乳腺叶状囊肉瘤的诊断与治疗

1961年~1991年我院共收治乳腺叶状囊肉瘤共120例,全部均行手术治疗。局部切除术71例的术后复发率为48.3%(34/71),其中低度恶性66例的复发率为43.9%(29/66)。中度恶性5例全部复发。全乳切除术58例中术后仅1例高度恶性,复发。高度恶性13例(全乳切除6例,根治术7例)术后局部复发1例,远处转移6例(肺、胸膜转移各2例,肝转移1例,骨及脑转移各1例)。本文就其诊断及治疗进行分析和探讨。     

Cystosarcoma phyllodes malignum: a case report of a successive triple modality treatment

This paper reports on a woman with a rapidly growing recurrent cystosarcoma phyllodes malignum after two major attempts of surgery. In this situation, neoadjuvant hyperfractionated radiotherapy, superficial hyperthermia and ifosfamide were administered. Toxicity was mild. Resection of the tumour bed revealed a pathologically complete response with an actual disease free followup of 48 months.     

乳腺叶状囊肉瘤的21例临床分析

目的 探讨乳腺叶状囊肉瘤的诊断和治疗方法。方法 对1987年9月至2004年12月收治的21例乳腺叶状囊肉瘤病人的临床资料进行回顾性分析。结果 21例中行局部切除术5例,局部扩大切除术9例,单纯乳房切除术4例,改良根治术2例,皮下腺体切除术加乳房再造术1例。随访至2004年12月,5年生存率95．2％,局部复发率9．5％。结论 乳腺叶状囊肉瘤是一种特殊类型的乳腺肿瘤,其预后与肿瘤的组织学分级、肿瘤大小以及手术切除是否彻底有关。     

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.     

Surgical treatment of phyllodes tumors of the breast: retrospective review of 172 cases

BACKGROUND AND OBJECTIVES: Phyllodes tumors (PTs) are uncommon biphasic breast tumors that usually occur in adult females. They are composed of a benign epithelial component and a cellular, spindle cell stroma forming a leaf-like structure. No one morphologic finding is reliable in predicting the clinical behavior of the tumor. The purpose of this study was to explore the clinicopathologic factors associated with outcome and metastasis. METHODS: We retrospectively reviewed the records of 172 patients seen at Mackay Memorial Hospital from January 1985 to December 2003. Clinical data analyzed included age, presenting symptoms and signs, tumor size, location, type of surgery, time to recurrence, and metastasis. The clinicopathologic factors associated with outcome and metastasis were analyzed statistically using the chi-square test with Yate correction. RESULTS: The mean follow-up was 71 months (range 7-237). The mean age was 37 years (range 11-73). The majority of tumors were found in the upper outer quadrant (46.0%), with an equal propensity to occur in either breast (48.8% vs. 50.0%). The pathologic diagnoses included 131 benign, 12 borderline, and 29 malignant lesions. Nineteen patients (11%) had a recurrence and three (1.7%) had metastases. The initial diagnosis of all 19 recurrent tumors were benign. Age, surgical approach, mitotic activity, and surgical margin were significantly correlated with recurrence (P = 0.029, 0.020, 0.048, and 0.00018, respectively). Stromal cellularity, stromal overgrowth, stromal atypia, mitotic activity, tumor margin, and heterologous stromal elements were significantly correlated with metastases (P = 0.032, 0.00008, 0.000002, 0.004, 0.005, and 0.046, respectively). Mammography and breast echo were not reliable for differentiating PTs from fibroadenomas (6.9% vs. 37.9% and 3.3% vs. 45%, respectively). Frozen section was of limited value (41.6%). The role of adjuvant radiotherapy and chemotherapy remains to be defined. Local excision, wide excision, or mastectomy with negative surgical margins yielded high local control rates (88.7%, 88.2%, and 100%, respectively), but local excision was associated with a relatively high percentage of positive surgical margins (18.3%). A total of 42 modified radical mastectomies were performed. The reasons for these procedures included a diagnosis of malignancy on frozen section or because the tumors were so large, they were assumed to be carcinomas. No axillary lymph node metastases were found. Fifteen patients in our series had tumors with infiltrating tumor margin, severe stromal overgrowth, atypia, and cellularity. All three patients with metastases (3/15) were in this group. Presence of metastases was significantly correlated with this group (P = 0.0000038). CONCLUSIONS: Wide excision with a clear margin may be the preferable initial therapy, even for malignant PTs. Routine axillary lymph node dissection is not recommended. Patients have tumors with infiltrating tumor margin, severe stromal overgrowth, atypia, and cellularity are at high risk for metastases.     

Infiltrating ductal carcinoma and synchronous malignant phyllodes tumour: Diagnostic and therapeutic approaches

Simultaneous presentation of breast cancer and malignant phyllodes tumour is rare. A female patient presented with a nodule in her left breast (infiltrating ductal carcinoma). On magnetic nuclear resonance another suspicious lesion (malignant phyllodes) was found in the right breast. Bilateral mastectomy was performed. Thirty two months later the patient is still free of disease. The approach to dealing with synchronous breast tumours should be the same as that normally used.     

乳腺叶状囊肉瘤1例报告并文献复习

目的：报道乳腺叶状囊肉瘤1例,并探讨其临床病理特征、诊断及治疗方法。方法：回顾性分析1例乳腺叶状囊肉瘤患者的临床资料,结合相关文献进行病理、病因、诊断及治疗等方面的讨论。结果：本例患者曾因“乳腺纤维瘤”先后6次手术,第6次手术诊断为恶性分叶状肿瘤,术后半年出现胸壁、牙龈转移,胸壁、牙龈肿物切除术后,发现颧面部、上颌窦、胸椎多发转移,行2周期化疗,临床症状获得缓解,后因经济原因放弃治疗,随访3个月患者死亡。结论：乳腺叶状囊肉瘤由良性上皮成分和富于细胞的恶性问质成分组成,细针穿刺细胞学检查诊断价值有限,常需要手术获取完整标本,并结合免疫组化进行诊断。一旦出现复发或远处转移,应行全面检查明确病灶范围,进行综合治疗。其临床表现具有异质性,复发及远处转移者预后较差。     

Cystosarcoma phyllodes metastatic to the mandible

Cystosarcoma phyllodes of the breast appears to encompass a diverse group of tumors with variable clinical behaviors. Although the tumor can behave in a malignant fashion, metastases to the head and neck region are distinctly uncommon. A case is presented of a solitary metastasis to the mandible appearing 1 year after mastectomy and in the absence of widespread disease. The metastatic potential of this neoplasm is discussed and involvement of the head and neck region is reviewed.     

Locally advanced cervix cancer: Chemotherapy prior to definitive surgery or radiotherapy. A single institutional experience

Primary or neoadjuvant chemotherapy prior to definitive local therapy has potential advantages for locally advanced cervix cancer. It can downstage a cancer and allow definitive local therapy to be technically possible (surgery), or potentially more effective (radiotherapy). It can also eradicate subclinical systemic metastases. This report reviews a single institution’s experience of neoadjuvant chemotherapy prior to definitive local therapy for cervix cancer over a 13‐year period. One hundred and six patients were treated with this intent. The patients were analysed for their response to chemotherapy, treatment received, survival, relapse and toxicity. The chemotherapy was feasible and the majority of patients had a complete or partial response (58.5%). Eight patients did not proceed to local treatment. Forty‐six patients had definitive surgery and 52 had definitive radiotherapy. The 5‐year overall survival was 27% and the majority of patients died with disease. The first site of relapse was usually in the pelvis (46.2%). Late complications that required ongoing medical therapy (n = 6) or surgical intervention (n = 2) were recorded in eight patients (7.5%). On univariate analysis stage (P = 0.04), tumour size (P = 0.01), lymph node status (P = 0.003), response to chemotherapy (P = 0.045) and treatment (P = 0.003) were all significant predictors of survival. On multivariate analysis, tumour size (P < 0.0001) and nodal status (P = 0.02) were significant predictors of survival. Despite the impressive responses to chemotherapy of advanced cervix cancer, there is evidence from randomized trials that it does not improve or compromise survival prior to radiotherapy. As its role prior to surgery remains unclear, it should not be used in this setting outside a prospective randomized trial.     

Cystosarcoma phyllodes: a clinicopathologic analysis of 42 cases.

A retrospective clinicopathologic evaluation of 42 patients with cystosarcoma phyllodes was undertaken to determine if tumor size, contour, degree of stromal atypia and mitotic activity were reliable indicators of clinical behavior. Excluding size, the latter three determinants showed a positive correlation with prognosis and served as the basis of a classification in which 18 benign, 5 borderline and 19 malignant cystosarcomas were diagnosed. The tumors occurred in women averaging 44.3 years of age who most often presented with a palpable occasionally painful mass with a median diameter of 5 cm. Excision or simple mastectomy were the more frequent forms of therapy. Local recurrences were experienced by 6 patients and occurred in all 3 categories of tumor. Only malignant neoplasms developed systemic metastases which was observed in 4 patients 3 of which have died. On borderline tumor recurred 14 times and eventually proved fatal as a result of contiguous pulmonary involvement. A plea is made to label the stroma of malignant cystosarcomas as to the cell(s) of origin so future investigators may evaluate the effect of various soft tissue patterns on prognosis.     

Management of non metastatic phyllodes tumors of the breast: review of the literature

Phyllodes tumors of the breast are rare tumors, accounting for less than 0.5% of all breast tumors. These tumors are comprised of both stromal and epithelial elements; and traditionally they are graded by the use of a set of histologic features into benign, borderline, and malignant subtypes. Unfortunately, the histologic classification of phyllodes tumors does not reliably predict clinical behavior.The mainstay of treatment of non metastatic phyllodes tumors of the breast is complete surgical resection with wide resection margins. Lumpectomy or partial mastectomy is the preferred surgical therapy. However, despite the complete surgical resection, local failure rate may be high; and 22% of malignant tumors may give rise to haematogenous metastases. The most frequent site of distant metastases is the lungs. Several predictive factors of recurrence and metastases have been described in the literature, such as positive surgical margins, increased stromal cellularity, stromal overgrowth, stromal atypia and increased mitotic activity.Nevertheless, the role of adjuvant therapies (radiotherapy and chemotherapy) is presently undefined and should be tested in multicenter, prospective, randomized trials.     

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。     

Bilateral malignant phyllodes tumours.

We report a rare example of bilateral primary malignant phyllodes tumours. The diagnosis was supported by the identification of a benign epithelial element in each lesion. The case illustrates the typical dimorphic features of malignant phyllodes tumours. A contralateral tumour should not be regarded as metastatic without histological confirmation.     

乳腺叶状囊肉瘤合并骨转移1例并文献复习

1 病例资料 患者女,55岁,2012年6月因"发现右乳腺肿块8年余,明显增大半年"到我院乳腺外科就诊,自诉初起时为一蚕豆大小肿物,质地较硬,近半年明显增大,查体可扪及右乳巨大肿块,大小约11 cm×11 cm×9 cm,肿瘤边界欠清,不伴局部皮肤发红、水肿、增厚、粗糙、溃烂,质地偏硬,CT提示乳腺叶状囊肉瘤(cytosarcoma phyllodes,CP)可能(图1),遂行右乳单纯切除术,术后病理示:①(右乳)叶状囊肉瘤,肉瘤成分主要为纤维肉瘤;②右乳低切缘、乳头、皮肤及肿物周围均未见瘤组织浸润(图2).     

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.     

Giant phyllodes tumor in both breasts:one case report and literature review

Breast phyllodes tumor (BPT),which is composed of epithelial cells and fibrous connective tissue,is a special type of tumor and yet not unified in biological behavior and histological classification,with high risk of recurrence and potential metastasis.BPT in one side has high incidence in clinic while BPT in both sides is rarely found.We treated one patient with giant BPT in both sides and reported as follows. 1 Case report The patient,female,aged 49,was admitted due to a rapidly increased lump in the right breast in June 2010,and she complained that the lump had increased from the size of "an egg" to the size of "a basketball" in August and had then been treated as suspected inflammation with anti-inflammatory treatment before admission but failed.Physical examination on admission showed an about 35 cm× 32 cm ×33 cm huge lump in the right breast,firm and poorly-movable,with reddish purple and mildly warm epidermis,swollen but not ulcerated (Fig.1).     

Tumeurs phyllodes et sarcomes du sein : mise au point

Phyllodes tumors and sarcomas of the breast are non-epithelial tumors of the breast. Phyllodes tumors are benign tumors, tumors of intermediate malignancy or malignant tumors. The differential diagnosis with a very proliferant fibroadenoma may be difficult. Histological sub-type, type of surgery (definitive or not) and stromal proliferation determine the prognosis. There is a risk of local relapse and distant metastases, in particular to the lung. Surgery (often radical) is the standard treatment. Radiotherapy is recommended in case of high-grade tumor and after conservativetreatment. Breast sarcomas are even rarer. All histological types exist with a predominance of histiofibrocytome type tumors. Grade, involved margins and sometimes tumor necrosis are major prognostics factors. Among the various sub-types, angiosarcoma is characterized by a high risk of occurrence in irradiated fields and by a poor prognosis with a high risk of lung metastases. The treatment is mostly based on mastectomy without lymph node dissection given the exceptional flooding axillary. In some situations, a conservative treatment can be discussed, based on tumor size, grade and volume of the breast. Locoregional radiotherapy is often proposed for tumors over 5 cm and/or of high grade. Systemic chemotherapy is not a standard but should be discussed in the forms at high risk of relapse (like angiosarcoma).     

Histologic examination of two cases of cystosarcoma phylloaes with pulmonary metastases

BACKGROUND: Cystosarcoma phyllodes (CP) is a rare neoplasm of the breast. Many studies of the histology of CP have been reported. However, few reports have included an evaluation of the histologic appearance of pulmonary metastases, or the change in histologic grade as a function of time in patients with recurrent tumors. METHODS: We treated two patients with pulmonary metastases, CP from 1973 to 1995. One patient died of respiratory failure. The other underwent six operations for CP. We evaluated the histologic characteristics of these metastases and changes in the histologic grade of recurrent tumors. RESULTS: The primary lesions in these two cases were typical high-grade malignant tumors. Case 1 had multiple pulmonary metastases and histologic findings indicated typical malignant CP. Case 2 had a solitary pulmonary metastasis and histologic findings showed low-grade malignant CP, which could be resected. The first patient died of respiratory failure ten months after surgery. The second had no further pulmonary metastases although she had frequent local recurrences, and the histologic features of these tumors became progressively worse. CONCLUSION: We suggest that patients with malignant CP be followed closely and that when pulmonary metastases are detected, they should be resected if possible, because pulmonary metastatic tumors may represent lower-grade malignant CP.