• 대한세포병리학회지
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• 제5권1호
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• pp.74-78
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• 1994

A case of retroperitoneal paraganglioma is presented with fine needle aspiration cytologic features. A 57-year-old woman complained of abdominal discomfort and left flank pain for 2 years. The abdominal CT scan revealed an ovoid mass left to the abdominal aorta. Percutaneous fine needle aspiration was performed from the mass. The smear revealed cellular sheets or groups on hemorrhagic background. The tumor cells were ovoid, round to spindle shaped with mild to moderate cellular pleomorphism. The nuclei were round to ovoid and had evenly dispersed chromatin and small nucleoli. The cytoplasm was amphophilic, finely granular and poorly defined. Cells having large or spindle nuclei were quite frequently observed, however, mitosis was not present The cytologic findings suggested paraganglioma, but the frequent spindle cell pattern and the pleomorphism made it difficult to exclude other endocrine tumors and sarcomas. The clinical, histological and ultrastructural findings as well as cytologic findings contributed to confirmatory diagnosis.

• Advances in pediatric surgery
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• 제11권2호
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• pp.99-106
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• 2005

Neuroblastoma treatment remains challenging, but treatment has become more effective due to the establishment of clinical and biological variables that determine prognostic risks. Initially, stage and age were the prime determinants of survival used in clinical practice. Risk-based therapy currently is the hallmark of neuroblastoma treatment. This study reviews one center's experience with the management of neuroblastoma. Sixty-three patients with neuroblastoma were treated from 1989 to 2003. All patients were graded according to the International Neuroblastoma Staging System (INSS) at diagnosis. There were 37 boys and 26 girls. The median age was 2.14 years (range, 33 days-10.2years). The primary site was the adrenal gland in 47, dumbbell shape extending into spinal canal in 6, retroperitoneum in 5, mediastinum in 3, and other sites in 2. The probability of 5-year overall survival (OS) and event free survival (EFS) were 46.7 % and 44.2 % by Kaplan-Meier method. According to INSS, there were stage 1 in 2 cases, stage 2 in 5, stage 3 in 12, stage 4 in 42, and stage 4s in 2. There were statistically significant differences in the survival rates between patients with stage1, 2 and stage 3, 4(P<0.05). For the stage 3 and 4, the extents of surgical resection, determined from the operative records and pathologies, were complete resection in 17 cases, minimal residual in 15, and partial resection 11, and the 5-year OS rate was 57.8, 51.4, and 13.6 %, respectively. There is a trend toward higher OS with more complete resection (P<0.05). We conclude that age and stage at diagnosis are prognostic factors, and complete excision of the primary tumor can provide better prognosis for patients with stage 3 and 4 neuroblastoma.

• Advances in pediatric surgery
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• 제14권2호
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• pp.153-163
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• 2008

Inflammatory myofibroblastic tumor (IMT) is a rare reactive lesion characterized by the feature of myofibroblasts and a mixed inflammatory infiltrate that rarely undergoes malignant transformation. Extrapulmonary IMTs in children have been described involving the mesentery, omentum, retroperitoneum, abdominal soft tissues, liver, bladder, mediastinum, head and neck, extremity, appendix, and kidney. Medical records of children treated with abdominal IMT between 1985 and 2005 were reviewed retrospectively. Seven children were treated for IMT with the mean age of 3 y 2 m (range, 1 y 1 m to 14 y). Tumors were located in transverse mesocolon (n=2), omentum (n=1), porta hepatis (n=2), complex site (antrum, duodenum, common bile duct, porta hepatis) (n=2). The symptoms included abdominal mass, fever, jaundice, abdominal pain and anemia. The masses were excised totally in transverse mesocolon, omentum IMT and there is no evidence of recurrence (follow-up periods: 6 y 8 m, 8 y 9 m, 4 y 10 m). In porta hepatis IMT, liver transplantations were performed and there is no evidence of recurrence (follow period: 6 y 8 m, 8 y 7 m). In one case of complex site IMT, partial excision of mass was performed and he still survived with no change of the residual tumor during follow-up period. The other one of complex site IMT denied further treatment after the biopsy. In conclusion, complete surgical excision including liver transplantation and close follow-up are mandatory for the abdominal IMT in child.

• Advances in pediatric surgery
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• 제14권2호
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• pp.164-172
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• 2008

The survival rate for rhabdomyosarcoma (RMS) has significantly improved after the introduction of combined multimodality treatment. We report the 20-year treatment outcome of pediatric rhabdomyosarcoma in a single institution. The medical records of 16 patients treated for rhabdomyosarcoma between December 1986 and August 2007 at the Department of Pediatric Surgery, Seoul National University Children's Hospital, were retrospectively reviewed. Mean age at diagnosis was 7.1 years (range: 1.3 -14.2 years). Retroperitoneum was the most common primary site (n=7, 43.8 %), and embryonal type was predominant (n=11, 6 %). Before the treatment, most patients were in advanced TNM stage (stage III 50 %, IV; 25 %). The patient distribution according to the Intergroup Rhabdomyosarcoma Study Clinical Grouping System (IRS-CGS) was as follows; Group I 31.3 %, Group II 12.5 %, Group III 31.3 % and Group IV 25 %. Patients were classified into three groups according to the extent of resection of the primary tumor; complete resection (CR, n=5; 31.3 %), gross total resection (GTR, n=7; 43.8 %) and incomplete resection (IR, n=4; 25 %). Recurrence was observed in 9 patients (56.3 %) while there was no recurrence in CR patients. All patients with recurrence were identified as moderate or high-risk according to the IRS-V Risk Group. Pre-treatment TNM stage of RMS in our institution was advanced with aggressive clinical feature, however postsurgical conditions according to IRS-CGS were similar to the previous reports by IRS. This suggests that down-staging of IRS-CGS was achieved with multimodality treatment with CR or GTR. It also suggests that complete resection is the most important prognostic factor in the treatment of RMS in children. Patients classified as moderate or high-risk need close follow-up due to high recurrence rate. In case of localized recurrence, better outcome may be achieved with multimodality treatment including limited surgery.

• 대한골관절종양학회지
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• 제13권2호
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• pp.130-134
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• 2007

부신경절종은 발생학적으로 신경 능선 세포로부터 기원하는 종양으로 두경부에서 가장 많이 발생하나 드물게 종격동, 후복막 등의 내장 기관에서도 생기며 대부분 양성 경과를 보인다. 그 중 흉요추부의 경막내에 발생한 경우는 매우 드물며, 경추나 흉추보다는 요추부에 대부분 발생한다고 보고되었다. 지금까지 흉요추부의 부신경절종이 보고된 예는 드물기에 저자들은 부신경절종이 흉요추부의 경막내 수막외에 발생한 1예를 경험하여, 자기 공명 영상 및 조직학적 특징과 그 치료 결과를 문헌 고찰과 함께 보고하고자 한다.

• Advances in pediatric surgery
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• 제8권2호
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• pp.138-142
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• 2002

Mesenteric and omental cysts are rare intra-abdominal lesions in childhood, and may present various clinical features such as an asymptomatic mass or an acute abdomen. Therefore, these entities are frequently misdiagnosed preoperatively or are found only incidentally at operation for other conditions. We analyzed our experiences of 19 cases in a 19 year period from 1981 to 1999, at College of Medicine, Catholic University of Korea. There were 12 boys and 7 girls with a mean age of 4.8 years (range, 3 days to 15 years). Common presenting symptoms were abdominal pain (47%), abdominal distension (31%), abdominal mass (24%), vomiting (15%) and fever (10%). Ultrasonography was the most preferred method of diagnosis. Other diagnostic modalities include CT, MRI, and abdominal ascites tapping in selected patients. Location of the mesenteric cysts was small bowel mesentery in nine, the right mesocolon and retroperitoneum in one, the left mesocolon in one, and the jejunum, sigmoid-colon mesentery in one. Most of the patients underwent cyst excision, but six patients required concomitant bowel resection for complete removal of the lesions, and two patients underwent unroofing and simple aspiration respectively. There was one mortality case due to sepsis.

• Advances in pediatric surgery
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• 제8권2호
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• pp.95-100
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• 2002

Lymphangiomas or cystic hygromas are quite commonly seen in children. During a 22-year period, from January 1980 to December 2001, 117 patients with lymphangioma were treated and followed in the Department of Pediatric Surgery at Hanyang University Hospital. The male-to-female ratio was 1.9:1(77:40) with a male preponderance. As for the age incidence at time of diagnosis, 10(8.5 %) patients were noted under 1 month of age, 37(31.6%) were between 1 month and 1 year of age, 12(10.3%) between 1 and 2 years of age, so 59(50.4%) were under 2 years of age. Sixty one (52.1%) lymphangioma-cases were located in the neck, including one case at the nape. Axilla was the second in frequency and the rest were scattered at various sites. The intrascrotal lymphangioma is very rare but we have experienced one case of primary intrascrotal lymphangioma. Eighteen (15.4%) cases were located in the intraabdominal area, 10 in the mesentery, 2 in the greater omentum and 6 in the retroperitoneum. The chief complaints of intraabdominal lymphangioma were abdominal pain, intestinal obstruction, inguinal hernia, palpable mass, and/or abdominal distension. Among 77 histologically proven cases, 14 cases were cavernous lymphangiomas and the rest were cystic lymphangiomas. Bleeding in the lymphangioma was noted in 20(17.1%) cases of all. As for the treatment, a complete excision was performed in 77(65.8%) patients and $AgNO_3$ sclerotherapy after incomplete excision was performed in 23(19.7%). Picibanil (OK-432) sclerotherapy was performed in 17(14.5%) patients. Recurrence rate was 7.7 % and mortality occurred in one case who had a large neck lymphangioma extending into the mediastinum.

• Advances in pediatric surgery
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• 제13권2호
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• pp.169-178
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• 2007

Teratomas arising from totipotential primitive germ cell are composed of 2 or 3 germ cell layers. We reviewed the records of 166 children who underwent the operation for teratoma from Jan, 1990 through April, 2007. There were 40 boys and 126 girls (average age $6.93{\pm}5.83$ years). Primary sites were ovary (n = 88), sacrococcygeum (n = 24), testis (n = 16), retroperitoneum (n = 16), mediastinum (n = 8), brain (n = 4), thymus (n = 3) and a single tumor involved the adrenal gland, neck, middle ear, oropharynx, stomach, pelvis, omentum, chest wall and scalp. Teratomas were mature in 141 patients, and immature in 25. Six patients who had ovarian immature teratomas grade 2 or 3 with peritoneal gliomatosis underwent adjuvant chemotherapy. During follow up period, 6 mature teratomas recurred at sacrococcygeal area (n = 1) and contralateral ovary (n = 5). Five patients of them underwent reoperation and diagnosed as a mature teratoma, but one who had underwent a left salpingooophrectomy with right ovary cystectomy at initial operation was observed carefully. Teratomas were dominant in female patients and developed at various organs. Complete excision was needed for good prognosis. In case of immature teratoma, complete excision and appropriate chemotherapy according to grading can contribute to favorable results.

• Tuberculosis and Respiratory Diseases
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• 제41권6호
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• pp.658-662
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• 1994

저자등은 종격동에서 늑막삼출액과 함께 종물이 있는 소견을 보였던 환자에서 세포진검사 및 면역화학적 검사를 통하여 평활근육종으로 진단된 1예를 경험하였기에 문헌고찰과 함께 이를 보고하는 바이다.

• 대한기관식도과학회지
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• 제6권1호
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• pp.102-107
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• 2000

Monocytoid B-cell lymphoma is uncommon, low grade lymphoma originating from monocytoid B lymphocytes. Monocytoid B-cell lymphoma usually presents as a localized lymphadenopathy. Peripheral lymph nodes are most often involved, particularly those in the frequent in the head and neck area. A distinctive feature is the association of monocytoid B-cell lymphoma with autoimmune diseases. Sjogren Syndrome had been present in 22% of patient with monocytoid lymphoma. Extranodal involvement by monocytoid lymphoma was reported in the salivary gland, breast, thyroid, and stomach. There were also occasional extensions to the liver and retroperitoneum. The bone marrow and peripheral blood involvement by monocytoid lymphoma is very rare, which is frequently seen in hairly cell leukemia. Fever, weight loss, and other constitutional signs are usually absent. Most patients have no symptoms, and the only sign is enlarged lymph nodes. The clinical course remains indolent; most patients are in complete remission and recurrence with progression to a high-grade lymphoma of large cell type was recorded only in a few cases. Authors experienced a case of monocytoid B-cell lymphoma associated with Sjogren Syndrome mistaken to simple cervical lymphadenitis in a 60-year-old female. We report this case with a review of literatures.