Blastic plasmacytoid dendritic cell neoplasm in Jinhua,China:Two case reports

BACKGROUND Blastic plasmacytoid dendritic cell neoplasm(BPDCN)is a rare and clinically aggressive hematologic malignancy originating from the precursors of plasmacytoid dendritic cells.BPDCN often involves the skin,lymph nodes,and bone marrow,with rapid clinical progression and a poor prognosis.The BPDCN diagnosis is mainly based on the immunophenotype.CASE SUMMARY In this paper,we retrospectively analyzed 2 cases of BPDCN.Both patients were elderly males.The lesions manifested as skin masses.Morphological manifestations included diffuse and dense tumor cell infiltration of the dermis and subcutaneous tissues.Immunohistochemistry staining showed that cluster of differentiation CD4,CD56,CD43,and CD123 were positive.CONCLUSION In this paper,we retrospectively analyzed 2 cases of BPDCN.Both patients were elderly males.The lesions manifested as skin masses.Morphological manifestations included diffuse and dense tumor cell infiltration of the dermis and subcutaneous tissues.Immunohistochemistry staining showed that cluster of differentiation CD4,CD56,CD43,and CD123 were positive.

Relationship between autophagy and perineural invasion, clinicopathological features, and prognosis in pancreatic cancer

AIM To investigate the relationship between autophagy and perineural invasion(PNI), clinical features, and prognosis in patients with pancreatic cancer. METHODS Clinical and pathological data were retrospectively collected from 109 patients with pancreatic ductal adenocarcinoma who underwent radical resection at the First Affiliated Hospital of Zhengzhou University from January 2011 to August 2016. Expression levels of the autophagy-related protein microtubuleassociated protein 1 A/1 B-light chain 3(LC3) and PNI marker ubiquitin carboxy-terminal hydrolase(UCH) in pancreatic cancer tissues were detected by immunohistochemistry. The correlations among LC3 expression, PNI, and clinical pathological features in pancreatic cancer were analyzed. The patients were followed for further survival analysis. RESULTS In 109 cases of pancreatic cancer, 68.8%(75/109) had evidence of PNI and 61.5%(67/109) had high LC3 expression. PNI was associated with lymph node metastasis, pancreatitis, and CA19-9 levels(P < 0.05). LC3 expression was related to lymph node metastasis(P < 0.05) and was positively correlated with neural invasion(P < 0.05, r = 0.227). Multivariate logistic regression analysis indicated that LC3 expression, lymph node metastasis, pancreatitis, and CA19-9 level were factors that influenced neural invasion, whereas only neural invasion itself was an independent factor for high LC3 expression. Univariate analysis showed that LC3 expression, neural invasion, and CA19-9 level were related to the overall survival of pancreatic cancer patients(P < 0.05). Multivariate COX regression analysis indicated that PNI and LC3 expression were independent risk factors for poor prognosis in pancreatic cancer(P < 0.05). CONCLUSION PNI in patients with pancreatic cancer is positively related to autophagy. Neural invasion and LC3 expression are independent risk factors for pancreatic cancer with a poor prognosis.

Clinical and pathological features and risk factors for primary breast cancer patients

BACKGROUND Breast cancer is the most common malignancy in women all around the world.According to the latest statistics in 2018,there were more than 2.08 million new breast cancer cases all around the world and more than 620000 deaths;the proportion of breast cancer deaths in women with cancer is 15%.By studying age,clinicopathological characteristics and molecular classification,age at menarche,age at birth,number of births,number of miscarriages,lactation time,surgical history of benign breast lesions,history of gynecological diseases,and other factors,we retrospectively summarized and compared the disease history of patients with primary breast cancer and patients with benign thyroid tumors admitted to our hospital in the past 10 years to explore the clinicopathological characteristics and risk factors for primary breast cancer.AIM To investigate the clinical and pathological features and risk factors for primary breast cancer treated at our center in order to provide a reference for the prevention and treatment of breast cancer in the Zhuhai-Macao region.METHODS Through a retrospective case-control study,149 patients with primary breast cancer diagnosed and treated at Zhuhai Hospital of Guangdong Provincial Hospital of Traditional Chinese Medicine from January 2013 to March 2020 were included as a case group,and 165 patients with benign breast tumors diagnosed and treated from January 2019 to March 2020 were included as a control group.The data collected included age,age at menarche,age at first birth,number of births,number of miscarriages,lactation time,history of surgery for benign breast lesions,history of familial malignant tumors,history of gynecological diseases,history of thyroid diseases,and the tumor characteristics of the patients in the case group including pathological diagnosis,pathological type,tumor size,lymph node metastasis,distant metastasis,stage,and molecular classification,among others.In the case group,the chi-square test was used to analyze the clinical and pathological features of patients in three age groups(<40,40-59,and≥60 years).A multifactor logistic regression analysis was used to analyze correlations between the two groups.RESULTS Among 149 patients with primary breast cancer,the average age was 48.20±12.06 years,and the proportion of patients at 40-59 years old was the highest,accounting for 61.8%of cases.The molecular type was mainly luminal B type,accounting for 69.2%of cases,and at the time of diagnosis,the tumor stage was mainly stage I/II,accounting for 62.4%of cases.There were no statistically significant differences in the distributions of tumor location,pathological type,tumor size,lymph node metastasis,stage,or molecular classification among the three age groups(<40,40-59,and≥60 years)(P≥0.05).The differences in the distribution of distant metastasis among the three age groups(<40,40-59,and≥60 years)were statistically significant(P<0.01).The differences in lactation time,history of familial malignant tumors,history of gynecological diseases,and history of thyroid diseases between the two groups were not statistically significant(P≥0.05).The differences in age at disease diagnosis,age at menarche,and history of surgery for benign breast lesions were statistically significant(P<0.01).The difference in age at first birth was also statistically significant(P<0.05).CONCLUSION The highest incidence of breast cancer in the Zhuhai-Macao region is present among women aged 40-59 years.There is a larger proportion of stage I/II patients,and the luminal B type is the most common molecular subtype.Distant metastasis occurs mainly in the≥60-year-old group at the first diagnosis;increased age,late age at menarche,and late age at first birth may be risk factors for primary breast cancer,and a history of surgery for benign breast lesions may be a protective factor for primary breast cancer.

Clinicopathological analysis of early-stage breast cancer patients that meet indications for BRCA 1/2 genetic testing

Objective:To investigate the clinicopathological characteristics and prognostic factors of early-stage breast cancer patients with indications for breast cancer susceptibility genes 1/2(BRCA1/2)genetic testing in China.Methods:Based on the indication criteria for BRCA genetic testing specified in the National Comprehensive Cancer Network(NCCN)clinical practice guidelines in oncology,genetic/familial high-risk assessment:Breast and ovarian(Version 2.2019),a retrospective analysis was performed on patients with early-stage invasive breast cancer treated at Breast Disease Center,Peking University First Hospital between January 2008 and December 2016.Clinicopathological characteristics of all patients were analyzed,and prognoses were calculated using the KaplanMeier method and a Cox proportionate hazards model.Results:A total of 906 early-stage breast cancer patients who had indications for BRCA genetic testing and had complete clinicopathological data and follow-up information were included in the study group,accounting for34.7%of all breast cancer patients treated in Breast Disease Center,Peking University First Hospital during the study period.Compared with breast cancer patients without indications for BRCA genetic testing,the overall survival(OS)and disease-free survival(DFS)of patients with indications were not significantly different.In the study group,patients with premenopausal status,high T stage,lymph node positive,estrogen receptor(ER)negative,Ki-67>20%and presence of a vascular tumor thrombus had worse prognosis.There were more family histories of gastrointestinal cancer in patients with related indications than in patients without such indications.Conclusions:Single-center data showed that more than 30%of patients with early-stage breast cancer had indications for BRCA genetic testing.There was no prognostic difference in patients with or without indications for BRCA genetic testing.Premenopausal status,high T stage,lymph node positive,ER negative,Ki-67>20%,and presence of a vascular tumor thrombus were associated with poor prognosis.

Clinical observation of pediatric-type follicular lymphomas in adult:Two case reports

BACKGROUND Pediatric-type follicular lymphoma(PTFL)is a unique pathological type in the 4th edition of hematopoiesis and lymphoid tissue tumor classification revised by World Health Organization.It is unique in clinical practice and seldom seen in adult.PTFL mainly occurs in the head and neck lymph nodes.Most of the cases are short of fever,night sweat,weight loss,and other B symptoms which substitute for lymphadenopathy as the main symptom.PTFL can be disposed of surgical resection and it can achieve long-term tumor-free survival,and it has an excellent outcome.CASE SUMMARY Two cases of PTFL were reported and their clinicopathological features,differential diagnosis,therapy and prognosis were discussed.PTFL showed graybrown tough texture in general performance.The histological manifestations of PTFL were similar to that of adult-follicular lymphoma(FL).Under low power microscope,the structure of lymph nodes was destroyed in different degree,the follicles were closely arranged,expanded and irregular,and the mantle zone became thin or disappeared.In addition,the“starry sky phenomenon”could be seen.At high magnification,the follicles were mainly composed of single medium-sized central cells,and some of them mainly consisted of centroblastic cells to characterize scattered chromatin and inconspicuous nucleoli.Immunohistochemical showed the tumor cells expressed CD20,PAX5,CD79a and CD10,BCL6,FOXP-1,which were limited in germinal center;Ki-67 was highly expressed in germinal center.CD21 and CD23 showed nodular and expanded follicular dendritic cells.Immunoglobulin gene rearrangement was positive for IGH and IGK.The two patients underwent surgical resection with no complications.After discharge,the two patients with a close review for 18 mo and 5 mo respectively and showed no evidence of recurrence.CONCLUSION PTFL in adult is generally supposed to be extremely rare.PTFL displayed characteristic morphological,immunophenotypic,and molecular biological changes which are a kind of neoplasm with satisfactory prognosis after surgical excision.

Tubular Carcinoma of the Breast: A Clinicopathological Analysis of Two Cases

Tubular Carcinoma (TC) of the breast, also known as tubular carcinoma or well-differentiated adenocarcinoma, is defined as a special type of breast cancer consisting of well-differentiated tubular structures with excellent prognosis by WHO (2019) pathological and genetic classification of breast neoplasms. Two cases of breast tubular carcinoma admitted to our hospital were reported. The relevant literature was reviewed and the clinical features, histological morphology (microscopic features and differential diagnosis), molecular changes and clinical prognosis were summarized.

Understanding Ehrlichia canis Infections in Dogs of Costa Rica: Hematological Findings and Indicative Clinical Signs

Background: A cross-sectional study combining different serological and molecular techniques for the detection of Ehrlichia canis in dogs was carried out to determine hemopathological findings and suggestive clinical signs associated with acute, subclinical and chronic infections in the dog population of Costa Rica. Objectives: The present study describes and analyzes, in a more representative sampling frame, the clinical and hematological presentation of E. canis infection in dogs of Costa Rica in all its clinical stages. Methods: A descriptive analysis of the clinical signs was performed from a 441-dog sample. Serological and molecular techniques for the detection of Ehrlichia canis in dogs were applied. One and two-way ANOVA were carried out to determine the effect of the infection status on the hematological parameters. Results: A total of 0.7% (3/407) dogs were found with acute (seronegative but PCR positive), 29.7% (121/407) with subclinical (seropositive and PCR negative), and 2.5% (10/407) with chronic (seropositive and PCR positive) E. canis infections. Significant hemopathological findings were determined in dogs with acute (thrombocytosis), subclinical and chronic (anemia, thrombocytopenia, leukopenia) E. canis infections. Conclusions: Future studies must determine if dogs with subclinical E. canis infections eliminated the agent without any medication, or if they continue to be persistently infected, and will develop the chronic disease at some point in their lives.

Prediction of Nipple and Areola Complex Invasion in Breast Cancer Patients. Clinical and Pathological Study of Surgical Specimens

Breast conservation surgery (BCS) and nipple-areola-sparing (NAS) mastectomy have been recognized as two milestones in this period. This study included 60 Egyptian female patients with breast cancer, all of them were subjected to modified radical mastectomy operation. Methods: This study included female patients > 18 years old who have breast cancer with healthy looking non invaded skin of nipple and areola and excluded patients < 18 years old, patients unfit for surger, patients previously subjected to chemo or radiotherapy for breast cancer. We peformed clinical examination of 60 patients with breast cancer. We studied the relevant factors that affect NAC invasion such as patient’s age, menstrual state, family history, tumor size, tumor location (central vs peripheral), tumor to nipple distance, lymphovascular invasion of NAC, lymph node metastasis, histological tumor type, tumor stage, multifocal/multicentric tumors and (ER, PR, HER2) status. Result: In our study, we have shown that NAC invasion is strongly associated with: 1) Nipple retraction as a patient’s complaint;2) Tumor site;3) Tumor-nipple-distance ≤ 4 cm;4) Multifocal/multicentric tumor;5) Tumor grade (grade III tumors);6) Positive lymph node invasion;7) ER and PR receptors negativity;8) HER2 positivity. This helps in preoperative planning for selecting patients for NAS mastectomy. Conclusion: The ideal patients for NAS mastectomy are with these criteria: 1) Clinically normal nipple areola complex;2) Distance from the tumor to the nipple is >4 cm;3) No multifocal/multicentric tumor;4) Absence of lymph node invasion;5) Tumor grade (grade I, II);6) Peripheral not central tumor;7) No sub-areolar lymphovascular invasion (LVI);8) ER receptor positive;9) PR receptor positive;10) HER2 negative.

Clinical pathologic analysis of urologic primary primitive neuroectodermal tumor

Objective To explore clinico-pathological features,immunophenotype,treatment and prognosis of urologic primary primitive neuroectodermal tumor ( PNET) . Methods The clinical data of 3 patients with urologic PNET were analyzed retrospectively. All patients were male,aged 29,32 and 75 years respectively.

双侧原发性乳腺癌的临床病理特征及预后分析

目的探讨双侧原发性乳腺癌的临床病理特征及其相关预后因素。方法回顾性分析45例原发性双侧乳腺癌病例资料,并运用多因素Cox比例风险回归模型筛选影响病人生存率的相关预后因素。结果45例双侧原发性乳腺癌病人占同期收治乳腺癌总病例数的2.3%。本组双侧原发性乳腺癌呈现特有的临床病理特征①发病年龄早首发癌病人的平均年龄为45岁,小于50岁者占总例数的64.4%(29/45)。②组织病理学恶性程度高本组45例共90个乳腺癌病灶中,浸润性导管癌占68.9%(62/90),其次为混合癌及单纯癌;③分期晚即使在第二原发癌中,II、III期癌肿也占73.3%(33/45)。Kaplan鄄Meier方法计算本组病例3年及5年的总体生存率分别为64.63%和60.59%。Cox回归结果提示,肿瘤TNM分期(P=0.0053)及双侧发病间隔(P=0.0059)是影响患者预后的独立指标。结论应针对具有高危因素的单侧乳腺癌病人进行筛查,早期发现和治疗第二癌,以改善双侧乳腺癌病人的预后。

Enhancement parameters of contrast-enhanced computed tomography for pancreatic ductal adenocarcinoma: Correlation with pathologic grading

BACKGROUND Pancreatic ductal adenocarcinoma(PDA)is a malignancy with a high mortality rate and short survival time.The conventional computed tomography(CT)has been worldwide used as a modality for diagnosis of PDA,as CT enhancement pattern has been thought to be related to tumor angiogenesis and pathologic grade of PDA.AIM To evaluate the relationship between the pathologic grade of pancreatic ductal adenocarcinoma and the enhancement parameters of contrast-enhanced CT.METHODS In this retrospective study,42 patients(Age,mean±SD:62.43±11.42 years)with PDA who underwent surgery after preoperative CT were selected.Two radiologists evaluated the CT images and calculated the value of attenuation at the aorta in the arterial phase and the pancreatic phase(VAarterial and VApancreatic)and of the tumor(VTarterial and VTpancreatic)by finding out four regions of interest.Ratio between the tumor and the aorta enhancement on the arterial phase and the pancreatic phase(TARarterial and TARpancreatic)was figured out through dividing VT arterial by VAarterial and VTpancreatic by VApancreatic.Tumor-to-aortic enhancement fraction(TAF)was expressed as the ratio of the difference between attenuation of the tumor on arterial and parenchymal images to that between attenuation of the aorta on arterial and pancreatic images.The Kruskal-Wallis analysis of variance and Mann-Whitney U test for statistical analysis were used.RESULTS Forty-two PDAs(23 men and 19 women)were divided into three groups:Welldifferentiated(n=13),moderately differentiated(n=21),and poorly differentiated(n=8).TAF differed significantly between the three groups(P=0.034)but TARarterial(P=0.164)and TARpancreatic(P=0.339)did not.The median value of TAF for poorly differentiated PDAs(0.1011;95%CI:0.01100-0.1796)was significantly higher than that for well-differentiated PDAs(0.1941;95%CI:0.1463-0.3194).CONCLUSION Calculation of TAF might be useful in predicting the pathologic grade of PDA.

Pathological characteristics and immunophenotype analysis of cervical intraepithelial neoplasia

Objective To explore the clinical pathological features and immunophenotypes of cervical intraepithelial neoplasia(CIN).Methods The protein expression of p16, p53, Bcl-2, and c-erb B-2 in 59 cases of CIN, 20 cases of cervical squamous cell carcinoma, and 20 cases of normal cervical tissues were tested using immunohistochemistry staining.Results The expression rates of p16, p53, Bcl-2, and c-erb B-2 in CIN tissues were 76.3%(45/59), 28.85(17/59), 61.0%(36/59), and 40.0%(23/59), respectively. The expression rates of p16, p53, Bcl-2, and c-erb B-2 in cervical squamous cell carcinoma tissues were 60.0%(12/20), 60.0%(12/20), 75.0%(15/20), and 65.0%(13/20), respectively. The expression rates of p16, p53, Bcl-2, and c-erb B-2 in normal cervical tissues were 0.0%(0/20), 0.0%(0/20), 35.0%(7/20), 0.0%(0/20), respectively. In comparison to normal tissues, the differential expressions of p16, p53, and Bcl-2 in squamous cell carcinoma and CIN were statistically significant(P <0.001), whereas the difference between the squamous cell carcinoma and CIN was not significant(P >0.05). In comparison to normal tissues, the differential expressions of c-erb B-2 and p53 in squamous cell carcinoma and CIN were statistically significant(P >0.05). The differential expressions of Bcl-2, c-erb B-2, and p53 in CIN 3 were statistically significant in comparison to CIN 1 and CIN 2(P <0.05). Conclusion Overexpression of Bcl-2 occurs early in the development of cervical cancer, whereas p16 and c-erb B-2 overexpression are markers for cell malignancy. The expression of p53 is correlated with the development of cervical cancer.

The prognostic value of clinical and pathologic features in nonmetastatic operable male breast cancer

Compared with female breast cancer, male breast cancer is a rare disease, and the relationship between clinical/pathologic features and prognosis is controversial, or even largely unknown. In this study, we performed a retrospective analysis using clinical and pathologic data from 109 nonmetastatic operable male breast cancer patients treated from January 1996 to December 2011 at Tianjin Medical University Cancer Institute and Hospital. Log-rank test showed that lower tumor stage, no lymph node involvement, and positive estrogen/progesterone receptor status were good predictors of both disease-free survival and overall survival on univariate analysis. However, hormonotherapy is only a good predictive factor of disease-free survival, and not of overall survival. In addition, based on a Cox proportional hazard regression model, only lymph node involvement, and estrogen/progesterone receptor status were statistically significant predictive factors on multivariate analysis. Our results demonstrated that although adjuvant systemic therapy is used extensively in male breast cancer patients and prognosis has improved over the last few decades, lymph node involvement, and estrogen/progesterone receptor status are still the most important prognostic factors. A prospective multi-center study with a larger sample size is urgently needed to further understand male breast cancer.

Clinicopathologic Features of Familial Nonmedullary Thyroic Carcinoma

Background： Familial nonmedullary thyroid carcinoma （FNMTC） is a variant of nonmedullary thyroid carcinoma（NMTC） with particular clinicopathologic features. In recent years, a number of studies have shown that FNMTC is more invasive than sporadic NMTC（SNMTC）. The purpose of this study was to explore the differences in clinicopathologic features of FNMTC between different types of families and to determine in which of these families more invasive FNMTC occurred. Methods： We retrospectively reviewed all patients with thyroid carcinoma admitted to Peking Union Medical College Hospital from January 2009 to July 2013 in the database. Of all 2000 cases, 55 met the inclusive criteria for FNMTC and were studied. There are two different grouping methods. The first is that all samples were allocated to families with three or more first-degree relatives affected （FNMTC-3 group） and families with only two affected first-degree relatives （FNMTC-2 group）. The second is that all patients were divided into families with three or more affected first-degree relatives over two generations （FNMTC-3-2 group） and the other families. We compared the clinicopathologic features such as sex, age, tumor size, multifocality, location, complications by thyroiditis, complications by benign thyroid nodules, surgical procedure, capsule invasion, histological type, lymph node metastases, tumor node metastasis stage, and BRAF mutation between FNMTC-2 group and FNMTC-3 group. We also made the same comparison between FNMTC-3-2 group and other families. Results： No pronounced differences in clinicopathological features were present between FNMTC-2 group and FNMTC-3 group. The proportion of FNMTC-3-2 group aged 〈45 years was significantly higher than that in the other families （58.8% vs. 26.3%, P = 0.021）. A similar difference was found in the proportion of lymph node metastasis （64.7% vs. 34.2%, P = 0.035）. Conclusions： FNMTC-3-2 is more invasive than the other families. Early screening and positive treatment for members of these families are recommended.

A clinicopathological study of 39 cases of atypical polypoid adenomyoma of the uterus

Objective:To clarify the clinical and pathological characteristics of atypical polypoid adenomyoma(APA)in order to improve the criteria used to diagnose and treat this disease.Study design:In 39 cases of APA,retrospective analysis was performed of theclinical data,pathological characteristics,and the follow-up findings in patients admitted to the Peking University People's Hospital from 2007 to 2019.Results:The median age was 39.6 years(range 24–60 years).Thirty seven patients were premenopausal(i.e.94.9%)and eighteen patients were nullipara(i.e.46.2%).Abnormal uterine bleeding was the most common presenting symptom.Hysteroscopic transcervical resection(i.e.TCR)identified lesions in 35 cases whereas:(a)hysterectomy identified them in 3 cases;(b)dilatation and curettage(D&C)identified them in 1 case.Immunohistochemical analysis was performed on 24 samples.In the glandular component,hormone receptors were positively expressed.In all cases,Ki67 expression was detectable in approximately 50%of the cases.In those samples,its presence was definitive in eighteen of the nineteen cases(94.7%,18/19),p53 positive expression was present in most cases(68.8%,11/16),and PTEN positive expression was detected in 40%(4/10).Stroma immunophenotype expression was exhibited as follows:(a)CD10-(12/12),Desminþ(6/7);(b)Vimentinþ(4/4);(c)α-SMAþ(3/3)and;d p16 positve staining in of 80%(4/5).The concurrent amount of atypical endometrial hyperplasia with atypical polypoid adenomyoma was 23.1%(9/39),while its concurrent level of endometrial carcinoma with atypical polypoid adenomyoma was 7.7%(3/39).Fertility preserving treatments were performed in 20 patients with strong childbearing desires.Their pregnancy success was 15%(3/20)and the live birth frequency was 10%(2/10).Follow-up findings were available in 36 cases(92.3%)with a medial follow-up of 48.1 months(range 4–122 months).Its pathological recurrence and frequency of progression into endometrial carcinoma were both 5%(1/20).One case died of another type of malignancy,while the other patients were alive.Conclusions:APA occurs mostly during the years of a women's reproductive period.Its diagnosis is based on the analysis of pathological and immunohistochemical findings.Individuals diagnosed with APA are at risk to coexist with endometrial carcinoma and atypical endometrial hyperplasia.For those individuals who desire retaining fertility,the treatment strategy involves performing TCR completely remove the lesions and close follow-up for surveillance of possible progressive APA recurrence.For those individuals who have no fertility desire,hysterectomy may be a preferred option.

Association of secretory IgA with clinical pathological characteristics and complement activation in IgA nephropathy

Objective To further investigate the association among clinical pathology,complement activation and renal secretory IgA(SIgA)deposition in patients with IgA nephropathy(IgAN).Methods The activation of serum complements was detected by immunoturbidimetry and ELISA.Renal deposition of SIgA and activation of complements were detected by immunofluorescence.

Sturge-Weber syndrome： a case report and review of literatures

Sturge-Weber syndrome （SWS）, or encephalo- trigeminal angiomatosis, is a rare, congenitalneurocutaneous syndrome characterized by unilateral facial cutaneous vascular malformation （nevus flammeus or port-wine stain） in association with ipsilateral leptomeningeal angiomatosis. Prevalence is approximately one per 50 000 live births. Males and females are equally affected and there is no racial bias. 1,2 The common clinical manifestations of SWS include progressive seizures, unilateral cutaneous vascular nevus following the ophthalmic divisions of the trigeminal nerve, ipsilateral glaucoma, contralateral hemiparesis, hemiatrophy, hemianopia and mental retardation. The radiographic hallmark of SWS is ＂tram-line＂ or gyriform calcifications usually involving the occipital and parietal lobes. Histologic studies have revealed that intracranial lesions of SWS display as leptomeningeal angiomatosis, and gyriform calcifications, neuronal loss, astrogliosis in underlying brain tissue.