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Malignant Cystosarcoma phyllodes: report of two cases.

Two cases of malignant Cystosarcoma phyllodes seen in Jos University Teaching Hospital over a 9-year period are being reported. One occurred in a 16-year girl now dead and the other in a 52-year old woman probably dead. Both cases showed metastases, the axillary nodes being involved in the deceased, cervical nodes in the elderly patient, and both had pulmonary and presumably hepatic metastasis. These were the 2 cases seen out of a total of 696 surgical breast specimens reviewed thus constituting 0.3 percent. It is not certain if chemotherapy has been beneficial but one might need more cases well organised trials to arrive at definitive conclusions.

Adolescent↗

Cystosarcoma phyllodes. Diagnosis and management.

The diagnosis and management of cystosarcoma phyllodes is analyzed by reviewing the literature and presenting cases from our institution. This rare neoplasm of the female breast represents less than 1% of all breast tumors. Most patients present in the fourth and fifth decades of life. The predominant complaint is a palpable mass in the breast. Systemic manifestations occur after metastases have developed. Treatment is surgical excision. There is some controversy, however, over which type of surgical procedure should be performed. We recommend total mastectomy. Local recurrence and metastases do occur and have been related to inadequate surgery and various histological characteristics (mitotic activity, tumor margin, and stromal cellular atypia).

Breast Neoplasms↗

Malignant cystosarcoma phyllodes of the breast metastatic to the oral cavity: report of a case and review of the literature.

A case report involving an oral lesion that was found to be histologically identical to the stromal component of malignant metastatic cystosarcoma phyllodes of the breast is described. This represents the first reported histologically confirmed oral manifestation of an extremely rare breast tumor. The literature regarding oral manifestations of metastatic disease in general and of cystosarcoma phyllodes in particular is reviewed.

Breast Neoplasms↗

Cystosarcoma phyllodes in young women.

A total of eight cases of cystosarcoma phyllodes in women under 25 years of age were reported to the Swedish Cancer Registry during a ten-year period (1960 to 1969). Only one of these tumors was malignant. It was successfully treated with simple mastectomy and postoperative radiation therapy. Of the remaining seven cases, six were treated with local excision of the lesion and one with simple mastectomy. In no case, did the tumor recur during a follow-up of an average of ten years. Local excision seems to be sufficient for the benign form of the tumor, whereas simple mastectomy is indicated for the malignant form. Radical mastectomy is indicated only of the tumor has invaded the pectoral fascia. The tumor rarely metastasizes to the axillary lymph nodes.

Adolescent↗

Progesterone receptor in cystosarcoma phyllodes.

A specific receptor for progesterone has been found in a cystosarcoma phyllodes, as determined by charcoal adsorption and sucrose gradient analysis. Similar assays for estrogen receptors were negative. The tumor consisted almost entirely of stroma that contained the progesterone receptors. The epidemiology and natural history of cystosarcoma do not strongly support the hypothesis that it is controlled by female sex hormones, but the presence of the progesterone receptors suggests that some cystosarcomas are hormonally regulated, and thus may be responsive to therapeutic hormonal manipulation.

Aged↗

Metastatic cystosarcoma phyllodes: a light and electron microscopic study.

Two cases of metastatic cystosarcoma phyllodes, one in lung and the other in axillary lymph nodes, were studied with the light and the electron microscopes. The malignant element of these tumors appears to be a poorly differentiated mesenchymal cell. No evidence supporting the presence of epithelial cells was found. The tumor metastatic to the axilla exhibited intracellular virus-like particles similar to those described in adenocarcinoma of breast and other sarcomas.

Cell Nucleolus↗

Malignant cystosarcoma phyllodes with lymph node metastasis--a case report.

A case of malignant cystosarcoma phyllodes with metastasis in the interpectoral lymph node (Rotter's) is presented in this paper. To the author's best knowledge, this is the first case in Japan on this disease, with lymph node metastasis. Although the surgical management of the disease has not yet been standardized, radical or modified radical mastectomy is thought to be the appropriate procedure.

Biopsy↗

Recurrent and metastatic cystosarcoma phyllodes.

During a 30 year period (1950 to 1980), 42 patients with cystosarcoma phyllodes were seen at the Mayo Clinic. Ten patients (24 percent) had recurrence of metastasis. The malignant or metastatic potential could not be identified or predicted by histologically evaluating tumor contour, stromal atypia, or mitotic activity. Spread was chiefly by a hematogenous route, and no patient with metastasis survived. Five of the 10 patients died from their disease 2 to 7 years after treatment. Simple mastectomy appears to be the surgical treatment of choice.

Abdominal Neoplasms↗

Cystosarcoma phyllodes in adolescent girls and young women: a study of 45 patients.

Cystosarcoma phyllodes (CP) is an uncommon tumor in adolescent girls and young women. This study seeks to define the clinical and pathologic features of CP in this unusual clinical setting. Forty-five CPs (34 benign and 11 malignant) in prepubertal and adolescent girls and young women were studied. Classification of the tumors was based on the following morphologic features: stromal cellularity, nuclear atypia, mitotic activity, necrosis, and the nature of tumor borders. Surgical therapy was local excision or mastectomy. The age of the patients ranged from 10 to 24 years (mean 17.7). The tumors measured 1.4 cm to 10.2 cm at their widest point (mean 4.6). Both breasts were affected equally. Thirty-two patients were treated by local excision with or without reexcision and four patients by mastectomy. Follow-up was available for 36 patients for a mean of 58.4 months. Local recurrence was reported in six of the 36 cases (16%) (four benign and two malignant). The six patients with recurrent disease had infiltrative tumor borders and positive surgical margins microscopically. There was a single instance of systemic metastases from a high-grade malignant tumor with rhabdomyosarcomatous stromal differentiation and a high mitotic rate. At last follow-up, 34 patients were alive with no evidence of disease, one patient was alive with pulmonary metastases, and one patient died of an unrelated cause. We concluded that CP in adolescent girls and young women is not more aggressive than in older patients. Infiltrative tumor borders and positive surgical margins are the best histologic predictors for local recurrence. Mitotic activity is the most important criterion for assessing the metastatic potential. CP in this age group should be treated to maximize breast conservation. Mastectomy may be required to obtain clear margins for CPs that cannot be managed by excision because of large tumor size relative to breast volume.

Adolescent↗

Cystosarcoma phyllodes: a clinicopathological analysis of 14 cases.

Experience with 14 cases of cystosarcoma phyllodes encountered at the 2nd Department of Propedeutic Surgery, Athens, University, between October, 1928 and December, 1978 is reported. Local excision including a wide cuff of uninvolved breast tissue is indicated for histologically proven benign and small tumors. For bulky or malignant tumors, a simple mastectomy is indicated. none of these neoplasms had metastasized to the axillary lymph nodes and, for this reason, and axillary node dissection was contraindicated.

Adolescent↗

Metastatic cystosarcoma phyllodes. A report of 2 cases presenting with neurological symptoms.

Two cases of cystosarcoma phyllodes of the breast are presented with central nervous system (CNS) metastases appearing several years after mastectomy for the primary lesion. Unusual features in these cases include the widespread metastases themselves, neurologic symptomatology and CNS involvement, metastases into a uterine leiomyoma and into an area of hepatic adenomatous hyperplasia, and glomus-like structures in one of the primaries with similar structures resembling glomus cells in metastases. The distant metastases were of stromal cells only and frequently surrounded epithelial cells to isolate the indigenous glandular structures. The concept that cytosarcoma is a peculiar stromal neoplasm rather than a tumor of dual neoplastic origin is discussed.

Breast Neoplasms↗

Malignant cystosarcomas phyllodes of the breast in adolescent females.

The authors report a case of cystosarcoma phyllodes occurring in an adolescent female, thus raising to 19 the number of reported cases. The prognosis of those composite fibro-epithelial tumours is ruled by the malignancy of the connective component: the diagnosis shows no distinctive feature compared with what can be observed in adults. The prognosis (classically better) appears in fact totally similar, justifying the same therapeutic rigour, particularly in the surgical field. A multidisciplinary approach should ensure complete recovery while limiting the harmful effects of the therapy in very young women.

Adolescent↗

Cystosarcoma phyllodes of the breast: prognostic significance of proliferation and apoptosis associated genes.

BACKGROUND: Cystosarcoma phyllodes (CP) is a rare fibroepithelial tumor of the breast. So far no valuable prognostic factors are available for predicting the biological behaviour and often unexpected clinical outcome of this tumor. PATIENTS AND METHODS: Using immuno-histochemistry, Mib-1 evaluated proliferative activity and expression of p53, Bcl-2, c-erbB1, c-erbB2, cytokeratin and vimentin were analysed in 17 benign and 12 malignant CP. RESULTS: Cell proliferation was significantly higher in malignant tumors compared to benign ones both in the epithelial and mesenchymal parts. In the epithelial part Bcl-2 positive cells were found more frequently in benign than in malignant lesions. Positive immunoreactivity for p53 was seen in only one malignant case. The reaction was positive in the both epithelial and mesenchymal compartments. Expression of c-erbB-proteins was not found in any case. The intermediate filament proteins indicate clearly the two components of the tumor. CONCLUSIONS: We concluded that increased proliferate activity and downregulation of Bcl-2 may be associated with dedifferentiation of cells and malignant progression. C-erbB-proteins are obviously not involved in carcinogenesis of CP.

Apoptosis↗

Recurrent cystosarcoma phyllodes: a clinicopathologic study of 32 cases.

A retrospective study was made of recurrent mammary cystosarcoma phyllodes seen at Memorial Sloan-Kettering Cancer Center from 1932 to 1972. Clinicopathologic findings, clinical course, treatment, and survival are described. It is apparent that cystosarcomas may recur and several years may elapse between reucrrences. We found that 18% of the benign and 8% of the malignant cystosarcomas recurred and malignant "transformation" occurred in two of the 28 recurrent benign cystosarcomas.

Adult↗

Surgical cure of hypoglycemia associated with cystosarcoma phyllodes and elevated nonsuppressible insulin-like protein.

A woman with a large, benign cystosarcoma phyllodes of the breast was found to have recurrent, profound hypoglycemia. Plasma levels of nonsuppressible insulin-like protein were increased (15.18 micrograms/ml; normal = 1 to 4 micrograms/ml), whereas levels of insulin and nonsuppressible insulin activity soluble in acid ethanol were normal. Surgical excision of the tumor resulted in immediate and lasting correction of the metabolic abnormalities, although nonsuppressible insulin-like protein levels did not completely return to normal. Surprisingly, preoperative nonsuppressible insulin-like protein levels were highest in blood distant from the tumor site.

Adult↗