Humans ; Lymphoma ; Tumor Microenvironment

Objective To analyze the clinical features of Weber-Christian disease (WCD) and to make a review of the literature for early diagnosis and treatment.Methods The clinical features of an atypical WCD patient who had been misdiagnosed as polymyositis were analyzed.Results WCD was characterized by subcutaneous nodules and systemic symptoms.Repeating physical examination and biopsy in time were important if the nodules were not obvious.Conclusion WCD is often misdiagnosed because of the complicated clinical manifestations.Carefully physical examination and timely biopsy are help for early diagnosis.

Objective To explore the clinical,endoscopic,pathologic and prognostic characteristics of primary gastric lymphoma (PGL) and to improve the level of diagnosis and treatment.Methods Sixtythree patients who were confirmed as PGL with operation and endoscopic biopsy pathology during January 2001 to December 2010 were retrospectively analyzed with respects of clinical,endoscopic and pathologic features.Survival analysis and prognosis were evaluated by kaplan-Meier and Cox proportional hazard model,respectively.Results In 63 PGL patients,the numbers of male and female were 40 and 23,respectively,and the average age was (59.8±13.3)years.The major symptoms were abdominal pain,abdominal distension,and gastrointestinal hemorrhage,accounting for 47.6 ％ (30/63),17.5 ％ (11/63),and 17.5 ％ (11/63),respectively.There were 39 (61.9 ％) PGL patients with endoscopic performance for ulcers,34 (54.0 ％) cases involved the gastric stomach antrum.The most immunohistochemistry analyses were diffuse large B-cell lymphoma (DLBCL) (71.4 ％,45/63),followed by mucosa-associated lymphoid tissue (MALT) lymphoma (22.2％,14/63).The frequency of Helicobacter pylori (H.pylori) positivity was lower in patients with DLBCL than that in patients with MALT lymphoma (37.8％(17/45) vs 10/14,x2 =4.872,P=0.027).The accumulate survival rates of one,three and five years were 74.6％,63.5％,55.6％,respectively,and the average survival time was (41.5±3.0) months (95％ confidence interval (CI) 35.7 to 47.4 months) in PGL patients.There was no difference in the average survival time between DLBCL patients treated with surgery combined chemotherapy and those with surgery or chemotherapy alone (38.33±5.21) months vs (50.17±8.98) months vs (41.39±4.40) months,P＞0.05).The patients diagnosed as DLBCL with H.pylori positive had longer average survival time than those with H.pylori negative ((51.90±4.30) months vs (33.30±4.50) months,t=-4.004,P＜0.01).Conclusions Male patients with PGL are slightly more than female.Abdominal pain is the most frequent symptom.Ulcerative lesions are the most common endoscopic demonstrations mostly at stomach sinus.DLBCL is the most pathologic characteristic.There is no significant difference in the survival rate between patients treated with surgery combined with chemotherapy and those treated with surgery or chemotherapy alone.

Purpose To study the clinicopathologic features, diagnosis and differential diagnosis of extranodal NK/T-cell lymphoma, nasal type ( EN-NK/TCL) . Methods Twenty-eight cases of EN-NK/TCL were analyzed according to WHO classification of tumors of haematopoietic and lymphoid tissues, the cases were studied by microscopy, immunohistochemistry and in situ hybridization. Results In 28 cases, the male to female ratio was 1 ∶ 1. 2. The mean age was 46 years, with the median of 44. 5. Eighteen cases involved nasal cavity, 5 cases of skin, and 1 case of tonsil, upper palate, root of tongue, adrenal glands and gastric, respectively. The main presentation was nasal obstruction in cases with nasal involvement, and the cases with skin involvement present with rash, ulcer, plaque or nodular mass. Patients might be accompanied by B-type symptoms, lymphoadenopathy or with multiple sites involvement. The tumor cells were small, medium, large-sized, or mixed, and angiocentric and angiodestructive growth pattern was frequently pres-ent. Most cases were mixed with inflammatory cells infiltration. The typical immunophenotype was T-cell ( the most sensitive, CD3ε) , CD56 and cytotoxic markers. In situ hybridization for EBER was positive. Ten cases were followed up, and 1 case died. Conclusions The most common site of EN-NK/TCL is nasal cavity, followed by skin. EN-NK/TCL can be definitely diagnosed by integration of pathological morphology, immunophenotype and in situ hybridization of EBER.

Epstein-Barr Virus Infections ; virology ; Herpesvirus 4, Human ; Humans ; Lymphoma, Large B-Cell, Diffuse ; pathology ; virology

Breast ; Breast Neoplasms ; pathology ; Carcinoma, Papillary ; pathology ; Female ; Humans

Objective To study the clinicopathological features of sarcomatoid hepatocellular carcinoma (SHC).Methods The clinical data of 42 patients with SHC who underwent surgical resection in the Peking University People's Hospital (n =33) and the Department of Pathology of the Peking University Health Science Center (n=9) from January 2008 to May 2017 were retrospectively analyzed.Results The average age was 58.3 (aged 32～84) years;the ratio of male to female was 2.2 ∶ 1;the average diameter of the lesions was 8.2 cm;the median AFP value was 45.2 ng/ml.The median survival time was 10.5 months,the average progression-free survival time was 2.9 months,and the 5-year survival rate was 25.0％.On histopathology,the tumor consisted of various degrees of different differentiated carcinomas with aligned sarcomatoid spindle cells.Immunohistochemical results in the sarcomatoid region expressed both mesenchymal markers and epithelial-derived markers.Conclusions SHC tumors were highly aggressive,with high rates of lymph node metastasis and poor prognosis.The diagnosis of SHC mainly depended on histopathology.Immunohistochemistry was very important for its diagnosis and differential diagnosis.Surgical resection was the treatment modality of choice to achieve prolonged survival time.

Objective:To study the clinicopathological features of metaplastic breast cancer (MBC) , and its prognostic factors.Methods:Data of 49 MBC cases and 30 cases of invasive ductal carcinoma (IDC) during the same period as matched control were collected. The immunohistochemistry staining of CK5/6, CK, P63, ER, HER2, Ki67 was performed in all MBC samples.Results:In MBC cases, the median age was 55 years. Median tumor size was 2.5 cm (range, 0.6-19 cm). Fifteen cases were classified as metaplastic carcinoma with heterologous mesenchymal differentiation (8 as matrix-producing carcinoma), 12 as spindle cell carcinoma, 7 as squamous cell carcinoma, 2 as low-grade adenosquamous carcinoma, 2 as fibromatosis-like metaplastic carcinoma, and 11 as mixed metaplastic carcinoma. The 5-, 10-year overall survival rate was 50%, 41%, respectively, lower than those of IDC (76%,63%) (all P<0.05). Lymph node metastasis rate, and expression of ER, PR, HER2 in MBC were lower than those in IDC (all P<0.05). Triple-negative cases in MBC were more than those of IDC ( χ2=26.244, P=0.000). The proliferative index of Ki67 was statistically different between the two groups ( t=2.624, P=0.011). Conclusions:MBC is a rare and heterogenous breast cancer. Compared to IDC, MBCs are usually larger, lower in lymph nodes metastasis, higher in proliferative index of Ki67, more triple-negative, hence with a poorer prognosis.

Objective: To study the clinicopathologic features, diagnosis and differential diagnosis of the tumors of lymphoidand hematopoietic tissue of the spleen(TLTS). Methods: Fifty-three cases of TLTS were selected from the pathologic files from Peking University People′s Hospital from April 2002 to April 2017. According to WHO classification of tumors of hematopoietic and lymphoid tissues (2008) and its updated classification (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination. Results: In 53 cases of TLTS, the male to female ratio was 3.4∶1.0; the mean age was 55.4 years (range 21-76 years), and all patients presented with variable degree of splenomegaly. Laboratory examination showed increased percentage of lymphocyte in peripheral blood in 22 cases, and elevated serum LDH level in 24 cases. Abnormal blood counts were seen in 26 cases pre-operatively, in which 22 cases showed complete or partial correction of these abnormalities post-operatively (84.6%, 22/26). The clinical symptoms included abdominal pain or distension, fatigue, fever, and weight loss, etc. Seventeen cases presented with lymphadenopathy of abdomen or other sites. Bone marrow biopsy was performed in 30 cases, and 19 cases were involved by tumor (63.3%). Of all 53 cases, 43 were diagnosed as primary splenic lymphoma (PSL), and the remaining 10 cases as secondary TLTS. According to Ann Arbor staging, 14 cases were stages Ⅰ or Ⅱ, 6 were stage Ⅲ and 28 were stage Ⅳ. By histopathologic classification, 43 cases of PSL were splenic B-cell marginal zone lymphoma (SMZL; 48.8%, 21/43), diffuse large B cell lymphoma (DLBCL; 23.3%, 10/43), splenic diffuse red pulp small B-cell lymphoma (11.6%, 5/43), mantle cell lymphoma (9.3%, 4/43), follicular lymphoma (4.7%, 2/43), and composite lymphoma (CL, DLBCL and classical Hodgkin lymphoma; 2.3%, 1/43). The remaining 10 cases were chronic lymphocytic leukaemia/small lymphocytic lymphoma (4 cases), hairy cell leukaemia (1 case), hepatosplenic T-cell lymphoma (HSTL; 5 cases), with lesions in other sites. Of the 53 cases of TLTS, 47 were B cell neoplasm (88.7%, 47/53), and the T cell neoplasms were all HSTL(5 cases, 9.4%, 5/53), 1 case was composite lymphoma. In 11 cases of TLTS, EBER in situ hybridization was performed and all cases were negative. Forty eight cases had follow-up data, and the median survival period was 17.0 months(range: 1-96 months). The survival of patients with SMZL and DLBCL were 25.7 and 18.6 months respectively. Thirteen patients died (27.1%, 13/48). The prognosis of those with elevated LDH level, high clinical stage, B symptoms and older than 60 years of age was worse. And the prognosis of DLBCL was worse than that of SMZL. There was no statistically significant difference between these factors and prognosis (P>0.05). Conclusions Most TLTS cases present with splenomegaly and abnormal blood counts, and complete or partial remission of blood counts isseen after splenectomy. The most common pathologic types of TLTS are SMZL and DLBCL. Definite diagnosis of TLTS could be made by combining clinical features, histopathology, immunophenotype, genetics, bone marrow biopsy and laboratory examination.

Objective To study the clinicopathologic features,diagnosis and differential diagnosis of tumors of haematopoietic and lymphoid tissue in the female productive tract. Methods Eleven cases of myeloid sarcoma and leukemia, 9 of non Hodgkin lymphoma(NHL),13 of cervical lymphoma-like lesions were selected from Peking University People′s Hospital from January 2006 to August 2017. According to WHO classification of tumors of haematopoietic and lymphoid tissues(2008)and updated classification(2016),the cases were studied by microscopy,immunohistochemistry and in situ hybridization.Results In 20 cases of tumors of haematopoietic and lymphoid tissue,the mean and median age was 48.5 and 56 years old(range:16-77 years old).In cases of lymphoma-like lesion of uterine cervix,the mean and median age was 45.9 and 48 years old(range:23-62 years old).The patients with neoplasm present as fever,fatigue, hypogastralgia, colporrhagia and mass etc. Eight cases had history of acute myeloid leukemia, and 3 had myeloid leukemia while pregnancy. One case of chronic lymphocytic leukaemia/small lymphocytic lymphoma(CLL/SLL)had history of ovary small cell carcinoma and high grade serous carcinoma resected with chemotherapy.One case of diffuse large B cell lymphoma(DLBCL)had history of renal transplantation. Lactic dehydrogenase(LDH)was elevated in 9 cases(9/18).The cases of lymphoma-like lesion present as contact bleeding in most cases and all located in cervix. Four cases of neoplasm located in vulva, 1 in vagina,4 in cervix, 4 in uterine corpus, 8 in ovary and 2 in placenta.Clinical staging of NHL: 4 case was stageⅠ,1 case of stageⅢ,and 4 cases of stageⅣ.Pathological morphology:9 cases were myeloid sarcoma, 2 cases were placenta invaded by myeloid leukemia. Six cases were DLBCL, and 1 case was CLL/SLL, 1 case was mucosa associated lymphoid tissuse lymphoma(MALToma), and 1 case was anaplastic large cell lymphoma. Resected mass, chemotherapy was performed in tumors of haematopoietic and lymphoid tissue. Five cases of myeloid sarcoma and 2 of NHL died. In 13 cases of lymphoma-like lesion of uterine cervix, the general condition was good as following up. Conclusions The clinical history, pathological morphology and immunohistochemistry are very important for diagnosing tumors of haematopoietic and lymphoid tissue in the female productive tract.Resection with chemotherapy is recommended in treatment. The prognosis of lymphoma-like lesion of uterine cervix is good,and should be differentiated from lymphoma.