breast fibromatosis

  • 文章类型: Journal Article
    乳腺纤维瘤病(也称为乳腺纤维瘤)是肿瘤学家和外科医生护理乳腺疾病患者的罕见实体。当前的文献高度依赖于病例系列和来自硬纤维瘤相关疾病其他部位的外推。关于病理起源仍不清楚,自然史,以及对这种情况的治疗反应。传统的治疗策略以手术切除为中心,这可能会导致显著毁容的外观和功能结果,经常需要重新手术,和相关的发病率。与药物治疗或观察等待策略相比,支持前期手术切除的优越性的数据有限。当前针对韧带样肿瘤的治疗指南并未将乳房作为疾病部位,并且由于缺乏可用的证据而有目的地模棱两可。我们的目标是回顾有关乳腺纤维瘤病的文献,并根据我们在高容量四元转诊中心的病理学经验,提出一种当前对这种罕见疾病进行循证治疗的算法。
    Desmoid fibromatosis of the breast (also known as desmoid tumor of the breast) is a rare entity infrequently encountered by oncologists and surgeons caring for patients with breast disease. The current body of literature is highly reliant on case series and extrapolations from other sites of desmoid tumor-related disease. Much remains unclear regarding the pathological origins, natural history, and response to treatment of this condition. Traditional treatment strategies have centered on surgical resection, which may result in significantly disfiguring cosmetic and functional outcomes, frequent need for re-operation, and associated morbidity. There are limited data to support the superiority of upfront surgical resection when compared to medical therapy or watchful waiting strategies. Current treatment guidelines for desmoid tumors do not focus on the breast as a site of disease and are purposefully ambiguous due to the paucity of evidence available. We aim to review the literature concerning desmoid fibromatosis of the breast and propose an algorithm for current evidence-based management of this rare disease in the context of our experience with this pathology at a high-volume quaternary referral center.
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  • 文章类型: Case Reports
    乳腺纤维瘤病是间质起源的良性乳腺肿瘤,占乳腺肿瘤的0.2%。本研究报告两例乳腺纤维瘤病突出其诊断,形态学,治疗和进化特征。在这两种情况下,这种肿瘤在临床和放射学上模仿癌症。诊断确认基于组织学检查。乳腺纤维瘤病的特点是局部进展和复发倾向,因此,在我们的患者中,具有自由手术切缘的手术切除的作用。局部治疗(放疗和冷冻治疗)和药物治疗的作用,特别是抗雌激素治疗,没有明确定义。总之,必须知道乳腺纤维瘤病,因为它模仿乳腺癌,具有非常高的复发率,没有发展转移。
    Breast fibromatosis is a benign breast tumor of mesenchymal origin, accounting for 0.2% of breast tumors. This study reports two cases of breast fibromatosis highlighting its diagnostic, morphological, therapeutic and evolutionary features. In both cases, this tumor clinically and radiologically mimicked a cancer. Diagnostic confirmation was based on histological examination. Breast fibromatosis was characterized by local progression and a tendency to recurrence, hence the role of surgical excision with free surgical margins in our patients. The role of locoregional treatments (radiotherapy and cryotherapy) and medical treatments, in particular anti-estrogen therapy, is not clearly defined. In conclusion, breast fibromatosis must be known as it mimicks breast cancer and is characterized by a very high recurrence rate, without ever developing metastases.
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  • 文章类型: Journal Article
    Studies suggest that surgical breast augmentation with implants is a risk factor for breast desmoid tumors. The statistical strength of this correlation is unknown, as evidence is limited to anecdotal reports.
    Patients with breast desmoid tumors and a history of breast implants seen at a single center between 2000 and 2021 were identified via radiology, breast, and sarcoma databases. The standardized incidence ratio (SIR) was calculated to assess the correlation between breast desmoid tumors and breast implants. The cases were pooled with published cases for analyses. Progression-free survival curves and hazard ratios were estimated using the Kaplan-Meier method and Cox proportional-hazards modeling.
    Fourteen patients from one institution and 66 cases in the literature were identified. All patients were female, and the mean age was 38 years old (range 20-66). 63 patients (82%) underwent resection, 9 (12%) received chemotherapy, 3 (4%) received sorafenib, 11 (14%) received hormonal therapy, and 3 (4%) underwent active surveillance. After resection, the 2-year recurrence-free survival rate was 77% (95% CI 65%-89%). The recurrence risk was lower for resection with no residual tumor (R0) compared to microscopic (R1) or macroscopic (R2) residual tumor (HR: 0.15; 95% CI 0.02-0.8; p < 0.05). The SIR was 482 (95% CI 259-775) to 823 (95% CI 442-1322), suggesting a 482-823 times higher risk of developing a breast desmoid tumor after breast augmentation than the general population.
    We present a nonrandom association between breast implants and desmoid tumors. Whether the tumors arise from the surgical trauma or the implant\'s biomaterial is unknown. When surgery is indicated, negative margins reduce the risk of recurrence.
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  • 文章类型: Case Reports
    We herein present a rare case of breast fibromatosis, the contrast-enhanced ultrasonography (CEUS) findings of which we believe have never been described. The high similarity between the clinical and imaging manifestations of breast cancer makes its differential diagnosis difficult. In this report, we describe the CEUS findings of a less common type of fibromatosis, discuss the potential value of CEUS to differentiate it from malignant breast lesions, and briefly review the literature.
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  • 文章类型: Journal Article
    Breast fibromatosis is a rare histologically benign tumor with local aggressive potential, and imaging and clinical findings of breast fibromatosis require attention. We retrospectively evaluated the images of 20 patients with histologically proven breast fibromatosis on mammography, magnetic resonance imaging (MRI), and ultrasonography. The lesions were assessed concerning the location, fascia involvement, imaging characteristics, and follow-up outcomes. Altogether, there were 22 lesions: 10 lesions involved the superficial fascia system including four lesions additionally involving the deep fascia and pectoralis major, and 12 lesions were inside the glandular parenchyma with two lesions originated from the prior surgery site. The detection rates of mammography, ultrasound, and MRI for breast fibromatosis were 33.3% (3/9), 90% (18/20), and 100% (3/3), respectively. We found that fascia involvement may be a characteristic of breast fibromatosis. The lesion located inside glandular parenchyma is prone to be underestimated, whereas combined MR with ultrasound is recommended for the diagnosis. The complete excision with negative margins is important for a good prognosis.
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  • 文章类型: Journal Article
    Fibromatosis of the breast is a rare condition that can be locally aggressive. The mainstay of treatment remains wide local excision, with varied adjuvant therapy as needed. The authors describe their experience in the treatment of a series of patients and propose the classification of primary and secondary breast fibromatosis. A single-institution retrospective analysis of patients treated for breast fibromatosis from 2003 to 2017 was completed. Demographic data, pertinent past medical history, and treatment modalities were reviewed. Primary breast fibromatosis was defined as arising in the absence of previous surgery or radiation therapy to the ipsilateral breast. Secondary breast fibromatosis was defined as arising in the setting of previous surgery or radiation therapy to the ipsilateral breast. A total of 16 patients were included with the median age 40 (28-64) years. The average size of the lesion was 6.37 cm (range of 1.5-15 cm). Mean follow-up time was 65 months. Surgical excision was completed in 14 patients, with two recurrences. There were no recurrences in patients with surgical margins >1 cm. Two patients were treated nonsurgically. There were seven patients with primary fibromatosis of the breast and nine patients with secondary fibromatosis of the breast. Fibromatosis of the breast is difficult to diagnose prior to surgical excision. We advocate for the multi-disciplinary treatment of this disease process with an aggressive surgical approach to achieve margins >1 cm.
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  • 文章类型: Case Reports
    Breast fibromatosis, also referred to as desmoid tumor or aggressive fibromatosis, is a very rare, locally aggressive disease that does not metastasize. Bilateral lesions are extremely rare and are found in only 4% of patients with breast fibromatosis. Tumor recurrence following surgery occurs in 18%-29% of patients, most often within the first 2 years after surgery. In this report, we discuss a case of breast fibromatosis, mimicking a breast carcinoma both clinically and radiologically, that presented clinically with dimpling of the skin of the left breast in a 31-year-old woman. The patient relapsed a few months after surgery, with a multicentric and bilateral disease.
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  • 文章类型: Case Reports
    Fibromatosis of the breast is a rare benign disease compromising <0.2% of all primary breast tumors. Although the chest wall is a common location, occurrences of implant-associated fibromatosis of the breast are extremely rare; only 33 cases have been reported. We present a case of a 42-year-old female who underwent breast augmentation with silicone breast implants, and 2 years later developed an aggressive implant-associated fibromatosis of the breast and chest wall. On imaging studies, the tumor mimicked breast carcinoma, and despite chemotherapy, the fibromatosis rapidly enlarged and was locally invasive requiring wide surgical excision. Unlike previously reported imaging findings, magnetic resonance imaging revealed an oval circumscribed mass with fringe-like internal architecture. We provide a review of the literature and discuss the imaging features of implant-associated fibromatosis of the breast.
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  • 文章类型: Case Reports
    背景:乳腺纤维瘤病是一种以单克隆成纤维细胞增殖为特征的罕见疾病。它没有转移能力,但局部复发率高,经常浸润周围组织。手术治疗是参考,但是最近,新的靶向疗法已经出现。我们报告了一名接受独家治疗的乳腺纤维瘤病患者的原始病例。我们的目的是根据临床和放射学结果分析这些治疗方法,医源性影响,和药理作用。
    方法:我们报告了一个19岁女性的病例,该患者发展为右乳房下内象限的纤维瘤,测量为50×25毫米(即,体积为27.4cm3)。由于潜在的审美和功能偏见,最初的手术是不可能的。因此,她接受了独家治疗,包括几行:NSAIDs与他莫昔芬和曲普瑞林,其次是索拉非尼,然后是干扰素α2b,最后是舒尼替尼。酪氨酸激酶抑制剂(TKIs)(舒尼替尼),观察到显著的部分反应(最大肿瘤体积减少57%).对于每一种治疗,我们提供了与已知药理作用相关的临床和放射学结果.
    结论:TKI是初始手术的一个有趣的替代选择,提供至少部分响应,并可能允许较少的残缺手术。然而,没有药理机制可以明确解释TKI的疗效。总的来说,乳腺纤维瘤病应与肿瘤学家和介入放射科医师一起以跨学科的方式进行治疗,从而为这种特殊的纤维瘤病定位提供了适应性治疗。
    BACKGROUND: Breast fibromatosis is a rare disease characterized by monoclonal fibroblast proliferation. It has no ability to metastasize but has a high local recurrence rate and often infiltrates surrounding tissues. Surgical treatment is the reference, but recently, new targeted therapies have emerged. We report an original case of a patient with breast fibromatosis who received exclusive medical treatment. Our aim was to analyze these treatments based on the clinical and radiological outcome, iatrogenic effects, and pharmacological action.
    METHODS: We report the case of a 19-year-old woman who developed a desmoid-type fibromatosis of the lower inner quadrant of the right breast, measuring 50 × 25 mm (i.e., a volume of 27.4 cm3). Initial surgery was not possible because of potential esthetic and functional prejudice. Thus, she had an exclusive medical treatment including several lines: NSAIDs with tamoxifen and triptorelin, followed by sorafenib, then interferon α2b, and finally sunitinib. With tyrosine-kinase inhibitors (TKIs) (sunitinib), a significant partial response was observed (57% reduction of the maximal tumoral volume). For each treatment, we provided the clinical and radiological outcome in association with known pharmacological action.
    CONCLUSIONS: TKI had been an interesting alternative option to initial surgery, providing at least a partial response and potentially allowing less mutilating surgery. However, no pharmacological mechanism can unequivocally explain TKI efficacy. In general, breast fibromatosis should be treated along with oncologist and interventional radiologists in a trans-disciplinary modality, thus offering an adapted treatment for this particular desmoid-type fibromatosis localization.
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  • 文章类型: Case Reports
    Breast fibromatosis is a rare entity responsible for 0.2% of all solid breast tumors. It has been associated with scars, pregnancy, implants, and familial adenomatous polyposis. We present an interesting case of breast fibromatosis in a 29 year old woman which encroached upon her saline implant and subsequently filled its cavity once the implant was removed. The patient was put on tamoxifen therapy and at 14 month follow-up there was a significant decrease in the size of the mass. Dynamic MRI images are offered for review and current treatment options are discussed.
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