Medulloblastoma accounts for 20 to 25+ACU- of all intracranial neoplasms in children. The significance of the presence of isochromosome 17q (i(17q)), proliferative potential, apoptotic activity, and expression of c-erbB-2, bd-2, and p53 proteins in predicting long-term survival of patients with medulloblastomas was investigated. Twenty children were divided into two groups (favorable and poor outcome groups). Ten children with favorable outcome (FO) were disease-free during the follow-up period (median: 61.5 months). The other ten children with poor outcome (PO) died of disease progression, having a median survival of 18 months. Fluorescent in situ hybridization (FISH) for i(17q), terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling (TUNEL), and immunohistochemistry for Ki-67, c-erbB-2, bcl-2, and p53 proteins was performed in these patients. Nine out of 17 children showed i(17q). There was no difference in the rate of positive i(17q) between the FO and PO groups. The presence of i(17q) was not significantly related to biological factors that we investigated. Unlike the prominent presence of the proliferative potential and p53 expression in children with PO, apoptotic activity and expression of c-erbB-2 and bcl-2 had no correlation with the outcome.
Adolescence ; Apoptosis ; Brain Neoplasms/pathology ; Brain Neoplasms/mortality ; Brain Neoplasms/genetics+ACo- ; Cell Division ; Child ; Child, Preschool ; Chromosomes, Human, Pair 17/ultrastructure+ACo- ; Chromosomes, Human, Pair 17/genetics ; Comparative Study ; Disease-Free Survival ; Female ; Follow-Up Studies ; Genes, bcl-2+ACo- ; Genes, erbB-2+ACo- ; Genes, p53+ACo- ; Human ; In Situ Hybridization, Fluorescence ; In Situ Nick-End Labeling ; Infant ; Ki-67 Antigen/analysis ; Male ; Medulloblastoma/pathology ; Medulloblastoma/mortality ; Medulloblastoma/genetics+ACo- ; Neoplasm Proteins/analysis ; Prognosis ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

Medulloblastoma accounts for 20 to 25+ACU- of all intracranial neoplasms in children. The significance of the presence of isochromosome 17q (i(17q)), proliferative potential, apoptotic activity, and expression of c-erbB-2, bd-2, and p53 proteins in predicting long-term survival of patients with medulloblastomas was investigated. Twenty children were divided into two groups (favorable and poor outcome groups). Ten children with favorable outcome (FO) were disease-free during the follow-up period (median: 61.5 months). The other ten children with poor outcome (PO) died of disease progression, having a median survival of 18 months. Fluorescent in situ hybridization (FISH) for i(17q), terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling (TUNEL), and immunohistochemistry for Ki-67, c-erbB-2, bcl-2, and p53 proteins was performed in these patients. Nine out of 17 children showed i(17q). There was no difference in the rate of positive i(17q) between the FO and PO groups. The presence of i(17q) was not significantly related to biological factors that we investigated. Unlike the prominent presence of the proliferative potential and p53 expression in children with PO, apoptotic activity and expression of c-erbB-2 and bcl-2 had no correlation with the outcome.
Adolescence ; Apoptosis ; Brain Neoplasms/pathology ; Brain Neoplasms/mortality ; Brain Neoplasms/genetics+ACo- ; Cell Division ; Child ; Child, Preschool ; Chromosomes, Human, Pair 17/ultrastructure+ACo- ; Chromosomes, Human, Pair 17/genetics ; Comparative Study ; Disease-Free Survival ; Female ; Follow-Up Studies ; Genes, bcl-2+ACo- ; Genes, erbB-2+ACo- ; Genes, p53+ACo- ; Human ; In Situ Hybridization, Fluorescence ; In Situ Nick-End Labeling ; Infant ; Ki-67 Antigen/analysis ; Male ; Medulloblastoma/pathology ; Medulloblastoma/mortality ; Medulloblastoma/genetics+ACo- ; Neoplasm Proteins/analysis ; Prognosis ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

Following improvements in therapy for childhood malignancies, the striking increase in survival rate over the past 30 years has led to the increase risk of developing second malignant neoplasms (SMNs). We report a case of colorectal carcinoma as a SMN, following treatment for rhabdomyosarcoma. The patient was diagnosed with rhabdomyosarcoma of the urinary bladder at his age of three years, and developed adenocarcinoma in the colon 13 years later. Histologic examination of the surgical specimen revealed adenocarcinoma involving the rectosigmoid area with radiation colitis in its background. The tumor cells showed strong immunoreactivity for p53 protein, suggesting the role of irradiation and p53 mutation in carcinogenesis. This case emphasizes the need for dose observation in survivors of early childhood malignancies treated with radiation and multiagent chemotherapy.
Adenocarcinoma/pathology ; Adenocarcinoma/genetics ; Adenocarcinoma/etiology+ACo- ; Adolescence ; Antineoplastic Agents, Combined/therapeutic use ; Antineoplastic Agents, Combined/adverse effects+ACo- ; Bladder Neoplasms+ACo-/radiotherapy ; Bladder Neoplasms+ACo-/drug therapy ; Case Report ; Colitis/pathology ; Colitis/etiology ; Colorectal Neoplasms/pathology ; Colorectal Neoplasms/genetics ; Colorectal Neoplasms/etiology+ACo- ; Combined Modality Therapy ; Cyclophosphamide/adverse effects ; Cyclophosphamide/administration +ACY- dosage ; Doxorubicin/adverse effects ; Doxorubicin/administration +ACY- dosage ; Genes, p53 ; Human ; Male ; Neoplasm Proteins/analysis ; Neoplasms, Radiation-Induced/pathology ; Neoplasms, Radiation-Induced/genetics ; Neoplasms, Radiation-Induced/etiology+ACo- ; Neoplasms, Second Primary/etiology+ACo- ; Protein p53/analysis ; Radiation Injuries/pathology ; Radiation Injuries/etiology ; Radiotherapy/adverse effects+ACo- ; Rhabdomyosarcoma+ACo-/radiotherapy ; Rhabdomyosarcoma+ACo-/drug therapy ; Sigmoid Neoplasms/pathology ; Sigmoid Neoplasms/genetics ; Sigmoid Neoplasms/etiology ; Time Factors ; Vincristine/adverse effects ; Vincristine/administration +ACY- dosage

Following improvements in therapy for childhood malignancies, the striking increase in survival rate over the past 30 years has led to the increase risk of developing second malignant neoplasms (SMNs). We report a case of colorectal carcinoma as a SMN, following treatment for rhabdomyosarcoma. The patient was diagnosed with rhabdomyosarcoma of the urinary bladder at his age of three years, and developed adenocarcinoma in the colon 13 years later. Histologic examination of the surgical specimen revealed adenocarcinoma involving the rectosigmoid area with radiation colitis in its background. The tumor cells showed strong immunoreactivity for p53 protein, suggesting the role of irradiation and p53 mutation in carcinogenesis. This case emphasizes the need for dose observation in survivors of early childhood malignancies treated with radiation and multiagent chemotherapy.
Adenocarcinoma/pathology ; Adenocarcinoma/genetics ; Adenocarcinoma/etiology+ACo- ; Adolescence ; Antineoplastic Agents, Combined/therapeutic use ; Antineoplastic Agents, Combined/adverse effects+ACo- ; Bladder Neoplasms+ACo-/radiotherapy ; Bladder Neoplasms+ACo-/drug therapy ; Case Report ; Colitis/pathology ; Colitis/etiology ; Colorectal Neoplasms/pathology ; Colorectal Neoplasms/genetics ; Colorectal Neoplasms/etiology+ACo- ; Combined Modality Therapy ; Cyclophosphamide/adverse effects ; Cyclophosphamide/administration +ACY- dosage ; Doxorubicin/adverse effects ; Doxorubicin/administration +ACY- dosage ; Genes, p53 ; Human ; Male ; Neoplasm Proteins/analysis ; Neoplasms, Radiation-Induced/pathology ; Neoplasms, Radiation-Induced/genetics ; Neoplasms, Radiation-Induced/etiology+ACo- ; Neoplasms, Second Primary/etiology+ACo- ; Protein p53/analysis ; Radiation Injuries/pathology ; Radiation Injuries/etiology ; Radiotherapy/adverse effects+ACo- ; Rhabdomyosarcoma+ACo-/radiotherapy ; Rhabdomyosarcoma+ACo-/drug therapy ; Sigmoid Neoplasms/pathology ; Sigmoid Neoplasms/genetics ; Sigmoid Neoplasms/etiology ; Time Factors ; Vincristine/adverse effects ; Vincristine/administration +ACY- dosage