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.     

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。     

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。     

Radiotherapy to pulmonary metastases of a malignant phyllodes tumor of the breast

A bulky right breast tumor (15 × 15 cm) was discovered in a 54-year-old woman, and a simple mastectomy was performed, followed by 50 Gy of postoperative radiotherapy. Histologically, the tumor was malignant phyllodes tumor of the breast. Nine months after the mastectomy, multiple pulmonary metastases were detected and three lesions were treated with radiotherapy of 60 Gy/30 fractions for 43–54 days. Two lesions (16 × 12 mm and 18 × 16 mm) showed a partial response and no progression 2–4 months after the radiotherapy. The other lesion (22 × 18 mm) showed a partial response but progressed again 3 months later. She died of respiratory failure 18 months after the mastectomy. She also had muscle metastasis, but there were no locoregional recurrences. The malignant phyllodes tumor in this patient, showed a partial response to radiotherapy. Radiotherapy is considered useful for local control or symptomatic treatment of malignant phyllodes tumor.     

61例乳腺叶状肿瘤诊疗分析

目的探讨乳腺叶状肿瘤的临床诊断、病理以及治疗和预后的关系。方法回顾分析1973年4月至2005年2月收治的61例经病理证实为乳腺分叶状肿瘤患者的临床资料。结果61例患者平均年龄43岁,按WHO分类标准,其中36例为良性,19例为交界性,6例为恶性;34例行局部扩大切除术,18例行单纯乳房切除术,3例行单纯切除+植皮术,6例行改良根治术(1例发现淋巴结转移)。随访52例,平均随访92个月(16~378个月),共13例局部复发和转移,死亡4例。结论局部扩大切除或单纯乳房切除术是治疗乳腺分叶状肿瘤的首选治疗方法,但术前检查证实同侧腋窝淋巴结肿大的患者,考虑行改良根治术。复发治疗以手术为主,需足够的手术范围,以根治为目的。     

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.     

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.     

A case of ductal carcinoma with squamous differentiation in malignant phyllodes tumor

Carcinoma derived from the lining epithelial cells in malignant phyllodes tumor is a rare neoplasm of the breast and belongs to the category of carcinosarcoma. We report a case of ductal carcinoma with squamous differentiation arising in malignant phyllodes tumor. A 54-year-old woman was admitted with a rapidly enlarging left breast mass. A breast tumor with a diameter of 6 cm was located mainly in the left outer area of the breast. Mammography revealed a high-density mass with an irregular margin and ultrasound showed a cystic tumor. A pathological diagnosis of ductal carcinoma with squamous differentiation was made by fine needle aspiration and a core needle biopsy. She underwent neoadjuvant chemotherapy followed by a modified radical mastectomy with a skin flap. Histopathological examination revealed that the invasive ductal carcinoma with squamous differentiation originated from the lining epithelial cells in malignant phyllodes tumor and that there was no transition area between the carcinomatous and the sarcomatous component. She experienced lung and facial bone metastases, microscopic features of which were consistent with the sarcomatous component of the original breast carcinosarcoma. This is an extremely rare case of carcinosarcoma and the histopathological findings and review of the literature are discussed.     

Giant malignant mammary phyllodes tumor: report of a case and review of the literature

BACKGROUND: To distinguish between a benign and malignant phyllodes tumor before surgery is difficult. Wide excision or mastectomy with adequate free margins is necessary in the case of a malignant phyllodes tumor. However, repairing the skin defect after removal of a giant malignant phyllodes tumor is a great challenge for the breast surgeon. CASE REPORT: We report the case of a 45-year-old Taiwanese woman with a giant malignant phyllodes tumor measuring 30 x 25 x 22 cm. Prior to surgery, we carefully designed a flap to cover the skin defect, following standard mastectomy with at least 2 cm free margins. Postoperatively, the patient recovered well without any wound infection or flap necrosis. During follow-up at our outpatient department, there was no evidence of local relapse or distant metastasis. CONCLUSION: Giant malignant phyllodes tumors can be treated by total mastectomy with adequate free margins, using a flap technique to cover the skin defect.     

Malignant transformation of breast fibroadenoma to malignant phyllodes tumor: long-term outcome of 36 malignant phyllodes tumors

Background  

Malignant phyllodes tumor of the breast is a rare neoplasm for which clinical findings remain insufficient for determination of optimal management. We examined the clinical behavior of these lesions in an attempt to determine appropriate management. We evaluated long-term outcome and clinical characteristics of malignant phyllodes tumors arising from fibroadenomas of the breast.     

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.     

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.     

Breast Carcinoma Arising in Cystosarcoma Phyllodes: Report of a Case with a Review of the Literature

An extremely rare case of coexistent carcinoma arising in acystosarcoma phyllodes of the breast is reported. A 41-year-oldJapanese woman was referred with a lump in the right breast.A tumor measuring 5.6x3.6x3.2 cm was removed by local excision.Histological examination revealed foci of papillo-tubular carcinomawithin the benign cystosarcoma phyllodes. Radical mastectomywas performed and the axillary lymph nodes showed no metastasis.While 22 cases of coexistent carcinoma and cystosarcoma phyllodesin the same breast have been reported, in only nine of thosecases, the carcinomas including this case have developed withinthe cystosarcoma phyllodes.     

乳腺叶状肿瘤研究进展

乳腺叶状肿瘤在乳腺肿瘤中较为罕见,其组织学上具有双向分化的特征,影像学有助于乳腺叶状肿瘤的诊断,组织病理学是诊断的金标准。该文通过对国内外乳腺叶状肿瘤的报道进行归纳、总结,从其临床特征、影像学表现、术前穿刺活检、标准手术方式的选择、术后是否行放化疗、内分泌治疗以及影响局部复发的因素进行总结,旨在提高该病的诊断率,避免过度治疗,为临床提供一定的参考意义。乳腺叶状肿瘤是否与相应基因有关,如何利用相关基因指导乳腺叶状肿瘤的治疗可能成为今后研究的重点之一。     

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。     

A case of noninvasive ductal carcinoma arising in malignant phyllodes tumor

We report on an exceedingly rare case of noninvasive ductal carcinoma arising in malignant phyllodes tumor of the breast. The patient was a 75-year-old woman who presented with a chief complaint of an indolent tumor mass of the left breast. Papillotubular carcinoma was diagnosed by aspiration cytology, and mastectomy with preservation of the pectoral muscle was subsequently performed (Bt+Ax+Ic, R2). Histopathological examination showed proliferation of monotonous, uniform tumor cells in a cribriform pattern amid atypical and spindle-shaped cells. Neither stromal invasion of the epithelial tumor cells nor clear transition between epithelial tumor cells and non-epithelial tumor cells was seen. Immunohistochemical staining revealed that the epithelial component was positive for antibodies such as CEA, EMA and keratin, while the non-epithelial component was negative for the same antibodies. Malignant phyllodes tumor with a noninvasive ductal carcinoma was diagnosed rather than true carcinosarcoma of the breast. No metastasis was detected in the axillary lymph nodes, and the patient was classified as stage II A (T2N0M0). Although neither chemoendocrine therapy nor irradiation was employed postoperatively, no recurrence was observed two years and two months after the surgery. There is little consensus on the treatment or prognosis of the disease. Careful observation of the present case is therefore important.     

Malignant phyllodes tumor of the breast with hypoglycemia: report of a case

A surgically resected case of giant malignant phyllodes tumor of the breast associated with a hypoglycemic attack is reported. A 54-year-old woman was referred to our hospital with loss of consciousness and a huge chest wall tumor. She was diagnosed as having a malignant phyllodes tumor by core needle biopsy and underwent palliative simple mastectomy because lung metastasis was detected on computed tomography and by other imaging modalities on admission. The preoperative laboratory data revealed a very low fasting blood sugar level of 37 mg/dl. After removal of the tumor, the blood sugar level gradually normalized (90-100 mg/dl) and the plasma insulin-like growth factor-II (IGF-II) level promptly decreased. The IGF-II level of tumor extracts was high (2500 ng/g wet weight) and the majority of atypical cells stained positively for IGF-II immunohistochemically. These findings suggested that the patient's hypoglycemia was associated with IGF-II produced by a giant malignant phyllodes tumor that consumed glucose.