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Unilateral virginal breast hypertrophy.

A case of unilateral virginal breast hypertrophy with a review of the etiological factors and treatment modalities is presented. A 16-year-old girl presented with progressive enlargement of the left breast of 5 months' duration. The result of the mammographic examination was consistent with cystosarcoma phyllodes. Fine-needle aspiration biopsy revealed giant fibroadenoma. Although some of the characteristics of the fine-needle aspiration biopsy specimen were suspicious for cystosarcoma phyllodes, there were no adequate epithelial structures, which are obligatory for the diagnosis. The patient was treated with subcutaneous mastectomy and subpectoral insertion of a silicone gel implant. The histopathological examination was consistent with virginal hypertrophy. The breast maintained its volume with no further growth in the affected or in the normal breast after 4 years of follow-up.

Adolescent↗

The use of the saddle prosthesis for reconstruction of the hip joint after tumor resection of the pelvis.

Reconstruction of the hip joint by a saddle prosthesis after excision of a malignant pelvic tumor is a relatively new method, which thus far has been mainly used for revision of infected hip arthroplasties. One patient with a metastatic cystosarcoma phyllodes and one patient with a chondrosarcoma of the pelvis were treated by local resection and reconstruction with a saddle prosthesis. Although the patient with the metastatic cystosarcoma phyllodes died 9 months after surgery due to metastatic disease, both patients had early recovery, with no difference in leg length and obtained early painless complete weight bearing with satisfactory functional result. These two case reports clearly illustrate the usefulness of the saddle prostheses in limb saving surgery for malignant tumors of the pelvis.

Adult↗

[Recurrences and metastases of cystosarcoma phylloides (phylloid tumor, WHO). On the 150th birthday of a controversial diagnostic concept].

134 Cystosarcoma phyllodes were been observed in the course of 12 years among the diagnostic and consultative (n = 121) material. 133 tumors occurred in the female, one in the male breast. In 21 cases local recurrences and in 4 cases metastases developed. 6 recurrences following local excision were observed more than once: in one case 10 subsequent recurrent tumors, the last of these in the chest wall after mastectomy. Histopathologically, the recurrences were identified as benign (1 case), as borderline tumors (2 cases) and as malignant cystosarcoma (8 cases). In 10 further cases (48% of the recurrences) a stromal sarcoma was predominant. In accordance with the literature, we found in most cases a more aggressive growth and an enhanced malignancy. It is emphasized, that angioinvasive growth in the recurrent tumors can indicate distant metastases. The frequency of the recurrent cystosarcoma in our series was 16%, multiple subsequent tumors occurred in 29% of them. Recurrences are caused by proliferative remnants of the primary tumor following local excision, or they are tumors-de-novo induced by a extratumoral stromal hypercellularity of the surroundings like a new benign cystosarcoma. Therefore an initial wide excision is recommended for the benign and a mastectomy for the malignant cystosarcoma. The site of distant metastases of the 4 cases were the lung and the skeleton (rib and femur). In one case an axillary lymph node was involved by a recurrent giant cell stromal sarcoma. The interval between primary and metastases was 7 months to 5 years, between recurrent tumor and metastases 6 to 24 months or both tumors were observed simultaneously. Following the recent literature, distant metastases occur at an average rate of 19% of the malignant cystosarcomas. In single cases it is impossible to make a sure prognosis of cystosarcoma phyllodes, because recurrences and metastases are observed in all subtypes, but predominantly in the malignant cystosarcomas.

Breast↗

[Cystosarcoma phylloides in adolescent women].

The authors describe four cases of juvenile fibroadenoma (benign form of cystosarcoma phyllodes) in pubertal girls aged 11-15 years. They reflect on the variable behaviour of this rare tumour and differentiate the relations between fibroadenoma, virginal hypertrophy of the breast, juvenile fibroadenoma, giant fibroadenoma and cystosarcoma phyllodes. A dominant position is held by surgical treatment which is strictly individualized. The authors recommend, similarly as many other authors, an extensive excision of the tumour with a safety margin and in case of repeated relapses or the very rare malignant form which frequently behaves as a benign tumour. The operation is made in the submammary groove or on the lateral margin of the breast which ensures an excellent cosmetic effect. The authors do not consider excision of superfluous skin or reconstruction of the breast necessary with regard to the elastic properties of the skin and the capacity of the mammary gland to develop.

Adolescent↗

Sarcomas of the breast: a multicenter series of 70 cases.

A multicenter retrospective series of 70 breast sarcomas (malignant cystosarcoma phyllodes (25), osteosarcoma (12), liposarcoma (10), stromal sarcoma (8), angiosarcoma (7), mixed types sarcoma (4), malignant histiocytoma (3), leiomyosarcoma (1)) was reviewed. The average follow-up was 5.9 years. Diagnostic tests (palpation, mammography, sonography and cytology) were poorly sensitive, and a large proportion of cases, appearing as regular, sharp bordered, rounded masses were diagnosed as benign fibroadenomas. Surgery (limited (29), mastectomy (41)) was the treatment of choice. Axillary nodes were rarely involved (2 of 31) at pathologic staging. No significant predictors of local recurrences (12 cases) were observed although recurrences were more frequent in larger lesions (0-20 mm = 1.1%, 21-50 mm = 1.7%, > 50 mm = 6.1% women-year) and in cases treated with limited surgery (limited surgery 4.6%, mastectomy 2.0% women-year). Distant metastases (16 cases) were less frequent in malignant cystosarcoma phyllodes or liposarcoma patients, but no other significant predictors of distant metastases were evidenced. Five-year disease-free or overall survival was 50% or 66%, respectively. The study confirms that breast sarcomas are rare, difficult to diagnose, but can be cured by surgical treatment in a considerable proportion of cases.

Adult↗

[Sarcoma of the breast].

Reports of 4 personal cases of breast sarcomas (3 cases of cystosarcoma phyllodes and 1 angiosarcoma), the authors review the literature concerning these rare tumors which account for less than 1% of malignant tumors of the breast. Whilst the usual clinical picture is that of a large tumor deforming the breast with pseudo-inflammatory signs, sarcomas 1 to 3 cm in diameter were discovered and treated with good chances of recovery. The natural history of breast sarcomas is marked above all by the risk of hematogenic metastatic spread (30% of cases) whilst very few (5%) local recurrences are seen following surgical treatment by mastectomy. Emphasis is placed upon problems of histological diagnosis of the variety of sarcoma and the demonstration of histoprognostic factors which can be used to predict the outcome (mitotic activity, grading) showing evidence of good correlation in the series analyzed. The various characteristics of cystosarcoma phyllodes are described emphasizing the conditions surrounding its development, relations with benign phyllodes tumors. The particular severity of this sarcomatous form in the very young woman is mentioned. Prevention lies in the sufficiently extensive excision of the phyllodes tumor. Mesenchymatous sarcomas of the breast are also accompanied by a fairly favorable prognosis (5 year actuarial survival of 50 to 80% according to series). By contrast, two special and rare histological varieties, angiosarcoma and lymphosarcoma, are characterized by a much more gloomy prognosis and require different treatment including radiotherapy and chemotherapy.

Adult↗

Malignant phyllodes tumor of the breast metastasizing to the pancreas: case report.

Phyllodes tumor of the breast, or cystosarcoma phyllodes, is an unusual breast tumor. It is usually considered a benign lesion but may have malignant potential. Only a small proportion of malignant phyllodes tumors will metastasize. A phyllodes tumor of the breast metastasizing to the pancreas is rare. This 39-year-old female patient initially presented to us with a 5 x 4 x 2 cm tumor of a right breast. After excision of the tumor, she received another wise excision for a local recurrence of the tumor. Three years had passed when she started having hematemesis and tarry stools intermittently over a period of about one month. Clinical evaluation disclosed a huge mass with an ulcerated and bloody base over the second portion of the duodenum. A pancreatoduodenectomy was then performed. During the postoperative recovery period, a rapidly enlarging tumor of the right breast was noted. She subsequently underwent a total mastectomy of the right breast. Both the duodenal tumor and the breast tumor were found to be malignant phyllodes tumors. The rarity of this kind of patient and presentation is discussed.

Adult↗

Primary sarcoma of the breast.

BACKGROUND AND OBJECTIVES: Primary sarcoma occurring in breast is rare and comprises 0.5-1% of all breast neoplasm. Majority of the series include both stromal and cystosarcoma phyllodes, only a few hundred cases of sarcomas other then cystosarcoma are reported. PATIENTS AND METHODS: We carried out a retrospective analysis of 19 patients with primary sarcoma of the breast treated between 1982 and 2002. RESULTS: Mean age of the patients was 38.6 years (12-70 years). Gradually progressive swelling was the commonest presenting feature. There were eight cases of angiosarcoma, four cases of spindle cell sarcoma, two each of pleomorphic sarcoma and stromal sarcoma, and one each of malignant fibrous histiocytoma, embryonal rhabdomyosarcoma, and sarcoma (NOS). Eight of these were high-grade (42%). Eight patients underwent either radical or modified mastectomy, three underwent wide excisions, and one underwent quadrantectomy. Ten (52.6%) patients received postoperative adjuvant radiation. Two patients received chemotherapy. After a mean follow-up time of 34.5 months (median 25 months), eight patients failed. Failure was local in five, opposite breast in one, and both local and distant in two. The disease free survival at 3-year was 39%. In univariate analysis only the margin of first surgery was found to be a significant predictor of survival (P = 0.05). CONCLUSIONS: Primary sarcomas of the breast are aggressive tumors. Surgical treatment should consist of at least simple mastectomy. All attempts should be made to achieve a negative margin as this appears to be the only factor influencing survival in these patients.

Adult↗

Malignant phyllodes tumor of the breast: review of the literature and case report of stromal overgrowth.

Cystosarcoma phyllodes constitutes only 0.3-0.9% of all breast tumors. The term "sarcoma" was initially used because of its fleshy appearance, a more modern term is Phyllodes tumor (PT). The behavior of PT constitutes a spectrum from benign and locally recurrent to malignant and metastatic. In a general surgical series, 6.2% of the tumors were malignant. The microscopic appearance of PT is that of epithelial elements and connective tissue stroma. Malignancy is determined by characteristics of the stroma. The metastatic spread of malignant PT is mainly hematogenous to lung, with infrequent lymphatic involvement. Wide local excision with 2 cm margins is the treatment of choice. In 20% of both benign and malignant cases, PT will locally recur. There is no proven benefit of radiation or chemotherapy, although radiotherapy may be useful in selected cases. We present a case of a sarcomatous overgrowth in a malignant phyllodes tumor involving multiple histologic types.

Aged↗

[Sarcoma phyllodes of the breast. Apropos of a case withan ultrastructural and histoenzymological study].

One case of cystosarcoma phyllodes is reported. Histologically, the neoplasm was composed of two elements: benign epithelial cells and malignant stromal cells. The stromal cells, spindle shaped, grow in solid sheets or in sparcely cellular, myxomatous and alcianophilic areas. Scanning electron microscopy demonstrates a mucous secretion, showing porelike openings and mucous droplets on the surface of tumoral cells.By transmission electron microscopy, sarcomatous cells look like very polymorphous. Secretory cells (showing dilated granular endoplasmic reticulum and abundant lysosomes) are mixed with primitive mesenchymal cells and with intermediate, myoepithelial cells. The myoid origin of these cells is demonstrated by the bundless of myofilaments terminated in marginal plaques on the plasma membrane, the numerous pinocytic vesicles and basal lamina investing irregularly the cells. Histoenzymology corroborates these findings, disclosing a high activity of alkaline phosphatase. Discovery of ambiguous myoepithelial cells associated with undifferentiated cells, likewise reported in epitheliomas, is of a great histogenetic interest. It suggests development of mammary neoplasm from stem cells coming under local of general influences, unknown today, but perhaps responsible of epithelial or conjunctive differentiation. The presence of a contralateral carcinoma in our case is consistent with this hypothesis.

Alkaline Phosphatase↗

[Phyllodes tumors of the breast. Apropos of 83 cases].

Twelve recurrences after surgical treatment of 83 cystosarcoma phyllodes of the breast were reported. The histology of the initial tumor was benign (n = 73), borderline (n = 6), sarcoma (n greater than 4). Nine recurrences were observed at the initial site of the tumor (2 in another quadrant of the same breast and I in the contralateral breast). The cause of local recurrence is related to incomplete tumorectomy (n = I), pseudo-capsule infiltration (n = 3), micro-nodules satellites (n = 2). No etiology for recurrence was defined in 3 cases. The importance of a careful microscopic examination of the initial tumor is emphasized because the infiltration of the pseudo-capsule and the presence of micro-nodules satellites can only be recorded on correctly prepared slides.

Adolescent↗

Recurrent phyllodes tumor in a man.

The case of a 35-year-old man with a borderline-type cystosarcoma phyllodes is presented. Four years after the primary excision of the tumor, wide excision of a local recurrence and postoperative radiotherapy were performed. No repeated relapse was observed during a 5-year follow-up. Neither significant endocrine changes nor genetic alteration could be proven. However, a slightly increased SHBG concentration was detected, resulting in a decreased biologically available androgen level reduced testosterone/SHBG index. This phenomenon might be a consequence of the chronic liver disease of the patient due to his type II diabetes mellitus and alcohol abuse. In addition to the conventional histopathological examinations, immunohistochemical and electron-microscopic investigations were carried out on tissue sections, and the steroid receptors, EGF receptors and EGF-like activity of the tumor were also studied.

Adult↗

[Phyllodes tumors of the breast].

Clinical pattern compared with ultrasound, radiology and fine needle acubiopsy allows an exact diagnosis of cystosarcoma phyllodes before surgical operation. Then, even using quite conservative procedures on low-grade tumours, we had very good results.

Adolescent↗

MRI in phyllodes tumor of the breast. Case reports.

The purpose of this study was to reassess the role of MRI in 3 cases of cystosarcoma phyllodes. Three patients with a palpable large lump were studied with mammography, ultrasonography and MRI. In the 3 patients mammography showed a nonspiculate soft-tissue mass while a hypoechoic lobulated mass with cystic areas was visualized on sonography. Dynamic MRI demonstrated a multi-lobulated lesion rapidly and markedly enhanced on dynamic studies of contrast-enhanced T1-weighted imaging. T2WI showed inhomogeneous signal intensity with a hypointense area and internal septation in the solid portion. MRI enabled complete visualization of the tumor even in the region close to the chest wall, as well as clear delineation against the healthy glandular tissue.

Adult↗

Phyllodes type of atypical prostatic hyperplasia.

A prostatic lesion characterized by atypical epithelial hyperplasia and pleomorphism of the stromal elements is presented. The features bear a strong resemblance to cystosarcoma phyllodes of the female breast and probably represent a comparable lesion. Such atypical hyperplasia has not been described previously.

Adult↗

Phyllodes tumors of the breast.

Phyllodes tumor is a rare fibroepithelial neoplasm of the breast with a very variable, but usually benign, course. Formerly known as cystosarcoma phyllodes, the designation "phyllodes tumor" with appropriate qualification regarding malignant potential based on pathologic features is now the agreed-upon term. The most important diagnostic distinction is from fibroadenoma--phyllodes tumors require complete excision with free margins even when pathologic features suggest benignity because of a proclivity to local recurrence. The most important component of therapy is wide surgical excision, and mastectomy is necessary only when free margins cannot be achieved without it. Involvement of axillary nodes is rare, and axillary dissection is not indicated. The role of radiation therapy and chemotherapy is not established and has not been studied in randomized trials due to the rarity of the tumor. At present, there is no consensus that patients with high-grade phyllodes tumors of the breast will benefit from either of these modalities.

Antineoplastic Agents↗

Malignant phyllodes tumor of the prostate. A case report with immunohistochemical and ultrastructural studies.

Phyllodes tumor of the prostate is a rare neoplasm with cellular or sarcomatoid stroma and hyperplastic glands. This lesion shares many histologic features with cystosarcoma phyllodes of the breast. Although a malignant variant of phyllodes tumor of the prostate has been described, the majority of cases have been clinically benign. We report an unusual case of phyllodes tumor of the prostate in which the stromal component underwent malignant degeneration, a finding not previously described (to our knowledge). Immunohistochemical and ultrastructural studies demonstrated smooth-muscle differentiation of the stromal cells.

Aged↗

Phyllodes type of atypical prostatic hyperplasia: a report of 3 new cases.

We report 3 new cases of phyllodes type of atypical prostatic hyperplasia. This lesion is characterized by epithelial and stromal proliferation. Stromal changes are the most characteristic finding in phyllodes type of atypical prostatic hyperplasia, which show atypical cells with enlarged, hyperchromatic sarcomatoid nuclei. Mitotic figures are not present. Although the histological appearance may mimic that of cystosarcoma phyllodes of the breast, this pattern is present only focally or not at all in phyllodes type of atypical prostatic hyperplasia. On computerized tomographic imaging phyllodes type of atypical prostatic hyperplasia has a distinct appearance. These patients can be expected to have a benign clinical course and distant metastases have not been reported. Treatment is by surgical excision as in benign prostatic hyperplasia.

Adult↗