ABSTRACT
BACKGROUND: Understanding the biologic behavior of a tumor is a prerequisite for tumor registration code assignment. The aim of this report was to propose appropriate behavior codes of the International Classification of Disease Oncology 3 (ICD-O3) to rare, yet pathologically interesting hematopoietic and soft tissue tumors. METHODS: The Study Group for Hematopathology, the Bone and Soft Tissue Pathology Study Group, and the Cancer Registration Committee prepared the questionnaire containing provisional behavior codes of selected diseases. RESULTS: In situ lesions of mantle cell and follicular lymphomas, dendritic cell tumors, and neoplasms with perivascular epithelioid cell differentiation (PEComa), not otherwise specified were classified as malignant (-/3). The fibromatosis group, with the exception of lipofibromatosis, was proposed as benign (-/0). Lipofibromatosis and several diseases that belong to the PEComa group were proposed as uncertain malignant potential (-/1). For the hematologic and soft tissue tumors, 274 and 288 members of the Korean Society of Pathologists, respectively, provided opinions through questionnaire, and most responders showed agreement with the provisional behavior code proposed. CONCLUSIONS: The determination of behavior codes for the rare diseases described in this study, especially those of the PEComa group or malignant lymphoma, could be viewed as impractical and premature, but this study provides the basis for future research on this topic.
Subject(s)
Dendritic Cells , Epithelioid Cells , Fibroma , Hematologic Neoplasms , Lymphoma , Lymphoma, Follicular , Perivascular Epithelioid Cell Neoplasms , Surveys and Questionnaires , Rare Diseases , Soft Tissue NeoplasmsABSTRACT
BACKGROUND: Post-transplantation lymphoproliferative disorder (PTLD) after liver transplantation is a rare but potentially fatal disease. Clinical manifestations and prevalence of PTLD after liver transplantation in Korea have not been investigated thoroughly. MATERIALS AND METHODS: A retrospective chart review was done for 284 liver transplant recipients at Samsung Medical Center, Seoul, Korea during the period from 1996 to 2003. RESULTS: The incidence of PTLD after liver transplantation was 3.9% (11/284). PTLDs were more prevalent in children (9/55, 16.4%) than in adults (2/237, 0.9%; P<0.01). Among the PTLD patients, four cases were male (36.3%) and seven were female (63.7%). Median time from the transplantation to PTLD diagnosis was 9 months. The type of PTLD was as follows:early lesion (6 cases, 54.5%), polymorphic PTLD (3 cases, 27.3%), and B cell lymphoma (2 cases, 18.2%). PTLDs were more prevalent in the patients with cyclosporine use (OR 13.28, 95% CI:1.29-136.31, P=0.03), acute rejection (OR 5.63, 95% CI:1.03-30.62, P=0.04), and negative serology for EBV VCA IgG (OR 19.15, 95% CI:1.99-183.98, P=0.01) by multivariate logistic regression. Three patients (27.3%) died of B cell lymphoma (2 cases) and polymorphic PTLD (1 case). The remaining patients were improved with reduction of immunosuppression and treatment with acyclovir. CONCLUSION: The incidence of PTLD was high in children. The risk factors of PTLD were negative serology for EBV VCA IgG, history of acute rejection, and cyclosporine use. Considering the poor prognosis of PTLD, effective strategies for prevention and early diagnosis for early treatment should be emphasized.
Subject(s)
Adult , Child , Female , Humans , Male , Acyclovir , Cyclosporine , Diagnosis , Early Diagnosis , Herpesvirus 4, Human , Immunoglobulin G , Immunosuppression Therapy , Incidence , Korea , Liver Transplantation , Liver , Logistic Models , Lymphoma, B-Cell , Lymphoproliferative Disorders , Prevalence , Prognosis , Retrospective Studies , Risk Factors , Seoul , TransplantationABSTRACT
BACKGROUND: Post-transplantation lymphoproliferative disorder (PTLD) after liver transplantation is a rare but potentially fatal disease. Clinical manifestations and prevalence of PTLD after liver transplantation in Korea have not been investigated thoroughly. MATERIALS AND METHODS: A retrospective chart review was done for 284 liver transplant recipients at Samsung Medical Center, Seoul, Korea during the period from 1996 to 2003. RESULTS: The incidence of PTLD after liver transplantation was 3.9% (11/284). PTLDs were more prevalent in children (9/55, 16.4%) than in adults (2/237, 0.9%; P<0.01). Among the PTLD patients, four cases were male (36.3%) and seven were female (63.7%). Median time from the transplantation to PTLD diagnosis was 9 months. The type of PTLD was as follows:early lesion (6 cases, 54.5%), polymorphic PTLD (3 cases, 27.3%), and B cell lymphoma (2 cases, 18.2%). PTLDs were more prevalent in the patients with cyclosporine use (OR 13.28, 95% CI:1.29-136.31, P=0.03), acute rejection (OR 5.63, 95% CI:1.03-30.62, P=0.04), and negative serology for EBV VCA IgG (OR 19.15, 95% CI:1.99-183.98, P=0.01) by multivariate logistic regression. Three patients (27.3%) died of B cell lymphoma (2 cases) and polymorphic PTLD (1 case). The remaining patients were improved with reduction of immunosuppression and treatment with acyclovir. CONCLUSION: The incidence of PTLD was high in children. The risk factors of PTLD were negative serology for EBV VCA IgG, history of acute rejection, and cyclosporine use. Considering the poor prognosis of PTLD, effective strategies for prevention and early diagnosis for early treatment should be emphasized.
Subject(s)
Adult , Child , Female , Humans , Male , Acyclovir , Cyclosporine , Diagnosis , Early Diagnosis , Herpesvirus 4, Human , Immunoglobulin G , Immunosuppression Therapy , Incidence , Korea , Liver Transplantation , Liver , Logistic Models , Lymphoma, B-Cell , Lymphoproliferative Disorders , Prevalence , Prognosis , Retrospective Studies , Risk Factors , Seoul , TransplantationABSTRACT
The survival rate after liver transplantation has greatly improved. However, complications such as infection and rejection remain major causes of mortality and morbidity. The fungal infections are associated with high mortality rates, despite having a relatively lower incidence, compared with bacterial and viral infections. Difficulty in establishing an early diagnosis, lack of effective therapy, difficult management of certain antifungal agent, limited data for antifungal prophylaxis presents remaining problems.3) We report our experience with one case of syatemic candidemia after orthotopic liver transplantation.
Subject(s)
Candidemia , Early Diagnosis , Incidence , Liver Transplantation , Liver , Mortality , Survival RateABSTRACT
The survival rate after liver transplantation has greatly improved. However, complications such as infection and rejection remain major causes of mortality and morbidity. The fungal infections are associated with high mortality rates, despite having a relatively lower incidence, compared with bacterial and viral infections. Difficulty in establishing an early diagnosis, lack of effective therapy, difficult management of certain antifungal agent, limited data for antifungal prophylaxis presents remaining problems.3) We report our experience with one case of syatemic candidemia after orthotopic liver transplantation.