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POEMS syndrome is a rare hematological disorder associated with plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Castleman disease is a lymphoproliferative disorder that can be present in POEMS patients, which can be defined as Castleman disease variant of POEMS syndrome. Herein, we described a 24-year-old male patient diagnosed with this syndrome and also suffered from multiple cerebral infarctions. This patient showed no evidence of monoclonal gammopathy and failed to have electromyography examined. The final diagnosis was established with the help of the axillary lymph node biopsy. As a rare case of POEMS syndrome without evidence fulfilling the major mandatory diagnostic criteria and with cerebrovascular involvement, its characteristics was discussed with a brief literature review in order to facilitate further understanding of the POEMS syndrome.

Background: Primary malignant tumors of the pericardium are rare, and most primary malignant pericardium tumors are mesotheliomas. Primary pericardial angiosarcoma is extremely rare, and it is associated with a poor prognosis., Case Presentation: We report of a 47-year-old woman who complained of activity-related chest tightness and shortness of breath. Computed tomography, magnetic resonance imaging, and transesophageal echocardiography revealed an enlarged pericardium with hematic and solid components. An exploratory pericardiotomy was performed, and the results of the histological examination were suggestive of spindle cell hemangioendothelioma. She survived for 9 months after surgery without chemotherapy and radiotherapy, and she had a relatively good quality of life., Conclusion: Primary pericardial angiosarcoma is difficult to diagnose, and it has a poor prognosis. Pericardiotomy, radiation therapy, and chemotherapy were associated with a prolongation of survival.

Objective: To explore the value of detecting mutations on epidermal growth factor receptor (EGFR) gene in non-small cell lung cancer (NSCLC) tissue by TaqMan-amplification refractory mutation system (TaqMan-ARMS)., Methods: TaqMan-ARMS and DNA sequencing were used to detect the EGFR exon 19 and 21 mutations in tumor tissues and the samples collected from 199 patients at 4 different 3A hospitals in Beijing from January 2008 to March 2011., Results: The rate of mutations in EGFR exon 19 and 21 was 19.1% (38/199), according to their different pathological types. Based upon TaqMan-ARMS, the classification was as followed: adenocarcinoma (35.0% (36/103)), squamous carcinoma (2.2% (2/93)) and adenosquamous carcinoma (0). According to DNA sequencing, they were 19.6% (39/199), 35.9% (37/103), 2.2% (2/93) and 0 respectively. Thus, no statistically significant difference existed between two methods (McNemar Test, P = 1.000, κ = 0.984). The mutation rate of adenocarcinoma was higher than those of squamous and adenosquamous carcinoma., Conclusion: The detection of EGFR mutations is highly consistent in the NSCLC tissue by the methods of TaqMan-ARMS and DNA sequencing.

Objective: To explore the clinicopathological characteristics of primary thyroid-like follicular carcinoma of the kidney., Methods: A case of primary thyroid-like follicular carcinoma of the kidney was studied with histology and immunohistochemical staining, and its clinical and pathological findings were further analyzed with review of the literature., Results: The patient was a 26-year-old asymptomatic woman who had a kidney mass during her annual physical examination. The tumor was well-circumscribed. Pathologically, the tumor showed follicular structures with colloid-like material in the lumina. Immunohistochemically, the tumor cells showed intense staining for CK7 and vimentin and negative for thyoid transcripation factor-1, thyroglobulin, thyoid peroxidase and RCC., Conclusions: The diagnosis of primary thyroid-like follicular carcinoma of the kidney is based on the characteristic follicular architecture with colloid-like material, and the metastasis from a thyroid follicular carcinoma must be excluded clinically and pathologically before making the final diagnosis.

Objective: To explore the significance of succinate dehydrogenase B (SDHB) mutation and EPAS1 overexpression in Zuckerkandl paragangliomas (PGL) and examine their correlations with malignant infiltration and metastasis., Methods: From March 2008 to July 2011, the clinical profiles of 16 Zuckerkandl PGL patients were analyzed retrospectively. For increased diagnostic specificity, a complex immunohistochemical panel of tissue microarray was performed for SDHB, EPAS1 and MIB-1. Positive expression identified as a granular cytoplasmic staining. Greater than or equal to 50% as strongly positive (+++), 1% to 10% as weakly positive (+)., Results: Tissue microarray immunohistochemical staining showed SDHB immunoreactivity in the cytoplasm, whereas EPAS1 and MIB-1 in the nuclear of tumor cells. Positive expression of EPAS1 in which 13 cases of Zuckerkandl PGL. And high expression strongly associated with malignancy. SDHB mutation of 7 cases are all EPAS1 positive staining. Non-gene mutation 9 cases tumor specimens, 6 cases were EPAS1 positive expression (P < 0.05). CgA positive expression in 11 cases benign Zuckerkandl PGL, strongly positive in 4 malignant cases (4/4). MIB-1 below 1% in 12 cases of benign Zuckerkandl PGL. And in 4 malignant cases, MIB-1 was about 3%. Malignant neoplasms had significantly higher EPAS1, CgA and MIB-1 expression compared to benign counterparts (P < 0.05)., Conclusions: The SDHB mutation causes the EPAS1 over expression in PGL and correlation with higher positive expression of CgA and MIB-1. It may be one of the mechanisms of malignant invasiveness and metastasis in PGL.

Objective: To study the pathologic characteristics of eutopic endometrium in patients with endometriosis., Methods: Pathologic characteristics of eutopic endometrium were studied in 176 patients with endometriosis in Peking Union Medical College Hospital from January 2007 to December 2008 retrospectively., Results: About 72.2% (127/176) of eutopic endometrium were in proliferative phase, 19.9% (35/176) of were observed as endometrial polyp, including 32 cases with simple endometrial polyp and 3 cases with abnormal hyperplasia combined with endometrial polyp. And 4.0% (7/176) showed abnormal hyperplasia. The incidence of pathologic changes in eutopic endometrium was 22.2% (39/176). Among 53 endometriosis patients combined with infertility, the incidence of pathologic changes of eutopic endometrium was 35.9% (19/53), which was significantly higher than 16.3% in non-infertile patients (χ(2) = 8.24, P = 0.004). Among 65 cases with irregular menstruation, the incidence of endometrial polypus and endometrial hyperplasia were 20.0% (13/65) and 10.8% (7/65), which were significantly higher than 17.1% (19/111) and 0 in normal menstruation patients (χ(2) = 13.839, P = 0.003)., Conclusions: The eutopic endometrium of endometriosis were in proliferative phase state. The pathologic changes of eutopic endometrium were more in patients combined with infertility and irregular menstruation.

Objective: To study the clinical and pathological features of pulmonary neuroendocrine cell hyperplasia and tumorlets with bronchiectasis., Methods: Both the clinicopathologic changes and immunohistochemical findings were examined with microscopy and EnVision method in 22 cases of pulmonary neuroendocrine cell hyperplasia and tumorlets., Results: The average age of the 22 patients was 53 years, with a male to female ratio of 9:13. On macroscopic examination the lungs showed bronchiectasis; one case was accompanied by gray-white, soft nodules (diameter < 5 mm). Microscopy of the HE sections showed the basic pathologic change was bronchiectasis, accompanied by neuroendocrine cell hyperplasia and tumorlet formation in the pulmonary parenchyma surrounding the bronchioles, presenting as single nodule (10 patients), or multifocal nodules (12 patients), with average size of 1.6 mm in diameter. No tumor cells were identified in the lymph nodes. Sixteen of 22 patients were disease-free after an average follow-up period of 58 months (17 - 117 months); one patient died suddenly after surgery; and five were loss of follow up. Immunohistologically, the tumor cells were positive for CgA (18/18), Syn (16/16), AE1/AE3 (16/16) , TTF-1 (14/15), and CD56 (14/14), and Ki-67 index was < 2% in 12 cases., Conclusions: Immunohistological staining for CgA, Syn, CD56, TTF-1 and AE1/AE3 can confirm the diagnosis. Early detection, pulmonary resection and follow-up help prevent the progression of these diseases.

Objective: To observe the clinical features and cardiac magnetic resonance imaging (CMR) characteristics of patients with endomyocardial biopsy (EMB)-proven cardiac amyloidosis (CA)., Methods: EMB proven CA patients underwent CMR examination from September 2006 to December 2010 were included. The findings of clinical manifestation, electrocardiogram, echocardiography and CMR were analyzed., Results: Among the 18 patients with EMB verified CA, 5 patients underwent CMR. All 5 patients had heart failure symptoms and electrocardiogram was abnormal. Echocardiogram showed concentric left ventricular hypertrophy, granular appearance of the myocardium, left atrial enlargement and moderate to severe left ventricular diastolic dysfunction. CMR revealed increased thickness of the left ventricular wall (especially at the inter-ventricular septum), enlarged bilateral auricle, restricted left ventricular filling with normal or mild to moderate reduced systolic function. Pleural and pericardial effusions were observed in 2 patients. Abnormal late gadolinium enhancement (LGE) was detected in all 5 patients. CMR revealed different patterns of LGE. Left ventricular global subendocardial delayed gadolinium enhancement or transmural delayed gadolinium enhancement were found, and patients also showed line-, granular- or patchy-like enhancement. The degree and range of LGE paralleled the disease course and were consistent with electrocardiogram changes., Conclusions: As a noninvasive diagnostic tool, CMR is valuable in the diagnosis of CA. For patients with clinical suspicion of CA, CMR could be a helpful diagnostic tool, especially in the hospitals where EMB is not available.

Objective: To summarize our experiences in the diagnosis and treatment of placenta accreta in the second trimester of pregnancy., Methods: We retrospectively analyzed the clinical data of 31 patients were admitted to Peking Union Medical College Hospital with placenta accreta in the second trimester of pregnancy from January 2002 to January 2010., Results: Among 31 cases, one case (3.2%) was suspected to be with placenta accreta by ultrasound examination and 30 cases (96.8%) were normal before delivery. Placenta accreta was identified during follow-up in 12 cases (38.7%) after delivery. Fourteen patients underwent curettage again after delivery,which was effective in 6 patients (42.9%) and failed in 8 patients,in whom uterine artery embolization (UAE) was further applied. Thirteen patients underwent UAE without curettage. In total,21 cases underwent UAE, which was effective in 19 patients (90.5%); one patient with abnormal β-human chorionic gonadotropin (β-HCG) 5 months after embolization underwent lesion resection and one case with slightly increased β-HCG were lost to follow-up. Hysteroscopy was effective in 3 patients,of whom two patients underwent lesion resection by hysteroscopy and one case who was suspected to be with trophoblastic disease by ultrasonography before surgery and confirmed to be placenta accreta during hysteroscopy examination underwent lesion resection. One case experienced hemorrhagic shock during vaginal delivery and underwent emergency laparotomy. Among all these 31 patients,massive hemorrhage occurred in 13 cases during delivery and hemorrhagic shock in 2 cases. Three cases had postpartum hemorrhage and stopped bleeding after UAE. None needed hysterectomy., Conclusions: Placenta accreta in the second trimester of pregnancy is usually diagnosed after childbirth,which may be delayed in some cases. Therefore,special attention should be paid to this disease during follow-up. Conservative treatment was the main therapy of placenta accreta in the second trimester of pregnancy. UAE is effective in stopping bleeding.

Objective: To evaluate the value of ultrasonic monitoring in induced abortion during the first trimester., Methods: Totally 110 healthy women with a singleton pregnancy between 9 and 11 gestational weeks were enrolled. All the procedures of induced abortion were performed routinely. Ultrasonography was performed when the procedure of induced abortion was completed. Patients with normal ultrasonographic results were assigned in the control group, while patients with abnormal ultrasonographic findings were enrolled in the study group,in which these patients underwent further operations until the ultrasonography showed clear endomembrane line. All the recurretaged tissue in the study group were sent for pathological examinations., Results: Of these 110 patients, 28 (25.5%) entered study group and 82 (74.5%) entered control group. In the study group, trophoblastic cell or chorion was found in the recurretaged tissue in 11 patients (39.3%), in which 3 had trophoblastic cell embedded in smooth muscular tissue, 6 had pathologic deciduas, and 11 had pathologic secretory endometria. In this control group,1 patient (0.9%) had retained products of conception. The operation duration [(20.6∓2.1) min vs.(11.5∓3.5) min, P0.05]., Conclusions: Ultrasonic monitoring in induced abortion during the first trimester can decrease the incidence of retained products of conception and will not induce endometrial damage. It is especially useful for women whose fetuses were at older gestational ages.

Objective: To investigate the diagnosis and treatment of cervical ectopic pregnancy., Methods: The clinical data of 27 patients with cervical ectopic pregnancy who were treated in Peking Union Medical College Hospital from January 1990 to November 2009 were retrospectively reviewed. The clinical effectiveness of three fertility-preserved treatment modes were compared., Results: Of these 27 patients,one underwent hysterectomy, while all the other 26 patients selected fertility-preserved treatment,which included curettage after uterine artery embolization (UAE) (n=14), curettage directly (n=8), and curettage after methotrexate (MTX) injection (n=4). The effectiveness rates of these three methods were 100%,75.0%,and 50.0%, respectively,which was significantly higher in curettage after UAE group than in curettage after MTX group (P=0.005). The duration of hospitalization was (17.0∓3.2) days in curettage after MTX group,which was significantly longer than that in curettage after UAE group (6.1∓2.9) d (P=0.004) and curettage directly group (4.9∓3.4) d (P=0.001). The mean hospitalization cost showed no significant difference among three groups (P=0.104)., Conclusion: Curettage after UAE is safe and effective for patients diagnosed or highly suspicious of cervical pregnancy.

Objective: restrictive cardiomyopathy (RCM) is characterized by impairment of ventricular filling during diastole with preserved systolic function. The clinical and histopathological profile on endomyocardial biopsy of 25 consecutive patients with RCM was analyzed in this study., Methods: twenty-five patients with diagnosis of RCM and underwent endomyocardial biopsy (EMB) were enrolled in the study. The clinical characteristics, electrocardiogram, serum chemistry, right heart catheter and cardiac pathology results were obtained., Results: heart failure symptom was present in all 25 patients and left ventricular size and function were normal or near normal while serum brain natriuretic peptide (577 pg/ml) was moderately elevated. Right atrial and ventricular end-diastolic as well as pulmonary capillary wedge pressures derived from right heart catheter examination were increased. Amyloid deposition were evidenced in 16 and eosinophilic myocarditis in 2 patients upon pathological examination of EMB. In the remaining 7 patients, 3 were diagnosed idiopathic RCM, 2 were diagnosed as amyloidosis by biopsy from non-cardiac tissue and etiology remained unknown in 2 patients. Thus, conclusive diagnosis was made on EMB samples in 84% (21/25) patients of RCM., Conclusion: RCM may result from various local and systemic disorders. EMB is helpful for identifying the underlying etiology.

Objective: To investigate the clinical and pathological features of eosinophilic diseases with cardiac involvement., Methods: We analyzed the clinical and cardiac pathological data of 7 patients with eosinophilic diseases with cardiac involvement under endomyocardial biopsy or autopsy., Results: Seven patients (5 male, average age 51 years) were enrolled. Four patients were diagnosed as idiopathic hypereosinophilic syndrome and three were Churg-Strauss syndrome. Peripheral blood eosinophilia count increased significantly in all patients. Cardiac involvement included angina pectoris, myocardial infarction, heart failure, presyncope and sudden death. Electrocardiogram showed cardiac ischemia, bundle branch block and third degree atrioventricular block. Echocardiography suggested ventricular and atrial enlargement, decreased ventricular systolic function, pulmonary hypertension, valvular prolapse and insufficiency and endocarditis. Pathology displayed infiltration of eosinophils, formation of granulomata, necrotizing vasculitis, myocardial necrosis and endomyocardial fibrosis in heart. Coronary artery could be also affected and led to myocardial infarction., Conclusions: Cardiovascular complications of eosinophilic diseases are a major source of morbidity and mortality in these disorders. The manifestations are multiple and early recognition and treatment with steroid and immunosuppressant can improve prognosis.

Objective: To detect the infection of human papillomavirus (HPV) 16/18 in patients with head and neck squamous cell carcinoma and explore the relationship between HPV infection and expressions of Ki-67 and P53 proteins in tumor tissue., Method: The level of HPV 16/18 DNA was measured by real time polymerase chain reaction, and Ki-67 and P53 proteins were measured by immunohistochemistry in tissues from head and neck squamous cell carcinoma., Results: HPV 16/18 DNA was detected in 62.8% of our patients. In each cancer tissue sample, Ki-67 protein was expressed between 2% to 70%. P53 protein was expressed in 46.15% of our patients. No significant relation was found between HPV 16/18 DNA level and sex, smoking, drinking, and tumor clinical stages. However, level of HPV 16/18 DNA was found to have positive relation with tumor pathological grades and negative relation with P53 protein expression. No relation with Ki-67 protein expression was found., Conclusion: Head and neck squamous cell carcinoma may be initiated by HPV 16/18 infection and the mechanism in carcinogenesis involves abnormal expression in P53 protein.

Objective: To distinguish choriocarcinoma from gestational or non-gestational choriocarcinoma and also identify the causative pregnancy of gestational choriocarcinoma by the genetic origin through molecular genetic analysis., Methods: Twelve patients with choriocarcinoma, who had experienced surgery prior to chemotherapy were enrolled in this study. All 12 cases were diagnosed pathologically as choriocarcinoma. Peripheral venous blood samples and formalin-fixed paraffin-embedded blocks of choriocarcinoma tissue microdissected from haematoxylin and eosin-stained sections of tissue by microdissection method were available from the patient and (or) her husband. DNA was then prepared from the couples' blood samples and choriocarcinoma tissue by using standard techniques. PCR amplification and fluorescent microsatellite genotyping were performed by using DNA from the couples and captured choriocarcinoma tissues. The genetic contributions to the choriocarcinoma tissue were determined by comparing the fragments of genes from the choriocarcinoma tissue to those from blood samples of the couples., Results: The primary lesion was ovary in 7 cases, but only 4 of them had the maternal contribution, indicating a non-gestational origin; the other three were gestational choriocarcinoma. The primary lesion was uterus in 5 cases, which were all gestational choriocarcinoma confirmed by genetic analyses. The causative pregnancies of the 8 cases with gestational choriocarcinoma were identified as androgenetic complete hydatidiform mole (AnCHM) in six cases and normal pregnancies in two cases, respectively., Conclusion: Microsatellite polymorphism analysis is a molecular approach for distinguishing the non-gestational choriocarcinoma from the gestational one, and also be used to identify the causative pregnancy of gestational choriocarcinoma.

Objective: To study the impact of neoadjuvant therapy on lymph nodes retrieval in locally advanced mid-low rectal carcinoma., Methods: Data collected from 120 patients with locally advanced mid-low rectal cancer (T2-4 and/or N1-2M0) treated from January 2005 to June 2008 was investigated. The patients were divided into two groups: the study group (n=54) was treated with neoadjuvant therapy (preoperative radiation with a total dosage of 50 Gy and synchronous 5-Fu-based chemotherapy) followed by radical tumor resection 4-6 weeks after;the control group (n=66) underwent primary surgery without neoadjuvant therapy. The clinical stage was evaluated before and after neoadjuvant therapy. The total lymph nodes yields, as well as the tumor-positive lymph nodes of each resected specimen was compared between the two groups statistically., Results: Clinical downstage was achieved in 30 cases (56%) in study group after neoadjuvant therapy. The number of total lymph nodes and positive lymph nodes harvested from each resected specimen in the control group were 14+/-7 and 2.2+/-3.7, meanwhile those were 9+/-6 and 0.7+/-2.4 in study group, which were all significantly lower than those in control group (P<0.01)., Conclusions: Preoperative radiotherapy combined with chemotherapy can downstage the tumor and reduce the retrieval rate of total lymph nodes and positive lymph nodes in locally advanced rectal cancer. It is necessary to retrieve as many lymph nodes as possible for it has some prognostic significance for the patients.

Objective: To identify differentially expression of microRNAs associated with expression of estrogen receptor alpha (ERalpha) and progesterone receptor (PR) between type I and type II endometrial adenocarcinoma., Methods: Two kinds of endometrial adenocarcinoma cell lines, Ishikawa and KLE, was transplanted into nude mice and biopsied to identify the expression of ERalpha, PR and p53, and test their response to estrogen and progesterone. Cultured the two cell lines under the estrogen-free and progesterone-free circumstance, total RNA was isolated to identify the differentially expressed microRNAs by microarray for prediction the microRNAs which target ESR1 and PGR by software miRANDA and TargetScan, and then was validated by real-time PCR in two cell lines cultured both in vivo and in vitro and ten specimens from patients., Results: Ishikawa cell line was confirmed from type I endometrial adenocarcinoma, KLE cell line was confirmed from type II endometrial adenocarcinoma. One hundred and twenty-six differentially expressed microRNAs between the two cell lines were identified by microRNA microarray, among of which may target ESR1 included hsa-miR-100, 99a, and may target PGR included hsa-miR-378, 768-3p. The differential expression of hsa-miR-100, 99a, 378, 768-3p identified by microarray between Ishikawa and KLE in vivo and in vitro was equal to that by real-time PCR, while Hsa-miR-100 was significantly down expressed in type I group specimens compared to type II group (P < 0.01)., Conclusion: Hsa-miR-100 is significantly down-expressed in type I endometrial adenocarcinoma compared to type II, which may be a great potential to target ESR1.

Objective: To study the efficacy and adverse events of intravenous bisphosphonates in the treatment of patients of primary hyperparathyroidism (PHPT) complicated by hypercalcemia crisis., Methods: From October 2003 to December 2007, 14 patients admitted into our hospital were diagnosed as PHPT complicated by hypercalcemia crisis, which was defined as a serum calcium concentration greater than 3.50 mmol/L. Of them, 6 cases had parathyroid adenoma, 1 had hyperplasia and 7 had parathyroid carcinoma. One of the intravenous bisphosphonates including pamidronate, ibandronate and zoledronic acid was given for 29 times in all the 14 cases. Serum calcium, parathyroid hormone, hematology, and other biochemical markers were monitored. Adverse events were recorded., Results: After intravenous bisphosphonates, the serum total calcium (Ca) levels decreased from (3.85 +/- 0.50) mmol/L to (2.86 +/- 0.39) mmol/L in (1.4 +/- 0.6) days, and were kept below 3.50 mmol/L for (10.14 +/- 8.54) days. There was no significant difference of the magnitude of decrease in serum Ca levels among the patients using pamidronate, ibandronate or zoledronic acid. The change of serum Ca level was associated with the serum Ca level before treatment. The response to intravenous bisphosphonates evaluated by the decrease of serum total calcium levels was more significant in patients with parathyroid adenoma or hyperplasia than those with parathyroid carcinoma. The most common adverse event was pyrexia, which occurred 15 times (51.7%) and 75% of the pyrexia events occurred after the first infusion. Other manifestations included fatigue, flu-like symptom, myalgia, arthralgia and diarrhea with an incidence of 3.4% each (one event in the 29 times of treatment). There were 2 events (6.7%) with mild increase of serum creatinine concentration., Conclusion: Bisphosphonates can decrease serum total calcium levels in hypercalcemia crisis caused by PHPT effectively with mild adverse events.