Tubular carcinoma developing within a recurring cystosarcoma phyllodes of the breast

Malignant alteration in cystosarcoma phyllodes is uncommon and almost always confined to the stromal component. A rare case of recurring cystosarcoma is reported. In the first recurrence, lobular carcinoma in situ was present within the tumor and the second recurrence revealed tubular carcinoma within the cystosarcoma. Epithelial proliferation of various sorts is not uncommon in cystosarcomas but only two previous instances of infiltrating carcinoma have been observed within these tumors. The problem of mammary carcinoma coexisting with cystosarcoma is reviewed and comparisons are made with a closely related neoplasm, the fibroadenoma.     

Primary chondrosarcoma of the breast: a case report

Mammary sarcomas are uncommon tumors. Of these pure chondrosarcoma without any other area of epithelial or mesenchymal differentiation feature as even rarer. This excludes tumors like malignant cystosarcoma phyllodes and metaplastic carcinoma where malignant cartilaginous areas may be present. Further primary chondrosarcoma arises from breast stroma and not from underlying bone or cartilage. Only five cases of pure and primary chondrosarcoma have been reported so far. The sixth case is reported here.     

Development of aggressive cystosarcoma phyllodes after conservative treatment of breast adenocarcinoma

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.     

Fatal metastatic cystosarcoma phyllodes in an adolescent female: case report and review of treatment approaches

The third reported case of fatal malignant cystosarcoma phyllodes in an adolescent female is described. The patterns of local recurrence and distant spread in this case, including the response to treatment, were similar to those reported in the first reported case in this age group. A review of the treatment recommendations for cystosarcoma phyllodes revealed that the surgical procedure of choice for the malignant variant has remained controversial, and the conclusions regarding the ineffectiveness of radiation and chemotherapy have been based on insufficient data handed down through the years. Our observations in this case and the information we have obtained from the literature have prompted us to recommend a multidisciplinary approach for malignant cystosarcoma phyllodes, particularly in young women, and we are calling for a multi-institutional study group to further investigate this disease.     

Malignant subtype of cystosarcoma phyllodes with brain metastases

Cystosarcoma phyllodes is an uncommon type of breast tumour. Benign, borderline, and malignant subtypes have been described. Central nervous system metastases of the malignant subtype of cystosarcoma phyllodes are rare and associated with poor prognosis. We report on a patient with malignant cystosarcoma phyllodes who developed metastatic disease six years after resection of the primary breast tumour. Partial regression of a brain metastasis was achieved using radiotherapy but the patient later died due to widespread metastatic disease which was uncontrollable by systemic chemotherapy. Because metastatic malignant cystosarcoma phyllodes are largely resistant to treatment, the most important objective is to provide optimal management of the primary tumour before dissemination occurs.     

乳腺叶状肿瘤和巨纤维腺瘤的临床病理特征——附89例临床分析

 目的　探讨乳腺叶状囊肉瘤和巨纤维腺瘤的诊断和治疗。方法　回顾分析我院 1979～ 2 0 0 2年间外科收治的 89例乳腺叶状囊肉瘤和巨纤维腺瘤的临床特点 ,病理诊断和治疗方式。结果　 2 7例乳腺叶状囊肉瘤 ,行乳腺改良根治术 4例 ,乳腺单纯切除术 16例 ,肿瘤局部切除术 4例 ,肿瘤复发行扩大切除 3例 ,复发率 33.3% (9/ 2 7) ;6 2例乳腺巨纤维腺瘤行肿瘤局部切除术 4 0例 ,乳房单纯切除术 18例 ,改良根治术 4例 ,复发率 11.3% (7/ 6 2 )。结论　按照WHO乳腺肿瘤分类的标准 ,使用乳腺叶状肿瘤的统一名称 ,并与乳腺巨纤维瘤相鉴别 ,根据肿瘤分类采用不同的手术方法。     

Intraductal carcinoma within a phyllodes tumor of the breast: a case report

The first reported case in the world literature of a ductal carcinoma in situ located within a phyllodes tumor of the breast is presented. Previously reported cases of carcinoma in phyllodes tumors are reviewed, and the relationship between carcinoma arising in fibroadenomas and phyllodes tumors is discussed.     

乳房叶状囊肉瘤的预测和治疗选择

涉及乳房叶状囊肉瘤的主要问题是预测恶性的潜在性。本文对２８例经病理证实的本病临床资料、ＡｇＮＯＲ、ＥＲ、ＰＲ结果进行研究。非复发病例中ＥＲ及ＰＲ阳性占３１．３％，ＡｇＮＯＲ平均２．４，介于乳癌（５．４８）和良性病变（０．９７）之间。差异有显著统计学意义。提示可作为预测本病生物学行为的定量指标和选择手术方式的指征。     

A malignant cystosarcoma phyllodes with positive estrogen receptor and its heterotransplantation into nude mice

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 of the breast: a case report in a 12-year-old girl

Breast tumors in adolescents are very rare and mostly benign. Fibroadenomas are the most frequent, but within the extensive differential diagnosis, the phyllodes tumor must be mentioned, which accounts for about 1% of breast tumors and the diagnosis of which is very rare in patients younger than 20 years. There are no specific symptoms or radiological images to distinguish phyllodes tumor from fibroadenoma; therefore, histological examination is mandatory for diagnosis. Histology also allows the classification of phyllodes tumor into benign, borderline, or malignant types for appropriate surgical treatment: freemargin excision in benign tumors and mastectomy in the other two types. Fortunately, the majority of these tumors are benign, and treatment maximizes breast conservation with free infiltration margins surgery, given that this fact is the most important factor to prevent local recurrence. In this article, we describe a rare case of borderline cystosarcoma phyllodes in a 12-year-old girl.     

乳腺增生性病变与其癌变的观察Ⅱ免疫组织化学研究

对１９９２年４月～１９９３年８月天津市肿瘤医院乳腺科收治的女性乳腺癌患者６３例，乳腺良性病变１２例，叶状囊肉瘤３例，分别采用免疫组化法进行癌胚抗原（ＣＥＡ）及ｃ－ｅｒｂＢ－２癌基因产物表达的测定。检测发现乳腺良性病变基本呈阴性着色，乳腺癌基本均为阳性或强阳性，增生性病变则多为弱阳性。少数ＨＥ染色尚未出现明显异型的重度乳头状瘤病和导管上皮不典型增生，其免疫组化ＣＥＡ或ｃ－ｅｒｂＢ－２呈阳性或强阳性着色，符合病理组织学考虑这两种病变均已处于早期癌变阶段的见解。     

Desmoid tumor associated with cystosarcoma phyllodes of the breast.

A case of desmoid tumor of 17 years' duration associated with cystosarcoma phyllodes of 17 months' duration is presented. Good initial response to radiation therapy of the desmoid tumor was shown. Poor response of cytosarcoma phyllodes to radiation therapy was noted. A comparison between the similarities and differences of the two tumors is presented.     

The unique association of mammary stromal sarcoma with intraductal carcinoma.

The unique combination of clinically unsuspected stromal sarcoma and noninfiltrating intraductal mammary carcinoma is presented. This association has not been previously documented. The clinicopathologic features of cystosarcoma phyllodes and mammary stromal sarcoma are reviewed to provide background perspectives to illuminate the unusual aspects of this case.     

Stromal neoplasms of the breast: a comparative flow cytometric study

We analyzed 42 mammary spindle cell neoplasms (10 conventional fibroadenomas, 8 giant fibroadenomas, 17 cystosarcoma phyllodes, and 7 stromal sarcomas) by flow cytometry to assess the diagnostic and prognostic relevance of DNA content analysis in the pathologic evaluation of these lesions. Our data indicate that all fibroadenomas were diploid, cystosarcoma phyllodes displayed diploid and aneuploid DNA content irrespective of their histological categorization, and that stromal sarcomas were all aneuploid and clinically aggressive. Diploid cystosarcoma phyllodes were biologically indolent, whereas most of the aneuploid neoplasms killed their hosts (P = 0.03). The present study indicates that a DNA content abnormality it is a reliable marker of malignancy in these neoplasms, and it may identify subsets of patients with variable biologic courses within the histopathologic spectrum of cystosarcoma phyllodes.     

A malignant phyllodes tumor of the breast in a 6-year old girl

We recently encountered a 6-year-old girl with a malignant phyllodes tumor of the breast. The patient's mother noticed a tumor on the right breast of her first doughter at 8 months after from her birth. The baby was brought to the Second Depertment of Surgery, University of Tokushima, at age 20 months. We recommended removal of the 3.5 x 2.9 cm tumor in the right breast, but it was not done. Next, she visited our department at 6 years of age because the tumor had gradually enlarged, reaching a size of 4.3 &timus; 4.0 cm. She underwent excisional resection of the tumor, and the tumor was diagnosed as a malignant phyllodes tumor of the breast coexisting with a borderline phyllodes tumor. To our knowledge, this is the youngest reported case of a malignant phyllodes tumor of the breast. Moreover, the malignant lesion was positive for estrogen receptor (ER) and showed strong proliferating cell nuclear antigen (PCNA) staining. On the other hand, the borderline part was negative for ER and showed weak PCNA staining. Thus, in the present case, the expression of ER, the exposure to estrogen (for example, the mother's estrogen during gestation) and increase in the proliferation rate may have played important roles in the mechanism of the transformation of the phyllodes tumor.     

Cystosarcoma phyllodes. Report of an unusual case, with death due to intraneural extension to the central nervous system

Cystosarcoma phyllodes tumors of the breast occasionally exhibit malignant behavior, including chest wall invasion, hematogenous spread or, rarely, metastasis by lymphatic routes. An unusual case of cystosarcoma is presented in which death was apparently caused by perineural and intraneural extension of the tumor along intercostal nerves, to the sympathetic chain, and then to the brain stem by spinal roots, with no evidence of embolic metastasis.     

Most cystosarcoma phyllodes and fibroadenomas have progesterone receptor but lack estrogen receptor: stromal localization of progesterone receptor

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.     

Dipeptidyl peptidase IV expression identifies a functional sub-population of breast fibroblasts.

The immunocytochemical distribution of the cell-surface enzyme dipeptidyl peptidase IV (DPP IV) has been studied in the human breast at the light and ultrastructural level. The presence of the enzyme was demonstrated on the cell membranes of interlobular fibroblasts, whilst intralobular fibroblasts were DPP-IV-negative. A fluorograph, after immunoprecipitation of 35S-methionine-labelled proteins of fibroblasts from primary breast cultures with an anti-serum to DPP IV, demonstrated a band at 135 kDa consistent with the presence of the enzyme. The clear delineation of 2 functionally distinct subpopulations of breast fibroblasts was maintained in benign fibro-adenomas and cystosarcoma phyllodes, both tumour types having growth characteristics of intralobular stroma. This observation has important implications for both normal breast biology and for breast carcinogenesis.     

Liposarcoma arising within a cystosarcoma phyllodes

A liposarcoma arising within a cystosarcoma phyllodes in a 17-year-old black female was treated by total mastectomy without the need of radiation and/or chemotherapy. The patient is alive and well with no evidence of recurrence or metastasis at 20 months recent follow-up. The clinicopathological features of malignant cystosarcoma phyllodes-liposarcoma are discussed.     

Phyllodes tumor showing intracystic growth: a case report

A phyllodes tumor often grows rapidly and occasionally contains microcysts; however, the tumor rarely shows a morphologically intracystic pattern. We experienced a rare case of a phyllodes tumor with a solid mass growing into the cyst. A 62-year-old female noticed a tumor in her right breast in January 1995. The tumor grew rapidly and she visited our out-patient clinic in February 1995. On physical examination, a 10 x 8 cm, well defined and movable mass with a smooth surface was palpated in the upper outer quadrant of the right breast. Mammography showed a large tumor shadow in the upper outer quadrant of the right breast without any microcalcification. Ultrasonography revealed a large cystic shadow with a low echoic lesion and solid component with heterogeneous internal echo in the cyst. Under general anesthesia, the tumor was widely excised. The resected specimen was 11.5 x 11 x 11 cm in size and the tumor was not invasive to surrounding tissues. Old bloody fluid was contained within the cyst. The gross appearance showed papillary process protrusions into a central cystic cavity. Histological examination revealed a borderline case of phyllodes tumor. Two years after the operation, she is doing well without any recurrence.