Fine needle aspiration has been effectively being applided to pediatric tumors since it renders a rapid diagnosis with minimal intervention. This measure is especially required for the large pediatric mass, which needs preoperative chemotherapy or radiotherapy to shrink the tumor to an operable size. A case of neuroblastoma of mediastinum, stage IV diagnosed by CT-guided FNA is described.
Biopsy, Fine-Needle* ; Diagnosis ; Drug Therapy ; Mediastinum* ; Neuroblastoma* ; Radiotherapy

PURPOSE: A case of proptosis due to orbital metastasis of neuroblstoma and review of the current literature on neuroblastoma METHODS: A 8-month-old female presented with proptosis due to neuroblastoma involving skull and bone marrow. RESULTS: Proptosis was improved following chemotherapy. CONCLUSIONS: In case of infant who has a proptosis and periorbital ecchymosis without a primary lesion or a specific history, neuroblastoma may be considered as a differential diagnosis.
Bone Marrow ; Diagnosis, Differential ; Drug Therapy* ; Ecchymosis ; Exophthalmos* ; Female ; Humans ; Infant ; Neoplasm Metastasis ; Neuroblastoma* ; Orbit ; Skull

Spontaneous rupture with internal bleeding of solid tumors has rarely been described at the time of diagnosis or during chemotherapy. This rare event must be regarded as a life threatening condition. In these emergency situations, control of hemorrhage, which is life-saving, can be achieved by transcatheter arterial embolization (TAE) and/or surgical resection. This report describes two infants presenting with acute hemorrhagic shock due to spontaneous tumor rupture of hepatoblastoma and neuroblastoma during chemotherapy. TAE successfully arrested the tumor bleeding and a visibly reduced the tumor size in both children. Spontaneous rupture of solid tumors occur infrequently in children, but is a life threatening situation. Careful monitoring for the occurrence of this rare event especially in very young children presenting with a large tumor mass.
Child ; Diagnosis ; Drug Therapy ; Emergencies ; Hemorrhage ; Hepatoblastoma ; Humans ; Infant ; Neuroblastoma ; Rupture ; Rupture, Spontaneous ; Shock, Hemorrhagic

Neuroblastoma originate in neural crest and is one of the most frequent tumor next to leukemia and brain tumor in child. The tumor is most highly malignant tumor due to early metastasis at diagnosis. It could be diagnosed by various diagnostic modalities such as roentgenologic examination, laboratory data and confirmed by pathologic diagnosis. Management of neuroblastoma is surgery, radiotherapy and chemotherapy. We experienced a case of adrenal neuroblastoma at age of 20-month male child and report with a brief review of literature
Adrenal Glands ; Brain Neoplasms ; Child ; Diagnosis ; Drug Therapy ; Humans ; Leukemia ; Male ; Neoplasm Metastasis ; Neural Crest ; Neuroblastoma* ; Radiotherapy

Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.
Adrenal Glands ; Adrenalectomy* ; Biopsy ; Drug Therapy ; Humans ; Liver* ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Neuroblastoma* ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography, Prenatal

Congenital mesoblastic nephroma is a rare renal tumor that is diagnosed during pregnancy and is associated with polyhydramnios, prematurity, and neonatal hypertension. Differential diagnoses include Wilms tumor, adrenal neuroblastoma, and other abdominal tumors. We report a case of congenital mesoblastic nephroma detected by prenatal ultrasonography as a large fetal renal mass with polyhydramnios at 32 weeks of gestation. Ultrasonography showed a 6x6-cm complex, solid, hyperechoic, round mass in the right kidney. At 35 weeks of gestation, the patient was admitted with preterm premature rupture of membranes and the baby was delivered vaginally. Postnatal ultrasonography and computed tomography showed a heterogeneous solid mass on the right kidney. At the end of the first week of life, a right nephrectomy was performed and subsequent pathological examination confirmed a cellular variant of congenital mesoblastic nephroma with a high mitotic count. Postoperative adjuvant chemotherapy was administered. The newborn was discharged in good condition.
Chemotherapy, Adjuvant ; Diagnosis, Differential ; Drug Therapy ; Humans ; Hypertension ; Infant, Newborn ; Kidney ; Membranes ; Nephrectomy ; Nephroma, Mesoblastic* ; Neuroblastoma ; Polyhydramnios ; Pregnancy ; Prenatal Diagnosis* ; Rupture ; Ultrasonography ; Ultrasonography, Prenatal ; Wilms Tumor

Extraskeletal Ewing's sarcoma is a rare primary malignant soft tissue tumor that has the same pathological characteristics of skeletal Ewing's sarcoma. It is known that the differential diagnosis of Ewing's sarcoma from undifferentiated rhabdomyosarcoma, neuroblastoma, reticulum cell sarcoma, and malignant lymphoma by the histologic studies only are not easy because their histologic findings are nearly similar each other. Therefore, immunohistochemical method, electron microscopic examination, cytogenetic study, or molecular gene study are used together to confirm the diagnosis. It chiefly involves the lower extremity, soft tissues of the paravertebral region, and adjacent chest wall. We recently experienced a case of extraskeletal Ewing's sarcoma of duodenum in a 21-year old female. The patient underwent Whipple's operation and postoperative combined chemotherapy were done.
Cytogenetics ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Duodenum* ; Female ; Humans ; Lower Extremity ; Lymphoma ; Lymphoma, Non-Hodgkin ; Neuroblastoma ; Rhabdomyosarcoma ; Sarcoma* ; Sarcoma, Ewing ; Thoracic Wall ; Young Adult

PURPOSE: Multidrug resistance (MDR) is one of the main obstacles in the successful anticancer chemotherapy. Classic MDR phenotype is characterized by overexpression of membrane bound permeability-glycoprotein (Pgp) drug-efflux pump, encoded by MDR1 gene. The non-Pgp MDR phenotype is caused by overexpression of multidrug resistance associated protein (MRP), another membrane transport protein, encoded by MRP gene. We examined the mRNA expression of MDR1 and MRP genes in various types of pediatric malignant solid tumors at diagnosis. METHODS: Five fresh frozen tissue and 15 primarily cultured cell samples from 20 children diagnosed as malignant solid tumors (8 neuroblastomas, 5 medulloblastomas, 3 Burkitt lymphomas, 2 Wilms tumors, 1 each of rhabdomyosarcoma and rhabdoid tumor) were analyzed by reverse transcription-polymerase chain reaction (RT-PCR). RESULTS: Among 20 pediatric solid tumors, MDR1 mRNA expression was observed in 14 cases (70%), and MRP mRNA expression was observed in 15 cases (75%). The co-expression of MDR1 and MRP was recognized in 12 (60%) of 20 cases. Event (death or relapse) occurred in 7 cases during observation period of median 9 months after diagnosis, and 6 of these 7 cases (86%) showed the co-expression of MDR1 and MRP. CONCLUSION: These data suggest that MRP, like MDR1, may have an important negative impact on the outcome of chemotherapy in pediatric malignant solid tumors, and it is possible that these two genes may collaborate in causing the appearance of MDR under certain circumstances.
Burkitt Lymphoma ; Cells, Cultured ; Child ; Diagnosis ; Drug Resistance, Multiple* ; Drug Therapy ; Humans ; Medulloblastoma ; Membranes ; Multidrug Resistance-Associated Proteins* ; Neuroblastoma ; Phenotype ; Rhabdomyosarcoma ; RNA, Messenger ; Wilms Tumor

BACKGROUND: The survival rate of children with neuroblastoma has been improved over 20 years, excluding the metastatic disease, in which it does not exceed 20% so far. New treatment modalities have been developed to improve the outcome in metastatic disease. Preoperative chemotherapy reduce the size, the vascularity and the adhesiveness, so increase the resectability of the primary tumor. This retrospective clinical study was designed to review the survival rate in neuroblastoma and to analyze the effect of preoperative chemotherapy in the view point of neoadjuvant therapy on long-term survival in advanced disease. METHODS: One hundred and thirty-four cases were reviewed from 135 patients with neuroblastoma registered at the Department of Pediatrics in Seoul National University Children's Hospital from January, 1985 till December, 1995. The survival rate was reviewed according to the stage. The age, sex of the patients, the stage, anatomical site of the tumor, the level of serum ferritin and neuron-specific enolase were analyzed for the risk factors on survival. RESULTS: Ranges of age at diagnosis were from 1 month to 166 months with the median of 39 months. Five year survival rates and five year disease-free survival rates were 100%, 100% in stage 1(n=5), 90.9%, 90.9% in stage 2(n=13), 43.4%, 40.6% in stage 3(n=19), 27.1%, 19.8% in stage 4(n=95) and 100%, 100% in stage 4S(n=2), respectively. In stage 3, five year survival rate was 52.5% in group receiving neoadjuvant chemotherapy, 28.6% in control group(P=0.02). Five year disease-free survival rate was also noted as 48.6%, 28.6% in each group(P=0.02). In stage 4, five year and ten year survival rates were 27.6%, 23.6% in group receiving neoadjuvant chemotherapy, 26.9%, 0% in control group(P=0.02). Five year and ten year disease-free survival rates were 14.3%, 14.3% in group receiving neoadjuvant chemotherapy, 20%, 0% in control group (P=0.11). In univariate analysis, the age, the stage, and the site of primary tumor appeared to affect the long-term survival. CONCLUSION: Neoadjuvant chemotherapy and delayed primary surgery contribute for advance in survival in advanced neuroblastoma via increasing the resectability of the primary tumor.
Adhesiveness ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Ferritins ; Humans ; Neoadjuvant Therapy ; Neuroblastoma ; Pediatrics ; Phosphopyruvate Hydratase ; Retrospective Studies ; Risk Factors ; Seoul ; Survival Rate

PURPOSE: Despite the use of improved therapeutic modalities, long-term survival remains poor in patients in stage lll or lV of neurobalstoma and for over 1 year olds at diagnosis. We evaluated the long-term survival rate and related prognostic factors in advanced neuroblastoma. METHODS: Twenty-nine children diagnosed with stage lll or lV neuroblastoma after 1 year of age, between May 1987 and December 1996, were enrolled. Therapy included surgery, radiotherapy, and chemotherapy with cyclophosphamide, DTIC, and vincristine (CDV) for stage lll, and CDV or intensified regimen for stage IV. RESULTS: Of the 25 patients, 18 male and 7 female, 23 (92%) were<5 years of age at diagnosis. The most commonly appearing initial presentation was abdominal mass, which was observed in 14 patients. Abdominal primary was found in 21 patients (84%) including 17 (68%) of adrenal origin and mediastinal primary in 4 (16%). The 5Y-EFS was 44% in all study patients with 80% for stage lll and 20% for stage lV. The age< years, mediastinal primary, low serum NSE, and low serum ferritin were associated with better outcome. In stage lll, progression of disease was observed in only two, both with unfavorable prognostic factors. In stage lV, no difference in long-term survival was noted between those treated with CDV regmen and those with intensified chemotherapeutic regimen. CONCLUSIONS: Long-term event free survival (EFS) rate for stage III was 80% with poor outcome for stage lll including certain unfavorable prognostic factors and for stage lV, which might need more effective innovative therapies, such as autologous bone marrow transplantation or peripheral blood stem cell transplantation.
Bone Marrow Transplantation ; Child* ; Cyclophosphamide ; Dacarbazine ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Female ; Ferritins ; Humans ; Male ; Neuroblastoma ; Peripheral Blood Stem Cell Transplantation ; Radiotherapy ; Survival Rate* ; Therapies, Investigational ; Vincristine