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1.  Hepatoblastoma: 15-year experience and role of surgical treatment 
Purpose
Hepatoblastoma is the most common malignant liver tumor in children. The aim of this study was to review our results of hepatoblastoma treatment and to determine the role of surgical treatment in hepatoblastoma.
Methods
This is a retrospective clinical study. The medical records of patients with hepatoblastoma, treated between October 1994 and October 2009, were reviewed. The patients were classified according to the pretreatment extent of disease (PRETEXT) grouping system. The main outcome variable was survival. Secondary outcome variables were complete, partial and no response to chemotherapy and surgery, when indicated.
Results
Twenty-seven patients were treated during the observation period. Eighteen were males. Five were PRETEXT group I, 8 group II, 13 group III and 1 group IV. Complete excision was achieved in all patients except in one case that underwent liver transplantation (group IV). Median follow-up and survival rate were 2.3 years and 100%, 6.6 years and 75%, 5.8 years and 92%, 7.7 years and 100%, for groups I to IV, respectively. Twenty patients are currently considered to be in complete response status and three patients are receiving postoperative chemotherapy. Four patients died; the causes of death were cytomegalovirus hepatitis, bone marrow suppression during adjuvant chemotherapy, primarynonfunction after the transplantation for recurrent tumor and metachronous rectal cancer, respectively.
Conclusion
Favorable long-term outcome could be expected for hepatoblastoma with complete tumor excision and adjuvant chemotherapy.
doi:10.4174/jkss.2011.81.2.134
PMCID: PMC3204570  PMID: 22066113
Hepatoblastoma; Surgery; Drug therapy; Transplantation
2.  Fibrous hamartoma of infancy: an experience of a single institute 
Purpose
Fibrous hamartoma (FH) of infancy is a distinctive fibrous growth that most frequently occurs at birth and during the postnatal period. It is important for clinicians and pathologists to recognize this entity to avoid an aggressive approach.
Methods
We herein describe the clinicopathologic features of 9 FHs diagnosed at a single institution between 1997 and 2010.
Results
There were 7 boys and 2 girls, and the mean age of presentation was 14.7 months. The common locations were the lower back and gluteal region (n = 3) and scrotum (n = 2). They were solitary lesions, and measured 1.0 to 7.0 cm in maximum diameter (mean, 4.9 cm). The excised masses tended to be poorly circumscribed, and consisted of an intimate mixture of firm, gray-white tissue with fat. Histologically, these lesions were composed of 3 components forming a vague, irregular, organoid pattern: well-defined intersecting trabeculae of fibrocollagenous tissue; loosely textured areas of small, rounded, primitive mesenchymal cells; and mature fat. Over a median follow-up of 72 months, no patient showed recurrence.
Conclusion
FH should be distinguished from other forms of fibromatosis and malignant tumors because it is benign and usually cured by local excision.
doi:10.4174/jkss.2011.81.1.61
PMCID: PMC3204559  PMID: 22066102
Hamartoma; Infant; Soft tissue neoplasms; Differential diagnosis; Fibromatosis
3.  Desmoplastic small round cell tumor of the stomach mimicking a gastric cancer in a child 
Journal of the Korean Surgical Society  2011;80(Suppl 1):S80-S84.
Intra-abdominal desmoplastic small round cell tumor (DSRCT) is a highly malignant tumor of uncertain histogenesis. Here we report a case of DSRCT involving the stomach, initially misdiagnosed as gastric cancer. A 12-year-old boy presented with upper abdominal pain developed 1 month prior. On gastroscopy, a 7-cm mass was noted involving the esophago-gastric junction to the fundus, and positron emission tomography showed multiple hot uptakes suggesting distant metastasis. Gastroscopic biopsy showed poorly differentiated malignant cells. We diagnosed as stage IV gastric cancer and treated with 6 cycles of chemotherapy. Laparotomy revealed a huge gastric mass along with peritoneal disseminations. Palliative proximal gastrectomy was performed. Pathological examination revealed transmural involvement of DSRCT, and t(11;22)(p12;q12) was demonstrated on fluorescence in situ hybridization test. The chemotherapeutic regimen was changed and the patient underwent 8 additional cycles of post-operative chemotherapy. The patient is now alive and the residual tumor shows no significant changes after chemotherapy.
doi:10.4174/jkss.2011.80.Suppl1.S80
PMCID: PMC3205364  PMID: 22066092
Stomach; Desmoplastic small round cell tumor; Children
4.  Graft-versus-host disease after kidney transplantation 
Journal of the Korean Surgical Society  2011;80(Suppl 1):S36-S39.
Graft-versus-host disease (GVHD) is a rare complication after kidney transplantation. We describe a 62-year-old female with end-stage renal disease due to hypertension. She received a kidney with 4 mismatched human leukocyte antigen (HLA) out of 6 HLA - A, B, DR from a deceased donor. After the procedure, the patient showed watery diarrhea on postoperative day (POD) 45. An endoscopic biopsy of the colon revealed some apoptotic cells consistent with GVHD. Thrombocytopenia was gradually developed on POD 54. She received steroid pulse therapy, and thrombocytopenia did not progress. However, pneumonia, renal failure, and cardiac failure occurred. She died due to multiple organ failure. We must consider GVHD in renal transplant recipients without homozygous or identical HLA, who had only watery diarrhea without other typical GVHD symptoms such as skin rash and fever, although GVHD is rare in renal transplant recipients.
doi:10.4174/jkss.2011.80.Suppl1.S36
PMCID: PMC3205370  PMID: 22066080
Graft-versus-host disease; Human leukocyte antigen; Kidney donation; Kidney transplantation
5.  Pneumatosis intestinalis after adult liver transplantation 
Journal of the Korean Surgical Society  2011;80(Suppl 1):S47-S50.
Pneumatosis intestinalis is an uncommon disorder characterized by an accumulation of gas in the bowel wall. We described three cases undertaking liver transplantation. The patients developed diarrhea in three cases and high fever in two. An abdominal X-ray and computed tomography scan demonstrated extensive pneumatosis intestinalis in the colon with pneumoperitoneum mimicking hollow organ perforation. However, the patients had no abdominal symptoms and there was no evidence of peritonitis. The infection work-up was negative except one case with cytomegalovirus antigenemia. After one week of conservative management including bowel rest and antibiotic therapy, their pneumoperitoneum resolved spontaneously without any complication. Pneumatosis intestinalis should be considered as a differential diagnosis after adult liver transplantation with patients suffering from watery diarrhea and fever. Pneumoperitoneum, air-density in mesentery and retroperitoneum in patients with pneumatosis intestinalis without signs of peritonitis improved with conservative management, which included bowel rest and antibiotic therapy.
doi:10.4174/jkss.2011.80.Suppl1.S47
PMCID: PMC3205374  PMID: 22066083
Liver transplantation; Pneumatosis intestinalis; Watery diarrhea
6.  Cardiac tamponade caused by tuberculosis pericarditis in renal transplant recipients 
Journal of the Korean Surgical Society  2011;80(Suppl 1):S40-S42.
A 50-year-old male, renal transplant recipient, was admitted with fever and chest discomfort. At admission, chest radiologic finding was negative and echocardiography showed minimal pericardial effusion. After 2 days of admission, chest pain worsened and blood pressure fell to 60/40 mmHg. Emergency echocardiography showed a large amount of pericardial effusion compressing the entire heart. Pericardiocentesis was performed immediately. Mycobacterium tuberculosis was isolated from pericardial fluid. Tuberculosis pericarditis should be considered as the cause of cardiac tamponade in renal transplant recipients, even with the absence of pericardial effusion in the initial study or suggestive history.
doi:10.4174/jkss.2011.80.Suppl1.S40
PMCID: PMC3205375  PMID: 22066081
Pericardiac tamponade; Renal transplantation; Tuberculosis; Tuberculosis pericarditis

Results 1-6 (6)