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Ifosfamide is an active drug for chemotherapy of metastatic cystosarcoma phyllodes.

Metastases from cystosarcoma phyllodes are rare, and treatment generally is ineffective. Four patients were treated with ifosfamide (alone in three and combined with doxorubicin in one). Two patients had complete remissions that lasted 26 and 61+ months. One other patient had a partial response that lasted 13 months. The complete responders were both treated as soon as metastases appeared, when they had only a small volume of disease. This appears to represent a significant improvement on other described regimens for this condition, and further trials of ifosfamide are warranted. Close follow-up of patients at high risk for metastases is suggested.

Adult↗

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.

ATP Binding Cassette Transporter, Subfamily B, Mem↗

Cystosarcoma phyllodes--Asian variations.

Cystosarcoma phyllodes afflicting 106 female patients in a multiracial Asian society is reviewed and some interesting variations are noted when compared with Western patients. The incidence of phyllodes tumour is 3.83% of surgically removed breast tumours compared with 0.5-2.5% in Western series. This lends weight to the belief that non-Caucasians are more prone to this enigmatic breast disease than the Caucasian population. The profile of the Asian phyllodes patient is that of a young female, aged 25-30 years, whereas her Western counterpart is in her 40s. As a result, she is liable to be mistaken clinically as having fibro-adenoma. The disease is notably rare among the females below 20 years of age but in the Asian context, as much as one-quarter-one-third may be in the adolescent group. There were only three malignant and six borderline phyllodes tumours out of 106 cases, and this incidence is far below that of many other studies in which the malignant cases are believed to constitute 23-27%. However, this may not be a racial variation but explicable by the fact that the present study is an unselected cohort from a general surgical unit. The presence of associated benign breast disease in 25.5% cases helps to reinforce the widely held belief that fibro-adenoma and phyllodes tumour are related to some degree.

Adult↗

Multifocal histogenesis of a cystosarcoma phyllodes.

An unusual multifocal cystosarcoma phyllodes is reported. It presented as a small, circumscribed tumour mass, which was associated with two diffuse neoplastic lesions which arose sequentially in two geographically separate parts of the mammary disc. Microscopically the tumour appeared to have been enlarging by the formation of isolated satellite tumour nodules within the adjacent normal breast tissue, which represents a third, though rarer way in which a cystosarcoma phyllodes may enlarge.

Breast Neoplasms↗

Infiltrating ductal carcinoma developing within cystosarcoma phyllodes--a case report.

Malignancy in cystosarcoma phyllodes is uncommon and often confined to the stromal element. An extremely rare case of infiltrating ductal carcinoma developing within the stroma of cystosarcoma phyllodes is reported herein. A breast tumor with a diameter of 15 cm, which was diagnosed as cystosarcoma phyllodes, was excised from the right breast of a 47-year old woman. The histopathological examination revealed that hyperplastic ductal epithelial cells with dark cytoplasm and enlarged hyperchromatic nuclei were infiltrating the stroma. Thus, a diagnosis of ductal carcinoma within cystosarcoma was made. Subsequently, a standard radical mastectomy was performed. No recurrence or metastasis has been observed over the post-operative period of 5 years and 6 months.

Breast↗

Cystosarcoma phyllodes of the prostate: MRI findings.

Cystosarcoma phyllodes of the prostate is a rare, relatively benign sarcoma of the prostate. We describe the magnetic resonance imaging findings in an unusual case of cystosarcoma phyllodes which resulted in extensive local recurrence and sarcomatous degeneration. Although uncommon, radiologists should be aware of the existence of cystosarcoma phyllodes of the prostate.

Aged↗

Cystosarcoma phyllodes.

A series of 27 cystosarcoma phyllodes diagnosed in 23 patients is presented. Treatment consisted of wide local excision with a rim of normal tissue for the histologically benign-appearing tumors, and simple mastectomy for the histologically malignant-appearing tumors. Of the three tumors with a malignant appearance, one metastasized distantly and the patient died; the other two patients were apparently cured. Of the 24 benign-appearing tumors, 3 recurred locally and were cured by reexcision. None behaved in a malignant fashion. There was one case of bilateral cystosarcoma phyllodes, and one tumor was associated with carcinoma of the breast. The local recurrence rate of 11% (3 out of 27) and mortality rate of 4% (1 out of 23) appears to justify this therapeutic approach. The precise indications for mastectomy in this disease are still unclear.

Adolescent↗

Molecular assessment of clonality leads to the identification of a new germ line TP53 mutation associated with malignant cystosarcoma phyllodes and soft tissue sarcoma.

Cystosarcoma phyllodes (CSP) is a rare breast neoplasm composed of stromal and epithelial elements. It usually runs a benign course but it may metastasize. In a 31-year-old patient with recurring CSP, a mesenchymal tumor in the leg developed. The question arose whether the latter tumor could be a metastasis from the CSP, which would have major treatment consequences. The problem was addressed using molecular methods, i.e., comparison of the pattern of polymorphic repeat markers on chromosome 17p as well as single strand conformation polymorphism analysis and sequencing of exons 5 to 8 of the TP53 gene in both tumor and normal tissue. An identical pattern of loss of heterozygosity in both breast tumors was demonstrated, but a different pattern was shown in the tumor in the leg. This led to the conclusion that the latter tumor had to be a new primary tumor. A mutation in codon 162 of the TP53 gene was found in the tumor tissue as well as in the normal tissue of this patient. This germ line mutation leads to the replacement of isoleucine by asparagine and most likely has functional consequences. In all four examined tumors of this patient, the normal TP53 allele was lost. This is strong evidence that this germ line TP53 mutation causes the genesis of these two rare primary mesenchymal tumors in this young patient. The current study exemplifies the power of molecular diagnostic methods in investigating the specific clinical problem of clonal relation between two separate tumors. The germ line mutation found in codon 162 of the TP53 gene and the association with cystosarcoma phyllodes have not been described previously.

Adult↗

Treatment and outcome of cystosarcoma phyllodes in Brunei: a 13-year experience.

Cystosarcoma phyllodes is a rare tumour of the breast whose clinical behaviour does not correlate well with histological findings. The optimal treatment of this tumour remains controversial. A retrospective study on the treatment and outcome of women diagnosed with cystosarcoma phyllodes between 1986 and 1998 in Brunei was undertaken. Twenty-seven women were diagnosed over the 13-year study period. Follow-up was complete in 26 cases. The mean age at diagnosis was 35 years. There were 19 (73%) histologically benign lesions, 3 (12%) borderline lesions and 4 (15%) malignant lesions. The mean follow-up period was 37 months. Four patients (16%) had recurrences after surgery (1 benign, 1 borderline and 2 malignant lesions). Mean time to recurrence was 9 months. Breast-conserving surgery with adequate resection margin is advocated in benign and borderline lesions. For malignant lesions, simple mastectomy without routine axillary dissection is recommended. More research is required to determine the role of adjuvant chemotherapy and radiotherapy in the management of malignant cystosarcoma phyllodes [corrected].

Adolescent↗

Dumbbell metastatic cystosarcoma phyllodes of the heart and lung.

Cystosarcoma phyllodes is an uncommon breast tumor that rarely metastasizes. A case of dumbbell cystosarcoma phyllodes metastatic to the heart and lung diagnosed antemortem is described. The roles of diagnostic echocardiography and surgical excision are discussed. Atrial extension of a lung malignancy through a pulmonary vein is not an absolute contraindication to operation, but careful patient selection and operative planning are required.

Breast Neoplasms↗

Grading of cystosarcoma phyllodes by texture analysis of tissue architecture.

Conventional histologic grading of cystosarcoma phyllodes of the breast has not been entirely successful in the prognosis of recurrence or metastasis. Our study first developed a tumor grade classification based on computerized texture features and then compared the classification to conventional grading of these tumors. Evaluation of the tissue architecture of histologic sections was obtained by measuring nine texture features on an image analysis system. Forty cases of cystosarcoma phyllodes were studied. Each parameter was calculated on subimages of 128 x 128 pixels. This size resulted from a preliminary study that confirmed that the difference between texture primitives depends on the area of subimages. We also compared our series to a panel of 20 extramammary sarcomas. The results show that tissue architecture evaluated by texture analysis allows good discrimination between benign, borderline and malignant cystosarcoma phyllodes. Furthermore, extramammary sarcomas and malignant cystosarcoma phyllodes were discriminated well in most cases.

Breast Neoplasms↗

Cystosarcoma phyllodes tumors in men.

Cystosarcoma phyllodes (CSP) tumors of the breast are rare, comprising less than 1 per cent of all mammary neoplasms. CSP tumors have typically been regarded as predominantly benign, unilateral lesions of the female breast. However, sporadic cases of CSP have been reported in males. We present the first case of CSP in a man occurring in bilateral axillae and review the literature on CSP in males.

Axilla↗

Cystosarcoma phyllodes.

Ten cases of cystosarcoma phyllodes are reported along with review of literature. The pathological features are outlined and principles of management are discussed.

Adolescent↗

Steroid receptors in cystosarcoma phyllodes.

Seven cases of cystosarcoma phyllodes were studied. All but one of the patients were premenopausal. Histologic appearance permitted us to classify 5 as low-grade and 2 as high-grade tumors; 40% of the low-grade tumors were estrogen-receptor-positive (ER-positive), 100% were progesterone receptor-positive (PR-positive), and 60% glucocorticoid receptor-positive (GR-positive). In these tumors, mean PR values were 26.4 fmoles/mg protein; mean GR values were 27.2 fmoles/mg protein; mean ER values were 10,0 fmoles/mg protein. ER and PR were present in one of the high-grade tumors at slightly higher values than in low-grade tumors. GR was present in both high-grade tumors at a mean concentration of 275 fmoles/mg protein. Sucrose gradient analyses showed only the presence of 4S-specific receptors for ER and PR. GR receptors sedimented faster (6-7S).

Breast Neoplasms↗

Bilateral cystosarcoma phyllodes of the breast: a case report of malignant form with contralateral benign form.

Bilateral cystosarcoma phyllode is uncommon; bilateral tumors, either synchronous or metachronous, are generally either benign or malignant. We report one case of bilateral synchronous cystosarcoma phyllode in a 32-year-old pregnant woman. The left breast mass, measuring 21 x 17 x 10 cm, was classified as malignant cystosarcoma phyllode (high cellularity, stromal overgrowth, marked nuclear atypia, necrosis, mitotic rate = 4 mitoses/10 high power field, infiltrative margin). The right 9 x 9 cm mass was a benign cystosarcoma phyllode tumor (low to moderate cellularity, discrete nuclear atypia, mitotic rate = 1 mitoses/10 high power field, no necrosis, pushing margin). The patient had a left-sided mastectomy and a complete local excision with clear margin of the right breast mass. The patient is free of disease with a 17-month follow-up.

Adult↗

Stromal proliferations of the breast: an ultrastructural and immunohistochemical evaluation of cystosarcoma phyllodes, juvenile fibroadenoma, and fibroadenoma.

The stromal cells of three cystosarcoma phyllodes, five typical fibroadenomas, and one juvenile fibroadenoma were studied by light and electron microscopy. Immunohistochemical staining for the S-100 protein also was performed on tissues from each of the three categories. On ultrastructural examination, cells comprising the three varieties of lesions were similar. Cells with fibroblastic features predominated in all cases. Myoid differentiation was present in two cases, one of cystosarcoma and one of fibroadenoma. Junctional complexes were present in the cystosarcomas but not in the fibroadenomas. Basal lamina was focally present around stromal cells in the cystosarcoma phyllodes but was not evident around cells of the typical fibroadenomas or the juvenile fibroadenoma. Stromal cells of the fibroadenomas and the cystosarcoma phyllodes did not stain for S-100 protein. The results support the hypothesis that the proliferating cells in all three tumor categories are similar and have features of fibroblasts. The lack of staining for S-100 protein would suggest an origin different from the myoepithelia. The latter conclusion, however, must be interpreted with a degree of reservation as we have shown that not all myoepithelial cells stain with certain monoclonal antibodies directed against the alpha and beta chain of S-100 protein.

Adenofibroma↗

Giant cystosarcoma phyllodes of the prostate associated with adenocarcinoma.

Cystosarcoma phyllodes of the prostate is a rare neoplasm, occurring in adult men. It closely resembles the not uncommon tumor of the female breast and usually behaves in a similar manner. This case of benign cystosarcoma phyllodes of the prostate occurred in a 53-year-old man who presented with increasing abdominal girth and underwent exploratory laparotomy and removal of the 11.2-kg tumor. It was remarkable for its very large size and the presence of foci of well-differentiated adenocarcinoma, prostatic acinar type. The glandular epithelium of both the phyllodes tumor and the carcinoma were immunoreactive for cytokeratin, epithelial membrane antigen, prostate-specific antigen, and prostate-specific acid phosphatase. The presence of typical prostatic type adenocarcinoma and this immunoreactivity pattern strongly supports a prostatic origin for this rare neoplasm.

Adenocarcinoma↗