Pseudoangiomatous stromal hyperplasia

假血管瘤间质增生
  • 文章类型: Case Reports
    背景:假性血管瘤性基质增生(PASH)是一种罕见的乳腺基质病变,通常在临床上表现为可触及的单侧,无痛肿块,可自由移动,并具有橡胶或牢固的一致性。诊断可以通过芯针活检(CNB)或手术切除来确认。治疗选择包括对无症状患者进行激素治疗或在极少数情况下进行局部切除和乳房缩小。
    方法:我们报道了一例24岁女性,有服用避孕药约一年的历史。检查显示极其扩大,疼痛,乳房肿胀,尤其是右侧,标志着她的第三次PASH复发。该患者使用一种新颖的技术进行了外科皮肤减少乳房切除术(SRM),该技术采用了直接由真皮皮瓣覆盖的胸前植入物,以重建由于较大的PASH病变的乳房形状,并旨在获得最佳的美容效果。随访18个月,手术无并发症,无复发。
    结论:乳房切除术后立即植入具有以下益处:迅速恢复乳房形状,减少手术。
    结论:本病例报告强调了立即植入在重建大型复发性良性乳腺疾病中的成功应用。结果表明,对于精心选择的大型患者,立即植入有望成为合适的选择,复发性双侧良性乳腺疾病。然而,由于常见的并发症,如感染和植入物丢失,一般不推荐用于良性病变。应根据具体情况作出决定,考虑到大小,复发,和个人适用性。
    BACKGROUND: Pseudoangiomatous stromal hyperplasia (PASH) is a rare breast stromal lesion that typically manifests clinically as a palpable unilateral, painless lump that is freely movable and has a rubbery or firm consistency. The diagnosis can be confirmed by a core needle biopsy (CNB) or surgical excision. Treatment options include medical treatment with hormonal management for asymptomatic patients or local excision and breast reduction in rare cases.
    METHODS: We reported the case of a 24-year-old woman with a history of taking contraceptive pills for about a year. Examination revealed extremely enlarged, sore, and swollen breasts, particularly the right side, marking her third PASH relapse. The patient underwent a surgical skin-reducing mastectomy (SRM) using a novel technique with an immediate prepectoral implant covered by a dermal flap to reconstruct the breast shape due to the large PASH lesions and aiming for optimal cosmetic outcomes. The procedure was complication-free with no recurrence after 18 months of follow-up.
    CONCLUSIONS: Mastectomy followed by immediate implantation offers benefits such as prompt restoration of breast shape with fewer surgeries.
    CONCLUSIONS: This case report highlights the successful use of immediate implantation in reconstructing large recurrent benign breast diseases. The outcomes indicate that immediate implantation shows promise as a suitable choice for carefully selected patients managing large, relapsing bilateral benign breast diseases. However, due to common complications such as infection and implant loss, it is not generally recommended for benign lesions. The decision should be made on a case-by-case basis, considering the size, recurrence, and individual suitability.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    假性血管瘤性间质增生(PASH)是一种良性的乳腺间质增生,通常发生在绝经前或围绝经期妇女中。它通常以局部病变或清晰的边界肿块为特征,弥漫性双乳房增大很少见。PASH被认为是激素依赖性疾病,通常与孕酮有关。没有成像特征,可以看到良性和可疑的恶性症状。PASH的最终诊断取决于病理诊断,在乳腺组织病理学相似的良性和恶性肿瘤之间的区别是必要的。这里,我们报告了一例23岁的多层患者,在怀孕期间患有双侧弥漫性假性血管瘤性乳腺间质增生,并回顾了文献以进一步了解其临床特征。病理诊断,鉴别诊断,乳腺假性血管瘤性间质增生的治疗和预后。
    Pseudoangiomatous stromal hyperplasia (PASH) is a benign interstitial hyperplasia of the breast that usually occurs in premenopausal or perimenopausal women. It is usually characterized by localized lesions or clear boundary masses, and diffuse double breast enlargement is rare. PASH is considered a hormone-dependent disease that is commonly progesterone related. There are no imaging characteristics, and both benign and suspicious malignant signs can be seen. The definitive diagnosis of PASH depends on a pathological diagnosis, and it is necessary to be vigilant in distinguishing between benign and malignant tumors with similar breast histopathology. Here, we report the case of a 23-year-old multipara patient with bilateral diffuse pseudoangiomatous stromal hyperplasia of the breast during pregnancy who presented with macromastia and reviewed the literature to further understand the clinical features, pathological diagnosis, differential diagnosis, treatment and prognosis of pseudoangiomatous stromal hyperplasia of the breast.
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  • 文章类型: Journal Article
    <b><br>目的:</b>本研究旨在系统分析近期与男性肿瘤假血管瘤性间质增生(PASH)相关的同行评议文献。</br><b><br>材料和方法:</b>对2012年至2023年在同行评审的知名期刊上发表的文章进行了八个变量的评估:1)患者的年龄,2)临床表现,3)侧向性(单边或双边),4)相关的乳房疾病,5)系统性合并症的存在,6)辅助诊断的成像方式,7)治疗,8)复发。</br><b>br>结果:</b>有10例患者年龄从1岁到67岁(平均:34.5岁)。8例(80%)有乳房肿块;在2例(20%)中,报告时有腋窝肿块。疼痛不是突出的症状。6例患者(60%)出现另一种乳房疾病,3例(30%)有明显的全身性合并症,包括糖尿病和肾功能不全。肿块的最大尺寸范围为2厘米至12厘米,80%的肿块大小在5厘米以下。用于评估的成像方式包括乳腺超声(USG),乳房X线照片,计算机断层扫描(CT),磁共振成像(MRI)。所有病例均经手术治疗,只有在对手术标本进行分析后才能做出最终诊断。2例(20%)病例记录复发。</br><b><br>结论:</b>TumanPASH是一种良性肌纤维母细胞增生性疾病,在男性中很少报道。这种情况应被视为乳腺或腋窝肿块的鉴别诊断,并应相应地开始评估。推荐的治疗过程是适当的手术切除,并定期进行临床和放射学随访。</br>.
    <b><br>Aim:</b> The study was conducted to systematically analyze the recent peer-reviewed literature related to tumoral pseudoangiomatous stromal hyperplasia (PASH) in males.</br> <b><br>Materials and methods:</b> Articles published in peer-reviewed journals of repute from 2012 to 2023 were evaluated for eight variables: 1) the age of the patient, 2) clinical presentation, 3) laterality (unilateral or bilateral), 4) associated breast disorder, 5) the presence of systemic co-morbidity, 6) imaging modalities aiding diagnosis, 7) treatment, and 8) recurrence.</br> <b><br>Results:</b> There were 10 patients ranging in age from 1 year to 67 years (mean: 34.5 years). In 8 cases (80%), there were breast lumps; in 2 cases (20%), there were axillary lumps at presentation. Pain was not a prominent symptom. Six patients (60%) had another breast disorder at presentation and 3 (30%) had significant systemic co-morbidities, including diabetes and renal insufficiency. The greatest dimension of the lumps ranged from 2 cm to 12 cm, with 80% of the lumps being below 5 cm in size. Imaging modalities utilized in evaluation included breast ultrasound (USG), mammograms, computerized tomography (CT), and magnetic resonance imaging (MRI). All the cases were treated surgically, and the final diagnosis was made only after the analysis of the surgical specimen. Recurrence was recorded in 2 (20%) cases.</br> <b><br>Conclusions:</b> Tumoral PASH is a benign myofibroblastic proliferation disorder rarely reported in males. This condition should be considered a differential diagnosis of breast or axillary lumps, and an evaluation should be initiated accordingly. The recommended course of treatment is adequate surgical excision with regular clinical and radiological follow-up.</br>.
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  • 文章类型: Letter
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  • 文章类型: Case Reports
    假性血管瘤性间质增生(PASH)是一种罕见的与激素失衡有关的特发性增生性乳腺间质病变,因此在儿童和青少年中极为罕见。此外,在某些情况下,PASH表现为双侧巨大畸形,没有明确的原因或治疗方法。这里,我们报告了一例14岁初潮前女性患者中快速发展的PASH表现为双侧巨大乳房发育症。考虑到患者的年龄和情绪,以及乳头-乳晕复合体重新定位的需要,尽管存在复发的可能性,但我们进行了乳房减量成形术而不是全乳房切除术.虽然有些肿块不能完全清除,没有并发症,如感染,伤口裂开,或血肿发生在术后。在18个月的随访期间,患者病情稳定,尽管超声检查发现了复发和残留疾病的证据。
    Pseudoangiomatous stromal hyperplasia (PASH) is a rare idiopathic proliferative mesenchymal breast disease related to hormonal imbalance, and thus extremely rare in children and adolescents. In addition, PASH manifests as a bilateral gigantomastia in some cases with no established cause or treatment. Here, we report a case of a rapidly developed PASH presenting with bilateral gigantomastia in a 14-year-old premenarchial female patient. Considering the patient\'s age and emotions and the need for nipple-areolar complex repositioning, we performed reduction mammoplasty rather than total mastectomy despite the possibility of recurrence. Although some masses could not be completely removed, no complications, such as infection, wound dehiscence, or hematoma occurred postoperatively. The patient was stable during the 18-month follow-up period, although an evidence of recurrent and residual disease was noted upon ultrasonography.
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  • 文章类型: Journal Article
    目的:青少年纤维腺瘤(JFA)是通常发生在青少年女性患者中的双相纤维上皮病变(FEL)。巨人(G)JFA,像其他FEL,可能表现出明显的假血管瘤间质增生(PASH)样变化。我们试图确定有和没有PASH的GJFA的临床病理和分子特征。
    结果:检索GJFA(1985-2020)病例的档案。全部进行雄激素受体(AR)染色,β-catenin,CD34和孕激素受体(PR)。使用定制的16基因面板-MED12(外显子1和2)对病例进行测序,TERT启动子(-124C>T和-146Ctable>T),SETD2,KMT2D,RARA(外显子5-9),FLNA,NF1,PIK3CA(外显子10、11和21),EGFR,RB1,BCOR,TP53,PTEN,ERBB4、IGF1R和MAP3K1。从21名年龄在10.1-25.2岁的女性患者中鉴定出27名GJFA。尺寸范围从5.2到21厘米。两个病人有多个,双侧和后来复发的GJFA。13例(48%)显示突出的PASH样基质。所有病例均为基质CD34阳性,AR和β-catenin阴性,1例显示局灶性PR表达。测序显示17个样本中的MAP3K1和SETD2突变,使用KMT2D,TP53和BCOR像差在10(45%),10例(45%)和7例(32%),分别。具有PASH样模式的肿瘤具有更高的SETD2(P=0.004)和TP53(P=0.029)突变患病率,而没有PASH的RB1突变较多(P=0.043)。在一个病例中鉴定出MED12突变。在四个(18%)中观察到TERT启动子突变,包括两次复发。
    结论:在GJFA中,沿着拟议的FEL致病途径的更晚期阶段的基因突变是不寻常的,并提出了在这些肿瘤中更积极生长的机制。
    OBJECTIVE: Juvenile fibroadenomas (JFA) are biphasic fibroepithelial lesions (FEL) usually occurring in adolescent female patients. Giant (G) JFA, like other FEL, may exhibit prominent pseudoangiomatous stromal hyperplasia (PASH)-like change. We sought to determine clinicopathological and molecular characteristics of GJFA with and without PASH.
    RESULTS: Archives were searched for cases of GJFA (1985-2020). All were stained for androgen receptor (AR), beta-catenin, CD34 and progesterone receptor (PR). Cases were sequenced using a custom 16-gene panel - MED12 (exons 1 and 2), TERT promoter (-124C>T and -146Ctable>T), SETD2, KMT2D, RARA (exons 5-9), FLNA, NF1, PIK3CA (exons 10, 11 and 21), EGFR, RB1, BCOR, TP53, PTEN, ERBB4, IGF1R and MAP3K1. Twenty-seven GJFA from 21 female patients aged 10.1-25.2 years were identified. Size ranged from 5.2 to 21 cm. Two patients had multiple, bilateral and later recurrent GJFA. Thirteen (48%) cases showed prominent PASH-like stroma. All were positive for stromal CD34, negative for AR and beta-catenin and one case showed focal PR expression. Sequencing showed MAP3K1 and SETD2 mutations in 17 samples, with KMT2D, TP53 and BCOR aberrations in 10 (45%), 10 (45%) and seven (32%) cases, respectively. Tumours with a PASH-like pattern had higher prevalence of SETD2 (P = 0.004) and TP53 (P = 0.029) mutations, while those without PASH had more RB1 mutations (P = 0.043). MED12 mutation was identified in one case. TERT promoter mutation was observed in four (18%), including two recurrences.
    CONCLUSIONS: Gene mutations along more advanced phases of the proposed FEL pathogenetic pathway in GJFA are unusual, and suggest a mechanism for more aggressive growth in these tumours.
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  • 文章类型: Journal Article
    背景:假血管瘤间质增生是一种罕见的乳腺良性乳腺间质增生性病变。临床表现范围从快速增长的质量到常规筛查中的偶然鉴定。表现上的这种差异及其稀有性使得难以成为标准的治疗方案。因此,我们的目的是分享我们在假性血管瘤间质增生的临床经验。
    方法:研究中纳入了2013年1月至2021年12月间因乳腺肿块而接受核心活检或手术切除并导致假血管瘤性间质增生的患者的档案。
    结果:17例中位年龄37岁(22-68岁)的患者经手术切除或穿刺活检证实为假性血管瘤间质增生。选择治疗方案8例(47.1%),而9例(52.9%)患者采用手术切除。平均随访时间为55.24±26.72(13~102)个月。在随访期间,没有患者观察到恶性转化。
    结论:对于乳腺假性血管瘤性基质增生,手术切除有清洁的边缘或通过组织活检确诊后密切随访是足够的。假性血管瘤性基质增生不是发生乳腺癌的危险因素。
    BACKGROUND: Pseudoangiomatous stromal hyperplasia is a rare benign breast stromal proliferative lesion of the breast. Clinical presentation ranges from rapidly growing mass to incidental identification in routine screening. This difference in manifestation and its rarity makes it difficult to be a standard treatment protocol. Therefore, we aimed to share our clinical experience in Pseudoangiomatous stromal hyperplasia.
    METHODS: The files of patients who underwent core biopsy or surgical excision due to a breast mass and resulted in pseudoangiomatous stromal hyperplasia between January 2013 and December 2021 were included in the study.
    RESULTS: 17 patients with a median age of 37 (22-68) were found Pseudoangiomatous stromal hyperplasia confirmed by surgical excision or core biopsy. Chosen treatment option was observation in 8 patients (47.1%), while surgical excision was used in 9 (52.9%) patients. The mean follow-up period was 55.24 ± 26.72 (13-102) months. None of the patients observed the Malignant transformation during the follow-up period.
    CONCLUSIONS: For Pseudoangiomatous Stromal Hyperplasia of the breast, surgical excision with clean margins or close follow-up after diagnosis confirmation by tissue biopsy is sufficient. Pseudoangiomatous Stromal Hyperplasia is not a risk factor for developing breast cancer.
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  • 文章类型: Journal Article
    这项研究的目的是确定浸润性乳腺癌(IBC)中的假血管瘤性基质增生(PASH)样外观与非肿瘤乳腺实质中PASH灶以及腋窝淋巴结受累的关系。
    在这项研究中,对200例连续IBC病例进行了复查。确定了在IBC中有和没有PASH样外观的病例。评估每个病例是否存在伴随的PASH病灶(CD34+,CD31-)在非肿瘤区域以及其他临床病理参数。
    IBC内的PASH样外观在200例中有22例(11%)存在,在178例(89%)中没有。与没有此类区域的组相比,在具有PASH样外观的IBC中,非肿瘤乳腺实质中PASH病灶的存在明显更常见。然而,在其他临床病理参数(年龄,肿瘤大小,核和组织学分级,雌激素受体/孕激素受体状态,HER2状态,和Ki-67增殖指数),淋巴管浸润(LVI),腋窝淋巴结受累。关于在非肿瘤区域观察到的组织病理学发现,两组之间没有显着差异。
    发现IBC内的PASH样外观与非肿瘤乳腺实质中PASH灶的发生率较高有关。然而,这些病例在LVI和腋窝淋巴结转移方面没有差异。
    UNASSIGNED: The aim of this study were to determine the relationship of pseudoangiomatous stromal hyperplasia (PASH)-like appearance in invasive breast carcinomas (IBCs) with PASH foci in the non-tumoral breast parenchyma as well as axillary lymph node involvement.
    UNASSIGNED: In this study, 200 consecutive cases with IBC were re-examined. Cases with and without PASH-like appearance in IBC were determined. Each case was assessed regarding the presence of accompanying PASH foci (CD34+, CD31-) in the non-tumoral areas in addition to other clinicopathological parameters.
    UNASSIGNED: PASH-like appearance within the IBC was present in 22 of the 200 cases (11%) and absent in 178 (89%). The presence of PASH foci in the non-tumoral breast parenchyma was significantly more common in IBC with PASH-like appearance compared to the group without such areas. However, there was no significant difference between the groups regarding other clinicopathological parameters (age, tumor size, nuclear and histological grade, Estrogen receptor/Progesterone receptor status, HER2 status, and Ki-67 proliferation index), lymphovascular invasion (LVI), and axillary lymph node involvement. There was no significant difference between the two groups regarding the histopathological findings observed in the non-tumoral areas.
    UNASSIGNED: PASH-like appearance within IBC was found to be associated with higher rate of PASH foci in the non-tumoral breast parenchyma. However, such cases do not show a difference as regards LVI and axillary lymph node metastasis.
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  • 文章类型: Journal Article
    背景:在个体特别容易受到同伴压力和社会规范的影响的时期,青少年的宏观性是身体和心理上的衰弱。儿科医生和外科医生的早期识别和干预对于避免不必要的痛苦至关重要。虽然减少乳房成形术是治疗成人有症状的巨大乳房畸形的金标准,儿科患者巨大乳房畸形的治疗仍存在争议.特别是,关于儿科患者乳房重建手术的时机有很多讨论。
    方法:对文献进行了全面回顾,以确定所有与≤16岁患者的巨乳症相关的文章,在美国通常实现全面发展的年龄。小儿巨大乳房症的病因,管理方法,和结果总结如下。
    结果:小儿病理性乳腺肥大是一种罕见的发现,可能继发于青少年乳腺肥大(JHB)和假性血管瘤性基质增生(PASH)。虽然已经尝试了对这些病症的医疗管理,但取得了不同的成功,减少乳房成形术对儿科患者是安全有效的。有,然而,手术前必须考虑的一些儿科特异性因素.我们提供了一种接近小儿巨大乳房症的算法。
    Macromastia in adolescents is both physically and psychologically debilitating during a period in life when individuals are particularly vulnerable to peer pressure and social norms. Early recognition and intervention by both pediatricians and surgeons are critical to avoid unnecessary suffering. While reduction mammaplasty is the gold standard for the management of symptomatic macromastia in adults, the management of macromastia in pediatric patients remains controversial. In particular, there is great discussion regarding the timing of reconstructive breast surgery in pediatric patients.
    A comprehensive review of the literature was performed to identify all articles related to macromastia in patients ≤16 years of age, the age at which full development is typically achieved in the United States. The etiologies of pediatric macromastia, approaches to management, and outcomes are summarized herein.
    Pathological breast hypertrophy in pediatric patients is a rare finding and may occur secondary to juvenile hypertrophy of the breast (JHB) and pseudoangiomatous stromal hyperplasia (PASH). While medical management of these pathologies has been attempted with varying success, reduction mammaplasty is safe and effective in pediatric patients. There are, however, a number of pediatric-specific considerations that must be taken into account prior to surgery. We provide an algorithm for approaching pediatric macromastia.
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