Phyllodes tumor of the breast with actin inclusions in stromal cells: diagnosis by fine-needle aspiration cytology

The occurrence of hyaline inclusions in stromal cells in fibroepithelial tumors of the breast is very uncommon. These inclusions, characteristic of infantile digital fibromatosis, are comprised of actin filaments. This report illustrates a case of a benign phyllodes tumor of the breast with inclusion bodies, identified by fine-needle aspiration. Histologically, many of the stromal cells contained round intracytoplasmic inclusions, with positivity for smooth muscle actin.     

Demonstration of polyclonal origin of giant fibroadenoma of the breast

We have shown that fibroadenoma of the breast is polyclonal and that phyllodes tumour is monoclonal in origin. It is not known whether a giant fibroadenoma which is histologically identical to the more usual type of fibroadenoma but grows to be a huge mass, like a phyllodes tumour, is polyclonal or monoclona. Clonal analysis was conducted on the DNA samples extracted from the paraffin sections of a giant fibroadenoma resected from a 21-year-old woman. The method used was based on trinucleotide repeat polymorphism of the X-chromosome-linked androgen receptor gene and on random inactivation of the gene by methylation. Clonal analysis showed that the giant fibroadenoma and the adjacent normal breast tissue are polyclonal in origin. Although the term giant fibroadenoma has often been used interchangeably with the term benign phyllodes tumour, because of their similarity in clinical appearance, our present results demonstrate that a giant fibroadenoma is a polyclonal fibroadenoma that has attained an immense size and is different from the monoclonal phyllodes tumour.     

乳腺叶状肿瘤的临床病理学研究

目的 探讨乳腺叶状肿瘤的病理形态学特点,分类和诊断标准,与复发转移的关系及其临床意义。方法 采用回顾性分析的方法对203例有随1访（6－372个月）资料的叶状肿瘤作了详细形态学持征的分析和分类研究,统计学聚类判别分析（SPSS软件10．0版）。结果 良性133例（复发28例）,交界性42例（复发19例,死亡2例）,恶性28例（复发18例,死亡15例）。统计学分析结果显示,肿瘤生长方式,瘤细胞异型性,核分裂象计数和肿瘤性坏死所组成的变量子集分类错判率为零。以此4项为主,完善了病理组织学诊断标准。良性,交界笥和恶性组间复发率,转移和死亡率差异均有显著性意义。肿瘤复发随术式的扩大而减少,2次以上复发占53．85％（35／65）。结论 此瘤可分为良性,低度恶性（交界性）及恶性三种类别。肿瘤生长方式,瘤细胞异型性,核分裂象和肿瘤性坏死是诊断此瘤并对其进行分级（分类）的重要依据。提示首次术式的选择的重要性,良性叶状肿瘤应选择肿物扩大切除术,对于复发的交界性和恶性肿瘤应作乳房切除术。     

Phyllodes tumours of the breast: a consensus review

Phyllodes tumours constitute an uncommon but complex group of mammary fibroepithelial lesions. Accurate and reproducible grading of these tumours has long been challenging, owing to the need to assess multiple stratified histological parameters, which may be weighted differently by individual pathologists. Distinction of benign phyllodes tumours from cellular fibroadenomas is fraught with difficulty, due to overlapping microscopic features. Similarly, separation of the malignant phyllodes tumour from spindle cell metaplastic carcinoma and primary breast sarcoma can be problematic. Phyllodes tumours are treated by surgical excision. However, there is no consensus on the definition of an appropriate surgical margin to ensure completeness of excision and reduction of recurrence risk. Interpretive subjectivity, overlapping histological diagnostic criteria, suboptimal correlation between histological classification and clinical behaviour and the lack of robust molecular predictors of outcome make further investigation of the pathogenesis of these fascinating tumours a matter of active research. This review consolidates the current understanding of their pathobiology and clinical behaviour, and includes proposals for a rational approach to the classification and management of phyllodes tumours.     

乳腺叶状肿瘤患者不同病理分型的超声表现

目的 探讨不同病理学分型的乳腺叶状肿瘤的彩色多普勒超声表现。方法 回顾性研究。纳入江阴市中医院及东南大学医学院附属江阴医院2008年1月—2018年9月49例经手术治疗及病理证实为乳腺叶状肿瘤的患者。49例患者均为女性,年龄17～74(45.78±10.50)岁;术后病理分型诊断为良性乳腺叶状肿瘤20例(40.81%)、交界性21例(42.86%)、恶性8例(16.33%)。采用美国放射学会超声乳腺影像报告和数据系统(BI-RADS)标准进行评估,并比较良性、交界性及恶性三种病理亚型的肿瘤病灶最大径线、超声BI-RADS分类、血供差异,以及不同大小肿瘤超声声像图表现差异。结果 三种病理亚型肿瘤病灶最大径线分别为(3.57±2.17)cm、(5.00±3.71)cm及(2.88±1.52)cm,差异无统计学意义(F=2.083, P＞0.05);49例患者超声BI-RADS分类,0类6例、3类24例、4类12例、5类7例,三种病理亚型的超声BI-RADS分类差异具有统计学意义(H=22.732, P＜0.05);良性、交界性及恶性肿瘤的富血供率分别为35.00%(7/20)、52.45%(11/21)及8/8,差异有统计学意义(χ²=12.782, P＜0.01)。超声灰阶声像图显示,病灶最大径≥5 cm的肿瘤内部液化发生率为6/10,高于最大径＜5 cm肿瘤液化发生率17.9%(7/39),差异有统计学意义(χ²=5.224, P＜0.05)。结论 当乳腺肿瘤在超声声像图上表现为分叶状、囊性变时要考虑叶状肿瘤的可能,应用超声BI-RADS分类可能有助于鉴别良性、交界性及恶性乳腺叶状肿瘤,当乳腺内病灶呈现富血供时要考虑恶性可能,但该病确诊仍需依靠病理。     

Phyllodes tumor with a benign heterologous osseous component: a diagnostic challenge

Phyllodes tumors (PTs) are rare fibroepithelial neoplasms of the breast and constitute 0.3–1% of all primary breast tumors. They should be characterized in to a benign, borderline or malignant category based on a combination of histological features. PTs can show heterologous components, typically sarcomatous, including osteosarcomatous and chondrosarcomatous. Benign heterologous components are exceedingly rare with only one prior reported case in the English literature. This case emphasizes how difficult establishing a correct diagnosis in PTs with heterologous components can be, especially when the tumor consists mainly of a benign heterologous component. We report the case of a 65 year old woman with a recurrent breast tumor initially misdiagnosed as benign osseous metaplasia. The tumor re‐occurred as a malignant PT dominated by benign osseous and chondroid metaplasia. Multiple metastases consisting of primarily mature bone and cartilage were seen in the lungs. On microscopic revision and considering the clinical course the primary breast tumor was re‐classified as a borderline PT.     

Expression of the breast differentiation antigen NY-BR-1 in a phyllodes tumor of the vulva

We describe a phyllodes tumor of borderline malignancy in the labium majus of a 49-year-old woman. The histogenetic origin of phyllodes tumors in the vulva is controversial. Strong immunoreactivity for NY-BR-1, a novel breast differentiation antigen, was demonstrated within the epithelial components of the phyllodes tumor. A similar expression pattern was observed in mammary-like glands of the vulva. These findings provide further evidence that phyllodes tumors of the vulva might derive from mammary-like glands in the labium majus or from ectopic breast tissue.     

胰腺实性-假乳头状瘤2例临床病理分析

目的 探讨胰腺实性-假乳头状瘤(SPT)的临床病理学特征及诊断、鉴别诊断。方法 对2例SPT行临床病理学分析及免疫组化研究。结果 2例患者均有腹部包块及腹痛症状。组织学：单形性肿瘤细胞构成实性及假乳头状结构，常伴有出血、囊性变，间质不同程度硬化。免疫表型：Vim( )、NSE( )、αl-AT( )、ER( )、PR( )；α-ACT灶性( )；上皮性标志(-)、Syn(-)、CgA(-)、CD68(-)。结论 胰腺SPT是较少见的、预后较好的交界性肿瘤，其诊断与鉴别诊断主要依据临床资料、组织学形态特征及免疫组化标记。     

伴菊形团形成的胶质神经元肿瘤5例临床病理分析

目的探讨伴菊形团形成的胶质神经元肿瘤(rosette-forming glioneuronal tumour,RGNT)的临床病理学特征。方法收集南京医科大学第一附属医院病理科2014年11月~2018年7月诊治的5例RGNT,行HE、免疫组化染色以及分子检测,并复习相关文献。结果5例均可见大小一致的神经细胞菊形团和(或)假菊形团结构,神经细胞核圆形,核仁不明显,胞质少,胞突纤细。例1、2、3可见胶质区呈毛细胞型星形细胞瘤样形态,其中例2、3见透明玻璃变的血管壁;例4部分胶质区可见少突胶质细胞瘤样形态;例4可见复杂的肾小球样血管,但细胞无明显异型性,胞核级别低,核分裂未见,无坏死。免疫表型:5例Syn在菊形团神经毡处均阳性,菊形团神经细胞Olig-2、S-100和ATRX均阳性,2例部分神经细胞NeuN阳性;5例胶质区GFAP、Olig-2和ATRX均阳性,4例S-100蛋白阳性;5例IDH1均阴性;Ki-67增殖指数<2%,其中例4球样血管增生区Ki-67增殖指数约10%。4例行PIK3CA分子检测,其中1例9号外显子(p.E545K)突变。结论RGNT是一种生长缓慢、罕见的肿瘤,有独特的组织形态,偶有恶变,目前以手术完整切除为主要治疗方法,但同时警惕潜在恶变危险,术后密切随访。     

PHYLLODES TUMOR (CYSTOSARCOMA PHYLLODES) OF THE BREAST: A Clinicopathologic Study of 45 Cases

The correlation between the histology and biologic behavior of phyllodes tumor was studied in order to establish the histologic criteria of this tumor, particularly its malignant counterpart. A preliminary analysis of five tumors with metastases revealed that a single essential indicator of malignant potential was the presence of a disproportionate overgrowth of the stromal elements at the expense of the ductal element. The presence or absence of such stromal overgrowth in the tumor showed a positive correlation with the degree of stromal atypism and the rate of mitoses. Seventeen out of 45 tumors were determined to be malignant, on the basis of our criteria. The arrangement of the stromal component in malignant phyllodes tumors was usually one of the particular sarcomatous types, with or without the focal areas of one of the specified types of sarcoma. On the other hand, 24 tumors recurred locally but never metastasized, and were preferentially interpreted to be benign phyllodes tumor. Histologically, these tumors had a general architecture resembling that of fibroadenoma but were characterized by high cellularity expressed by the distinct formation of bundles or fascicles of stromal tumor cells. The remaining four tumors seemed to form a borderline phyllodes tumor subset. A follow-up study revealed that, irrespective of the mode of therapy, the outcome of patients with phyllodes tumor was well correlated with the histologic criteria we proposed. ACTA PATHOL JPN 38: 21–33, 1988.     

儿童颅内肿瘤254例临床病理分析

目的分析儿童颅内肿瘤的临床特点和病理类型,提高儿童颅内肿瘤的早期诊断率和诊疗效果。方法对254例儿童颅内肿瘤的临床及病理资料进行总结分析。结果本组病例占我院同期脑肿瘤病例的11.70%,以男性患儿居多,占64.57%;发病年龄以9～12岁为高发,占27.95%;临床表现以头痛、头晕、呕吐为主,占73.23%;儿童颅内肿瘤以胶质细胞瘤为多见,占50.79%,其次为髓母细胞瘤,占24.42%;发病部位幕下多于幕上,占57.09%;CT检查阳性率为94.09%,MR检查阳性率为100%。结论儿童颅内肿瘤发病男多于女,肿瘤好发于幕下,以胶质瘤和髓母细胞瘤最为多见,其临床表现、发病部位、肿瘤性质和组织学类型均与成人有很大不同;影像学检查仍是目前儿童颅内肿瘤的常规检查方法,MR诊断阳性率高于CT。     

Phyllodes tumor of the prostate

In this report, we describe a case of phyllodes tumor of the prostate with a high value of prostate-specific antigen (PSA). A 47-year-old man with symptoms of hematospermia presented with a steadily elevated serum PSA value of 60.76 ng/mL (normal range, < 4 ng/mL). A needle biopsy revealed atypical stromal cells without any evidence of malignancy. After radical prostatectomy, the tumor measured 2.9 cm in diameter and consisted of a single nodule composed of irregular, elongated epithelial ducts and atypical stromal cells with enlarged, occasionally multinucleated, pleomorphic, or hyperchromatic nuclei. Immunohistochemistry showed that the atypical stromal cells were positive for vimentin, androgen receptor, estrogen receptor, progesterone, and 5α-reductase, but negative for MIB-1, PSA, SMA, p53, desmin, CD34, c-kit, CD10, S-100, and EGFR. Excess PSA might be secreted by hyperplastic luminal cells driven by 5α-reductase-positive stromal and epithelial cells. Array-comparative genomic hybridization (array CGH) for genomic alterations revealed a gain of 11p13, which includes the WT1 gene, and a loss of 1p36.23 and 12p12.1. After surgery, the serum PSA value rapidly decreased to within the normal range; no recurrence or distant metastasis was noted after 2 years of follow up.     

骨小细胞恶性肿瘤34例病理形态学研究

目的 研究骨小细胞恶性肿瘤(SCMT)的病理形态和免疫组化特点。方法 应用免疫组化SP法对34例SCMT进行组织学观察。结果 34例SCMT中22例为弥漫型非霍奇金恶性淋巴瘤，其中21例B细胞性，1例T细胞性；瘤组织表达CD45(LCA)、CD20(L26)或C1345RO(UCHL—1)。7例浆细胞肿瘤，其中5例为多发性骨髓瘤、2例为孤立性浆细胞瘤，表现为单一的不同分化程度的肿瘤性浆细胞；免疫组化示6例CD38( )。2例Ewing肉瘤显示排列密集、大小较一致的圆形细胞；肿瘤表达CD99和Vim。1例小细胞骨肉瘤，肿瘤由丰富密集的小细胞和网格状的骨样组织组成，瘤细胞Vim阳性。1例间叶性软骨肉瘤示富于血管的圆形或梭形细胞和透明软骨；瘤细胞表达Vim，软骨细胞表达S—100蛋白。1例小细胞癌示小细胞紧密片巢状排列，表达CK和EMA。结论 骨SCMT组织学类型各有不同的病理形态和免疫组化特征，结合临床和X线表现可作出正确的病理诊断。     

乳腺叶状肿瘤的形态学和免疫组织化学研究

目的观察乳腺叶状肿瘤（phyllodes tumours,PTs）的形态学和免疫组织化学特征,并探讨其诊断标准。方法对21例PTs进行组织学观察和免疫组化SP法检测,并选取5例乳腺纤维腺瘤和5例乳腺腺病标本作为对照组。使用一抗包括CK-pan、EMA、SMA、p53、S-100蛋白、CD117、CD34、CD99、bc l-2、ER、PR、K i-67、CD10。结果21例PTs大体上均表现为界限清楚的肿块,且呈分叶状。肿瘤由具有双层排列的上皮成分以及过度生长的间质成分组成。根据间质的过度生长程度、细胞的异型程度、核分裂数、肿瘤边缘情况、有无异源性成分以及肿瘤性坏死等继发性改变将其分为良性、交界性和恶性3个级别。间质细胞免疫组化表达情况：CKpan：0/21、EMA：0/21、SMA：17/21、CD117：6/21、CD34：18/21、S-100蛋白：2/21、CD99：13/21、CD10：8/21、PR：5/21、ER：4/21、p53：18/21、K i-67：3%~10%、bc l-2：15/21。结论PTs的诊断主要依据组织学观察,免疫组化CD117、CD34、CD99、CD10、bc l-2的检测可以起到一定的辅助作用。     

Immunohistochemical study of MIB1 expression in phyllodes tumor and fibroadenoma

The histogenesis of phyllodes tumor (PT) and fibroadenoma (FA) is closely related, and discrimination between them by histopathological analysis is sometimes problematic. Moreover, objective criteria by which to categorize the grade of malignancy in PT are still controversial. The aim in this study is to clarify whether immunohistochemical evaluation using the MIB1 antibody, which reacts with the ki-67 antigen, correlates with the histological grade of malignancy in PT, and can discriminate between PT and FA. The 47 cases of phyllodes tumor (PT) were categorized into three groups (malignant, 4 cases; borderline, 6 cases; benign, 37 cases) according to the criteria proposed by Azzopardi (1979) and were investigated by immunohistochemistry. There were significant differences in stromal MIB1-index among the three groups (P < 0. 0001), and, unexpectedly, benign PT was easily divided into two groups according only to the MIB1-index. There were significant differences in the stromal MIB1-index (P < 0.001) and stromal cellularity (P < 0.01) between the two benign PT groups. A total of 478 cases of FA was reviewed and these were divided into 403 conventional fibroadenomas (CFA), 36 cellular fibroadenomas (CEFA) and 39 fibroadenomas with focal phyllodes structure (FAPS). All cases of CEFA and FAPS, and 140 cases of CFA were studied by immunohistochemistry. The 21/215 (9.8%) cases of FA, which were designated as FAMIB, showed a high stromal MlB1-index (more than 10/0.0625 mm2). Conversely, 77% cases of FA showed no MIB1-positive stromal cells. The incidence of MIB1-positive epithelium of FAMIB was much higher than that of FA. These results suggest that high proliferative activity may be present in both stromal and epithelial cells of FAMIB. Our study suggests that immunohistochemical evaluation using MIB1 antibody correlates with the histological grade of malignancy in PT, and can select FA with high proliferative activity. However, new objective diagnostic factors useful for discriminating FA from benign PT with a low MIB1-index should be developed.     

Histological features useful in the distinction of phyllodes tumour and fibroadenoma on needle core biopsy of the breast

AIM: To identify features useful in distinguishing phyllodes tumours from fibroadenomas on core biopsy. METHODS AND RESULTS: Starting from the diagnosis made on the surgical specimen, 12 features in the previous core biopsy specimens were analysed. Thirty-six phyllodes tumours had 44 previous core biopsy specimens, which were reported as fibroadenoma in 11 and spindle cell lesion of uncertain nature in one, and included phyllodes tumour in the differential diagnosis in 32. The lesions with a core diagnosis of fibroadenoma were excised largely because they were growing or exceeded 30 mm; review of the corresponding surgical specimen showed heterogeneous stromal cellularity. Thirty-eight fibroadenomas had previous core biopsy specimens reported as fibroadenoma in 37, and one of which included phyllodes tumour in the differential diagnosis. The following four features were significantly more common in cores from phyllodes tumours and had a kappa statistic of > 0.6 in a reproducibility study: stromal cellularity increased in at least 50% compared with typical fibroadenoma, stromal overgrowth (x10 field with no epithelium), fragmentation and adipose tissue within stroma. CONCLUSIONS: This study describes features useful in the diagnosis of phyllodes tumour on core biopsy. Some core biopsy specimens from phyllodes tumours show features of fibroadenoma on core biopsy because of tumour heterogeneity.     

Benign notochordal cell tumor: a retrospective study of 11 cases with 13 vertebra bodies

Purpose: To analyze the clinical data, MRI, pathological diagnosis, treatment and long-term effects of benign notochordal cell tumor (BNCT), a newly described novel spine tumor. Methods: We retrospectively studied 11 patients’ clinical data of the above. Results: The ratio of males to females was 4:7, and the average age was 49.2 years (range, 18-74 years). Cervical vertebra (5; 38.5%) and thoracic vertebra (5; 38.5%) were the most frequent site followed by the lumbar vertebra (3; 23%). Pain was the main symptom except case 2 who were diagnosed accidently because of prostate cancer. The mean delay from first clinical symptoms to diagnosis was ranged from 2 months to 20 years. MRI showed all BNCTs were osteolytic lesions with hypointense on T1-weighted sequences, hyperintense on T2-weighted sequences. There were 4 vertebral bodies with wedge fracture. There were two cases that had two noncontiguous vertebral bodies with BNCT. In histology, marrow replacement was noted by multivacuolated physaliphorous cells immunoreactive for CK, EMA and S100 protein. All 10 cases except case 2 had vertebral reconstruction and fixation with different methods. Of the 11 patients, 9 had full follow-up data which showed no evidence of recurrence or metastasis without further treatment. Conclusion: Noncontiguous multi-centricity BNCTs are rare. No specific vertebrae are more frequently involved. Once BNCT is diagnosed by pathology, the surgical intervention is necessary for the patients with obvious clinical symptoms although it is benign. There is no evidence of BNCT recurrence or metastasis.     

Phyllodes tumor of the breast clinical experience and outcomes: A retrospective cohort tertiary hospital experience

BackgroundPhyllodes tumor (PT) accounts for <1% of all breast tumors worldwide. Based on their microscopic features, these tumors are classified into benign, borderline, and malignant. This study aimed at evaluating the clinical experience and the clinicopathologic features of PT.MethodsA retrospective cohort study of 46 female patients with histologically diagnosed PT. Data collection and evaluation was done on patient demographics, preoperative radiological assessment and pathology, surgical procedure, post-surgery pathological evaluation, radiation therapy (RT), and follow-up.ResultsThe median age at diagnosis was 42 years and young premenopausal patients (median age 35 years) had malignant PT. Forty-five patients underwent core needle biopsy (CNB) with high sensitivity and the positive predictive value (82.2% and 97.4% respectively). Thirty-nine patients (86.7%) underwent conservative surgery and 6 (13.3%) had a mastectomy. Twenty-seven (58.6%) were classified as benign, 11 (23.9%) as borderline and only 8 (17.4%) as malignant PT. Malignant PT had the greatest median tumor size (13 cm). Mortality and recurrence rates were 4.3% and 2.2% respectively. RT was administered in 6 patients (13.0%), 5 having malignant and 1 borderline PT. The metastatic rate was found to be 6.5%.ConclusionPT are rare breast tumors with variable biologic behavior and heterogenous clinicopathological findings. Young, premenopausal women with large tumors may have malignant PT with a risk of recurrence and metastasis. Core needle biopsy is a reliable tool for diagnosis of PT with strict follow-up recommended for large tumors diagnosed as fibroadenoma on CNB. Surgical management must ensure a tumor-free margin on excision to reduce recurrence.