Clinicopathological analysis of lung transplantation in the treatment of end-stage lung disease complicated with neuroendocrine carcinoma / 中华器官移植杂志

Objective:To explore the clinicopathological features of end-stage lung disease complicated with neuroendocrine carcinoma after lung transplantation (LT).Methods:From April 2017 to December 2021, 5 cases of neuroendocrine cancer were diagnosed as end-stage lung disease by hematoxylin-eosin (HE) and immunohistochemical stain.Clinical follow-up data, histological characteristics and immunophenotyping were analyzed retrospectively.Results:The ratio of male-to-female in five recipients was 4: 1 and the average age 64(56-73) years.Three cases were idiopathic pulmonary fibrosis concomitant with small cell carcinoma (including 1 case of combined small cell carcinoma), bronchiectasis plus carcinoid carcinoma (n=1) and connective tissue disease-related fibrosis plus carcinoid carcinoma (n=1). HE stain indicated that morphological spectrum changed from neuroendocrine cell hyperplasia to carcinoid in transplanted lung of bronchiectasis.Immunohistochemical stain indicated that neuroendocrine markers CgA, Syn, CD56 and epithelial markers AE1/AE3, TTF-1 were positive for small cell carcinoma and carcinoid.Ki-67 index of small cell carcinoma (n=2) and combined small cell carcinoma (n=1) was 80% and Ki-67 index of carcinoid (n=2) was ≤1%.Until the last follow-up, 3/5 patients survived and the remaining 2 died of Klebsiella pneumoniae, Corynebacterium striatus and Acinetobacter baumannii infections at Days 33 and 196 post-transplantation.Conclusions:Neuroendocrine carcinoma in transplanted lung is more common in elderly males and end-stage lung disease is mostly idiopathic pulmonary fibrosis.Small cell carcinoma is a major type of neuroendocrine carcinoma.Specific neuroendocrine markers and TTF-1 aid in a definite diagnosis of neuroendocrine carcinoma.Postoperative infection is an important prognostic factor.

Value of diagnosis and efficacy evaluation of folate receptor-positive circulating tumor cells in patients with small cell lung cancer / 肿瘤研究与临床

Objective:To analyze the value of folate receptor-positive circulating tumor cells (FR +-CTC) in the diagnosis and efficacy evaluation of patients with small cell lung cancer (SCLC). Methods:The data of 59 patients with SCLC and 14 patients with benign pulmonary diseases treated in China-Japan Friendship Hospital from May 2017 to October 2019 were retrospectively analyzed. Folate receptor targeted detection was used to detect the level of FR +-CTC in the blood of SCLC patients. The levels of serum progastrin-releasing peptide (Pro-GRP), neuron-specific enolase (NSE), cytokeratin 19 fragment 21-1 (Cyfra21-1) , and carcinoembryonic antigen (CEA) were detected by using chemiluminescence. The median ( P25, P75) was used as all the detection indexes. Mann-Whitney U test was used for pairwise comparison, Spearman correlation test was used to analyze the correlation between two variables, and receiver operator characteristic (ROC) curve was used to evaluate the diagnostic efficacy. Results:The level of FR +-CTC in 59 patients with SCLC was 11.00 FU/3 ml (7.10 FU/3 ml, 14.50 FU/3 ml), and the positive rate of FR +-CTC in patients with SCLC was 66.10% (30/59); the level of FR +-CTC in 14 patients with benign pulmonary diseases was 6.75 FU/3 ml (5.03 FU/3 ml, 7.85 FU/3 ml), and the positive rate of FR +-CTC in 14 patients with benign pulmonary diseases was 14.29% (2/14). The level of FR +-CTC in patients with SCLC was higher than that in patients with benign pulmonary diseases, and the difference was statistically different ( U = 33.50, P < 0.01). The expression level of FR +-CTC was not related to age, gender and smoking history in SCLC patients (all P>0.05). The expression level of FR +-CTC in patients with extensive-stage was higher than that in patients with limited-stage, and the difference was statistically significant ( P < 0.05). Tumor markers Pro-GRP, NSE, Cyfra21-1 and CEA were compared with FR +-CTC, and the ROC curve was drawn; the results showed that FR +-CTC had better sensitivity (71.2%) and specificity (92.90%) in the diagnosis of SCLC. For SCLC patients who received chemotherapy, the decrease range of FR +-CTC in patients with partial remission and stable disease was greater than that in patients with the progression of disease, and the differences were statistically significant (all P < 0.05). Conclusion:FR +-CTC can assist the diagnosis and disease staging of SCLC. For patients receiving chemotherapy, continuous detection of circulating tumor cells can help to evaluate the efficacy of chemotherapy and provide a reference for the choice of clinical treatment.

Interpretation of BRAF gene mutation detection by real-time PCR: a study of 8 644 thyroid fine-needle aspiration specimens / 中华病理学杂志

Objective@#To study common problems in BRAF gene mutation detection, and conditions for repetition testing using thyroid fine needle aspiration specimens.@*Methods@#A total of 8 644 cases of thyroid fine-needle aspiration specimens at China-Japan Friendship Hospital were collected between February, 2012 and July, 2018. BRAF gene mutation was detected by real-time PCR. Repeat testing was performed in 237 cases when the results were inconsistent with clinical or cytological diagnosis or when uncertain results were obtained.@*Results@#The final positive rates of BRAF mutation was 22.0% (1 897/8 625). Nineteen cases were excluded due to inadequate DNA samples. The average Ct value of internal quality control was 16.061, and the average Ct value of the positive samples was 19.147. Among 237 repeat tests, 51.4% (19/37) continued to have poor DNA quality and 48.6% (18/37) had adequate DNA resulting in 1 positive case and 17 negative cases. In 40 repetition of initial negative cases, results were unchanged. In initial positive cases, 40.4% (40/99) with a difference of Ct value (between BRAF gene and internal quality control) between 8 to 12 turned negative after repetition, 69.8% (37/53) of these cases with a difference of more than 12 turned negative after repetition. The sensitivity and specificity of BRAF mutation were 83.97% and 96.94%, respectively.@*Conclusions@#Difference between BRAF gene Ct value and internal quality control Ct value is recommended as a reliability index for the test result. Cases with a difference greater than 8 should be subjected to repeat testing.

Clinicopathological and molecular features of Erdheim-Chester disease accompanied with Langerhans cell histiocytosis / 中华病理学杂志

Objective@#To investigate the clinicpathological and molecular features of Erdheim-Chester disease (ECD) as well langerhans cell histiocytosis (LCH).@*Methods@#The clinical, histopathological, molecular findings, immunophenotype, treatment and prognosis in 4 cases of ECD combined LCH were evaluated from February 2015 to September 2018 with review of the relevant literature.@*Results@#2 cases were male, and 2 were female, aged from 7-55 years. Microscopically, there were two different areas, in the first area, the lesions were composed of foamy histiocytes, spindle-shaped fibroblasts, scattered multinucleated giant cells. Lymphocytes, plasma cells, and giant cells were also found. In the other, the lesions were composed of histiocytes with obvious nuclear groove, associated with a variable number of eosinophils, lymphocytes and plasma cells. Immunephenotype, In the second area, histiocytes were positive for CD1a (4/4), S-100 (4/4),CD207/Langerin (4/4), cyclin D1(4/4), and in the two different area, the histiocytes were positive for CD68, CD163, Braf. Ki-67 positive index 1%-10% BRAF V600E gene mutation was detected in three cases.@*Conclusion@#ECD combined LCH was a very rare histiocytosis tumor and its correct diagnosis relies on histopathologic features, immunohistochemical staining, and BRAF V600E gene detection.

Clinical pathologic characteristics of extranodal follicular dendritic cell sarcoma / 中华肿瘤杂志

Objective@#To investigate the clinical pathologic characteristics of extranodal follicular dendritic cell sarcoma (FDCS).@*Methods@#We collected 7 cases of extranodal FDCS, HE staining, immunohistochemical study were performed. The V600E mutation of BRAF in 7 cases were detected by real-time PCR and EBER in situ hybridization was performed on 4 cases.@*Results@#Among the 7 cases of FDCS, 5 cases were male and 2 cases were female, the median age was 55 years old, including 4 cases of low-grade FDCS and 3 cases of high-grade FDCS. The tumor location of 2 cases was in mediastinum, the tumor locations of others were in nasopharynx, kidney, lung, rectum and liver, respectively. The results of immunohistochemistry showed that, the tumor cells were diffusely or focally positive for CD21, CD23, CD35, D2-40, EGFR and CXCL13, but negative for S-100, CD68, HMB45, SMA, Desmin, CD117, Dog-1, CD34, CD30, EMA and CK.Five cases were positive for PD-L1 and the its expression in high-grade FDCS were higher than that in low-grade FDCS.Two cases of low-grade FDCS were positive for BRAF V600E, but the BRAF V600E mutation weren′t detected in all of 7 cases. The result of EBER in-situ hybridization showed that only the nasopharynx FDCS was positive.The follow-up information of 5 patients were available (7~43 months), 4 patients died and 1 still alive with rectum metastasis.@*Conclusions@#FDCS is a rare malignant disease with relapse and metastatic tendency. The combined applications of the first-line antibodies including CD21, CD23, CD35 and second-line antibodies including D2-40, CXCL13, EGFR are helpful for its diagnosis and differential diagnosis. The high expression of PD-L1 implicates the potential benefit of FDCS patients acquired from immunotherapy.

MRI Features and Pathological Basis of Aggressive Angiomyxoma in Pelvic and Perineal Areas / 中国医学影像学杂志

Purpose To summarize the clinical features, MRI features and pathological features of aggressive angiomyxoma (AAM) occurring in pelvic and perineal areas. Materials and Methods The clinical, MR and pathological data of 7 patients with AAM pathologically confirmed were retrospectively analyzed. Results Seven cases of AAM were all females, aged from 12 to 45 years old. Among them, 5 cases were treated due to perineal area tumors, and 2 cases due to relapses after surgical treatment of perineal area AAM in other hospitals. MRI findings: 7 cases were single occurrence with the maximum diameter of 5.9-15.7 cm; 6 cases were in irregular shape, with no envelope and unclear borders with adjacent tissues or organs; 1 case was in long column shape with clear boundary and pseudocapsule visible. On T1WI, AAM showed equal signal (4/7) or equal, low mixed signal (3/7). On T2WI, AAM showed high and low mixed signals with 5 cases of "vortex sign". 5 cases underwent routine enhancement examinations, all demonstrating significant enhancement, 4 cases of which displayed heterogeneous enhancement ("vortex sign" was detected in 2 cases) and 1 case homogeneous enhancement. On diffusion weighted imaging, AAM manifested as homogeneous (3/7) or heterogeneous (4/7) high signal. Pathological examination showed that a large amount of mucin-like matrix was contained in AAM, and there were scattered fusiform and astral tumor cells and abundant arterial and venous blood vessels seen locally. Tumor cells infiltrated the peripheral fat or muscle tissues at the border. Conclusion The characteristic MRI of AAM is manifested as "vortex sign", which corresponds to its pathological features such as sparse tumor cells, mucin-like matrix in large amount, and locally rich blood vessels, suggesting great significance for imaging diagnosis.

Clinical and immunohistopathologic study of phosphaturic mesenchymal tumor / 中华病理学杂志

Objective@#To study the clinicopathological characteristics and immunohistochemical phenotype of phosphaturic mesenchymal tumor (PMT) .@*Methods@#The clinicopathological data and immunohistochemical profiles were obtained retrospectively from 206 patients diagnosed with PMT at Peking Union Medical College Hospital (PUMCH) during July 2008 to September 2017, with a review of literature.@*Results@#The mean age of PMT patients was 42 years (range 13 to 70 years), with a male to female ratio of 1.1∶1.0. All patients presented with different degree of bone pain, muscle weakness, shorten of stature, thoracic deformity and pathological fractures, with hypophosphatemia and high serum ALP. Phosphatemia returned to normal within 1 week after operation in all cases underwent complete tumor resection. The duration of osteomalacia before resection (documented in 197 cases) ranged from 20 days to 40 years (average 5.7 years). The average blood phosphorus concentration raised from 0.49 mmol/L to 0.92 mmol/L before and after tumor resection (P<0.01), with 147 cases (84.0%, 147/175) returned to normal range within 2 weeks. The rate or blood phosphorus concentration recovery in 15 days after operation was 79.6% in average, displayed significant differences between patients with complete resection and those with partial resection (85.4% vs. 21.1%, P<0.01). PMT lesions mainly involved lower extremities (55.8%), followed by head and neck (29.1%). In immunohistochemical study, all cases were positive for vimentin (100.0%), while most cases were positive for NSE (96.3%), CD56 (94.2%), FGF23(88.4%), CD68 (88.3%), D2-40 (70.9%), CD34 (23.1%), SMA (55.5%), bcl-2 (59.8%) and CD99 (47.1%). The Ki-67 positive index of tumor varied from less than 2% (51.4%), 3% to 10% (41.3%) to >10% (7.2%).@*Conclusions@#PMT mainly occurs in lower limbs or head and neck, with unique clinical characteristics and blood biochemical indexes. The tumor expresses a variety of immunohistochemical markers, indicating the potential of multi-directional differentiation. Clinical profile, blood biochemistry testing and immunohistochemical phenotype is helpful for diagnosis of PMT.

Clinical characteristics of intravascular large B cell lymphoma: a single-center retrospective study / 中华血液学杂志

Objective@#To investigate clinic-pathological characteristics, diagnosis, treatment and prognosis of intravascular large B cell lymphoma (IVLBCL) in China.@*Methods@#Clinical and pathological records were analyzed from 12 IVLBCL patients diagnosed between Jan 2010 to Jun 2016. Kaplan-Meier method was used to estimate overall survival (OS), and univariate analysis was performed to identify prognostic factors.@*Results@#A series of 12 patients with IVLBCL (median age, 53.8 years; range, 32-76 years; 6 males and 6 females) was reviewed. Fever was the most common symptom (10/12), respiratory symptoms (cough, pleural effusion, dyspnea, 50%) and hemophagocytic lymphohistiocytosis (50%) were frequently observed, and only 12 patients had neurological symptom. All patients had elevated lactic dehydrogenase and serum ferritin. International Prognostic Index score was high in 75% of total patients. All patients had extra-nodal involved, pulmonary (6/12) and bone marrow (4/12) were frequently involved. Large lymphoid cells within vessel lumina or sinuses were observed in all patients. These cells were large, with scant cytoplasm, vesicular nuclei, and one or more nucleoli, and the structures of vessels and sinus were reserved. CD20 and CD79a were positive in all cases. 11patients received rituximab combined CHOP regimen chemotherapies, overall response rate (ORR) was 90.1%, and complete response rate was 66.7%. Median survival time and median progression time were not reached after a median follow-up of 20 months. Univariate analysis revealed that no clinical characters were associated with OS.@*Conclusion@#As a rare variant of DLBCL, IVLBCL presented with pulmonary involved frequently, and trans-bronchial lung biopsy had good positive rates. Rituximab contained chemotherapy was the backbone for IVLBCL.

The 453rd case:diarrhea, weight loss and diabetes mellitus / 中华内科杂志

Chronic diarrhea is a common complaint in gastroentology department,which is classified into a variety of types.While as eosinophilic gastroenteritis is a rare cause.A 50-year-old male patient was admitted with a 2-year history of diarrhea worsened for 10 months.Fasting could alleviate the diarrhea.Laboratory findings demonstrated anemia,low vitamins and minerals,fat malabsorption and abnormal D-xylose absorption test,delayed gastric emptying.According to the endoscopic biopsy of gastrointestinal mucosa,the diagnosis of eosinophilic gastroenteritis was made.Diabetic neuropathy may aggravate the symptom.The diarrhea resolved after the administration of steroids.

Expression of CD133 in rectal cancer tissues and its relationship with neoadjuvant chemoradiotherapy / 中华胃肠外科杂志

<p><b>OBJECTIVE</b>To investigate the association of CD133 expression in rectal cancer tissues with neoadjuvant chemoradiotherapy (nCRT) and tumor regression grading (TRG) after nCRT.</p><p><b>METHODS</b>Radical resected rectal cancer specimens and clinicopathological data of 105 patients, including 60 men and 45 women with median age of 59 years, diagnosed as locally advanced rectal cancer in Peking Union Medical College Hospital from January 2008 to December 2014 were collected retrospectively. Thirty-nine and 66 cases were histologically classified as good-moderate and poor differentiation respectively. Sixty-eight and 37 cases were clinically graded as stage I(-II( and III(-IIII( in preoperative assessment respectively. NCRT was administered in 61 cases before surgery (nCRT group). The nCRT consisted of preoperative pelvic radiotherapy using 50 Gy (2 Gy once, for 25 sessions) with FOLFOX regimen (5-fluorouracil plus oxaliplatin) for 2-3 cycles or XELOX regimen (capecitabine plus oxaliplatin) for 2 cycles. Patients underwent surgery after 6 courses of nCRT, and then received the same previous chemotherapy regimen. In nCRT group, biopsy specimens before nCRT were obtained in 45 cases. Forty-four cases received surgery alone without nCRT (surgery alone group). CD133 expression was tested by immunohistochemical Envision two-step methods. The histological TRG evaluation was performed in the nCRT group. TRG score 0-2 was defined as insensitivity to nCRT, whereas TRG score 3-4 was defined as sensitivity. CD133 expression in rectal cancer samples before and after nCRT was compared. Association of CD133 expression with TRG after nCRT was examined.</p><p><b>RESULTS</b>No significant differences of baseline parameters were found between nCRT group and surgery alone group (all P>0.05). The positive rate of CD133 in nCRT group was 70.4%(43/61,) which was significantly higher than that in surgery alone group (47.7%, 21/44)(χ(2)=5.566, P=0.018) and that in biopsy samples before nCRT group (44.4%, 20/45)(χ(2)=7.287, P=0.007). Twenty-two cases (36.1%, 22/61) in nCRT group had TRG score of 3-4 . Among these 22 cases, 11 cases were negative CD133, and constituted 61.1% (11/18) of all CD133-low expression cases in nCRT group, whereas the other 11 cases were positive CD133, and constituted 25.6%(11/43) of all CD133-high expression cases in nCRT group (χ(2)=6.974, P=0.008).</p><p><b>CONCLUSION</b>The CD133 expression up-regulates markedly in rectal cancer after nCRT and nCRT may have potential positive modulation on CD133 expression. CD133-positive cancer reveals lower response to nCRT, suggesting CD133 may be a potential target for improving efficacy of nCRT in rectal cancer.</p>

Expression and prognostic value of CARD11 in diffuse large B cell lymphoma / 中华血液学杂志

<p><b>OBJECTIVE</b>To determine the CARD11 expression and its prognostic value in diffuse large B cell lymphoma (DLBCL).</p><p><b>METHODS</b>This retrospective study included previously untreated patients diagnosed with DLBCL from January 2007 to December 2012. Formalin-fixed, paraffin-embedded blocks of these patients were collected. Tissue microarray was built and expression of CARD11 was examined immunohistochemically. Subtype of DLBCL was determined by Hans algorithm (CD10, BCL6, MUM1). The pattern of CARD11 was further studied and their correlation with outcome was analyzed.</p><p><b>RESULTS</b>79 patients with DLBCL were enrolled and two reactive lymph nodes were used as control. The positive rate of high CARD11 expression in DLBCL was 65.33%, which showed no significant associations with patients' characteristics. Positive CARD11 expression was associated with an inferior event free survival (EFS)(2- year EFS: 52.03%vs 86.12%,P=0.036). Even in patients with a high international prognostic index (IPI, 3-5 points), this difference still remained significant (Median EFS not reached vs 557 days,P=0.033).</p><p><b>CONCLUSION</b>DLBCL patients with high CARD11 expression had a shorter EFS compared with low level of CARD11. This difference remained significant when patients were in high IPI (3-5 points), which might indicate the value of CARD11 in stratification of high-risk DLBCL patients.</p>

Classification of amyloidosis by laser micro-dissection and mass spectrometry based proteomic analysis / 中华血液学杂志

<p><b>OBJECTIVE</b>To establish a novel method to determine specific type of amyloidosis through laser microdissection and mass spectrometry (LMD/MS) based proteomic analysis.</p><p><b>METHODS</b>There were 138 formalin-fixed and paraffin-embedded (FFPE) biopsy samples of patients who were diagnosed as systemic amyloidosis used in this study. For each case, a 10 μm section stained with congo-red and positive amyloid deposits were identified under fluorescent light, followed by micro-dissection and mass spectrometry analysis. The amyloidosis subtype was confirmed based on the most abundant amyloid protein.</p><p><b>RESULTS</b>The tissue types of 138 specimens were as following: subcutaneous abdominal fat accounted for 26%, tongue for 19%, gingiva for 11%, kidney for 9%, intestine for 9%, heart for 6% and others for 20%. Specific types of amyloid were accurately detected in 121 cases, including 106 (87.6%) amyloid light chain (AL) type, 7 (5.8%) amyloid trans-thy-retin (ATTR), 2 (1.7%) amyloidogenic protein A (AA), 2 (1.7%) amyloid heavy chain (AH)/AL+AH, 2 (1.7%) fibrinogen alpha chain (AFib), 1(0.8%) amyloid apolipoprotein A-type II (AApoA-II) and one (0.8%) amyloid lysozyme (ALys). Diagnosis of amyloidosis was excluded in 5 cases. The types of twelve cases were indeterminate by LMD/MS. On the whole, LMD/MS reached 91.3% accuracy rate in amyloid typing. Commonly involved organs (for example, heart, kidney and liver) turned out to be suitable sources of FFPE samples with typing success rate of almost 100%. In contrast, MS analysis was successful in only 83.3% of subcutaneous abdominal fat samples.</p><p><b>CONCLUSION</b>LMD/MS method provided a more direct technique for accurate typing of amyloidosis in a single procedure.</p>

Phosphaturic mesenchymal tumor:clinicopathological analysis of 12 cases / 临床与实验病理学杂志

Purpose To study the clinicopathological features of the phosphaturic mesenchymal tumors. Methods The clinicopatho-logical data, histology and immunohistochemical findings of 12 cases of phosphaturic mesenchymal tumors were retrospectively analyzed with review of the relevant literature. Results Among the 12 patients studied, 8 were males and 4 were females. Their age at the time of operation ranged from 23 to 63 years (mean=40. 5 years). The duration of symptoms ranged from 1 to 14 years (mean =5. 6 years) . A history of long-standing bone pain, arthralgia, limitation in movement, accompanied with hypophosphatemia and hyperphos-phaturia were present in all cases. The tumors size ranged from 1 to 7. 5 cm ( mean size=2. 7 cm) . The tumors were primarily com-posed of spindle cells, and were accompanied with osteoclast-like giant cells partly. Their background were rich in blood vessels. In addition, there were scattered tufed thick vascular anomaly, thin vascular, islands of mature adipocytes and chondroid cells. In 7 of the 12 cases, there were dystrophic calcification in an unusual flocculent. Spindle epithelium were noted in 2 cases. Mitotic figures were rare in 10 cases. In 2 of the 12 cases however, mitotic figures were commonly encountered, and in 1 of the 2 cases, heterotypic cells were notablely observed as well. On immunohistochemical study, the tumor cells were all positive for vimentin and CD56. The positivi-ty for NSE, CD99, BCL-2 in 11, 8 and 7 cases were expressed in 12 cases. In 4 and 6 of the 12 cases, CD34 and SMA were positive in various degree. Ki-67 proliferation index were less than 5% in 10 cases, and 10% and 25% in 2 cases. The duration of follow-up ranged from 2 to 108 months ( mean=22 months) . 2 cases were recurred at 72 and 84 months after the operation, respectively. So far, the remaining 10 cases had no recurrence and metastasis. Conclusioin The phosphaturic mesenchymal tumors are either benign or low-grade malignant. Their histomorphology are multivariable and short of specificity. It is important to make the correct diagnosis in combination with their common features and clinical datas.

Pathologic analysis of primary cardiac inflammatory myofibroblastic tumor / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinical and pathologic features of primary cardiac inflammatory myofibroblastic tumor.</p><p><b>METHODS</b>A total of 4 patients with primary cardiac inflammatory myofibroblastic tumor were encountered during the period from 1993 to 2013 in National Center for Cardiovascular Disease. The clinical features, imaging findings and outcomes of the 4 patients were evaluated. ALK protein expression and ALK gene status were studied using the archival tumor tissues.</p><p><b>RESULTS</b>There were 1 female and 3 male patients. The age of patients ranged from 5 months to 30 years (mean = 16 years). The tumor was located in right ventricle (n = 2), right atrium (n = 1) or pericardium (n = 1). Histologic patterns included 2 cases of fibrous histiocytoma type, 1 case of granulomatous type and 1 case of sclerosing type. Immunohistochemical study showed that 2 cases expressed ALK protein. Fluorescence in-situ hybridization however did not reveal any ALK gene rearrangement.</p><p><b>CONCLUSIONS</b>Inflammatory myofibroblastic tumor of the heart is rarely encountered and easily misdiagnosed. It carries distinctive clinical and pathologic features. ALK protein expression is helpful in arriving at the correct diagnosis.</p>

Clinical and pathologic characteristics of Erdheim-Chester disease / 中华病理学杂志

<p><b>OBJECTIVE</b>To explore the clinicopathologic features, immunophenotype, differential diagnosis and gene mutation status of the Erdheim-Chester disease (ECD).</p><p><b>METHODS</b>Clinical and pathologic findings of 3 ECD cases were examined by gross, microscopic, immunohistochemical methods and BRAF V600E mutation. Related literatures were reviewed.</p><p><b>RESULTS</b>Two male patients and one female patient presented clinically with multiple skin nodules, bone pain and bony lesions by imaging study. Microscopically, the lesions were composed of spindle-shaped fibroblasts, foamy histiocytes and scattered Touton-type giant cells embedded in reactive fibrous tissue. Lymphocytes, plasma cells, and multinucleated giant cells were also found. Immunohistochemically, all histiocytes were positive for CD68, none of which expressed CD1a, although 2 cases focally expressed weak S-100 stain. In 2 cases,BRAF V600E mutation was detected.</p><p><b>CONCLUSIONS</b>ECD is a rare disease of xanthogranulomatous histiocytosis.Its diagnosis relies on pathological and immunohistochemical findings, but correlation with clinical information, especially radiographic findings should be performed.No effective treatment of the disease is currently available.</p>

A clinical analysis of AIDS-related non-Hodgkin lymphoma in 5 patients and review of literature / 中华内科杂志

Objective To analyze the clinical characteristics of AIDS-related non-Hodgkin lymphoma(ARL)and review relative literature for the diagnosis and treatment of ARL.Method The clinical data of ARL patients admitted to Peking Union Medical College Hospital from April 2009 to April 2011 were retrospectively analyzed.Results Five male ARL patients aged 32 to 65 years old were included in this retrospective study.Among them,two patients were found to be HIV-positive for the first time,three were on regular highly active anti-retroviral therapy(HAART)for 7-8 months before the emergence of lymphoma-related symptoms.CD4+ T cell count was(69-232)× 106/L at presentation.Two patients firstly presented with sore throat and throat ulcer,one with cervical nodules,one with pelvic mass,one with fever and edema in right thigh.Through pathological analysis,four patients had B cell-originated lymphoma,with one Burkitt lymphoma and three diffuse large B cell lymphomas; one patient had T-cell lymphoma.Four patients were treated with chemotherapy,with one complete remission,one relapse,one non-response,and one death.One patient had radiotherapy only and had progressed disease.Bone marrow suppression and gastrointestinal disturbance were the main adverse effects of chemotherapy.Conclusions Lymphoma should be considered in any HIV-infected patients presented with unexplainable adenopathy,recurrent sore throat or throat ulcer,or fever of unknown origin.Biopsy should be rigorously carried out.Appropriate chemotherapy,together with HAART,may improve the prognosis greatly.

Endoscopic ultrasonography for restaging and predicting pathological response to advanced gastric cancer after neoadjuvant chemotherapy / 中华消化内镜杂志

Objective To evaluate endoscopic ultrasonography (EUS) for TN restaging and predicting response to advanced gastric cancer after neoadjuvant chemotherapy. Methods A total of 22 patients,15 males and 7 females, mean age 64 (36-80 years ), with advanced gastric cancer were recruited to the study from June 2007 to December 2009 with written informed consents. All patients underwent 3 cycles of neoadjuvant chemotherapy ( Folfox 6 ), and subsequent surgery ( R0 resction) in 3-4 weeks after chemotherapy. EUS was performed 1-2 weeks before and 1-2 weeks after chemotherapy. EUS TN staging was compared with pathological findings. The correlation of peri-chemotherapy EUS TN staging with postoperative pathological response was evaluated. Results After chemotherapy, the overall accuracy of EUS T staging was 63.6% (14/22), with overstaging (36. 4%, 8/22) more frequent than understaging (0). The overall accuracy of N staging was 54. 5% (12/22) with 4 ( 18. 2%, 4/22) overstaging and 6 ( 27. 3%, 6/22 ) understaging. EUS revealed T and/or N downstaging ( concyrrence of T and N downstaging was accounted once) after chemotherapy in 10 patients, with 9 T downstaging (4 from T3 to T2, 5 from T4 to T3) and 4 N downstaging (4 from N1 to N0). TN downstaging was correlated with pathological response, with 7 patients achieving pathological response 2 and 1 patient 3. Conclusion T and N restaging by EUS after neoadjuvant chemotherapy in patients with locally advanced gastric cancer is not accurate enough. However, T and/or N downstaging confirmed by EUS is well correlated with a better degree of pathological response to chemotherapy.

Prenatal sonographic diagnosis of hemivertebra / 中华超声影像学杂志

Objective To investigate the diagnostic value of prenatal ultrasonography in the fetal hemivertebra. Methods The ultrasonographic findings of three fetuses with hemivertebra diagnosed in our hospital were reviewed and compared with those of postnatal ultrasonography,other image modalities,and autopsy. Results In all fetuses,a distortion of the spine was observed where only one half of the vertebra could be identified. The parents opted for termination of the pregnancy in one case and the deformity was confirmed by autopsy. The other two fetuses were delivered and in one fetus the diagnosis was confirmed by radiological assessment. Conclusions Hemivertebra can be diagnosed accurately by second-trimester ultrasonography. The prognosis is mostly favorable when no other anomalies are associated. Meticulous examination may disclose the lesion and help decide the fate of pregnancy.

A clinical analysis of 20 cases with bone marrow granulomas / 中华内科杂志

Objective To study the clinical feature and original diseases of bone marrow granulomas. Methods A total of 5217 bone marrow biopsies were retrospecitively analyzed in Peking Union Medical College Hospital from January 2001 to December 2007. Results Bone marrow granulomas were present in 20 cases, representing an incidence of 0.38% in the series and an annual incidence of 2. 9 cases per year. Finally, 13 of these cases (65%) were diagnosed with tuberculosis. Hematological neoplasms and viral hepatitis were found in 4 and 2 cases respectively. Acute interstitial nephritis was diagnosed in one case. Conclusions The finding of a granuloma in a bone marrow biopsy is not common and unspecific.Tuberculosis, hematological neoplasms and viral hepatitis are the most common underlying diseases. Bone marrow biopsy is an important method for the diagnosis of disseminated tuberculosis.

Prenatal ultrasound diagnosis of fetal hand malformations / 中华超声影像学杂志

Objective To determine the diagnostic value of two and three dimensional ultrasound in detecting fetal hand malformations. Methods In the retrospectively analysis,the severe fetal hand malformations detected by prenatal ultrasound during the recent three years in our hospital were classified according to the prenatal sonographic characteristics,family history,karyotype analysis and autopsy results,etc.Results Fourteen fetuses with hand-anomaly were detected during the 16-28th week of gestation,both hands were affected in 10 cases,with the same morphology bilaterally in 8 cases.Thirteen cases had other abnormal sonographic findings.They were detected and classified into three categories,Wrist deformity (9 cases),three fetuses were associated with total absence of radius and radial clubhand,and six fetuses had palmar deviated hands,with various etiologies including familial hereditary arthrogryposis multiplex congenita,distal type 1 (AMCD1),amniotic band syndrome,body stalk anomaly,trisomy18 and micromelia.Hand (figer) hypoplasia or aphasia (3 cases),one hand was absent in one fetus without associated anomaly,absence of five fingers with ipsilateral multicystic dysplastic kidney in the second fetus,and the third fetus had split hand/foot malformation (SHFM).Overlapping fingers (4 cases),three of them were trisomy18,and two fetuses had both wrist deformity and overlapping fingers.Conclusions Prenatal two and three dimensional ultrasound play an important role in detecting and diagnosing severe type of fetal hand malformations.