[Fibroadenoma intracanaliculare phyllodes (cystosarcoma phyllodes) of the breast].
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Cystosarcoma phyllodes is a rare tumour of the breast which is notoriously difficult to diagnose accurately preoperatively. In this review we report the clinical, imaging and histopathological features of 20 patients who have presented in our Centre over a 10-year period.
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Cystosarcoma phylloides is characterized by frequent local recurrences, by questionable tumour status and by the often large size or extension. This very rare tumour is mentioned in the literature mostly as a case report. During 1974-1989 a group of 15 cases could be collected. Starting with the different pathological and anatomical features, the typical mammographic and sonographic criteria are described. The outline of the tumour is smooth and often lobulated. The solid component has echo levels lower than the surrounding breast tissue and is mostly homogeneous. Some parts may be cystic. Endotumoral calcifications are very rare. In all cases a zone of dorsal acoustic enhancement was seen. Differential diagnosis is discussed. In large breast tumours with slow and episodic growth, the tentative diagnosis cystosarcoma phylloides should be considered more often.
Thirty-three cases of phyllode cystosarcoma were diagnosed in the course of 21 years at the Tumour Department of the Berlin School of Medicine (Charité). Ten of them were found to be in sarcomatous degeneration. Metastasation was positively established in four of these ten cases, and locally delimited recurrences were recorded from two. Metastasation in cases of phyllode cystosarcoma has been found to occur rarely through regional lymph nodes. Therefore, routine removal of those lymph nodes is not considered necessary.
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The correlation between the histology and biologic behavior of phyllodes tumor was studied in order to establish the histologic criteria of this tumor, particularly its malignant counterpart. A preliminary analysis of five tumors with metastases revealed that a single essential indicator of malignant potential was the presence of a disproportionate overgrowth of the stromal elements at the expense of the ductal element. The presence or absence of such stromal overgrowth in the tumor showed a positive correlation with the degree of stromal atypism and the rate of mitoses. Seventeen out of 45 tumors were determined to be malignant, on the basis of our criteria. The arrangement of the stromal component in malignant phyllodes tumors was usually one of the particular sarcomatous types, with or without the focal areas of one of the specified types of sarcoma. On the other hand, 24 tumors recurred locally but never metastasized, and were preferentially interpreted to be benign phyllodes tumor. Histologically, these tumors had a general architecture resembling that of fibroadenoma but were characterized by high cellularity expressed by the distinct formation of bundles or fascicles of stromal tumor cells. The remaining four tumors seemed to form a borderline phyllodes tumor subset. A follow-up study revealed that, irrespective of the mode of therapy, the outcome of patients with phyllodes tumor was well correlated with the histologic criteria we proposed.
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BACKGROUND: Cystosarcoma phyllodes is a rare mesenchymal tumor in the breast with a quite different prognosis depending on its specific dignity. There are almost no data in the literature concerning therapeutic strategies of metastasized cystosarcoma phyllodes. PATIENTS AND METHODS: We report the unique case of a young female patient with advanced multiple liver metastases of a cystosarcoma phyllodes malignum. RESULTS: After irradiation of the total liver combined with simultaneous chemotherapy containing ifosfamide and adriamycin an impressing 12 months lasting remission and control of the liver metastases was achieved. No relevant treatment side effects were observed. CONCLUSION: Combined liver irradiation and simultaneous chemotherapy should be considered as effective treatment strategy for comparable situations in young patients with good physical conditions.
An unique case of high-grade phyllodes tumor (cystosarcoma phyllodes) arising in the seminal vesicle is reported. A 61-year-old man had symptoms of urinary obstruction, and ultrasonography and computed tomography showed a large mass posterior to the bladder. Using cystoprostatoseminovesiculectomy, a high-grade phyllodes tumor of the left seminal vesicle was found that did not involve the bladder or prostate. Histologically, the tumor consisted of benign irregular slit-like glands set in a mitotically active cellular sarcomatous stroma. The epithelium displayed intense cytoplasmic immunoreactivity with all keratin proteins (AE1/AE3, CAM 5.2, and high-molecular-weight keratin [clone 34 beta E12]). The stromal cells were strongly positive for vimentin, and approximately 30% were positive for muscle-specific actin and desmin. Four years after resection, a lung metastasis was removed, which was histologically and immunohistochemically identical to the seminal vesicle tumor. This case represents the malignant end of the range of phyllodes tumors (cystosarcoma phyllodes). Like similar tumors in the breast and prostate, phyllodes tumors of the seminal vesicle should be considered high-grade lesions (malignant) in which there is significant mitotic activity, stromal pleomorphism, and stromal overgrowth.
BACKGROUND: Cystosarcoma phyllodes (CP) is an uncommon fibroepithelial breast neoplasm that rarely involves the central nervous system (CNS). METHODS: The authors encountered two patients with CNS metastases and reviewed the literature to identify reports of CP affecting the nervous system. Patients must have been reported in sufficient detail to characterize clinical course, extent of tumor burden, and histologic type of the primary tumor and metastasis to be included in the study. RESULTS: Eleven cases were identified from the literature, but only six were presented in sufficient detail for analysis. Including the current patients, the mean age of patients with CP metastases was 52 years. The time between initial diagnosis and CNS involvement was 5 years. Parenchymal brain metastases respond poorly to treatment, with a mean survival of 29 days, whereas survival is significantly longer with CNS involvement secondary to epidural extension of tumor from adjacent bony structures. Local recurrence or widespread metastases nearly always were present at the time of CNS involvement. CONCLUSIONS: Brain metastases from CP are refractory to therapy and carry a dismal prognosis. Patients with CP and brain metastases should undergo brain biopsy to exclude metastases from associated epithelial breast tumors, which have significantly better prognoses. Patients with compressive lesions arising from bone have a better prognosis and may benefit from aggressive management.
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Cystosarcoma phyllodes is a rare breast lesion representing between 0.5 and 2.5 percent of all breast tumors. Its microscopic and clinical features are impressive. When first described by J. Mueller in 1838, the lesion was thought to be a benign neoplasm without metastatic potential. Nevertheless, more recent reports have shown either recurrent or metastatic cystosarcoma phyllodes. Some Authors have attempted to predict biologic behavior from histological appearance so that the extent of surgical management could be determined. However, only a small percentage of histologically malignant tumors have recurred or metastasized. The above observations stimulated the present analysis of the histology, clinical behavior, appropriate therapy and prognosis in ten patients with cystosarcoma phyllodes seen at our unit, from 1965 to 1979.
Thirty-five cases of benign cystosarcoma phyllodes, 13 cases of malignant cystosarcoma phyllodes, and ten cases of giant fibroadenoma were studied. The diagnosis of benign or malignant cystosarcoma phyllodes was based on a combination of histological features. Clinical and gross pathologic findings were not found to be useful in distinguishing between benign and malignant tumors. Giant fibroadenomas occurred primarily in black adolescents and were histologically distinct. Positive surgical margins were found to be the best predictor of local recurrence of benign or malignant cystosarcoma phyllodes. Systemic metastases occurred in only one case of malignant cystosarcoma phyllodes. Most benign and malignant cystosarcoma phyllodes may be treated by wide local excision with tumor-free margins. Giant fibroadenomas should be treated by simple excision to preserve normal breast tissue.
BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND: 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. METHODS: Incidence data were collected from 1972-1989 by the Cancer Surveillance Program, the population-based cancer registry for Los Angeles County. RESULTS: 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. CONCLUSIONS: 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.