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The presence of a malignant cystosarcoma phyllodes in one breast and a scirrhous carcinoma in the opposite breast in a premenopausal Black woman is described. Malignant cystosarcoma phyllodes is uncommon and its rare association with carcinoma of the breast is mentioned.
A case of cystosarcoma phyllodes with liposarcomatous stroma is presented. Electron-microscopic study revealed lipid-containing tumor cells admixed with other mesenchymal cells devoid of fat, correlating well with the light-microscopic findings. The literature is briefly reviewed.
A case of malignant cystosarcoma phyllodes of the prostate is reported in a 45-year-old male. This tumor was composed of benign columnar or squamous cystic folds and sarcomatous stroma including rhabdomyomatous elements. The prostatic origin of the tumor was clearly proved by the unlabeled immunoperoxidase method.
Clinical and morphological characteristics of two malignant cystosarcoma phyllodes mammae were reported with the review of recent literature. One of the patients went to see her doctor 2 years after she had noticed the tumour because of "problems in her family". In the removed giant fibroadenoma osteo-chondrosarcomatous transformation was found. The other patient tried "to mobilise her inner energies against the growth" for 3 years. The removed tumour weighed 2100 grams and there were metastases in the axillary lymph nodes. Histological examination showed malignant cystosarcoma phyllodes with vasoformative tendency. Electron microscopical studies confirmed in both cases that the tumors with varying structures originated from immature mesenchymal cells. Authors agree on wide extirpation of the tumour. According to the literature adjuvant chemo- or radiotherapy do not improve patients survival. In case of early radical operation the malignant transformation is less probable.
The authors present a case of giant pulmonary metastasis from cystosarcoma phyllodes of the breast, and correlate the CT and MR findings with the pathological findings. Although CT showed homogenous low density in most parts of the tumor, T 2-weighted MR images presented a more variegated appearance, namely, peculiar slit-like areas of low intensity between areas of high intensity. The MR findings reflected the characteristic gross pathology of cystosarcoma phyllodes, showing narrow cavities filled with bloody fluid surrounding intracystic protruding tumors with abundant necrotic change.
For the last 150 years, cystosarcoma phyllodes of the mammary gland has had 62 different synonyms. At the present time, the most logical name for it seems to be tumor phyllodes. The result of the histological analysis of every single tumor should contain information about the degree of its maturity. The classical description of cystosarcoma phyllodes given by Johannes Müller (1838) is still considered the basis for understanding the clinical and histogenetical aspects of this neoplasm.
We detected cytosol estrogen receptors in a malignant cystosarcoma phyllodes and succeeded in serial transplantations into athymic nude mice. The tumors of this transplantable strain (MC-3-JCK) have the same histologic features as the original tumors, and retain significant amounts of cytosol estrogen receptors. This strain appears to provide a useful experimental model for the study of biologic and therapeutic aspects of human cystosarcoma phyllodes of the breast.
Cystosarcoma phyllodes is an unusual breast neoplasm that rarely metastasizes. Most series report chemotherapy, radiation, and hormonal therapy to be ineffective. Three patients were treated with cisplatin and etoposide combination chemotherapy with effective palliation in two patients. Radiation therapy was effective in controlling symptomatic metastasis in all three patients. Hormonal therapy was ineffective in two patients despite the presence of positive hormone receptors. Chemotherapy and radiotherapy may be more effective in the treatment of this tumor than has been reported, although there is no apparent role for hormonal therapy. Functional hormone receptors are probably not present.
A retrospective study of 33 patients with cystosarcoma phyllodes was done. Eight of these patients had metastases, and the clinical and histologic criteria predicting the development of metastases were examined. The most reliable predictor was the presence of stromal overgrowth; this appears to be necessary for metastasis to occur. Other useful indicators of clinical behavior were the degree of mitotic activity, nuclear pleomorphism, and infiltrating margins. Based on these data and a literature review, the authors suggest close follow-up of patients whose primary tumors contain areas of stromal overgrowth because, in all series combined, the risk of metastatic spread in such patients was 72% within 5 years. Among these high-risk patients, local recurrence is another indication that metastasis is likely.
Of 106 females treated surgically for cystosarcoma phyllodes, 20 had one or more local recurrences, while seven other patients had "pseudorecurrences" in that the histologic findings showed benign disease of the breast. Histologic transformation into a histologically more aggressive lesion was noted in three patients. There was a higher incidence of recurrence among the younger patients (those less than 20 years of age) and in those with the larger tumors (greater than 5 centimeters diameter). The reasons for these findings are discussed. The phenomenon of recurrence was reviewed with regard to age of the patient, size of the lesion, histologic features of the tumor and extent of the operation.
A case of malignant cystosarcoma phyllodes metastatic to the sacral bone and sacral area and diagnosed by fine needle aspiration cytology is presented. A review of the initial histologic sections was undertaken in an attempt to correlate our findings with the criteria suggested by other authors for predicting the clinical and metastatic behavior of this tumor.
Biochemical study of fibroepithelial tumors of the female breast showed presence of progesterone receptor (PgR) in all five cystosarcoma phyllodes (two malignant, three benign), and in 11 of 13 fibroadenomas tested. Estrogen receptor (ER) was detected in only one of five cystosarcomas and 2 of 13 fibroadenomas. The relative volumes occupied by epithelium and stroma in each tumor were measured from histologic sections. The results were consistent with presence of PgR in the stroma and ER in the epithelium. Different types of cystosarcoma (benign and malignant) and different types of fibroadenomas (intracanalicular, pericanalicular, and mixed) did not differ significantly in content of PgR, and mean levels of PgR in cystosarcoma were comparable with those in fibroadenomas. The presence of PgR in cystosarcomas suggests that progestational therapy, and possibly other forms of hormonal therapy, should be tested in the treatment of advanced, malignant cystosarcoma phyllodes.
The case report is that of patient who underwent tumorectomy plus X-ray therapy for mammary invasive ductal carcinoma. Eight years later a large cystosarcoma phyllodes, developed and quickly recurred after conservative surgery. Reviewing previous mammograms, the authors discovered that at the time of irradiation, there was a small lump which had the appearance of fibroadenoma. It may be that heavy X-ray therapy on a benign epithelial and stromal tumour of the breast, played a major role in the later development of cystosarcoma phyllodes.
A case of malignant cystosarcoma phyllodes metastatic to the maxilla is reported, representing the only known case with a maxillary metastasis.
Twenty-six patients of Cystosarcoma phyllodes, treated between July 1994 and July 2001, were analysed retrospectively. Median age at presentation was 38 years (range 13-61 years). Mean size of the lesion was 6 cm. There were 77% left-sided lesions and 23% right-sided lesions. Histologically, 58% lesions were benign, 11% borderline and 31% malignant lesions. All patients underwent definitive surgical procedure in the form of wide local excision or mastectomy. Four patients received postoperative radiotherapy. Median follow-up period was 35 months. Six patients showed recurrence, and four of these were malignant. Median disease-free survival period was 34 months.
Cystosarcoma phyllodes (CP) of the breast is a rare biphasic tumor composed of benign epithelium and a spindle cell stroma. Biologic behavior of CP cannot be predicted with certainty on the basis of morphologic criteria only. We studied immunohistochemical expression of basic fibroblastic growth factor (bFGF), urokinase, Ki67, p53 protein, and microvessel density in stromal and epithelial components of 14 low-grade CP (LCP) and 9 high-grade CP (HCP). bFGF was more often positive in LCP than in HCP. The stroma was positive for bFGF in 86% of LCP and 67% of HCP, and the epithelium was positive in 64% of LCP and 14% of HCP. Urokinase was positive in stromal cells of 86% of LCP and 93% of HCP. The epithelial positivity for urokinase in both groups resembled closely that of the stroma. p53 protein was more often positive in stromal cells of HCP (67%) than in LCP (50%). Ki67 was positive in the stroma of 43% of LCP and 89% of HCP and in the epithelium of 14% of LCP and 33% of HCP. There was no significant difference in microvessel density (MVD) in low- and high-grade lesions. Our study demonstrates that stromal Ki67 and p53 immunohistochemical positivity are more often associated with high-grade tumors. The positive immunostaining for bFGF, urokinase, Ki67, and p53 in stroma and epithelium of the majority of CP supports the existence of epithelial-stromal interactions and recognizes epithelium as an integral part of this tumor.
An unusual invasive breast carcinoma, arising in a cystosarcoma phyllodes and characterized by a variable cytoplasmic appearance and mucin content, was evaluated to determine the nature of the secretory material within the cells as well as the type of secretory organelle at the ultrastructural level. Histochemical studies revealed both acidic (sialic acid) and neutral mucin within the tumor cells. Ultrastructural analysis revealed secretory material within membrane-bound vesicles in some cells and within intracytoplasmic lumens in others; some cells contained both membrane-bound vesicles and intracytoplasmic lumens simultaneously. The Golgi derivation of the intracytoplasmic lumens was supported by their presence within or near hyperplastic Golgi complexes. The histochemical characteristics of the secretory material is correlated with their ultrastructural site of accumulation.
In this paper we report a case of cystosarcoma phyllodes malignum in a 45 years old female patient. A local relapse occurred 20 months after ablatio mammae. This tumor was histologically a benign fibroadenoma. 69 months postoperative metastases of the pleural diaphragma were found: the appearance was similar to that of a mesothelioma. The CEA was helpful in revealing the metastases and in indicating the progression. This case is discussed and compared to the review of the literature. Because of the high rate of local relapses a postoperative irradiation of the chest wall is proposed.