ABSTRACT
We report two rare cases of botryoid Wilms' tumor (BWT) occupying the renal collecting system with no macroscopic parenchymal mass. In case 1, a 3-year-old boy presented with a mass in the right flank, low-grade fever, abdominal pain, and macrohematuria. Radiology revealed an enlarged right kidney with a heterogeneous mass occupying a large part of the dilated renal calyx, pelvis, and ureter. Radical right nephroureterectomy was performed. The histopathologic diagnosis was nephroblastoma, and the pedicle of the tumor was attached to the renal parenchyma near the pelvic wall. In case 2, a 2-year-old boy presented with macrohematuria, and the clinical course was almost the same as in case 1. No local recurrence or metastatic spread has been detected for 4 years postoperatively in case 1 and for 9 months postoperatively in case 2.
Subject(s)
Kidney Neoplasms/pathology , Wilms Tumor/pathology , Child, Preschool , Diagnosis, Differential , Follow-Up Studies , Hematuria/diagnosis , Hematuria/surgery , Humans , Kidney/diagnostic imaging , Kidney/pathology , Kidney Neoplasms/surgery , Kidney Pelvis , Magnetic Resonance Imaging , Male , Nephrectomy , Sarcoma/diagnosis , Tomography, X-Ray Computed , Ultrasonography , Wilms Tumor/surgeryABSTRACT
A neonate with stage IV bilateral adrenal neuroblastoma associated with metastases to the skin, liver, bone marrow, and right ocular conjunctiva is reported. At birth, skin metastases were present all over the body. He underwent a combination of chemotherapy, surgery, and peripheral blood stem cell transplantation (PBSCT). Histopathology was unfavorable with diploid tumor cell DNA content and low levels of TRK-A mRNA expression. Skin and conjunctival metastases disappeared after PBSCT, and there have been no signs of recurrence after 5 years of follow-up. Accurate staging of disease and histologic examination followed by intensive management are essential even in infants with neuroblastoma to ensure successful outcome.
Subject(s)
Adrenal Gland Neoplasms/pathology , Neoplasms, Multiple Primary/pathology , Neuroblastoma/secondary , Skin Neoplasms/secondary , Follow-Up Studies , Humans , Infant, Newborn , Male , Neoplasm Staging , Neuroblastoma/diagnosis , Neuroblastoma/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/therapySubject(s)
Cord Blood Stem Cell Transplantation , Cytomegalovirus Infections/blood , DiGeorge Syndrome/complications , DiGeorge Syndrome/therapy , Viral Load , Antigens, Viral/blood , Antigens, Viral/urine , Cytomegalovirus/isolation & purification , Cytomegalovirus Infections/complications , Cytomegalovirus Infections/immunology , DNA, Viral/blood , DNA, Viral/urine , DiGeorge Syndrome/diagnosis , DiGeorge Syndrome/immunology , Humans , Immunocompromised Host , Infant , Male , T-Lymphocytes/immunologyABSTRACT
The authors recently encountered a lethal case of Down syndrome with transient abnormal myelopoiesis (TAM). Although the peripheral white blood cell count and blast cells had improved without specific treatment, the patient died of severe coagulopathy due to liver fibrosis when he was 5 years old. The prognosis of TAM with liver fibrosis was poor. The patient had high levels of N-terminal peptide of III procollagen, type IV collagen, and hyaluronic acid. These serum makers are noninvasive indicators of liver fibrosis and may be useful as prognostic indicators of TAM in Down syndrome.
Subject(s)
Down Syndrome/complications , Liver Failure/etiology , Myelopoiesis , Biomarkers/blood , Collagen Type IV/blood , Disseminated Intravascular Coagulation , Down Syndrome/blood , Down Syndrome/diagnosis , Fatal Outcome , Female , Fetal Diseases/blood , Fetal Diseases/diagnosis , Humans , Hyaluronic Acid/blood , Infant, Newborn , Liver Cirrhosis/blood , Liver Cirrhosis/diagnosis , Liver Cirrhosis/etiology , Liver Failure/blood , Liver Failure/diagnosis , Male , Peptide Fragments/blood , Pregnancy , Procollagen/blood , PrognosisABSTRACT
Hepatoblastoma is a common hepatic tumor in children. Although evidence regarding cytogenetic and molecular genetic alterations in hepatoblastomas has been reported, the molecular events affecting the biologic characteristics of this tumor, including alterations of the gene expression profile, are largely unknown. To identify genes differentially expressed between nondiseased liver (NDL) and hepatoblastoma tumor (HBT), we analyzed the gene expression profile in 14 NDL and 16 HBT samples using a high-density oligonucleotide DNA array. Using Mann-Whitney U test followed by the k-nearest neighbor algorithm, we identified 26 genes (predictor genes) that were able to assign unknown samples derived from NDL and HBT to either the NDL group or HBT group with 100% accuracy. Using a cross-validation approach, we confirmed that the k-nearest neighbor algorithm assigned the particular samples derived from NDL and HBT to either the NDL or HBT group with 93.3% (28/30 samples) accuracy. In the 26 predictor genes, we found alteration of the expression of genes regulating cell division (NAP1L1, STMN1, CCNG2, and CDC7L1) and tumor cell growth (IGF2 and IGFBP4) in HBT. Four predictor genes (ETV3, TPR, CD34, and NR1I3) were also found to be mapped to the chromosomal region 1q21 approximately q32, which has been reported to be frequently involved in the development of hepatoblastoma. The findings obtained in this study suggest that alteration of the expression of some genes regulating cell division and tumor cell growth may be characteristics of the gene expression profile in HBT, and that alteration of the expression of the four predictor genes mapped to chromosomal region 1q21 approximately q32 may also contribute to the differences in gene expression profile between NDL and HBT.
Subject(s)
Gene Expression Profiling , Hepatoblastoma/genetics , Oligonucleotide Array Sequence Analysis , Child , Child, Preschool , Chromosome Mapping , Constitutive Androstane Receptor , Female , Hepatoblastoma/physiopathology , Humans , Infant , Infant, Newborn , Insulin-Like Growth Factor II/metabolism , Male , PhylogenyABSTRACT
OBJECTIVE: Recent reports have indicated that monocytes express receptors for the granulocyte colony-stimulating factor (G-CSF). The direct effects of G-CSF on cytokine secretion in monocytes were examined. MATERIALS AND METHODS: A monocytic cell line NOMO-1 that secretes multiple cytokines upon stimulation with lipopolysaccharide (LPS) was used. Normal human monocytes were purified by negative selection using magnetic beads. Cells pretreated with or without G-CSF were stimulated with LPS, and the subsequent concentrations of cytokines and chemokines in supernatants were determined by sandwich enzyme-linked immunosorbent assay. RESULTS: NOMO-1 cells were found to express receptors for G-CSF. Although G-CSF stimulation did not induce cytokine secretion, pretreatment with G-CSF significantly attenuated LPS-stimulated secretion of the proinflammatory cytokines tumor necrosis factor-alpha and interleukin (IL)-12 in NOMO-1 cells. Simultaneously, however, G-CSF pretreatment apparently enhanced LPS-induced secretion of IL-10 and monocyte chemoattractant protein-1, whereas secretions of IL-1beta, IL-6, and IL-8 were unaffected. When normal human monocytes from healthy volunteers were similarly examined, marked individual variations in LPS-induced secretion of cytokines were observed. Although some exceptions exist, a similar tendency as to the effects of G-CSF treatment on cytokine secretions as that in NOMO-1 cells was observed in human monocytes. CONCLUSIONS: Our data suggest that G-CSF directly affects monocytes and modulates their cytokine secretion. NOMO-1 cells can provide an alternate model for in vitro culture of monocytes to investigate the effects of G-CSF on cytokine secretion by these cells.
