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.     

Chromosomal aberrations and genetic relations in benign, borderline and malignant phyllodes tumors of the breast: a comparative genomic hybridization study

Phyllodes tumors are not quite rare fibroepithelial neoplasms of the breast that show a broad spectrum of clinical behaviour. The molecular genetic features of the heterogenous groups of neoplasms have not been studied in detail yet. We have used comparative genomic hybridization to analyze chromosomal copy number changes in 36 cases of phyllodes tumors (including benign, borderline and malignant phyllodes tumors, 12 cases each). The average number of chromosome copy changes (range) in benign, borderline and malignant phyllodes tumors were 5.58 (0–20), 14.08 (3–23), and 12.42 (0–29) respectively. In benign phyllodes tumors the number of gains and losses was in balance (2.50 vs 3.08), while in borderline and malignant phyllodes tumors gains occurred more often than losses (9.25 vs 4.83, 9.5 vs 2.92). The result suggests the molecular cytogenetics of borderline and malignant phyllodes tumors is similar, and the most striking difference with benign phyllodes tumors is an increased number of chromosomal gains in a nonrandom distribution. Gains of 4q12 seem especially to be involved in the progression of benign to borderline and malignant phyllodes tumors, possibly because of overexpression of oncogenes at these loci.     

Liposarcoma of the breast arising in a malignant phyllodes tumor: A case report and review of the literature

Liposarcoma of the breast is a very rare malignant tumor. It can clinically manifest as a palpable breast mass and mimic primary breast cancer. We report an unusual case of a 51-year-old female who presented with an asymptomatic right breast mass, which was histologically diagnosed as well differentiated liposarcoma arisen within malignant phyllodes tumor. The patient underwent breast conserving surgery, received no adjuvant treatment and is disease-free after 2 years. Radiological and histopathological features are presented and described in detail. Data from the literature are presented and therapy recommendations discussed.     

Cervical cancer metastatic to the breast: a case report and review of the literature

Metastasis to the breast from primary cervical cancer is rare. We describe one such patient, who developed breast metastasis 6 months after diagnosis. She was free of pelvic disease at the time of metastases but had evidence of lung involvement. She died of progressive disease 2 months later. Data on 23 patients collected from the literature via Medline are reviewed.     

乳腺叶状肿瘤复发的治疗及预后分析

目的 探讨乳腺叶状肿瘤复发的治疗方法以及预后.方法 回顾分析1972年3月至2006年6月收治的26例乳腺叶状肿瘤复发患者的临床资料.结果 26例患者的中位生存时间为96个月.原发肿瘤≥5 cm者10例,其中6例出现局部复发,4例死亡;<5 cm者16例,其中5例出现局部复发,3例死亡.复发肿瘤≥5 cm者14例,其中5例出现局部复发,3例死亡;<5 cm者12例,其中6例出现局部复发,4例死亡.原发肿瘤和复发肿瘤的大小对肿瘤再次复发(P=0.094,P=0.383)和预后(P=0.142,P=0.486)影响不显著.良性肿瘤组12例患者中,3例出现局部复发,2例死亡;恶性肿瘤组14例患者中,8例小现局部复发,5例死亡.乳腺叶状肿瘤的良恶性与肿瘤再次复发(P=0.046)和预后(P=0.028)存存相关性.结论 乳腺叶状肿瘤的恶性程度对肿瘤再次局部复发和患者预后有显著影响,而肿瘤大小与再次局部复发和预后无关.乳腺叶状肿瘤复发后的补救手术十分重要,手术必须保证一定的安全范围;多次局部复发患者的于术治疗效果较好.     

Consecutively occurring multiple fibroadenomas of the breast distinguished from phyllodes tumors by clonality analysis of stromal tissue

We present a woman with consecutively occurring multiple fibroadenomas of the breast distinguished from phyllodes tumor by clonality analysis of stromal tissue. Fifty-three masses developed in her right breast in an 11.5 year period since first onset. A clonality analysis, performed by polymerase chain reaction (PCR), focused on theHpa II site within exon 1 of the androgen receptor (AR) gene. Estrogen receptor (ER) α immunohistochemistry was performed with a specific polyclonal antibody against the hormone binding domain of the protein. Since all of the nodules were polyclonal in stromal tissues, they were hyperplastic rather than true neoplasms such as phyllodes tumors. The co-existence of abundant adenosis foci and numerous sub-clinical small nodules in the adjacent breast tissue also suggested proliferative stimuli throughout the breast. ER α protein was expressed only in the nuclei of epithelial cells. The possible influence of ER positive epithelial cells on stromal cell growth is discussed.     

乳腺叶状肿瘤的诊断及治疗

目的探讨乳腺叶状肿瘤的诊断和治疗.方法对经手术和病理诊断的48例乳腺叶状肿瘤的临床病理资料进行回顾性分析.结果48例中平均年龄为43.2岁.根据肿瘤生长方式,瘤细胞异型,核分裂和肿瘤坏死进行乳腺叶状肿瘤分级,良性26例,交界性12例,恶性10例.全部病例采用手术治疗,肿瘤单纯切除术23例,良性、交界性、恶性复发分别为3/18、2/3、2/2;肿瘤扩大切除术15例,良性、交界性、恶性复发分别为0/8、1/5、1/2;乳房切除术10例,交界性、恶性复发转移分别为0/4、2/6.结论乳腺叶状肿瘤可分良性、交界性和恶性三种类别,确诊和分级主要依靠病理组织检查.提示首次肿瘤彻底切除是治疗的关键,应根据组织分级及肿瘤大小决定手术方式.     

Increased epidermal growth factor receptor (EGFR) expression in malignant mammary phyllodes tumors

Mammary phyllodes tumors are uncommon stromal-epithelial neoplasms, and are divided into benign, borderline malignant and frankly malignant groups on the basis of their histological features. Accumulating evidence shows that epidermal growth factor receptor (EGFR) is involved in the pathogenesis and progression of many malignancies. This study investigated 453 phyllodes tumors (296 benign, 98 borderline, 59 malignant) for EGFR expression using immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) for gene amplification. The staining was correlated to tumor margin status, degree of malignancy, stromal cellularity, mitotic activity, nuclear pleomorphism and stromal overgrowth. Cases with strong positive IHC staining were selected for FISH. The overall positive rate for EGFR was 16.2% (48/296), 30.6% (30/98) and 56% (33/59) for benign, borderline malignant and frankly malignant phyllodes tumors, respectively. FISH demonstrated egfr gene amplification in 8% of immunohistochemically positive cases. The results of this study provide strong evidence that EGFR overexpression is involved in the pathogenesis of phyllodes tumors, although gene amplification may not be the major underlying mechanism for overexpression.     

Phyllodes tumors of the breast: The British Columbia Cancer Agency experience

Purpose

Phyllodes tumors of the breast are uncommon fibroepithelial lesions for which optimal management remains unclear. This retrospective population-based study reports treatment and outcomes for patients with phyllodes tumors and evaluates characteristics that influence outcome.

Phyllodes tumor of the breast

PURPOSE: To better identify prognostic factors for local control and survival, as well as the role of different therapeutic options, for phyllodes tumors, a rare fibroepithelial neoplasm of the breast. METHODS AND MATERIALS: Data from 443 women treated between 1971 and 2003 were collected from the Rare Cancer Network. The median age was 40 years (range, 12-87 years). Tumors were benign in 284 cases (64%), borderline in 80 cases (18%), and malignant in 79 cases (18%). Surgery consisted of breast-conserving surgery (BCS) in 377 cases (85%) and total mastectomy (TM) in 66 cases (15%). Thirty-nine patients (9%) received adjuvant radiotherapy (RT). RESULTS: After a median follow-up of 106 months, local recurrence (LR) and distant metastases rates were 19% and 3.4%, respectively. In the malignant and borderline group (n = 159), RT significantly decreased LR (p = 0.02), and TM had better results than BCS (p = 0.0019). Multivariate analysis revealed benign histology, negative margins, and no residual disease (no RD) after initial treatment and RT delivery as independent favorable prognostic factors for local control; benign histology and low number of mitosis for disease-free survival; and pathologic tumor size < or = 3 cm and no tumor necrosis for overall survival. In the malignant and borderline subgroup multivariate analysis TM was the only favorable independent prognostic factor for disease-free survival. CONCLUSIONS: This study showed that phyllodes tumor patients with no RD after treatment have better local control. Benign tumors have a good prognosis after surgery alone. In borderline and malignant tumors, TM had better results than BCS. Thus, in these forms adjuvant RT should be considered according to histologic criteria.     

A very unusual breast lump

A 77 year old woman presenting with a malignant phyllodes tumour of the right breast with exclusively stromal metastases to axillary lymph nodes refractory to both radiotherapy and chemotherapy.     

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.     

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。     

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。     

Regression of metastatic carcinoid tumors with octreotide therapy: two case reports and a review of the literature

BACKGROUND: The antiproliferative effect of the somatostatin analogue, octreotide, on metastatic carcinoid tumors is poorly understood. Partial tumor regression seen radiographically has been reported with the use of octreotide therapy for neuroendocrine tumors. Complete regression of carcinoid tumors is rarely reported. RESULTS: Two patients with metastatic midgut carcinoid tumors were treated with subcutaneous octreotide 300 microg/day for symptomatic control of their carcinoid syndrome before debulking palliative surgery. During the laporatomies, both patients were found to have complete macroscopic regression of the metastatic lesions that had been identified radiologically before surgery, including liver metastases in one patient and periportal and retrocaval lymph nodes in the other. After surgery, the patients were evaluated every 3 months, and had no detectable disease at 30 and 43 months, respectively. Thirty cases of partial tumor regression with octreotide administered with or without other treatment modalities have been reported in the literature. Most of the patients involved received other treatment modalities. Only one other case reported in the literature showed complete regression with octreotide monotherapy. CONCLUSIONS: We report two cases of metastatic midgut carcinoid tumors that demonstrated a significant anti-proliferative response to octreotide monotherapy. Review of the literature failed to identify any specific prognostic factors with which the response to octreotide can be predicted. Possible mechanisms for this antiproliferative effect of octreotide on carcinoid tumors are discussed.     

A case of phyllodes tumor of the breast with a lesion mimicking fibroadenoma

We report a case of phyllodes tumor (PT) with multiple lesions partly mimicking fibroadenoma (FA). A 43-year-old woman was referred to our institute because of a left breast mass which was diagnosed as FA clinically. During the follow-up, the mass gradually enlarged and excisional biopsy was performed to rule out malignant lesion. On histological examination, the tumor consisted of three different components; a lesion diagnosed as FA, another lesion as benign PT, and the other lesion as borderline PT. Finally, the tumor was diagnosed as borderline PT. Stromal cellularity may vary from area to area in any given PT. If there are some disagreements among the findings of imagings and cytology for the diagnosis of FA, as was seen in our case, the possibility of coexistence of PT should be considered.     

妊娠合并乳腺巨大恶性分叶状肿瘤2例报告及文献复习

目的:探讨乳腺恶性分叶状肿瘤的临床特点、治疗方法和预后。方法:回顾性分析2例妊娠合并乳腺巨大恶性分叶状肿瘤的诊治过程,并复习相关文献。结果:第1例患者经手术切除后局部复发,再次手术切除、局部放疗后随访至今,未见肿瘤复发转移;第2例患者,手术后短期内局部复发,再次手术后迅速出现双肺、胸膜转移,化疗疗效不显著,患者最终死亡。结论:乳腺恶性分叶状肿瘤的异质性显著,其诊治方法仍需要不断探索。     

Malignant phyllodes tumor of the breast: local control rates with surgery alone

PURPOSE: Patients with malignant phyllodes tumors of the breast (MPTB) are routinely treated with surgery alone. We performed a retrospective study to determine local control rates based on tumor size and type of surgery performed. METHODS AND MATERIALS: We reviewed records of 478 patients with MPTB treated between March 1964, and August 2005. The data were extracted from the IMPAC National Oncology Database consisting of merged tumor registries from 130 hospitals. RESULTS: Median follow-up was 64 months (range, 0-410 months). Actuarial 5-year local control rates were 79.4% for 169 lumpectomy patients and 91.2% for 207 mastectomy patients treated by surgery alone. Five-year local control rates for lumpectomy based on tumor size were 91% for 0-2 cm tumors, 85% for 2-5 cm tumors, and 59% for 5-10 cm tumors. For mastectomy patients, 5-year local control rates were 100% for 0-2 cm tumors, 95% for 2-5 cm tumors, 88% for 5-10 cm tumors, and 85% for 10-20 cm tumors. Multivariate analysis of overall survival found several factors to be significant including advancing age with each decade after 50 years of age, appearance of distant metastases, larger primary tumor size, and local control vs. local recurrence (Hazard Ratio [HR] 2.5, p < 0.05). CONCLUSIONS: Malignant phyllodes tumors of the breast local recurrence rates are 15% or greater for patients with tumors >2 cm treated by lumpectomy alone and tumors >10 cm treated by mastectomy alone. Adjuvant radiation therapy should be evaluated for these patients. This may be especially important because our study showed that local recurrence impacted on survival rates.     

年轻女性乳腺恶性叶状肿瘤3例及文献复习

目的：探讨年轻女性乳腺恶性叶状肿瘤的临床病理学特点及诊断要点。方法对3例年轻女性乳腺恶性叶状肿瘤的临床资料、病理形态学及免疫组化结果进行分析,并结合相关文献进行讨论。结果3例女性患者,年龄14～20岁;镜下肿瘤细胞异型明显,核分裂＞10／10HPF。结论年轻女性乳腺恶性叶状肿瘤少见,提高对该病临床病理特点的认识,对避免误诊是至关重要的。