OBJECTIVETo investigate the clinical and pathological characteristics of patients with primary Waldeyer's ring lymphomas (PWRL), and to analyze its therapeutic efficacy and prognostic factors.

METHODSA total of 112 patients with PWRL confirmed by pathological and immunohistochemical methods between January 2009 and January 2014 were studied. Clinical data were collected and analyzed retrospectively.

RESULTSPWRL accounted for 3.9% of lymphoma over the same period. Median age of patients with PWRL was 51.5 years old. The affected areas were tonsil, nasopharynx, tongue base and oropharynx, which accounted for 63.4% (71/112), 22.3% (25/112), 5.3% (6/112) and 4.5% (5/112) respectively. The most common pathological types of these four areas were diffused large B-cell lymphoma (DLBCL) and extranodal NK/T cell lymphoma (NKTCL) which accounted for 58% and 15.2%. The overall response rate (CR/CRu = 51.4%; PR = 30.8%) in all patients was 82.2%, the estimated 5-year overall survival (OS) rate were 71.6%. The 5-year OS rate were 94.7% in the group used Rituximab. Meanwhile, chemotherapy combined with radiotherapy could improve the outcome of T-cell PWRL patients and the 5-year OS rate were 88.9%. Age, disease stages, pathological types, IPI scores, LDH level, β2-MG level and the efficacy of initial therapy were prognostic factors with statistical significance. Cox multivariate analysis showed that age of more than 60 years, LDH level, pathological types and the efficacy of the initial therapy were independently associated with OS.

CONCLUSIONPWRL has a relatively good prognosis. The pathological types affect the prognosis directly and guide treatment. Combined modality therapy should be chosen for patients with PWRL. Patients with T-cell PWRL should accept chemotherapy combined with radiotherapy, while rituximab may be better for B-cell PWRL. The efficacy of initial therapy is crucial for the outcome of patients. Age and LDH level are also important prognostic factors.

Combined Modality Therapy ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; pathology ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; pathology ; Middle Aged ; Multivariate Analysis ; Prognosis ; Retrospective Studies ; Rituximab ; Survival Rate

No abstract available.
Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Biopsy ; Chemoradiotherapy, Adjuvant ; Diagnostic Errors ; Dupuytren Contracture/diagnosis ; *Fingers/pathology/ultrasonography/virology ; Hematopoietic Stem Cell Transplantation ; Herpesvirus 4, Human/genetics ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; *Lymphoma, Extranodal NK-T-Cell/diagnosis/therapy/virology ; Male ; Middle Aged ; Neoadjuvant Therapy ; Predictive Value of Tests ; RNA, Viral/genetics ; *Tendons/chemistry/pathology/ultrasonography/virology ; Treatment Outcome ; Tumor Markers, Biological/analysis ; Ultrasonography, Doppler, Color

Adult ; CD56 Antigen ; metabolism ; Diagnosis, Differential ; Granzymes ; metabolism ; Humans ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; surgery ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; Male ; Pectoralis Muscles ; pathology ; surgery ; RNA, Viral ; metabolism ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Thoracic Wall ; pathology ; surgery

A 68-year-old Chinese man presented with an eight-month history of pyrexia of unknown origin and chronic sinusitis despite multiple courses of antibiotics. He underwent extensive investigations, including workups for infections, chronic granulomatous diseases and malignancy. Nasal biopsies were performed twice under local anaesthesia, but did not show any evidence of malignancy. Eventually, the patient was diagnosed with natural killer (NK)/T-cell lymphoma, nasal variant, based on histopathological findings from harvested deep tissue obtained via functional endoscopic sinus surgery. This study highlights that, for patients presenting with pyrexia of unknown origin and nasal symptoms, NK/T-cell lymphoma must be considered as a differential diagnosis. Generous amounts of tissue should be harvested under general anaesthesia rather than limited tissue under local anaesthesia, in order to facilitate and ensure a definitive diagnosis.
Aged ; Anti-Bacterial Agents ; chemistry ; Biopsy ; China ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; diagnostic imaging ; pathology ; Male ; Nasal Cavity ; pathology ; Nose Neoplasms ; complications ; diagnosis ; radiotherapy ; Prognosis ; Radiography ; Radiotherapy ; Sinusitis ; complications ; diagnosis ; Treatment Outcome

OBJECTIVETo study the clinicopathologic features, diagnosis and differential diagnosis of intestinal natural killer (NK)/T-cell lymphoma.

METHODSThe clinical features, histopathology, immunohistochemical findings and follow-up data of 14 cases of intestinal NK/T-cell lymphoma were retrospectively reviewed.

RESULTSThe male-to-female ratio was 9:5. The medium age of patients was 45 years. The sites of involvement included small intestine (6 cases), colon (6 cases) or both (2 cases). The main clinical manifestations were an abdominal mass, other gastrointestinal symptoms such as abdominal pain, as well as systemic symptoms such as fever and cachexia. Intestinal perforation complicated by acute peritonitis might occur in advanced disease. Histologically, the intestinal wall showed full-thickness infiltration by medium-sized atypical lymphoid cells with pleomorphic nuclei, prominent inflammatory background, angiocentric/angiodestructive growth pattern and coagulative necrosis. Immunohistochemical study showed that the tumor cells were positive for CD3ε, CD43, CD56, granzyme B and perforin. They were negative for CD20, CD79α and MPO. In-situ hybridization for Epstein-Barr virus encoded RNA (EBER) showed negative signals. A high proliferative index was demonstrated by Ki-67 immunostaining. Follow-up data of 8 cases were available, with duration of follow up ranging from 0.5 to 36 months. Five patients died within 20 months.

CONCLUSIONSExtranodal NK/T-cell lymphoma, nasal-type primarily involving intestine is rare and tends to carry an aggressive clinical course. The relatively non-specific clinical manifestations of intestinal NK/T-cell lymphoma may result in misdiagnosis in some cases. A comprehensive evaluation of clinical manifestations, pathologic features and immunohistochemical findings is essential for definitive diagnosis.

Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; CD3 Complex ; metabolism ; CD56 Antigen ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Granzymes ; metabolism ; Humans ; Intestinal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Intestines ; pathology ; Ki-67 Antigen ; metabolism ; Leukosialin ; metabolism ; Lymphoma, Extranodal NK-T-Cell ; drug therapy ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Perforin ; metabolism ; Retrospective Studies ; Treatment Outcome ; Young Adult

CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Hematologic Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Leukemia, Myeloid ; pathology ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Skin Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery

OBJECTIVETo study the clinicopathologic features, immunohistochemical findings, differential diagnosis and prognosis of type II enteropathy-associated T-cell lymphoma (EATL).

METHODSFourteen cases of type II EATL encountered in Department of Pathology, Nanjing General Hospital were retrospectively reviewed. The clinical data, histologic features, immunohistochemical findings and follow-up information were analyzed, with literature review.

RESULTSThere were altogether 12 males and 2 females. The median age of patient was 49 years. The sites of involvement included jejunum (10 cases) and ileum/colon (4 cases). The patients often presented with an abdominal mass, abdominal pain, diarrhea and constitutional symptoms such as fever, night sweating and cachexia. There was no clinical evidence of gluten-sensitive enteropathy. Histologically, the lymphoma cells showed full-thickness infiltration of the intestinal wall. They contained round hyperchromatic nuclei and pale cytoplasm. The stroma was minimally inflamed, with or without associated coagulative necrosis. A remarkable finding was the presence of villous atrophy, cryptal hyperplasia and intraepithelial lymphocytosis. Immunohistochemical study showed that the tumor cells expressed CD3, CD43 and CD8 (14/14). Some of them were also positive for CD56 (11/14) and CD30 (2/14). The staining for CD4, CD20, CD79a and myeloperoxidase was negative. A high proliferation index was demonstrated by Ki-67 immunostain. In-situ hybridization for EBER was negative. Follow-up data were available in 9 cases. The duration of follow-up ranged from 6 months to 36 months. Seven patients died within 14 months.

CONCLUSIONSEATL is a rare type of lymphoma with intestinal involvement. Associated enteropathy is not demonstrated, in contrast to cases encountered in Nordic countries. A correct diagnosis requires evaluation of clinical manifestations, pathologic features and ancillary study results.

Adolescent ; Adult ; Aged ; CD3 Complex ; metabolism ; CD8 Antigens ; metabolism ; Diagnosis, Differential ; Enteropathy-Associated T-Cell Lymphoma ; genetics ; immunology ; pathology ; surgery ; Female ; Follow-Up Studies ; Gene Rearrangement, T-Lymphocyte ; Humans ; Ileal Neoplasms ; genetics ; immunology ; pathology ; surgery ; Jejunal Neoplasms ; genetics ; immunology ; pathology ; surgery ; Leukosialin ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Ki-1 Antigen ; metabolism ; Leukocyte Common Antigens ; metabolism ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; drug therapy ; metabolism ; pathology ; Male ; Melanoma ; pathology ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Prednisone ; therapeutic use ; Receptor Protein-Tyrosine Kinases ; metabolism ; Retrospective Studies ; Vincristine ; therapeutic use ; Young Adult

OBJECTIVETo study the clinicopathologic features and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm.

METHODSThe clinical, morphology and immunophenotypic features were analyzed in 3 cases of blastic plasmacytoid dendritic cell neoplasm, with review of literature.

RESULTSThe pathologic changes of these tumors accorded with that of blastic plasmacytoid dendritic cell neoplasm, and they also had new characteristics, including lineage other than T, B, myeloid and NK cells, and immunophenotypes of CD56(+) CD4(-) CD123(+) TdT(+) CD43(+) CD68(+) , CD56(+) CD4(+) CD123(-) TdT(+) CD43(+) CD68(-) and CD56(+) CD4(+) CD123(-/+) TdT(-) CD43(+) CD68(+) in the 3 cases, respectively. Bone marrow involvement was found 5 years later in case 1, and was then stable after chemotherapy; case 2 and case 3 were died 5 and 2 months after diagnosis, respectively.

CONCLUSIONBlastic plasmacytoid dendritic cell neoplasm is a heterogeneous group of lymphoproliferative disorders, with different clinical, morphologic and immunophenotypic features.

Adolescent ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bleomycin ; therapeutic use ; CD56 Antigen ; metabolism ; Cyclophosphamide ; therapeutic use ; Dendritic Cells ; metabolism ; pathology ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Hematologic Neoplasms ; drug therapy ; metabolism ; pathology ; Humans ; Interleukin-3 Receptor alpha Subunit ; metabolism ; Leukemia, Myeloid ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; Male ; Middle Aged ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Prednisone ; therapeutic use ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; Treatment Outcome ; Vincristine ; therapeutic use

Antigens, CD20 ; metabolism ; Diagnosis, Differential ; Humans ; Infectious Mononucleosis ; metabolism ; pathology ; Leukocyte Common Antigens ; metabolism ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; metabolism ; pathology ; Nose Neoplasms ; diagnosis ; metabolism ; pathology ; Oral Ulcer ; metabolism ; pathology ; RNA, Viral ; metabolism ; Rhinoscleroma ; metabolism ; pathology