The Chinese guidelines for the diagnosis and treatment of pancreatic neuroendocrine neoplasms (2020) / 中华消化外科杂志

Pancreatic neuroendocrine neoplasms (pNENs) are highly heterogeneous, and the management of pNENs patients can be intractable. To address this challenge, an expert committee was established on behalf of the Chinese Pancreatic Surgery Association, Chinese Society of Surgery, Chinese Medical Association, which consisted of surgical oncologists, gastroenterologists, medical oncologists, endocrinologists, radiologists, pathologists, and nuclear medicine specialists. By reviewing the important issues regarding the diagnosis and treatment of pNENs, the committee concluded evidence-based statements and recommendations in this article, in order to further improve the management of pNENs patients in China.

SMARCA4-deficient primary thoracic sarcoma:a clinicopathological analysis of five cases / 中华病理学杂志

Objective@#To investigate the clinicopathological characteristics of SMARCA4- deficient thoracic sarcomas.@*Methods@#The clinical features and CT scans of SMARCA4-deficient thoracic sarcomas (n=5) diagnosed at Fudan University Cancer Hospital from December 2016 to October 2018 were reviewed. Hematoxylin-eosin staining, immunohistochemistry and targeted next generation sequencing were performed in available cases along with a literature review.@*Results@#All 5 patients were males with age ranging from 32 to 65 years (average 54 years; median 61 years). Four patients were smokers except one with unknown smoking history. The average maximum diameter of tumor was 5.6 cm. Tumor primary sites included thoracic wall,thoracic cavity,lung and mediastinum. Histologically,tumor cells formed solid sheets or anastomosing islands with brisk mitotic figures accompanying with large areas of necrosis. Three cases focally exhibited rhabdoid morphology and vesicular chromatin. Immunohistochemically, SMARCA4, SMARCA2 and Claudin-4 were negative in all cases and all tumors demonstrated SOX2 and SMARCB1 nuclear positive staining. Among 3 cases analyzed by targeted next generation sequencing, concurrent SMARCA4 and p53 mutation was detected in all three cases. Mutations of STK11, ERBB4, NF2, GNAS, MTOR,MET and FGFR1 amplification were also detected among the three cases. The follow-up information was available in all 5 cases. Two patients died of the tumor. One relapsed multiple times after surgeries but was alive with disease. Two patients received radical excisions without relapse.@*Conclusions@#SMARCA4-deficient thoracic sarcoma is a rare but highly-aggressive tumor with dismal prognosis. The tumor is featured by rhabdoid morphology histologically and distinctive immunohistochemical and molecular phenotype.

Expression of P16 and CD44 in gastrointestinal stromal tumors and their relationship with the prognosis ;of patients / 实用医学杂志

Objective To investigate the expression of P16 and CD44 in gastrointestinal stromal tumors (GIST) and their relationship with the prognosis of patients. Methods The GIST specimens of seventy patients who received surgical excision were collected. Tissue microarray of the seventy GIST samples was constructed. The expression of P16 and CD44 were detected by the immunohistochemical staining. The patients were followed up via out-patient examination and telephone. Results All the patients were followed up for 2-212 months, and the median time for follow-up was 68 months. The differences of the expression of P16 in GISTs among NIH risk ranks were insignificant (P > 0.05). The differences of the expression of CD44 in GISTs among NIH risk ranks were statistically significant (P < 0.05). Univariate analysis showed that tumor size, mitotic count, tumor location, NIH risk rank, the expression of P16 and CD44 were related to the prognosis of GIST patients. Multivariate showed that tumor size, mitotic count, tumor location, and the expression of CD44 was independent prognosis factors of GIST patients. Conclusion CD44 could be used as a biomarker in predicting the prognosis of GIST patients.

Grey zone lymphoma with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: clinicopathologic characterization of 16 cases showing different patterns / 中华病理学杂志

<p><b>OBJECTIVE</b>To profile the clinicopathologic features of a series of grey zone lymphoma (GZL) cases with hybrid features of diffuse large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma (CHL), with a purpose to gain an in-depth understanding of the borderline B-cell neoplasm.</p><p><b>METHODS</b>The clinical, morphologic and immunophenotyical characteristics of 16 cases were retrospectively analyzed.</p><p><b>RESULTS</b>The patients were mostly male adults, with a male to female ratio of 1.7: 1.0 and a mean age of 40.2 years. Eight patients presented with peripheral nodal lesions and five cases with mediastinal involvement. Histologically and immunophenotypically, the 16 cases were classified into three sub-categories. In 4 cases, the morphologic features resembled CHL more closely, but the neoplastic cells showed uniform and intense positive staining of CD20 (pattern 1). Although the initial impression of the other 8 cases was that of DLBCL, the expression levels of CD20 and PAX5 were variable, and CD30 or CD15 was positive (pattern 2). A characteristic feature of pattern 3, observed in the remaining 4 cases, demonstrated a broad spectrum of morphology with hybrid features of both CHL and DLBCL. The neoplastic cells in pattern 3 were positive for CD20, CD30 and CD15. EBV-LMP1 was detected in 6 of the 11 tested cases. Clinically, most patients with GZL seemed insensitive to immuno-chemotherapy of the R-CHOP regimen.</p><p><b>CONCLUSIONS</b>The diagnostic criteria for GZL with features intermediate between DLBCL and CHL is proposed by the three histologic patterns commonly seen in these lesions. Cases presented with peripheral lesions might differ from those with mediastinal presentation pathologically. At current time, there is no effective treatment for these borderline B-cell lymphomas and the prognosis is poor.</p>

Immunohistochemical classification and prognosis of diffuse large B-cell lymphoma in China / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the immunohistochemical classification and prognosis of diffuse large B-cell lymphoma (DLBCL).</p><p><b>METHODS</b>A total of 148 cases of DLBCL were classified into germinal center B-cell-like (GCB) and non-GCB/activated B-cell-like (ABC) subtypes by Hans, Choi and Tally immunohistochemical stain algorithms. The clinical features and survival data of GCB and non-GCB/ABC subtypes were compared. Multivariate analysis about clinical features and results of immunohistochemical stain algorithms was carried out by using Cox regression, with overall survival as the outcome.</p><p><b>RESULTS</b>The prevalence of GCB subtype was significantly lower than that of non-GCB/ABC subtype, as classified by whichever algorithms in the 148 DLBCL cases studied. The prevalence of GCB subtype by Tally algorithm was lowest. The prevalence of GCB subtype (19 cases, 16.7%) was also significantly lower than non-GCB/ABC subtype (95 cases, 83.3%; P = 0.000 1) in the 114 (77.0%) concordant cases by the three algorithms. There was no difference between GCB and non-GCB/ABC subtypes by the three algorithms in five-year overall survival rate and survival curve of the 80 DLBCL patients with follow-up data available (P > 0.05). Primary gastric DLBCL tended to show a higher prevalence of GCB subtype, a better five-year overall survival rate and survival curve than the other groups. Multivariate analysis showed that patient age (HR = 1.036, P = 0.001) and tumor stage (HR = 1.997, P = 0) were also significantly adverse predictors of overall survival.</p><p><b>CONCLUSION</b>The Hans, Choi and Tally immunohistochemical stain algorithms cannot effectively classify Chinese DLBCL into different prognostic subtypes. Primary gastric DLBCL has different immunophenotype and outcome, as compared with DLCBL in other sites.</p>

Primary and metastatic hepatic neuroendocrine carcinoma: a clinicopathological study / 中华肝胆外科杂志

Objective To study the clinicopathological features of primary and metastatic hepatic neuroendocrine carcinoma.Methods The records of 35 patients with primary hepatic neuroendocrine carcinoma and 35 patients with metastatic hepatic neuroendocrine carcinoma were retrospectively reviewed.These patients served as the primary group(priNET,n=35)and the metastasis group (metNET,n=35),respectively.Results There were significant differences between the two groups of patients in gender,site,size and number of tumor(P＜0.05).Although there was no significant difference between the two groups in the distribution of the tumors in the two lobes of liver (P＞0.05),priNET had more tumors localized to one lobe of liver while metNET had more tumors involving both lobes of liver(P＜0.05).Conclusions Gender,size,site and number of tumor may play an important role in the differentiation of primary or metastatic hepatic neuroendocrine tumor.

Chondroblastoma in the long bone diaphysis: a report of two cases with literature review / 癌症

To investigate the clinical characteristics of chondroblastoma with an emphasis on lesions located in the long bone diaphysis, we reviewed the clinical data of 7 patients with histologically proven chondroblastoma treated in Tianjin Medical University Cancer Hospital and Fudan University Cancer Hospital between January 1995 and May 2009. There were two rare cases of chondroblastoma in the long bone diaphysis. One patient with a lesion in the tibial diaphysis underwent intralesional curettage and bone grafting, and the postoperative bone function was measured as excellent according to the Enneking scoring system. The patient was still alive upon follow-up at 60 months. The other patient with a lesion in the humeral diaphysis underwent resection, and the postoperative bone function was excellent at 48 months, at which there was no evidence of recurrence or metastasis. Thus, except for the distinctive site of the long bone diaphysis, which made diagnosis difficult, the patients' ages, symptoms, X-ray and CT images, treatment, and prognosis were in accordance with typical lesions in the epiphysis and metaphysis. The diagnosis of chondroblastoma in the long bone diaphysis significantly depends on histopathologic characteristics.

Primary Hodgkin disease of the supermaxilla: a case report and review of the literatures / 白血病·淋巴瘤

Objective To intensify the diagnosis and treatment of primary osseous Hodgkin disease (HD) and reinforce the impression of its features of pathology and imaging. Methods The clinical manifestation, laboratory examination, treatment and outcome of a patient with primary Hodgkin disease of the supermaxilla were first reported and the pertinent literatures were reviewed. Results Pain of the right supermaxilla was the first clinicM symptom. Plain X-rays showed mixed osteolytic and partially osteosclerotie lesions in the right supermaxiUa. The tumor was removed and the pathohistology was HD lymphocyte-depletion. The clinical diagnosis was primary HD of the supermaxilla (Stage Ⅰ ). The case was treated with ABVD regimen and no obviously adverse reaction appeared. Conclusion Primary osseous HD rarely presents as a malignant bone lymphoma and is easily misdiagnoed. Pathological and immunohistologic studies can be useful to confirm the diagnosis of primary osseous HD. Early diagnosis and the differentiation from other disease should be performed and the prognosis of the present-day chemotherapy regimen appears good.

Advances in research on extranodal NK/T cell lymphoma(nasal type) / 中国癌症杂志

Extranodal NK/T cell lymphoma(nasal type) is a common,high malignant degree and poor prognosis entity of the nasal lymphoma.It is the most important to understand its genesis,development,clinical manifestation,pathologic diagnosis,therapy and prognosis.

Molecular and biologic study of gastrointestinal stromal tumors / 肿瘤研究与临床

Gastrointestinal stromal tumors is a new kind of tumor known recently, especially the important progress in the molecular and biologic study of GIST were used quickly in the clinical diagnosis and treatment, and made a close combination of basic research and clinical applications. The study and application of target therapy of new molecules on the mechanism of molecular genetics of GIST was another successful example. Therefore, GIST was paid extensive attention by the scholars. The progress on the molecular and biological study was summarized.

Study on the clinical pathology of gastrointestinal stromal tumors / 肿瘤研究与临床

The content of gastrointestinal stromal tumors had changed many times since the appearance of this idea. Along with the progress of molecular and biological study in recent years, it was a kind of tumor with specific clinical, pathological and molecular genetic characteristics, and was different from other specific tumors originated from the smooth muscle or nerves. Along with the success of targeted therapy of GIST, the disease was paid more attention. Therefore, a review of the clinical pathological study of GIST was needed.

Reversal of nomegestrol acetate on multidrug resistance in drug-resistant human breast cancer cell line MCF7/ADR / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To study the reversal effect of nomegestrol acetate (NOM) on mutidrug resistance (MDR) in MCF7/ADR and its mechanism.</p><p><b>METHODS</b>Using tetrazolium dye assay, effects of various concentrations of NOM on sensitivity to ADR in MCF7/ADR was studied. Expression of MDR related genes MDR1, glutathoine S-transferase Pi (GSTpi), Topoisomerase II alpha (Topo II alpha) and MDR related protein (MRP) were assayed by reverse transcription-polymerase chain reaction (RT-PCR) and immunocytochemistry assay. Using flow cytometry (FCM), intracellular ADR concentration effects on cell cycle were observed.</p><p><b>RESULTS</b>NOM significantly reversed MDR in MCF7/ADR. After NOM 20, 10 and 5 micromol/L treatment, the chemosensitivity to ADR increased to 21, 12 and 8 times. The reversal activity of NOM was stronger than that of the precursor compound megestrol acetate, and was comparable to that of verapamail. After treatment with NOM 5 micromol/L both MDR1 and GSTpi mRNA genes expression began to decline on D2 (P < 0.05, & P < 0.01) and reached the lowest level on D3 (both P < 0.01), but the expression levels began to rise on D6 again (both P < 0.05). The expression of MRP and Topo II alpha gave no significant change. Changes of P-gp and GSTpi protein expressions were similar to those of their mRNA expressions, showing early decline and late rise. Two hours after NOM 20, 10, and 5 micromol/L treatment, intracellular ADR concentration increased 2.7, 2.3 and 1.5 times, respectively. FCM data showed that after forty-eight hours, combined administration of NOM (20 micromol/L) and ADR (from low concentration to high concentration), MCF7/ADR cells showed gradual arrest in the G(2)M phase with the increase of ADR dose.</p><p><b>CONCLUSION</b>NOM has strong reversal effects on MDR in MCF7/ADR. The reversal takes place via different routes, i.e. down regulating mRNA and protein expression levels of MDR1 and GSTpi, increasing intracellular drug concentration, and enhancing the arrest of ADR in cells at G(2)M phase.</p>

Detection of SYT-SSX fusion transcripts in paraffin-embedded tissues of synovial sarcoma by reverse transcription-polymerase chain reaction / 中华医学杂志(英文版)

<p><b>OBJECTIVE</b>To assess the feasibility of detecting SYT-SSX fusion transcripts in paraffin-embedded tissues of synovial sarcoma by reverse transcription-polymerase chain reaction (RT-PCR).</p><p><b>METHODS</b>RT-PCR was used to amplify the SYT-SSX fusion transcripts using archival formalin-fixed paraffin-embedded tumor specimens from a series of 37 synovial sarcoma cases. To investigate the specificity of the SYT-SSX fusion transcripts, a variety of non-synovial sarcoma tumors were included in the study as negative controls. The detected messages derived from fusion genes were confirmed by subsequent sequence analysis.</p><p><b>RESULTS</b>SYT-SSX fusion transcripts were detected in 33 of 37 (89.2%) synovial sarcomas. None of the 34 cases of non-synovial sarcoma tumors showed amplified products of SYT-SSX fusion transcripts, although PBGD mRNA was detected in all specimens. Among 33 SYT-SSX-positive synovial sarcomas, 22 tumors had an SYT-SSX 1 fusion transcript, whereas 6 tumors had an SYT-SSX2 fusion transcript. Fusion types can not be distinguished in the remaining 5 cases. There was a significant relationship between SYT-SSX fusion type and histologic subtype. All 10 biphasic synovial sarcomas had the SYT-SSX1 fusion, whereas all tumors with SYT-SSX2 were of monophasic morphology (P < 0.05).</p><p><b>CONCLUSIONS</b>RT-PCR can be applied to archival formalin-fixed paraffin-embedded tumor tissues as a sensitive and reliable technique for the diagnosis and differential diagnosis of synovial sarcoma. There is an association between SYT-SSX fusion type and histological subtype. SYT-SSX2 fusion transcripts can only be found in monophasic synovial sarcoma.</p>

A clinicopathologic and immunohistochemical study on 76 cases of gastrointestinal stromal tumors / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the morphologic and immunohistochemical features of gastrointestinal stromal tumors (GISTs) and to explore the reference parameters for malignancy.</p><p><b>METHODS</b>Seventy six (76) cases of primary GISTs were distinguished from a group of gastrointestinal mesenchymal tumors by use of a panel of antibodies such as CD117, CD34 by immunohistochemical EnVision method, their biologic behaviors were analyzed by including their follow-up data.</p><p><b>RESULTS</b>All patients were adults, age range 32 to 81 years (mean 54 year), male 39 cases and female 37 cases; the tumors were situated in stomach (36 cases), in small intestine (23 cases), colon (2 cases) and rectum (15 cases). The most common symptoms were abdomen mass, vague pain and GI bleeding. Forty eight (48) cases were mainly located within the muscularis propria, 25 cases outside the serosa, and 3 cases below the mucosa. Grossly, they were of soft consistency often with hemorrhage, cystification or necrosis. Microscopically, the tumors were composed of spindle cells (46 cases) or epithelioid cells (9 cases) and of both cells (21 cases), arranged in interlacing fasicles, diffusing sheets, pallisading, whirling, alveolar and giant pseudo-rosette shapes. Tumor cells often had abundant cytoplasm with light to moderate eosinophilic or slight basophilic in staining, the nuclei generally showed spindle, blunted ends, round or signet in shape with nucleoli. Immunohistochemically, CD117 and CD34 showed diffuse strong expression, the positive rates were 98.7% and 68.4% respectively, alpha-SMA, MSA, S-100, PGP9.5 showed focal expression, the positive rates were 25.0%, 19.7%, 23.7% and 17.1% respectively, vimentin were all positive and desmin, GFAP, NF were all negative. Nine cases were benign, 19 cases borderline and 48 cases malignant. Follow-up of 20 cases with benign and borderline tumors found patients alive without tumor. In the malignant group of 34 cases, 10 cases were alive without tumor, 10 cases developed recurrence or metastasis, and 14 cases died of tumor. Coagulative necrosis, mitotic activity over 10/50HPF, high cellularity and obvious pleomorphism were all in the malignant group. In this group, tumor necrosis, adhesion in operation, tumor, over 5 cm in diameter, mitotic activity over 5/50HPF had significant differences among three groups and the 3 years survival rate had a significant difference in tumors with or without coagulative necrosis and also in tumors with or without mitotic activity over 5/50HPF.</p><p><b>CONCLUSIONS</b>GISTs predominantly occurred in middle aged or old patients, the tumors had varied cell types and different arrangements, the immunohistochemical characters were positive for CD117 and CD34, negative for desmin, occasional positive for alpha-SMA, MSA, S-100 and PGP9.5, which were helpful to differentiate GIST from leiomyomas and Schwannomas. Coagulative necrosis, mitotic activity over 10/50HPF, high cellularity with obvious pleomorphism were also helpful parameters for diagnosis of malignancy aside from metastasis and invasion. Adhesion, over 5 cm in diameter and mitotic activity over 5/50HPF but less than 10/50HPF might be the potential malignant parameters.</p>

Giant cell fibroblastoma: a clinicopathologic analysis of seven cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinical, pathological and immunohistochemical features of giant cell fibroblastoma (GCF), with emphasis on its differential diagnosis and histogenesis.</p><p><b>METHODS</b>Seven cases of GCF were investigated by light microscopy and immunohistochemistry.</p><p><b>RESULTS</b>Six cases occurred in children, and one occurred in a 35 year-old adult (mean 9.4 years). Five were male and two were female. Clinically, all cases appeared as slowly growing painless nodules located in the dermis or subcutis of the trunk and extremities. Microscopically, the poorly circumscribed tumor was composed of a proliferation of slightly to moderately atypical spindle cells which were arranged in parallel or wavy fascicles, and embedded in a fibromyxoid to collagenous background. The pathognomonic feature consisted of irregular distributed cleft-like or sinusoid-like pseudovascular spaces lined with a discontinuous layer of pleomorphic spindle cells and multinucleate giant cells. There was transition in shape between these two cells. Immunohistochemially, both cells expressed vimentin and CD34. Follow-up information in five cases showed local recurrences in two cases.</p><p><b>CONCLUSIONS</b>(1) GCF is a distinctive fibroblastic tumor of intermediate malignancy that occurs predominantly in children. Recognizing its clinical and pathological characteristics is important to avoid misdiagnosis with other lesions with similar features. (2) GCF shared clinical, immunohistochemical and cytogenetic features with its adult counterpart-dermatofibrosarcoma protuberans (DFSP). The additional coexistence of GCF and DFSP areas in some primary cases and the reciprocal transformation in recurrent tumors all suggest that they are two closely related entities, possibly representing two members of the CD34 positive dendritic neoplasms.</p>

A clinicopathologic study of CD30-positive sinusoidal large B-cell lymphoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To explore the clinicopathologic features of CD30-positive sinusoidal large B-cell lymphoma (CD30 + SLBCL) and its relative correlation with Epstein-Barr virus (EBV).</p><p><b>METHODS</b>Two cases of CD30 + SLBCL, a 65-year-old men and a 85-year-old women were morphologically and immunophenotypically analyzed. EBV status was also evaluated through not only the polymerase chain reaction (PCR) amplification to the EBV Bam HIW DNA sequence, but also an immunohistochemical detection of the latent membrane protein 1 (LMP1).</p><p><b>RESULTS</b>The patients presented with similarly superficial lymphadenopathy. One of them died of the tumor within 10 months. Microscopically, both of the neoplasms were characterized by a cohesive sinus growth pattern and the monomorphic cytology of the tumor cells. Immunohistochemically, They were both positive for CD45, CD30, and CD20 or CD79alpha, whereas neither expressed EMA, ALK1, nor any histiocytic/T-lineage markers. No evidence of EBV-infection could be found either.</p><p><b>CONCLUSIONS</b>CD30 + SLBCL is a morphologically and immunophenotypically distinctive variant of diffuse large B-cell lymphoma, which should be distinguished from T/null cell type anaplastic large cell lymphoma and some other nodal lesions with a predominantly sinusoidal infiltrative pattern. CD30 + SLBCL may not be correlation with EBV.</p>

A clinicopathologic and immunohistochemical study of diffuse large B-cell lymphoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic and immunohistochemical features of diffuse large B-cell lymphoma (DLBCL) and the significance of immunohistochemistry in diagnosis and differential diagnosis of DLBCL.</p><p><b>METHODS</b>60 cases of DLBCL were studied and immunohistochemical staining for LCA, L26, BLA-36, CD30, bcl-6 were carried out with the EnVision 2 step method.</p><p><b>RESULTS</b>The age range of 76.7% (46/60) patients was 40 - 70 years. The location of the lesion includes nodal and extranodal sites. 90.0% (54/60) were in clinical stages of II (24/54), III (21/54), IV (9/54). Histopathologic morphology presented as centroblastic (88.3%, 53/60), immunoblastic (3.3%, 2/60), anaplastic large B cell type (3.3%, 2/60) and T cell rich B cell type (5.0%, 3/60). Immunostaining showed 100% (60/60) DLBCL were positive for LCA, L26, BLA36, 3.3% (2/60) DLBCL positive for CD30, 95% (57/60) expressed bcl-6 protein.</p><p><b>CONCLUSIONS</b>DLBCL is an aggressive lymphoma which shows cytologic variability from case to case. The evaluation of pathologic features and immunohistochemistry in DLBCL are useful and practical for diagnostic purposes, but cannot delineate distinctive morphologic subtypes.</p>

A comparative study of esophageal stromal tumors and smooth muscle tumors / 中华病理学杂志

<p><b>OBJECTIVE</b>To explore the clinicopathological, immunohistochemical, and molecular biologic characteristics of esophageal stromal tumors and smooth muscle tumors.</p><p><b>METHODS</b>Twenty four cases of esophageal mesenchymal tumors were reclassified by a panel of antibodies such as CD117, CD34 etc. The sequence of 11 exon of c-kit gene were detected in some cases.</p><p><b>RESULTS</b>There were 3 cases of esophageal stromal tumors, 20 leiomyomas, and 1 leiomyosarcoma. The 3 esophageal stromal tumors occurred in 3 men aged 71, 56 and 60 years respectively. The tumors originated from muscularis propria with the size of 4 cm, 8 cm and 14 cm in diameter. Microscopically, the tumor cells were spindle and epithelioid shaped with slightly basophilic appearance, arranged in intersecting fascicles, diffusing and palisading patterns. Immunohistochemically, the tumors were positive for CD117 and CD34. The mutation of 11 exon of c-kit gene was detected in one case. In comparison, esophageal leiomyomas occurred in a younger population. The age ranged from 30 to 60 years (mean age 41.6 years), 12 male cases, 8 female cases. 15 cases of esophageal leiomyomas were intramural tumors with a diameter of 0.8 - 10.5 cm (mean 4.5 cm) originating from muscularis propria and 5 cases which were intraluminal polyps with a diameter of 0.2 - 1.0 cm originating from muscularis mucosae. Leiomyomas were strongly eosinophilic in appearance, diffuse positivity for alpha-SMA, MSA, and desmin, and no c-kit gene mutation. One male case of leiomyosarcoma had a diameter of 5 cm and originated from muscularis mucosae and displayed a sausage-shaped polyp.</p><p><b>CONCLUSIONS</b>Leiomyoma is still the most common mesenchymal tumor of the esophagus, the stromal tumor can be similar to gastrointestinal stromal tumors. Typical esophageal leiomyosarcoma is very rare and has different clinicopathologic and molecular biologic features.</p>

Diagnosis and differential diagnosis of nodular lymphocyte-predominant Hodgkin's lymphoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the diagnosis and the differential diagnosis of nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHL).</p><p><b>METHODS</b>245 cases of Hodgkin's lymphoma (HL) diagnosed between 1980 and 2000 from 3 hospitals in Guangzhou were reviewed. Four cases of NLPHL were confirmed according to the WHO classification of lymphoid neoplasms. Among the other 3 cases of NLPHL, 2 collected from other clinical centers and 1 from Fudan University Cancer Hospital. Immunohistochemistry (IHC) were performed on paraffin sections through SP technique using a panel of markers to define the large neoplastic cells (CD45, CD20, CD15, CD30 and vimentin) as well as the non-neoplastic background cells (CD3, CD20, CD45RO, CD57, CD68 and TIA-1).</p><p><b>RESULTS</b>Seven patients with NLPHL were 4 males and 3 females, age 29 to 70 years, average 43.8 years. All patients had lymphadenopathy. Histologically, in NLPHL, instead of the structure of normal lymph nodes, the tumor tissue became nodular in architecture. Characteristic lymphocytic and histiocytic (L&H) cells with scant cytoplasm and large multilobulated nuclei distributed among a predominant population of small lymphoid cells. The large cells exhibited a CD45+, CD20+, but CD15-, CD30- and vimentin-phenotype. The background cellularity was relatively rich in B cells and the majority of T-cells infiltrated were CD57(+) cells. TIA-1+ cells were few.</p><p><b>CONCLUSIONS</b>NLPHL can be diagnosed according to the morphologic and immunophenotypic features rather than by morphology alone. It is important to distinguish this tumor from its morphologic mimics, such as lymphocyte-rich classical Hodgkin's lymphoma (LRCHL) and T-cell rich B-cell lymphoma (TCRBCL). The immunophenotype of neoplastic cells and background cells are the helpful criteria for the differential diagnosis.</p>

Sequence analysis of translocation t (X; 18) genomic breakpoints characterized in synovial sarcoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To analyze the DNA sequence characteristics of translocation t (X; 18) genomic breakpoints and to study the mechanism underlying chromosomal translocation t (X; 18) in synovial sarcoma.</p><p><b>METHODS</b>Two cases of synovial sarcoma were studied utilizing long-distance polymerase chain reaction (PCR) and sequence analysis to amplify the genomic DNA of translocation t (X; 18) breakpoints.</p><p><b>RESULTS</b>Translocation t (X; 18) was detected in both cases, which generated SYT-SSX1 and SYT-SSX2 fusion gene respectively. Sequence analysis revealed that intron 10 of SYT was fused to the intron 4 of SSX1 or SSX2. Sequences highly homologous to consensus recognition motifs of translin were found adjacent to breakpoints in all three genes. Breakpoints in the three genes were close to or even at several palindromic oligomer sequences. The breaks in intron 4 of SSX1 and SSX2 were near an Alu sequence. No Alu or other repetitive sequences were found 500 bp upstream or downstream from the break in intron 10 of SYT. One topoisomerase II consensus site was found between the two breakpoints but with considerable distance from intron 10 of SYT.</p><p><b>CONCLUSIONS</b>All three genes involved in synovial sarcomas contain characteristic sequence motifs in the breakpoint regions which may play an important role in the genesis of chromosomal translocation in synovial sarcoma.</p>