Jejunal intussusception due to malignant phyllodes tumor of the breast

Phyllodes tumors are rare fibroepithelial neoplasms of the breast; classified as benign, borderline, or malignant based on the mitotic activity, cellular atypia, and stromal overgrowth. Wide surgical excision is the treatment of choice. The most common locations for metastasis are lung, bone, and liver; small intestinal metastasis is extremely rare. Here we present the first patient with jejunal metastases and intussusception due to malignant phyllodes tumor of the breast. Adjuvant treatment of malignant phyllodes tumor needs to be investigated.     

Histologic features predict local recurrence after breast conserving therapy of phyllodes tumors

Background: Pathologists can distinguish benign phyllodes tumors, which very rarely metastasize, from malignant phyllodes tumors, which metastasize in approximately one fourth of patients. However, whether these same histologic criteria can be used to predict the likelihood that a phyllodes tumor will locally recur after breast conserving therapy remains controversial.Study Design: Since few patients with malignant phyllodes tumors have been treated with breast conserving surgery in any individual series, the literature was reviewed using a Medline search.Results: After local excision, 21 (111/540), 46 (18/39), and 65 (26/40) of patients with benign, borderline, and malignant phyllodes tumors, respectively, recurred in the breast. Following wide local excision, 8 (17/212), 29 (20/68), and 36 (16/45) of patients with benign, borderline, and malignant phyllodes tumors recurred in the breast.Conclusions: Malignant phyllodes tumors are much more likely than benign phyllodes tumors to recur in the breast after breast conserving surgery. This high rate of local recurrence of borderline and malignant phyllodes tumors suggests that wide local excision is less than optimal therapy, and challenges us to look for methods to improve local tumor control.     

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.     

乳腺叶状肿瘤的X线表现

目的探讨乳腺叶状肿瘤X线影像学特点,并与其病理对照,以提高对该病的诊断准确率。方法回顾性分析经手术病理证实有完整乳腺X线资料的乳腺叶状肿瘤50例共54个病灶,其中良性26个、交界性22个、恶性6个。结果 54个病灶中表现为伴或不伴钙化的肿块50个(92.6%),假阴性4个(7.4%)。50个肿块病变中良性23个、交界性21个、恶性6个。形状以分叶状、圆形或卵圆形最多见,分别占62.0%(31/50)、10.0%(5/50)、26.0%(13/50)。肿块边缘主要表现为清楚或部分清楚部分模糊,占80%(40/50),其余肿块呈浸润状及细小分叶状边缘,分别占18.0%(9/50)和2.0%(1/50)。肿块表现为高密度占74.0%(37/50),等密度占26.0%(13/50)。3个病灶肿块内含有钙化。边缘特征在良性与恶性肿瘤、交界性肿瘤与恶性肿瘤之间有明显差异,P_(良-恶)=0.003,P_(良-恶)=0.044。结论乳腺叶状肿瘤主要X线表现为分叶状或圆形、卵圆形的高密度肿块,钙化少见;边缘特征可在一定程度上提示良恶性叶状肿瘤的区别。     

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.     

乳腺复发性叶状肿瘤临床病理分析

目的:探讨乳腺复发性叶状肿瘤临床及病理特征。方法:收集2011年01月至2019年12月在我院进行手术治疗的叶状肿瘤病例,并找出其中复发的病例,分析复发病例的临床及病理组织学特征。结果:叶状肿瘤137例,共有10例为复发病例,其中9例为单次复发,1例复发两次,复发病例中良性叶状肿瘤7例,交界性叶状肿瘤2例,恶性叶状肿瘤1例。所有的肿物均为局部复发,良性、交界及恶性叶状肿瘤复发率分别为5.9%、15.4%、20%。其中3例（30%）出现组织学升级,1例良性叶状肿瘤复发为交界性叶状肿瘤,1例交界性叶状肿瘤复发为恶性叶状肿瘤,1例良性叶状肿瘤第一次复发为交界性叶状肿瘤,第二次复发为恶性叶状肿瘤。免疫组化标记CD117、CD34、CD10、p53、p16在原发及复发肿瘤中表达无差异。Ki67增殖指数在复发病例中均升高,并且核分裂数也增多。结论:良性、交界性、恶性叶状肿瘤均可复发,其中恶性叶状肿瘤复发率最高,肿瘤多为局部复发,部分肿瘤复发后出现组织学升级,复发后肿瘤细胞增殖活性增强。     

46例乳腺分叶状肿瘤临床病理特点及误诊分析

目的探讨乳腺分叶状肿瘤的临床病理特点及误诊原因。方法对46例经病理复诊明确诊断的乳腺分叶状肿瘤病例进行回顾性分析,并用免疫组化法检测AE1／AE3、VIM、平滑肌肌动蛋白（Act）和Ki-67表达。结果46例乳腺分叶状肿瘤术前临床诊断相符者24例,其余术前诊断分别为：乳腺纤维腺瘤15例,乳腺增生或混合性囊肿4例,乳腺癌或疑似乳腺癌3例。术前临床诊断误诊率为47．83％（22／46）。术中冰冻病理诊断为乳腺分叶状肿瘤40例,漏诊率为13．04％（6／46）。术后石蜡切片病理诊断：46例乳腺分叶状肿瘤中良性35例,交界性7例,恶性4例。免疫组化表达结果：VIM表达均为阳性,表达在肿瘤问质细胞;Act在部分病例（36．96％,17／46）表达于间质细胞;AEl／AE3均无间质细胞表达（用于排除梭形细胞癌）;Ki-67平均阳性细胞指数在良性、交界性和恶性中分别为13％、28％和69％,Ki-67表达在三者之间差异有统计学意义（P〈0．05）。结论乳腺分叶状肿瘤存在较高的临床误诊率和冰冻病理漏诊率,而其治疗方式的选择与病理分型密切相关;免疫组化对判断其良、恶性有重要意义;肿瘤的及时确诊和手术方式选择是决定治疗成功的关键。     

Phyllodes tumor of the breast: an update

Phyllodes tumor is an uncommon biphasic breast tumor, with the ability to recur and metastasize, and it behaves biologically like a stromal neoplasm. Traditionally, phyllodes tumors are graded by the use of a set of histologic data into benign, borderline, and malignant. In most series, all phyllodes tumors may recur, but only the borderline and malignant phyllodes tumors metastasize. On the basis of histologic features, prediction of behavior is difficult. The expression of many biological markers, including p53, hormone receptors, proliferation markers, angiogenesis group of markers, c-kit, CD10 and epidermal growth factor receptor have been explored, and many have been shown to be variably expressed, depending on the grade of the tumor. These markers are, however, of limited value in predicting the behavior of the tumor. Recently investigators have reported a plethora of genetic changes in phyllodes tumors, the most consistent of which seems to be 1q gain by comparative genomic hybridization. Some candidate genes have been mapped to various sites, and preliminary data suggest that some of these changes may be related to recurrence. It is foreseeable that more exciting data will be generated to help us to understand the etiology and pathogenesis of phyllodes tumor.     

乳腺叶状肿瘤17例诊治分析

[目的]总结乳腺叶状肿瘤的诊治情况。[方法]回顾分析17例经病理证实乳腺叶状肿瘤的临床资料。[结果]17例中良性11例,交界性3例,恶性3例。行乳腺肿瘤局部切除3例,乳腺肿瘤扩大切除7例,乳房切除术4例,乳房改良根治术3例,3例乳腺肿瘤术后出现复发。[结论]乳腺叶状肿瘤术前诊断较困难,主要依靠组织学检查,其局部复发与是否手术彻底切除密切相关。     

乳腺叶状肿瘤17例诊疗分析

目的:探讨乳腺叶状肿瘤的治疗原则和影响预后因素。方法:对经手术和病理诊断确诊的17例乳腺叶状肿瘤的临床资料进行回顾性分析。结果:17例患者中良性叶状肿瘤9例,交界性叶状肿瘤5例,恶性3例。行局部肿块切除7例,单纯乳房切除术6例,改良根治术3例,姑息性肿块切除术1例。其中随访13例,平均随访时间21(5~84)个月,1例行乳腺癌改良根治术后2.5年死于远处转移,2例行局部肿块切除术后复发。结论:乳腺叶状肿瘤的预后与手术方式有关,良性和交界性应首选扩大区段切除术,切除肿瘤边缘不少于2cm;复发的交界性和恶性应尽早行单纯乳房切除术。     

Molecular classification of breast phyllodes tumors: validation of the histologic grading scheme and insights into malignant progression

Phyllodes tumors of the breast are rare fibroepithelial neoplasms with a potential for recurrence. Current histological classification is not always predictive of clinical behavior. The aim of this study was to identify genetic changes associated with the development of borderline and malignant phyllodes tumors in an Asian population, and to assess if genetic data supported the categorization of these tumors into the existing three grades of benign, borderline, and malignant. Expression profiling of 21 phyllodes tumors (6 benign, 10 borderline, 5 malignant) was performed using Affymetrix U133Plus 2.0 GeneChips^®. Gene expression among benign, borderline, and malignant tumors was compared and a 29 gene list was able to classify them according to their histologic grade. Among these 29 genes are those responsible for matrix formation, cell adhesion, epidermis formation, and cell proliferation. Comparative genomic microarray analysis showed that the most common chromosomal alteration associated with borderline and malignant tumors was 1q gain, and an increasing number of chromosomal changes was noted with increasing histological grade. Upregulation of HOXB13 was seen in malignant relative to borderline phyllodes tumors and further investigated by immunohistochemistry in a corresponding set of formalin-fixed, paraffin-embedded tumors. HOXB13 protein overexpression was found to be correlated with stromal hypercellularity and atypia (P = 0.03, P = 0.039, respectively) and may be implicated in the development of malignant phyllodes tumors.     

Comparison of Clinical Characteristics Between Benign Borderline and Malignant Phyllodes Tumors of the Breast

Background: Phyllodes tumors of the breast are rare fibroepithelial lesions, so relatively little is known aboutthis disease entity. The present study was designed to identify differences in clinical features between benignborderline and malignant phyllodes tumors. Materials and Methods: Data from 246 women with phyllodestumors of the breast treated in Cancer Hospital Chinese Academy of Medical Sciences between 2002 and 2012were collected and analyzed, including age at presentation, age at treatment, course, size of primary tumor,location, histological type, type of surgery and treatment, local recurrence, distant metastasis, fibroadenomahistory, disease-free survival and number of mitosis per 10hpf. There are 125 (55%) benign, 55 (24%) borderlineand 47 (21%) malignant tumors. Results: In univariate analysis, average age at presentation, average age attreatment, size of primary tumor, ulceration or not, type of primary surgery, distant metastasis and number ofmitosis per 10 hpf turned out to be statistically different among the three PT types (p=0.014, 0.018, <0.000, 0.003,<0.000, 0.001 and <0.000, respectively), while recurrence and disease-free survival (DFS) demonstrated trendsfor statistical significance (P =0.055 and 0.060, respectively). Multivariate analysis revealed distant metastasisand excision were significantly different in benign, borderline and malignant phyllodes tumors of the breast(p=0.041 and 0.018, OR=0.061 and 0.051). At the same time, size of primary tumor with p=0.052 tended to bedifferent between groups (OR=1.127). However, age at treatment, ulceration and DFS showed no statisticallysignificant variation (p=0.400, 0.286 and 0.413, respectively). Conclusions: Benign borderline and malignantphyllode tumors have different distant metastasis risk, different primary tumor size and different surgicalprocedures, and malignant PTs are more likely to be bigger and to metastasize.     

Phyllodes tumors of the breast

Opinion statement 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.     

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.     

乳腺叶状肿瘤局部复发的临床风险因素分析

目的分析乳腺叶状肿瘤局部复发的临床风险因素。方法使用SPSSCox比例风险模型分析2002年12月至2008年12月中国医科大学附属第一医院66例乳腺叶状肿瘤患者各种临床风险因素与局部复发的关系。单因素分析采用X2检验。结果本组患者的发病年龄为17—83岁（中位年龄41岁）,良性40例,交界性24例,恶性2例。局部复发15例,其中12例复发发生于区段切除术。在不同手术方式、不同组织学等级的叶状肿瘤患者之间,复发率的差异均有统计学意义（P〈0．05）。手术方式为保护因素（RR=0．151）,扩大切除术和乳房切除术者的复发风险较区段切除术者下降至66／1000和53／1000;组织学等级为危险因素（RR=5．803）,恶性和交界性叶状肿瘤患者的局部复发风险分别为良性肿瘤者的12．26倍和4．37倍。结论恶性程度高和手术方式选择不当影响预后。叶状肿瘤以手术治疗为首选,应选择切除范围扩大的手术方式,尤其是对恶性程度高的患者。     

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.     

Phyllodes tumors of the breast diagnostic and therapeutic dilemmas

BACKGROUND: This article compares experiences in the diagnosis and treatment of phyllodes tumors from 2 regional institutions with the relevant literature. PATIENTS AND METHODS: From 1991 to 2005, 2,848 breast cancer patients were treated in our institutions, 36 (1.44%) for phyllodes tumors. The average tumor size was 5.1 cm (range 1.4-19.6). Triple assessment was the standard diagnostic algorithm. Wide excision with tumor-free margins was carried out in 29 (80.5%) cases and mastectomy in 7 (19.4%) cases. Axillary lymphadenectomy was performed in patients with positive lymph nodes. RESULTS: Histology showed the phyllodes tumors to be benign in 27 (75.0%), malignant in 6 (16.6%), and borderline in 3 (8.3%) cases. Follow-up was from 5 months to 16 years. In this period, recurrences of 3 (8.3%) malignant and 2 (5.6%) benign phyllodes tumors were diagnosed and treated. 10 (27.7%) patients treated with wide local excision showed deformities in the form of scarring. The steroid receptor status was of no prognostic value in our patients, and chemotherapy was used in only 1 (2.7%) patient. 5-year survival was 86.2%. CONCLUSION: Our study shows that tumor size, margin infiltration, mitotic activity and degree of cellular atypia are important prognostic factors. Problems in diagnosing this condition arise from its similarity to fibroadenoma. Although wide local excision is usually the treatment of choice, tumor recurrence is common. Axillary lymphadenectomy in malignant phyllodes tumors is, in our opinion, still controversial.     

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.     

巨大良性乳腺叶状肿瘤1例报道及文献复习

目的:提高对乳腺叶状肿瘤临床特点和诊疗现状的认识。方法:回顾我科收治的1例巨大良性乳腺叶状肿瘤的临床资料及诊治过程,并结合文献进行复习。结果:患者右乳肿块7年,B超示:右侧乳腺可探及大小约20cm×20cm混合回声包块,内可见液性暗区。全麻下行右侧乳腺单纯切除术,术中冰冻考虑恶性病变,组织来源不能确定,遂加行右侧腋窝淋巴结清扫术。结论:乳腺叶状肿瘤临床上发病罕见,仅以乳腺肿块为首发症状,术前诊断困难,易误诊,明确诊断主要依靠术后病理学检查,故易导致过度治疗或治疗不当。     

巨大良性乳腺叶状肿瘤1例报道及文献复习

目的：提高对乳腺叶状肿瘤临床特点和诊疗现状的认识。方法：回顾我科收治的1例巨大良性乳腺叶状肿瘤的临床资料及诊治过程,并结合文献进行复习。结果：患者右乳肿块7年,B超示：右侧乳腺可探及大小约20cm×20cm混合回声包块,内可见液性暗区。全麻下行右侧乳腺单纯切除术,术中冰冻考虑恶性病变,组织来源不能确定,遂加行右侧腋窝淋巴结清扫术。结论：乳腺叶状肿瘤临床上发病罕见,仅以乳腺肿块为首发症状,术前诊断困难,易误诊,明确诊断主要依靠术后病理学检查,故易导致过度治疗或治疗不当。