of the epithelial-stromal junction. The epithelial cells were basically similar in all cases. The basal lamina of fibroadenomas tended to be reduplicated, whereas in cystosarcoma phyllodes the basal lamina was less reduplicated and even focally absent. A continuous layer of delimiting fibroblasts was not present in either type of tumor. Stromal cells were generally fibroblasts, with 2 of 15 being fibroadenomas and 2 of 11 cystosarcoma phyllodes showing myofibroblastic differentiation. The stromal sarcoma also showed smooth muscle differentiation. The authors conclude that the epithelial-stromal junction is abnormal in both fibroadenomas and cystosarcomas, but the abnormality is different in these two tumors. Increasing prominence of stromal cell junctions and of lysosomes also correlated with the diagnosis of cystosarcoma, particularly the malignant variant.
Cystosarcoma phyllodes is a rare tumor. The histologic criteria used for its classification are controversial, and usually do not correlate with the clinical behavior. In this article, we present an unusual case of metastatic cystosarcoma phyllodes, and review the literature concerning this rare entity.
Only three cases of carcinoma infiltrating the stroma of cystosarcoma phyllodes have been reported. An additional case is presented. Unlike the treatment of cystosarcoma phyllodes which is limited to complete removal of the tumor, carcinosarcomas should be treated in the same manner as carcinoma by mastectomy and axillary dissection.
Clinicopathologic characteristics were evaluated in 20 cases of cystosarcoma phyllodes in relation to clinical outcome. Flow cytometric DNA studies were carried out in 15 of these 20 cases. Stromal overgrowth and an infiltrating tumor border emerged as prominent histopathologic features that were associated with an unfavorable clinical outcome. Flow cytometric S fractions greater than 0.05 were associated with poor clinical outcome. There was no correlation between tumor ploidy and clinical outcome. There were no direct correlations between clinicopathologic features and flow cytometric measurements. Our data suggest that flow cytometric S fractions may be a useful predictor of clinical outcome in cystosarcoma phyllodes that can complement the traditional histologic analysis of these rare breast tumors.