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.     

Long-term results of surgical therapy for phyllodes tumor of the breast

We report that the long-term results of surgical therapy for phyllodes tumor of the breast. During ten years, we experienced twenty-eight patients of phyllodes tumors of the breast. They were treated by surgical operation. Twenty-one cases were performed local excision with at least 1 cm surgical margin, and seven cases were performed muscle preserving mastectomy without axillary lymph nodes dissection. Pathological surgical margins of all patients were negative. Five patients were recognized the local recurrence, and only one patient was recognized a distant metastasis. The total recurrence rate was 21.4%. The patients with local recurrence were diagnosed the benign phyllodes tumor of the breast at the first operation. The mean time to recurrence was 5.4 years. If the pathological diagnosis was benign phyllodes tumor and surgical margin was negative at the first operation, it is necessary to follow up the patients, because there is a risk of local and distant metastasis.     

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.     

Le sarcome stromal périductal : une nouvelle observation d’une tumeur rare

Periductal stromal sarcoma is an extremely rare malignant fibroepithelial breast tumor, long confused with phyllodes tumors. This confusion is justified by its biphasic histology comprising two components: ductal or epithelial benign, surrounded by a sarcomatous periductal stroma made of spindle cells. Currently, it is a distinct pathological entity. Wide surgery with free margins is enough and is the only treatment currently validated, the value of adjuvant therapy based on radiation and/or chemotherapy remains to be demonstrated. The prognosis is marked by local recurrence sometimes as phyllodes tumors or specific soft tissue sarcoma more aggressive, which requires close monitoring based on clinical examination.     

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.     

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。     

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.     

Primary treatment of cystosarcoma phyllodes of the breast

BACKGROUND: Cystosarcoma phyllodes is a rare sarcoma of the breast. Although surgical removal is the mainstay of treatment, the extent of surgery required (excision vs. mastectomy) and the need for additional local therapy, such as radiotherapy, are unclear. The current study evaluated the rate of local and distant failure, as well as potential prognostic factors, to better define appropriate treatment strategies. METHODS: One hundred one patients treated primarily for cystosarcoma phyllodes of the breast were evaluated. These tumors were classified histologically into benign (58%), indeterminate (12%), and malignant (30%) based on well defined criteria. Stromal overgrowth (29%) was considered separately. Surgery was comprised of local excision with breast conservation (47%) or mastectomy (53%). Microscopic surgical margins were negative in 99% of cases. Six patients received adjuvant radiotherapy. RESULTS: Overall survival for the 101 patients was 88%, 79%, and 62% at 5, 10, and 15 years, respectively. For patients with nonmalignant (benign or indeterminate) and malignant cystosarcoma phyllodes, the overall survival was 91% and 82%, respectively, at 5 years, and 79% and 42%, respectively, at 10 years. Similar rates were observed based on the presence or absence of stromal overgrowth. Local recurrence occurred in 4 patients, with an actuarial 10-year rate of 8%. Eight patients developed distant metastases, with an actuarial 10-year rate of 13%. Multivariate analysis using Cox proportional hazards regression revealed stromal overgrowth to be the only independent predictor of distant failure. CONCLUSIONS: Local failure in this group of largely margin negative patients with cystosarcoma phyllodes of the breast was low, showing that breast-conserving surgery with appropriate margins is the preferred primary therapy. The current study data do not support the use of adjuvant radiotherapy for patients with adequately resected disease. Patients with stromal overgrowth, particularly when the tumor size was > 5 cm, were found to have a high rate of distant failure; such patients merit consideration of a trial that examines the efficacy of systemic therapy.     

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.     

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.     

乳腺分叶状肿瘤八例报告

回顾分析8例乳腺分叶状肿瘤患者的临床病理资料，良性2例，交界性2例，恶性4例，患者年龄29～50岁，平均年龄41岁。肿块最大径平均6．6cm（3～38cm）。4例有肿块短时间内迅速增大的病史，4例曾接受2～3次肿瘤局部切除术，1例行局部扩大切除术，2例行乳腺单纯切除术，3例行乳腺改良根治术，1例胸壁复发的巨大肿瘤行皮肤与肋骨的全层切除、背阔肌-侧胸-腹直肌跨区皮瓣转移修复，1例在20个月内做过5次手术。随访3～72个月，8例患者均生存，其中2例分别于首次手术后8和21个月出现肺转移。回顾分析提示，乳腺分叶状肿瘤术前诊断困难，易局部复发，保证切缘阴性的局部扩大切除术是治疗乳腺分叶状肿瘤的合适的手段。     

Elderly breast cancer patients treated by conservative surgery alone plus adjuvant tamoxifen: fifteen-year results of a prospective study

BACKGROUND: In elderly patients with early breast cancer and a clinically clear axilla, axillary surgery, sentinel lymph node biopsy, and postoperative radiotherapy to the residual breast may not be necessary because of reduced life expectancy, effectiveness of hormone therapy in achieving long-term disease control, and generally favorable biologic behavior of breast cancer in elderly patients. METHODS: The authors followed 354 prospectively recruited women aged > or =70 years who had primary, operable breast cancer and no palpable axillary lymph nodes. All 354 women were treated with conservative surgery and adjuvant tamoxifen and without axillary dissection or postoperative radiotherapy. Women who had resection margins in tumor tissue were excluded. Endpoints were cumulative incidence of axillary disease, cumulative incidence of ipsilateral breast tumor recurrence (IBTR), and breast cancer mortality. RESULTS: After a median follow-up of 15 years (interquartile range, 14-17 years), the crude cumulative incidence was 4.2% (4% in pathologic T1 [pT1] tumors) for axillary disease, 8.3% (7.3% in pT1 tumors) for IBTR, and 17% for breast cancer mortality. Of the 268 patients who died during follow-up, 222 patients (83%) died from causes unrelated to breast cancer. CONCLUSIONS: Elderly patients with early breast cancer and no palpable axillary lymph nodes may be safely treated safety by conservative surgery without axillary dissection and without postoperative radiotherapy, provided that surgical margins are in tumor-free tissue and that hormone therapy is administered. Sentinel lymph node biopsy is also unnecessary because of the low cumulative incidence of axillary disease, and axillary surgery can be reserved for the small proportion of patients who later develop overt axillary disease.     

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.     

En Bloc Excision of Phyllodes Tumor of the Breast: Radical Approach Heralds Better Outcome

IntroductionSurgery is the primary treatment of phyllodes tumor of the breast, and margins are the most important risk factor associated with local recurrence. We conducted a retrospective audit of 433 patients treated at our center.Patients and MethodsWomen who presented with phyllodes tumors between 1999 and 2017 were included in the analysis. Data was collected from the hospital medical records, telephonic interviews, and electronic mail.ResultsOf the 433 women included in this study, 177 (40.9%) had benign phyllodes tumors, 84 (19.4%) were borderline, 131 (30.3%) were malignant, and 41 (9.5%) had sarcoma. A history of previous excision was noted in 154 (35.6%) patients, of which 104 presented with local recurrence. Of the total patients, 209 (48.3%) underwent breast conservation surgery; the median pT was 6 cm. At a median follow-up of 37.9 months, the 5-year disease-free survival (DFS) was 82.9%. On multivariate analysis, the factors that impacted DFS were histology (hazard ratio, 4.1; 95% confidence interval [CI], 1.5-10.9; P = .005) and history of previous excision biopsy (hazard ratio, 3.39; 95% CI, 1.76-6.52; P < .001). We analyzed 231 women who presented without any prior excision separately, wherein at a median follow-up of 44.1 months, the DFS was 92.1% (95% CI, 92.05%-92.15%). In addition, less recurrences were noted in this cohort (5.6% [13/231] in no-excision biopsy vs. 12.5% with surgery done prior to presentation to our institute).ConclusionA previous history of excision and the histologic subtype of phyllodes tumor are factors that have an impact on DFS, thus emphasizing the need for appropriate surgical planning and en bloc excision of the phyllodes at presentation.     

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。     

Malignant phyllodes tumor of the female breast: association of primary therapy with cause-specific survival from the Surveillance, Epidemiology, and End Results (SEER) program

BACKGROUND: Malignant phyllodes tumor is a rare and potentially aggressive breast neoplasm. Little information is available regarding the optimal management of these lesions and rarer still are data regarding survival. The current study used a large population database to determine prognostic factors that predict cause-specific survival (CSS). METHODS: Data were obtained from the Surveillance, Epidemiology, and End Results Program (SEER) for the years 1983-2002. Women receiving resection for primary nonmetastatic malignant phyllodes tumor of the breast were included (n = 821). Analyses of patient, pathologic, and treatment characteristics were performed using univariate and multivariate Cox regression analyses for the CSS endpoint. RESULTS: With a median follow-up of 5.7 years, CSS was 91%, 89%, and 89%, at 5, 10, and 15 years, respectively. Mastectomy was performed in 428 women (52%) and wide excision or lumpectomy in 393 (48%). Women undergoing mastectomy were significantly older (P = .004) and had larger tumors (P = .009). Wide excision was associated with equivalent or improved CSS relative to mastectomy on univariate and multivariate analyses. Older age predicted for cause-specific mortality on multivariate analysis. Adjuvant radiotherapy (RT) predicted for worse CSS when implemented compared with surgery alone. CONCLUSIONS: Mastectomy was not found to provide a benefit in CSS compared with wide excision in malignant phyllodes tumor of the breast. Women undergoing wide excision had at the minimum similar cancer-specific mortality compared with those who received mastectomy. The role of adjuvant RT is uncertain and requires further investigation.     

Phyllodes tumor arising in ectopic breast tissue of the axilla

We examined a 31-year-old woman with a solid mass in her left axilla. Physical examination and ultrasonography confirmed a 2 cm well-defined mass. Fine needle aspiration biopsy suggested fibroadenoma of breast. Excisional biopsy revealed benign phyllodes tumor of the ectopic breast tissue. Phyllodes tumor in ectopic breast tissue is an extremely rare occurrence. Only nine cases have been reported, including tumors of the vulva, inguinal region and axilla. This is the second case in the axillary region.     

Management of Rare Breast Tumors and Male Breast Cancer

Purpose of Review

There is very little clinical trial data available to guide the management of uncommon breast cancers. This review draws on classic and recent retrospective data to synthesize a clearer understanding of the biology and behavior of these rare tumors.

Recent Findings

Avoid disfiguring surgery in the pursuit of very wide margins for benign phyllodes tumors unless there are adverse histological features. Encapsulated papillary carcinoma is very good prognosis breast cancer; do not over treat. Metaplastic carcinoma is a basal type breast cancer that may be more responsive to “BRCA1-like” treatments. Complete excision of the capsule is curative for most implant-associated anaplastic large cell lymphomas. Aromatase inhibitors are still controversial for male breast cancer; tamoxifen is standard.

Summary

Available data are useful for guiding the treatment of rare breast cancers, but it must be recognized that there is still a great deal to be learned about each of these entities.

     

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。