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A malignant cystosarcoma phyllodes, with simultaneous intraductal and infiltrating duct carcinoma involving the surrounding breast tissue, was found in a 31-year-old black woman. Careful histologic examination of the tissue surrounding the cystosarcoma phyllodes is important in detecting coexistent carcinoma.
A case is presented of cystosarcoma phyllodes of the breast metastatic to the pancreas. Solitary metastasis to the pancreas has not been reported. The lesion was excised in toto with a good result. Clinical and pathological features of cystosarcoma phyllodes are discussed.
Cystosarcoma phyllodes is a rare form of breast cancer that rarely metastasizes. This case represents the first reported case of a pathological spine fracture and neurologic injury as a result of metastatic cystosarcoma phyllodes. Physicians should be aware of a recently identified histological subtype of cystosarcoma that exhibits an increased incidence of hematogenous metastasis.
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.
There have been described three cases of the breast phyllodes tumour, i.e., fibroepithelial tumour, histologically both benign and on borderline, or expressively malignant (sarcoma). The mean age of patients concerned is of about 50 years old at the time of neoplasm diagnosis. If the surgical removal is incomplete, therein a large number of recurrences occurs. Diagnostic procedures are based on clinical examination (it is remarked the abnormal wideness of the tumour) as well as mammography, echography, needle biopsy. Surgical treatment is always based on large tumorectomy which results are sufficient in benign tumours. In malignant phylloides a total mastectomy is required without axillary lymphadenectomy owing to the hematic dissemination. Usually, after surgical treatment, survival rate seems better as compared to that of breast cancer.
We present four cases of cystosarcoma phyllodes in which mammograms revealed a small or large lobulated mass occupying the entire breast. Preoperative diagnosis was a benign lesion with a low probability of nodular carcinoma. Histologically, all tumors were classified as malignant. Although uncommon, cystosarcoma should be considered in the differential diagnosis of these masses, since local recurrence is frequent unless wide wedge resection or simple mastectomy is done.
Five biphasic tumors, four fibroadenomas and one cystosarcoma phyllodes, are reported in the male breast. Gynecomastia and lobular development were present in all five cases. The rarity of fibroadenoma and cystosarcoma phyllodes in men is attributed to the absence of lobules in the normal male breast. We propose that lobular development precedes biphasic tumors in the male breast. The development of lobules apparently requires a certain length and/or intensity of endogenous or exogenous estrogenic stimulation not frequently attained at the levels that commonly induce gynecomastia.
BACKGROUND: Cystosarcoma phyllodes is a rare breast tumour whose behaviour is not well understood by many clinicians. METHODS: In 1998 a retrospective study was undertaken of women diagnosed with phyllodes tumour of the breast who had their initial surgery between 1983 and 1994 in Western Australian public hospitals. RESULTS: Forty women were diagnosed and treated over this period; however, only 28 received ongoing follow-up (70%). Follow-up was obtained on 26 of these. The median age at diagnosis in this group was 46 years. Cases were predominantly Caucasian (85.5%). Postmenopausal women were affected in 26.9% of cases. Four patients had recurrences after surgery (one malignant and three benign). No patient had a recurrence with primary tumours with a diameter of < or = 2 cm. Mean time to recurrence was 35.8 months. CONCLUSIONS: Breast-conserving surgery was used in almost all cases (96.2% of first operations).
Recurrence and metastases of a cystosarcoma phyllodes are poorly correlated with the histologic type and treatment used. There is some evidence, however, that the prognosis is more favorable for small tumors and minimal cellular atypism. Local excision seems to be associated with a higher incidence of recurrence of the tumor. Metastases can develop later from what appears primarily a benign, as well as a malignant, type. Mastectomy as primary treatment was not always effective in preventing the local recurrence of the tumor. If the tumor recurred after mastectomy, it gave rise to fatal metastases. The recurrence of the tumor after local excision is secondary to microscopic foci retained after enucleation. If a local excision is done, it should include a wide margin of mammary tissue around the tumor. Wide local excision, for small, slow growing and clinically benign tumors can be used as a first operation, with wider re-excision and later mastectomy if the tumor recurs. Large and rapidly growing tumors that suggest malignant disease should be treated primarily by mastectomy.
From a total number of 31 patients with the diagnosis of cystosarcoma phyllodes recorded by the authors, in two thirds the benign and proliferating form of the tumour was involved, in one third a malignant type of tumour. In benign tumours it is sufficient to extirpate the tumour completely, incl. capsule of the tumour. In malignant forms radical modified mastectomy with revision of the axilla must be performed. When the histological findings have a high grading (non-differentiated anaplastic), adjuvant radiotherapy or a combination of radiotherapy and chemotherapy must be used. Dispensarization of patients with the diagnosis of cystosarcoma phyllodes is essential.
Twenty-six cases of cystosarcoma phyllodes diagnosed at M. D. Anderson Hospital were reviewed. The following criteria were evaluated for possible correlation with local recurrence, uncontrolled local recurrence, metastasis, and tumor death: tumor size, stromal overgrowth, tumor necrosis, mitotic rate, stromal cellularity, nuclear size and pleomorphism, the presence of specialized stroma, and initial therapy. Of the 26 tumors, seven caused death. Five patients developed metastatic spread, and all of them died of tumor. Five patients had local recurrence, which was uncontrolled in three (two patients died with uncontrolled recurrence alone, and one with uncontrolled recurrence and metastasis). Stromal overgrowth was present in eight cases. Six of the seven patients who died of tumor had stromal overgrowth, including all five with metastasis. Correlation of stromal overgrowth with metastatic spread and tumor death was significant at P levels of 0.0014 and 0.02, respectively. It is concluded that stromal overgrowth is a significant histologic indicator of malignant behavior in cystosarcoma phyllodes.
A 47-year-old woman was transferred to our hospital under endotracheal intubation with severe hemodynamic deterioration. A left mastectomy had been performed 8 months earlier and histology of the specimen showed malignant cystosarcoma phyllodes. An angiography and echocardiography suggested massive mass of the right ventricle extending to the pulmonary artery. An emergent life-saving operation was required using cardiopulmonary bypass. A huge multilobular tumor was resected from the right ventricle through the pulmonary artery as much as possible and a tricuspid valvuloplasty was also performed. The patient was weaned from cardiopulmonary bypass, then satisfactorily recovered from hemodynamic disturbances. Nevertheless, she died 15 days later due to multiple organ failure. Microscopic examination of resected specimen showed the neoplastic stromal cells that increase mitotic activity and have sarcomatous characteristics, consistent with a metastatic cystosarcoma phyllodes. This is a second case of operation for cardiac metastasis of cystosarcoma phyllodes using cardiopulmonary bypass, so far presented in the literature of the world reviewed by the authors.
Cystosarcoma phyllodes is a rare breast tumor with variable malignant potential. Metastasis has been reported in a small percentage of cases. We describe the case of a 52-year-old woman who developed a large facial tumor 1 year after she had undergone a mastectomy for a rapidly enlarging breast neoplasm. The facial lesion was found to be a malignant cystosarcoma phyllodes metastatic to the mandible, and the patient died shortly after diagnosis. To our knowledge, this patient represents only the third reported case of a phyllodes tumor metastatic to the mandible.
UNLABELLED: This study reviewed 26 women who had resection of a malignant cystosarcoma phyllodes. Clinical presentations were palpable mass, 25; pain, 11; and ulceration, 2. Definitive surgical therapy was radical mastectomy (RM), 2; modified radical mastectomy (MRM), 14; total mastectomy (TM), 4; and partial mastectomy (PM), 6. Tumors ranged in size from 1-20 cm (median, 7 cm). Eight patients developed recurrent disease after 10-45 months. Local recurrence was more likely after TM and PM than after MRM and RM (P < 0.05). Patients who developed local recurrence only were treated by wide re-excision, and all such patients are alive with no evidence of disease at 5-25 years. Only one of 16 patients undergoing axillary dissection had involved lymph nodes. Four patients whose tumors ranged from 5-8 cm and who underwent one RM and three MRM developed systemic recurrence; all died of their disease after 15-48 months. CONCLUSION: 1) Although there is a higher local recurrence after PM and TM as compared with MRM and RM for malignant cystosarcoma phyllodes, the local recurrence can be treated with wide excision without affecting long-term survival; 2) systemic recurrence was not related to size or extent of resection; 3) axillary dissection does not predict for or prevent recurrence.
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.
Pathologically involved ectopic breast tissue is not commonly encountered. There have been, however, intermittent reports of involvement of ectopic breast tissue by many of the same benign and malignant processes that occur in normally located breast tissue. This article records what, to our knowledge, is the first case of cystosarcoma phyllodes arising in ectopic breast tissue of the axillary regions and is the second reported case of three primary cystosarcoma phyllodes arising synchronously. A review of the available literature pertaining to ectopic breast tissue and multiple cystosarcoma phyllodes is also presented.
BACKGROUND: We present cytogenetics and fibroblast growth factor immunohistochemistry in one case of cystosarcoma phyllodes with localized disease and one with metastatic spread. The p53 gene was sequenced in the malignant case. METHODS AND RESULTS: Karyotype analysis used trypsin-Giemsa banding. Immunohistochemistry of FGF1, FGF2, FGFR1 and p53 used avidin-biotin detection of the primary antibody. One case had a mosaic female karyotype and three clones: one normal, one with trisomy 7, and one with both trisomy 5 and a rearranged chromosome 1. In the second case, a resected pulmonary metastasis had the karyotype 43-47,XX,+mar1,+mar2[6]/43-46,XX, +del(7)(p10)[3],+mar2[1][cp3]/46,XX[10]. These tumors expressed FGF1, FGF2, and FGFR1. The malignant case showed immunostaining for p53 protein, but a wild-type gene sequence. CONCLUSION: The karyotype of cystosarcoma phyllodes is complex, with wide case-to-case variation. These tumors express members of the FGF family. Metastatic behavior can occur in the presence of a wild-type p53 gene.
The surgical treatment and outcome of 31 women with a pathologic diagnosis of cystosarcoma phyllodes at Roswell Park Memorial Institute were reviewed. Surgical excision was performed upon 20 patients with a mean tumor size of 4.5 centimeters (range of 1.0 to 9.0 centimeters). Mastectomy was performed initially upon 11 patients with a mean tumor size of 15 centimeters (range of 3 to 40 centimeters). Local recurrence was seen in five patients treated by excision and one patient treated by mastectomy. All patients retreated by repeat excisions were controlled; one patient retreated with mastectomy died with local recurrence and distant disease. Routine axillary dissections were not performed. Involvement of the axillary lymph nodes occurred in one patient and was associated with advanced systemic disease. Local recurrences did not imply associated systemic spread and were controlled in five of six patients by repeat excision or mastectomy. Although the benign behavior of this tumor is commonly emphasized, four of the patients we studied died of malignant spread of cystosarcoma phyllodes.