Solitary lymphomatoid granulomatosis at the maxilla

Lymphomatoid granulomatosis (LYG) is an uncommon Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder distinct from lymphoma. LYG primarily occurs in the lung with or without accompanied lesions in the skin, central nervous system, kidney, gastrointestinal tract, nose, eyes, liver and oral cavity. Solitary extrapulmonary LYG is extremely rare, and whether solitary lesions progress onto pulmonary development and dysfunction is controversial. Herein, we report a case on a solitary LYG in the maxilla gingiva with bone exposure in a patient who had been taking methotrexate for rheumatoid arthritis.ope

Gingival Juvenile Xanthogranuloma

Juvenile xanthogranuloma (JXG) is a benign histiocytosis that occurs in the pediatric population. Cutaneous JXG is the most common form, while extracutaneous lesions, including oral JXG, is extremely rare. Cutaneous JXG can occur as multiple lesions and may have systemic visceral involvement, but this is not seen in oral JXG. In this case, we report a solitary oral JXG at the gingiva in a 3-year old male.ope

Gemtuzumab Ozogamicin (MylotargⓇ) as a Single Consolidation Therapy in Relapsed Pediatric Acute Myeloid Leukemia Patient: a Patient Who Refused Transfusion and Stem Cell Transplantation for Religious Belief

Despite improvement of long-term survival rate in pediatric acute myeloid leukemia (AML) during the last two decades, relapse/refractory disease remains a major obstacle to further improvement of prognosis. Moreover, there are only few therapeutic options which stimulate investigations for targeted, less toxic therapy. Gemtuzumab ozogamicin (GO, MylotargⓇ) is an anti-CD33 monoclonal antibody and there are ongoing studies evaluating safety and efficacy of this drug in relapsed/refractory AML patients. Still, the response rate is only 30% and the response does not last more than a year. We present a case of pediatric central nervous system relapsed AML who was treated with GO as a single consolidation therapy without stem cell transplantation because of religious belief and maintained CR status for more than 3 years.ope

Total spondylectomy for giant cell tumor of cervical spine

Giant cell tumor which is arisen at vertebra is rare and this tumor of the cervical vertebra has been very rarely reported tumor which is less than 1% of all giant cell tumor. When the treatment option is considered, the curretage is often selected rather than total resection because the anatomic relationship of adjacent structures is complicated and there are major vessels and organs around the cervical vertebra. The prognosis of this tumor is decided by degree of resection so, total sponylectomy should be considered as primary surgical option. We report a case of cervical giant cell tumor in which the total spondylectomy was performed successfully and discuss the feasibility of this procedure at cervical region.ope

Nasal NK/T-cell lymphoma: epidemiology and pathogenesis.

Nasal NK/T-cell lymphoma (NKTCL) is an uncommon disease, but usually shows a highly aggressive clinical course. The disease is much more frequent in Asian and Latin American countries than in Western countries, and is universally associated with Epstein-Barr virus (EBV) infection. Analyses of gene mutations, especially p53 and c-KIT, revealed the different frequencies by district. Epidemiological studies revealed the changes of the disease frequency in Korea during the period from 1977-1989 to 1990-1996. Case-control study showed that the exposure to pesticides and chemical solvents could be causative of NKTCL. Further studies including HLA antigen typing of patients is necessary to further clarify the disease mechanism.ope

Immunophenotypic and genotypic characterization of nasal lymphoma with polymorphic reticulosis morphology.

Nasal lymphoma with polymorphic reticulosis (PR) morphology is now categorized as T/natural killer (T/NK) cell lymphoma. In this study, immunophenotypes and genotypes of proliferating cells in 21 cases with PR were examined. The patients included 13 men and 8 women ranging in age from 20 to 74 (median 37) years. All patients presented with lesions in the upper respiratory tract, mostly in the nasal cavity. Histological specimens obtained from the primary lesions (19 cases) and metastatic cervical lymph nodes (2 cases) were used for analyses. Histologically, polymorphous proliferation was found in 20 cases, and these were thus diagnosed as PR. A monomorphous pattern was found in the remaining last case. Immunohistochemical analysis revealed that the proliferating cells were CD56 (123C3)+ and/or CD16 (2H7)+, TIA-1+ and frequently stained CD3 epsilon+. Tumor cells were frequently stained positively with monoclonal antibodies (mAbs) for T lymphocytes, but were negative for T-cell receptor (TCR) beta and delta chain expression. In situ hybridization analysis using an Epstein-Barr virus-encoded early RNA 1 (EBER-1) probe revealed positive signals in 13 of the 15 cases examined. Southern blotting analysis for clonality of the Epstein-Barr virus (EBV) genome in 12 positive cases confirmed the presence of monoclonal proliferation in 7 cases. The pattern of TCR gamma chain gene rearrangement was examined by PCR analysis of DNA from tumor tissues by the denaturing gradient gel electrophoresis method. The results demonstrated no clonal rearrangement in any of the 21 cases examined, including 7 cases with proven clonal proliferation of EBV-infected cells, indicating the absence of T-cell clones. Our findings strongly suggested that nasal T-cell lymphoma is in fact a NK cell lymphoma.ope

Expression of Anaphase Promoting Complex (APC) and APC Regulatory Proteins in Invasive Ductal Carcinoma Associated with Paget's Disease

Background : Oncogene expression in Paget's disease of the breast is not well known. To characterize invasive ductal carcinoma associated with Paget's disease, we studied expres-sion of anaphase promoting complex (APC) with its regulatory proteins. Methods : Immuno-histochemical stainings were done with 10 cases of invasive ductal carcinoma associated with Paget's disease for APC, pituitary tumor transforming gene (PTTG), cyclin B1, p53, cyclin D1, and c-erbB-2. The expressions of these markers in Paget's disease were compared with those in the associated with carcinoma. Results : APC, PTTG, cyclin B1, and c-erbB-2 were positive in all of the cases with both Paget's disease and underlying carcinoma. p53 was expressed in Paget's disease of 6 cases (60%) and in carcinoma of 7 cases (70%). Cyclin D1 was positive in Paget's disease of 8 cases (80%) and in carcinoma of 9 cases (90%). Conclusions : Breast carcinomas with Paget's disease seem to be distinguished by the high expression of APC, cyclin B1, PTTG, c-erbB2, and cyclin D1 in contrast to breast can-cers without Paget's disease. Furthermore, the similar expression pattems of APC and APC regulatory proteins in both Paget’s disease and underlying breast cancer support the epider-motropic theory as its pathogenetic mechanism.ope

Effect of Probucol and Verapamil on Injury to Myocardium and Nerve Fibers in Rat Heart Induced by Doxorubicin

Background: Doxorubicin is considered to be one of the most effective drugs to treat a variety of human cancers. However, the dose-dependent cardiotoxicity of doxorubicin limits its clinical usefulness. This study aimed to evaluate the effect of probucol and verapamil on the cardiac neurotoxicity and cardiomyopathy induced by the long-term use of doxorubicin. Methods: Sprague-Dawley male rats were grouped as the control group, the doxorubicin treated group, the doxorubicin treated with probucol group, and the doxorubicin treated with verapamil group. The rats were treated for 4, 6, 8 and 10 weeks. H&E staining and immunohistochemical staining for protein gene product 9.5, caspase-3, heat shock protein 70, and hsp 25 were performed. Results: The degree of interstitial inflammatory cell infiltration was mildest in the probucol treated group. The reduction in the number of nerve fibers in the probucol treated group was less than the other treatment groups. There was a negative correlation between the treatment duration and stained nerve fibers in all the treatment groups. The number of caspase-3 positive cells was more increased in the doxorubicin group and the verapamil treated group than in the control and probucol treated group. Conclusions: It is suggested that probucol partly contributed to the inhibition of doxorubicin-induced cardiac neurotoxicity and cardiomyopathy, whereas the verapamil had no effect.ope

Follicular dendritic cell sarcoma presenting as a submucosal tumor of the stomach

Follicular dendritic cell (FDC) sarcomas, especially those of extranodal origin, are extremely rare, and this entity could easily be missed without a high index of suspicion. We report a case of FDC sarcoma presenting as a submucosal tumor of the stomach in a 45-year-old man. The mass was a spindle and epithelioid mesenchymal tumor with many individually scattered and perivascular aggregates of lymphocytes. Immunohistochemical and ultrastructural studies confirmed the diagnosis. Although more than 50 cases of this tumor have been documented in the English literature, to our knowledge the presentation of FDC sarcoma as a submucosal tumor of the stomach has never been recorded. This case highlights the occurrence of FDC sarcoma as a submucosal tumor of the gastrointestinal tract. We believe that FDC sarcoma should be included in the differential diagnosis of spindle or epithelioid cell tumors of the gastrointestinal hollow viscus to prevent this still under-recognized tumor from being overlooked.ope

Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin.

Sinus histiocytosis with massive lymphadenopathy (SHML) is an idiopathic proliferation of unique histiocytes that have vesicular nuclei and voluminous pale cytoplasm, often with emperipolesis. Pure cutaneous involvement is very rare. We describe a patient with SHML limited to the skin whose lesion has spontaneously regressed. A 35-year-old Korean male visited the Department of Dermatology due to facial rash for 2 months. A 3 x 3.5 cm-sized well-demarcated dark erythematous nontender plaque was noted on the right cheek. Skin biopsy showed dense, nodular infiltrates of histiocytes with abundant cytoplasm and vesicular nuclei rimmed by lymphoplasma cell aggregates throughout the upper and mid-dermis. The histiocytes were immunohistochemically positive for S-100 protein and CD68, but negative for CD1a. Laboratory tests and a thorough physical examination revealed no abnormalities. These findings suggested that this was a case of SHML clinically limited to the skin. The skin lesion was initially resistant to steroid therapy, but began to regress 10 months after the onset without further treatment.ope