• Advances in pediatric surgery
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• v.5 no.1
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• pp.45-52
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• 1999

Pediatric solid tumors have many histologic similarity. These tumors contained small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the differentiation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumors. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is a useful tool for rapid and objective diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.

• Advances in pediatric surgery
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• v.12 no.2
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• pp.221-231
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• 2006

Solid pseudopapillary tumor (SPT) of the pancreas occurs most frequently in the second or third decades of life, and is prevalent in females. Unlike other pancreatic malignancy, SPT usually has a low malignancy potential. This study reviews our clinical experience and surgical treatment of pancreatic SPT. Admission records and follow-up data were analyzed retrospectively for the period between January 1996 and January 2003. Five patients with a pancreatic mass were operated upon and SPT was confirmed by pathology in each case. The male to female ratio was 1: 4. The median age was 13.8 years. Findings were vague upper abdominal pain (n=5, 100 %) and an abdominal palpable mass (n=3, 60 %). The median tumor diameter was 6.8cm and the locations were 2 in the pancreatic head (40 %) and 3 in the pancreatic tail (60 %). Extra-pancreatic invasion or distant metastasis was not found at the initial operation in all five cases. A pyloruspreserving pancreaticoduodenectomy (n=1) and a mass enucleation (n=1) were performed in two patients of pancreatic head tumors. For three cases of tumors in pancreatic tail, distal pancreatectomy (n=2) and combined distal pancreatectomy and splenectomy (n=1) were performed. The median follow-up period was 60 months(12-117month). During the follow-up period, there was no local recurrence, nor distant metastasis. Postoperative adjuvant chemotherapy or radiotherapy was not carried out. All five children were alive during the follow up period without any evidence of disease relapse. SPT of the pancreas in childhood has good prognosis and surgical resection of the tumor is usually curative.

• Advances in pediatric surgery
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• v.11 no.1
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• pp.40-45
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• 2005

Solid pseudopapillary tumor of pancreas in children is a tumor with low malignant potentiality. and is rarely associated with distant metastasis. A 13-year-old girl was hospitalized because of abdominal pain of one week duration. Abdominal CT revealed not only a $12{\times}6cm$ sized mass at the pancreatic body and tail but also a 1cm sized mass in left lobe of the liver. The patient underwent a near-total pancreatectomy and tumorectomy of the liver. A solid pseudopapillary tumor with liver metastasis was confirmed by pathology. She has undergone 13 courses of chemotherapy and has been well for 13 monthswithout any sign of recurrence.

• Advances in pediatric surgery
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• v.15 no.2
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• pp.180-185
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• 2009

We report a case of nonfunctioning neuroendocrine tumor of the pancreas in a 15-year-old girl who presented with back pain. On physical examination, there was mild tenderness in the left upper quadrant of the abdomen. The patient had no pancreatic hormone-associated symptoms. An abdominal ultrasonography showed a well-demarcated hypervascular solid mass with calcification in the tail of the pancreas. An abdominal computed tomography scan showed a $6{\times}5cm$ sized well-encapsulated enhancing solid mass with cystic component in the tail of the pancreas. Distal pancreatectomy was performed. Pathology revealed awelldifferentiated nonfunctioning low grade malignant neuroendocrine tumor of the pancreas. The postoperative course was uneventful.

• Advances in pediatric surgery
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• v.15 no.1
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• pp.73-79
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• 2009

Sertoli-Leydig cell tumor is a rare sex-cord stromal tumor of the ovary. They make up less than 0.5 % of all ovarian tumors. We experienced a case of an ovarian Sertoli-Leydig cell tumor in a 4 year-old girl who presented with nausea, vomiting, and lower abdominal pain of 2 days' duration. On physical examination, there was mild tenderness in the right lower quadrant of the abdomen. Abdominal ultrasonography and computed tomography (CT) scan revealed a pelvic mass measuring $5{\times}3cm$ that appeared to arise from the right ovary. At exploratory laparotomy, a $6{\times}5{\times}3cm$ solid right ovarian mass without torsion was found. A right salpingo-oophorectomy was performed. The postoperative course was uneventful. The child was discharged 5 days after surgery.

• Neonatal Medicine
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• v.25 no.1
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• pp.23-28
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• 2018

Purpose: Abdominoperineal solid tumors presenting in neonates often require surgical intervention during the neonatal period. Although we report our single-center experience, this study would be meaningful to understand the clinical implications of these neoplasms. Methods: We retrospectively reviewed and analyzed the clinical data and characteristics of 22 patients (${\leq}28$ days old) diagnosed with histopathologically confirmed abdominoperineal solid neoplasms (benign or malignant) after surgical resection. Results: The mean gestational age and postnatal age at the time of operation were $38.3{\pm}1.8weeks$ and $13.5{\pm}8.3days$, respectively. Most patients (18/22, 81.8%) were diagnosed during antenatal care visits; however, 4 (18.2%) were identified after birth. The mean tumor size was $6.4{\times}5.3cm$ (3.5-17.0 cm), and tumors occurred most frequently within the sacrococcygeal region (8/22, 36.4%). Histopathologically, 14 patients (63.6%) demonstrated benign tumors and 8 (36.4%) demonstrated malignant tumors. Germ cell tumors and hepatoblastomas were the most commonly observed tumors. Fortunately, all patients showed a localized pattern of tumor involvement without distant metastasis. No recurrence or mortality was observed during the follow-up period (mean $66.4{\pm}44.2months$). Conclusion: Abdominoperineal solid tumors occurring in neonates show variable clinical patterns during the antenatal and postnatal monitoring/screening periods. We conclude that aggressive and multidisciplinary approaches could achieve good clinical results in these patients.

• Advances in pediatric surgery
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• v.12 no.1
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• pp.32-40
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• 2006

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.

• Advances in pediatric surgery
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• v.5 no.1
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• pp.58-63
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• 1999

Neuroblastoma is a solid tumor derived from neural crest cells of the sympathetic nervous system. It is the most common extracranial solid tumor in children. Although it has the highest rate of spontaneous regression, it has a bad prognosis. Recent reports indicate a much improved outcomes utilizing the multitreatment approaches and early diagnosis as a result of patient screening. We have studied 42 patients managed in the last decade at the Severance and Yongdong Severance Hospitals. The patients were followed until January 1998 and analyzed in terms of age, sex, admission period, stage, diagnostic studies, clinical symptoms, physical findings, operative time, treatment modalities, and 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 one year of age. The patients were initially diagnosed as a result of symptoms and signs. An abdominal mass was the most common clinical finding. Eighty six percent of the patients were in advanced stages (>Stage III of INSS). The 2 year survival rate was 59.2 % and the 5 year survival rate was 29.6 %.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.2
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• pp.116-120
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• 2014

Inflammatory myofibroblastic tumor (IMT) is rare mesenchymal solid tumor that consists of proliferating myofibroblasts with an inflammatory infiltrate background. It has a very low prevalence in infants and occurs mainly in children and young adults. IMT are mainly located in the thoracic cavity, but intra-abdominal lesions are rare. IMT can exhibit locally aggressive neoplastic processes and metastases similar to malignancies, so, have clinical importance. Herein, we describe two infantile intra-abdominal IMT cases presenting with incidentally found palpable abdominal mass. A 4-month-old male infant had IMT at the ileal mesentery and a 5-month-old male infant had IMT at liver. Both cases were successfully treated by complete surgical resection without complication or recurrence. Considering the biological behavior of the intermediate type of neoplasm in IMT, we expect good survivals when achieving appropriate surgical resection without adjuvant therapy in infantile intra-abdominal IMT.

• Advances in pediatric surgery
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• v.6 no.2
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• pp.134-138
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• 2000

Solid and papillary cystic neoplasm of pancreas is an uncommon low grade malignant tumor. It is predominant in young female between the second and third decades of life, and amenable to cure by surgical treatment. The authors report two cases of solid and papillary neoplasm of pancreas pathologically verified at Kyung Hee University Hospital. The first case was an 11-years old female patient and the other case was a 12-years old male. Symptoms were abdominal discomfort, nausea and vomiting in both cases and abdominal pain in the female patient. CT finding included a solid and papillary neoplasm of pancreas. The mass was well-demarcated with solid and cystic necrosis components. In the female patient, a large hematoma was found. Gross findings revealed apparent encapsulation, cystic degeneration and hemorrhagic necrosis. Microscopically the tumors were characterized by distinctive solid and papillary patterns of cellular arrangement without local invasion. Both patients were discharged after surgery and followed up without any problem.