Four children with solid and papillary epithelial neoplasm of the pancreas are reported. Three were girls. Mean age at operation was 12 years and 7 months (10-13 years). Clinical presentation included nausea, vomiting, and apalpable mass. One had hemoperitoneum due to tumor rupture. In two cases, tumors were in the body of the pancreas, and one the body and tail, and in one,the tail. Mean diameter of the tumors was 10.8 cm (8-15cm). Surgical procedures were distal pancreatectomy and splenectomy in 2 cases, distal pancreatectomy in one, and subtotal pancreatectomy and splenectomy in one. Mean follow-up period was 61 months (6-121 months). Three patients are still alive without any recurrence. However, in the one case of ruptured tumor, portal vein thrombosis and liver metastasis developed after subtotal pancreatectomy and splenectomy during the course of postoperative adjuvant chemotherapy.

A newborn male baby was transferred to our hospital with a left inguinal mass. The mass was huge measuring 10 x 10 cm, engorged, and dark-blue colored as a result of internal hemorrhage. Unstable vital signs were combined with DIC and acute renal failure. Emergency operation was performed because of the suspicion of bowel perforation. The peritoneal cavity was full of ascitis and the distal jejunum had a 0.5 cm perforation. Segmental resection of the jejunum and incisional biopsy of the inguinal mass were performed. On pelvic and thigh MRI, the mass protruded into pelvic cavity and encircled large vessels and nerves of the thigh. Pathologic diagnosis was congenital infantile fibrosarcoma. Fifteen days after operation, primary tumor excision was undertaken. The second look operation, performed after 6 times VAC chemotherapy, revealed no remained malignant cell on microscopic section. The baby has been followed closely for the last eight months.

The teratoma is a unique complex neoplasm and is one of the most frequent pediatric tumors originated from the extragonadal germ cells. Mature teratoma is composed of mature differentiated tissues, while immature teratoma always contains embryonic tissues of variable degrees of immaturity, especially in the neuroepithelial elements. Diagnosis of teratoma is relatively easy by conventional radiologic study, but the immaturity can be identified only by histopathological examination. Between January 1993 to December 2002, 63 cases of teratoma were operated and analysed retrospectively at the Chonnam University Hospital Female to male ratio was about 3:1 and age distribution was relatively even. Among 63 cases, gonadal teratoma was the most common (52.4%), followed by sacrococcygeal (25.4%), retroperitoneal (9.5%) and mediastinal teratoma (9.5%). Fifty-six cases were mature teratomas and seven were immature teratomas. Alpha-fetoprotein (AFP) was elevated in 4 of 6 immature cases, but in 2 of 51 mature ones Elevated AFP progressively returned to normal range by 1 month after operation in all. Complete excision of the mass was performed, and major complication was not noticed. In five immature cases, PEB chemotherapy (Cisplatin, Etoposide, Bleomycin) was performed. Two of 2 cases in histological grade II were well tolerated to the aggressive chemotherapy. One of three cases in grade III expired due to severe bone marrow depression, and two of them expired by tumor recurrences. In conclusion, immature teratoma in histological grade III showed high potentiality of recurrence. Therefore, postoperative chemotherapy has to be applied to the high graded immature tumors.

The purpose of our study was to evaluate children who underwent hepatic resection for primary malignant hepatic tumor in the period from January 1994 to December 2001. A total of 8 patients, seven with hepatoblastoma (HB) and one with hepatocellular carcinoma (HCC). were studied. One HCC was respectable at the initial diagnosis, but five cases of unresectable HB received two cycles of transarterial chemoembolization (TACE) before operation. One patient with an unresectable HB with bone marrow metastasis was operated after one cycle of TACE and one cycle of systemic chemotherapy based on CCG-823F protocol. All 6 patients who underwent TACE and neoadjuvant chemotherapy showed marked redcuction in tumor volume and a clear outline of the lesion. Major complication was not noticed. Mean alpha-fetoprotei (alpha-FP) level at diagnosis, after neoadjuvant chemotherapy and after postoperative chemotherapy was 9,818 (42-35,350), 664, and 10.1 ng/mL, respectively. Half life of the alpha-FP after complete resection was 5.1 days (3.0-8.7 days). Median follow up period was 57.1 months (10-97 months) and all the patients are alive with NED. In conclusion, preoperative chemotherapy, especially TACE, is effective, safe, and useful to treat initially unresectable hepatoblastoma, and serial level of the serum alpha-FP is a useful tumor marker for diagnosis and monitoring therapeutic responses.

In hepatoblastoma, encouraging cure rates have been achieved with recent advances in chemotherapy and surgical techniques. The aim of this study is to evaluate the role of combined therapeutic modalities and surgical resection in hepatoblastoma. Fifteen cases of hepatoblastoma were treated from January 1993 to August 2000. Six patients had resectable tumors at initial diagnosis. All underwent surgical resection and in four patients postoperative adjuvant chemotherapy was needed. Nine out of 15 patients had unresectbale tumors at initial diagnosis, and preoperative chemotherapy was applied. There was one operative mortality and 14 patients showed good prognosis after surgery. Although various treatment modalities should be combined for the unresectable hepatoblastoma, surgical resection remains the major curative procedure.

Neuroblastoma derived from neural crest cells of the sympathetic nervous system, is the most common extracranial solid tumor in children with many interesting features. It has the highest rate of spontaneous regression, yet has one of the poorest outcome results. Recent updates have shown much increased outcomes in multitreatment approaches and early diagnosis with screening. Authors have studied 42 patients who have been diagnosed and treated in the last decade in the Severance and Yongdong Severance Hospital respectively. They were followed up till January 1998 and was analyzed in terms of age, sexual difference, admission period, staging, diagnostic studies, clinical symptoms and physical examinations, operative time, treatment modalities, survival rate. Twenty eight patients underwent operative procedures, 16 patients had postoperative chemotherapy, and 19 patients had preoperative chemotherapy. Sexual difference was 1.33:1 in favor of males, and 43% of patients were under the age of one year. The patients were initially diagnosed symptomatically, and the abdominal mass was the most common clinical symptom. Eighty six percent of the patients were of advanced stages (>Stage III of INSS), and the 2 year survival rate showed 59.2% and the 5 year survival rate was 29.6%.

Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage. With the neoajuvant chemotherapy, improved resectability and survival have been reported. Twenty children with biopsy proven hepatoblastoma were treated during the period between January 1987 and June 1995. Median age at diagnosis was 13 months(2 months to 7 year and 10 months), and 13 were male. Histologic profile was 13 epithelia1(5 fetal, 4 mixed, 1 embryonal, 3 undetermined), and 5 mixed mesenchymal and epithelial and 2 of undetermined type. Chemotherapy effectively reduced the tumor volume(p=0.008), and' was able to convert 7 out of 9 initially unresectable cases(78%) to resectable ones. Twelve radical and 2 palliative operations were done with or without adjuvant chemotherapy. The Median follow up period was 33 months and the median survival was 26 months. The group with curative resection had a 61.1 % 5 year survival rate, but none of palliative resection grpup survived more than 13 months(p=0.000l). In univariate analysis for prognostic factors revealed, large tumor size at diagnosis and abscence of thrombocytopenia were associated with poor survival, but these differences were not statistically significant. Histological pure fetal type did not mean a better prognosis. Even with a recent neoadjuvant chemotherapy, the strategy should be focused on the radical resection as early as possible.