A seven months old male infant visited Severance Children's Hospital for evaluation of anterior mediastinal mass. With chest computed tomography (CT) image and biopsy, precursor T-cell lymphoblastic lymphoma was suspected but the ultrasonography guided biopsy specimen was insufficient to confirm the disease. Because there was a life-threatening risk to perform open biopsy to the small infant, we started chemotherapy empirically. The mass decreased, however, the lesion increased again and did not respond to the drugs. Finally we decided to resect the anterior mass with sternostomy and the pathology report finally resulted in thymoma.
Biopsy ; Drug Therapy ; Humans ; Infant ; Male ; Pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Thorax ; Thymoma ; Ultrasonography

OBJECTIVETo study the clinical features, diagnosis and therapy of hydroa vacciniforme-like cutaneous T cell lymphoma.

METHODSThe clinical presentations and the findings of laboratory examinations and skin biopsy of affected tissue in a child with hydroa vacciniforme-like cutaneous T cell lymphoma were retrospectively reviewed.

RESULTSThe child manifested as rash, fever and lymph node intumesce. Rash was pantomorphia, including edematous erythema, vesicles, crusts, necrosis and depressed scar, and it was mild in winter and severe in summer, mainly involving in the face and extremities. Epstein-Barre virus (EBV)-IgM was positive. Histopathological findings revealed focal lymphocyte invasion in subcutaneous panniculus adiposus, mainly surrounding the blood vessels. Immunohistochemistry showed CD3 (+), CD43 (+), CD20 (-), pax-5 (-), TIA (+), CD5 (+), CD8 (+), Granmye (+) and CD4 (-). The clinical symptoms were improved after glucocorticoid treatment in this child.

CONCLUSIONSHydroa vacciniforme-like cutaneous T cell lymphoma has special clinical manifestations. This disorder may be definitely diagnosed by skin biopsy of affected tissue and immunohistochemistry assay. Glucocorticoid treatment is effective. EBV infection may be related to the development of this disorder.

Child, Preschool ; Female ; Humans ; Hydroa Vacciniforme ; pathology ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; immunology ; pathology ; Skin ; pathology ; Skin Neoplasms ; drug therapy ; immunology ; pathology

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

Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cisplatin ; administration & dosage ; Dexamethasone ; administration & dosage ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; pathology ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; pathology ; Lymphomatoid Granulomatosis ; pathology ; Male ; Middle Aged ; Natural Killer T-Cells ; pathology ; Neoplasm Recurrence, Local ; Skin Neoplasms ; drug therapy ; pathology ; Young Adult

This study was purposed to evaluate the clinical and pathological features, prognosis of patients with subcutaneous panniculitis-time T cell lymphoma (SPTCL). The clinicopathologic features, immunophenotypes and treatment of 10 SPTCL patients which confirmed by pathology were analyzed retrospectively. The results showed that the main clinical manifestations of SPTCL were the single or multiple subcutaneous nodules. Of them 8 cases were found with recurrent high fever, weight loss, injury of liver function, bone marrow involvement and pancytopenia. This disease rapidly advanced. Pathologically, atypical large, medium-size and small-lymphocytes rounded the lipocytes look like rosettes. The reactive proliferation of histiocytes accompanied by phagothrocytic phenomena, polynuclear giant cells and granulomatous reaction. The tumor cells infiltrated into the lipolubuls. This lymphoma expressed the cytotoxic T-cell immunophenotype. CHOP regimen was the most common chemotherapy regimen used. 60% patients achieved a good initial response to chemotherapy. 3-year survival was 10%, with median survival time of 10 months. It is concluded that SPTCL is a specific type of lymphoma involving primarily in subcutaneous fatty tissues, most cases of SPTCL display an aggressive course, the disease may progress rapidly and accompanies with unfavorable prognosis. And the prognosis is poor in SPTCL patients with hemophagocytic syndrome. but the allo-HSCT can improve the outcome of this disease.
Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Lymphoma, T-Cell ; drug therapy ; pathology ; Male ; Middle Aged ; Panniculitis ; drug therapy ; pathology ; Retrospective Studies ; Young Adult

Adult ; Drug Therapy, Combination ; Female ; Granuloma ; diagnosis ; drug therapy ; pathology ; Humans ; Interferon-gamma ; therapeutic use ; Lymphoma, T-Cell, Cutaneous ; diagnosis ; drug therapy ; pathology ; Prednisone ; therapeutic use ; Skin Neoplasms ; diagnosis ; drug therapy ; pathology

OBJECTIVETo summarize and analyze the diagnosis, clinical features and therapy of primary colorectal non-Hodgkin's lymphoma (NHL).

METHODSThe clinicopathological data of 52 patients with primary colorectal NHL diagnosed and treated in our department from January 2000 to January 2010 were reviewed and analyzed retrospectively in this study.

RESULTSThis group of patients was composed of 45 cases of B cell and 7 T cell lymphomas, including 33 males and 19 females, with a male to female ratio of 1.7:1, and the age at diagnosis was 16 - 74 years old, with a median age of 50 years. The ileocecal region was most frequently involved site, acounted for 48.1%. The common symptoms encountered were abdominal pain (66.7%), diarrhea (15.6%), blood stool (24.4%), and body weight loss (8.9%). All patients were eventually diagnosed by histopathology, and the DLBCL subtype took up 64.4%. Among the 45 cases of B cell subtype, 33 cases (73.3%) were of early stage (IE and IIE confirmed), and the 5-year survival rate was 78.1%, while those of stage IIIE and IVE comprised 26.7%, with a 5-year survival rate of 45.5% (P < 0.05). The 5-year survival rate of all patients was 71.1%. Surgery was employed in 36 cases, and 9 patients received chemotherapy alone. Radical surgery could significantly increase the patients' overall survival rate, as compared with the chemotherapy alone group and palliative surgery group (P < 0.05).

CONCLUSIONSColorectal non-Hodgkin's lymphoma is a rare malignancy of the gastrointestinal tract. B cell type, male predominance and DLBCL subtype are most encountered manifestations in clinics. Multi-modality management with radical surgical resection of the primary lesion followed by standard chemotherapy, affords better local disease control, and a better survival outcome. Early detection and tailored immunotherapy can obviously prolong the long-term survival time.

Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Chemotherapy, Adjuvant ; Colorectal Neoplasms ; diagnosis ; drug therapy ; pathology ; surgery ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Lymphoma, B-Cell ; diagnosis ; drug therapy ; pathology ; surgery ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; drug therapy ; pathology ; surgery ; Lymphoma, Non-Hodgkin ; diagnosis ; drug therapy ; pathology ; surgery ; Lymphoma, T-Cell ; diagnosis ; drug therapy ; pathology ; surgery ; Male ; Middle Aged ; Neoplasm Staging ; Prednisone ; therapeutic use ; Retrospective Studies ; Salvage Therapy ; Survival Rate ; Vincristine ; therapeutic use ; Young Adult

OBJECTIVETo study the clinicopathologic features of peripheral T-cell lymphoma, unspecified (PTL-U) with follicular pattern.

METHODSThe clinical data, hematoxylin and eosin-stained sections of lymph node biopsies and follow-up data of 18 cases of PTL-U associated with follicular growth pattern were reviewed and studied. Eight cases of reactive lymphoid hyperplasia were used as controls. Semi-quantitative observation by retiform micrometer rule was carried out. Immunohistochemical study was also performed in all cases. T-cell receptor and immunoglobulin heavy chain gene rearrangement studies were conducted by polymerase chain reaction-based method.

RESULTSThe median age of the patients was 53 years. The male-to-female ratio was 1.57:1 in lymphoma group. All of the lymphoma patients presented with superficial lymphadenopathy, with (8/18) or without B symptoms. Histologically, the lymphoma was characterized by follicles of various sizes and shapes. The T zones were expanded by medium-sized lymphoma cells which contained clear cytoplasm and irregular nuclei. Mitotic figures were commonly identified. Immunohistochemical study confirmed that the lymphoma cells were of T-lineage. The proliferative index, as highlighted by Ki-67, was higher [average = (38.24 +/- 13.42)%/mm2] than that in the control group. T-cell receptor gene rearrangement was demonstrated in 71.4% (10/14) of the lymphoma cases.

CONCLUSIONSA definitive diagnosis of PTL-U with follicular pattern can be made on the basis of morphologic examination, immunohistochemical assessment and clinical features. Cases with atypical features can further be delineated by molecular analysis. Long-term follow up of these patients is prudent.

Adolescent ; Adult ; Aged ; CD3 Complex ; metabolism ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Gene Rearrangement, T-Lymphocyte ; Humans ; Ki-67 Antigen ; metabolism ; Lymphatic Diseases ; pathology ; Lymphoma, B-Cell, Marginal Zone ; pathology ; Lymphoma, Follicular ; drug therapy ; metabolism ; pathology ; Lymphoma, T-Cell, Peripheral ; drug therapy ; metabolism ; pathology ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Remission Induction ; Young Adult

OBJECTIVETo study the clinicopathologic features of malignant tumors in head and neck region complicated by fungal infection.

METHODSTwenty-one cases of malignant tumors occurring in head and neck region complicated by fungal infection were retrieved from the archival file. The light microscopic findings were reviewed. Histochemical (for PAS and GMS) and immunohistochemical (for MUC5B) studies were carried out. Fungal culture results were available in 13 of the 21 cases.

RESULTSThe age of the patients ranged from 12 to 72 years (median = 48 years). The male-to-female ratio was 17:4. Eight cases (38.1%) were complicated by invasive fungal sinusitis, with orbital involvement in 6 cases and brain involvement in 1 case. The primary tumors in such cases included leukemia (n = 7) and nasopharyngeal carcinoma (n = 1). The fungi belonged to Zygomycete in 5 cases and Aspergillus in 3 cases. These patients had history of chemotherapy/radiotherapy or antibiotics usage. The remaining 13 cases of fungal infection often affected necrotic tumor tissue in nasal cavity, paranasal sinuses, pharynx, larynx and palate. The fungi involved were Aspergillus (n = 6) and Candida (n = 4). Seven of such patients had received radiotherapy. Fungal culture was positive in 9 cases. Fourteen patients had follow-up information available and six of them died of the disease.

CONCLUSIONSMalignant tumors occurring in head and neck region can be complicated by fungal infection. Invasive fungal sinusitis (due to Zygomycetes and Aspergillus) often occurs in patients with leukemia, tends to involve orbit and is associated with poor prognosis. On the other hand, Aspergillus and Candida are the commonest fungi found in the necrotic tumor tissue. Pathologic examination remains the hallmark in confirming the diagnosis and fungal typing.

Adolescent ; Adult ; Aged ; Antifungal Agents ; therapeutic use ; Aspergillosis ; drug therapy ; microbiology ; pathology ; Aspergillus ; isolation & purification ; Candida ; isolation & purification ; Candidiasis ; drug therapy ; microbiology ; pathology ; Carcinoma, Squamous Cell ; drug therapy ; microbiology ; pathology ; Child ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; drug therapy ; microbiology ; pathology ; Humans ; Leukemia ; drug therapy ; microbiology ; pathology ; Lymphoma, Extranodal NK-T-Cell ; drug therapy ; microbiology ; pathology ; Male ; Middle Aged ; Mycoses ; drug therapy ; microbiology ; pathology ; Retrospective Studies ; Sinusitis ; drug therapy ; microbiology ; pathology ; Young Adult ; Zygomycosis ; drug therapy ; microbiology ; pathology

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