- Differential Expression of Promyelocytic Leukemia Protein in Autoimmune Liver Diseases.
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Hyun Jung Kim, Jung Sun Kim, Yong Sang Lee, Young Hwa Chung, Han Joo Lee, Dong Jin Suh, Chong Jai Kim, Eunsil Yu
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Korean J Pathol. 2004;38(6):357-363.
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- BACKGROUND
Promyelocytic leukemia protein (PML) is a primary biliary cirrhosis (PBC)-specific autoantigen.
Anti-PML antibody is analyzed using cultured cells with patient sera, however, PML expression has rarely been examined in liver tissues.
METHODS
In the present study, PML expression was examined immunohistochemically in paraffin embedded liver needle biopsy specimens obtained from 20 cases of PBC, 10 cases of autoimmune cholangitis, 36 cases of autoimmune hepatitis and from 5 cases of noninflammatory livers.
RESULTS
Variable PML immunopositivity was detected in the bile duct epithelial cells of 18 (90.0%) of 20 PBC cases and in all 10 cases (100.0%) of autoimmune cholangitis, whereas it was only present in 6 (16.7%) of 36 cases of autoimmune hepatitis (p<0.001). In contrast, hepatocyte PML immunopositivity was higher in autoimmune hepatitis (33/36 cases, 90.8%), than in PBC (10/20 cases, 50.0%) or autoimmune cholangitis (3/10 cases, 30.0%) (p<0.05).
CONCLUSION
Our data indicate that the differential expression of PML is closely related to autoimmune liver diseases type, and suggest that the overexpression of PML protein in bile duct cells is associated with the development of autoantibodies in patients with PBC or autoimmune cholangitis. Furthermore, PML immunoreactivity may be useful for the diagnosis of autoimmune cholangitis and overlap syndrome.
- Dedifferentiated Chondrosarcoma with Giant Cell-rich Sarcomatous Component Resembling Giant Cell Tumor: A Case Report.
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Pil Gyu Hwang, Jae Kyung Won, Min A Kim, Han Soo Kim, Sang Hoon Lee, Chong Jai Kim
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Korean J Pathol. 2004;38(5):345-349.
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- Dedifferentiated chondrosarcoma is an uncommon bone tumor, defined as a tumor in which two components -a low-grade chondrosarcoma and a high-grade non-cartilaginous sarcoma-coexist with abrupt interface. We report a rare case of giant-cell rich dedifferentiated chondrosarcoma occurred in the right distal femur shaft of a 60 year-old female. The plain X-ray film showed an irregular radiolucent mass. The T2-weighted MRI revealed a heterogeneous high signal intensity. It was an irregular mass composed of bluish-white, translucent chondroid elements and yellowish solid components with extraosseous invasion.
Microscopically, a low-grade chondrosarcoma and a giant-cell rich spindle cell sarcoma with areas resembling giant cell tumor were recognized with abrupt transition.
Immunohistochemical staining revealed a S100 protein positivity in chondroid cells and a few spindle cells. CD68 was strongly positive in giant cells. Vimentin was positive in both components and smooth muscle actin was positive in some spindle cells. There was no cytokeratin, desmin and myogenin immunopositivity. It is important to be aware of this rare variant of dedifferentiated chondrosarcoma to avoid the misdiagnosis of more common bone tumors including giant cell tumors.
- Bilateral Mammary Metastasis of Alveolar Soft Part Sarcoma: A Case Report.
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Soo Youn Cho, Ho Chang Lee, Chong Jai Kim, Min Suk Kim, Sun Hoo Park, Eui Keun Ham, In Ae Park
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Korean J Pathol. 2003;37(5):365-368.
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- An alveolar soft part sarcoma(ASPS) is a rare malignant soft tissue tumor, which metastasizes to the lung, bone and brain. Recently, we encountered an unusual case of a metastatic ASPS to the bilateral breasts in a 27-year-old woman. She had undergone surgery for an ASPS in her right thigh two years ago, which metastasized to the breast on three occasions, 15 months, 20 months and two years after surgery.
- Undifferentiated Sarcoma of the Liver: Clinical and Pathologic Study of 9 Cases.
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Kyung Chul Moon, Chong Jai Kim, Je G Chi, Gyeong Hoon Kang
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Korean J Pathol. 2003;37(1):50-57.
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- BACKGROUND
Undifferentiated sarcoma of the liver (USL) is a rare malignant tumor that is found in children and young adults.
METHODS
We performed a clinicopathologic analysis of 9 cases (M:F=4:5) of USL using immunohistochemical staining for vimentin, desmin, -smooth muscle actin (SMA), CD68, CD117, S-100, cytokeratin, epithelial membrane antigen (EMA), and p53.
RESULTS
Grossly, the tumors were large, single, and well demarcated with areas of hemorrhage and necrosis.
Microscopically, the tumors were composed of spindle to stellate cells and variable numbers of multinucleated giant cells with a myxoid background. The tumors had eosinophilic globules, small cystic spaces and fibrous pseudocapsule.
Under immunohistochemical study, the tumor cells were positive for vimentin, CD68 and desmin, but negative for S-100 protein. p53 overexpression was noted in most cases, and four cases showed immunoreactivity for CD117. All patients received chemotherapy before or after the excision of the tumors. Two patients died during chemotherapy, but six patients survived without recurrence for 18, 35, 53, 57, 65 and 126 months after the initial diagnosis. The remaining one patient survived with recurrence for 20 months after the initial diagnosis.
CONCLUSION
Our cases showed unique pathological and immunohistochemical features similar to the cases of previous reports. In contrast to the previous reports, the outcome of our cases were not poor.
Modern multimodal treatment including surgical resection combined with multiagent chemotherapy may contribute to the better prognoses.
- Mesenchymal Hamartoma of the Liver in Beckwith-Wiedemann Syndrome: A Case Report.
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Seong Ho Yoo, Hyo Jin Park, Soo Yoen Cho, Chong Jai Kim
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Korean J Pathol. 2002;36(6):425-428.
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- Beckwith-Wiedemann syndrome is a well-known overgrowth syndrome associated with the presence of a wide variety of anomalies and increased risk of cancers. Less frequently, benign neoplasms also develop. We report a female infant with Beckwith-Wiedemann syndrome who developed a mesenchymal hamartoma of the liver. The patient was born with macroglossia, ear lobe crease, and abdominal distension.
Laboratory data showed hypoglycemia, and magnetic resonance image revealed both adrenal enlargement, enhancing mass of the pancreas, and multiple hepatic nodules. The histologic findings of the resected distal pancreas and both adrenals were those of Beckwith-Wiedemann syndrome. Microscopic findings of the liver biopsy specimens were compatible with mesenchymal hamartoma. Hamartoma of the urinary bladder, cardiac fibrous hamartoma, and mixed hamartoma of the liver have been documented previously in association with Beckwith-Wiedemann syndrome. However, to our knowledge, this is the first case report of hepatic mesenchymal hamartoma in Beckwith-Wiedemann syndrome. Because of the paucity of hamartomas in childhood, we should be cautious of other features of Beckwith-Wiedemann syndrome and the present case extends the spectrum of tumor formation in this syndrome.
- Intestinal Metastasis of Osteosarcoma Presenting with Intussusception: A Case Report.
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Seong Ho Yoo, Min A Kim, Kyu Joo Park, Joon Koo Han, Sang Hoon Lee, Chong Jai Kim, Eui Keun Ham
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Korean J Pathol. 2002;36(4):271-273.
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- Metastatic osteosarcoma most commonly affects the lungs and other bones. Intestinal intussusception caused by metastatic osteosarcoma is very rare. We report a case of metastatic osteosarcoma of the intestine in a 39-year-old female. She underwent surgical resection of the left femur due to osteosarcoma and received additional chemotherapy 3 years ago. Pulmonary metastasis was found two years later and the patient complained of abdominal pain, nausea and vomiting after 8 months following excision of the lung nodules.
Abdominal computed tomography revealed intussusception with a suspected polypoid mass in the distal portion of the jejunum. The histologic findings of the resected bowel were those of osteosarcoma. This is the first case of documented intestinal metastasis of osteosarcoma in Korea. It is suggested that the tumor metastasis to the small intestine should be considered in patients with previous osteosarcoma, when the patient presents with acute abdominal symptoms and intussusception.
- Juvenile Hyaline Fibromatosis in an Adult.
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Young A Kim, Seoung Wan Chae, Chong Jai Kim, Je G Chi
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Korean J Pathol. 2000;34(3):239-242.
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- Juvenile hyaline fibromatosis is a rare disorder probably inherited as an autosomal recessive trait. It is characterized by multiple slowly growing subcutaneous nodules, hypertrophy of gingiva, flexion contracture, and radiolucent bone destruction.
The histological features of the tumor-like lesions are characterized by the deposition of amorphous hyaline material in which spindle shaped cells are embedded. We report a case of juvenile hyaline fibromatosis in a 26 year-old-woman. She had multiple subcutaneous nodules in scalp, ear, forearms, right knee, and back. Surgical excision of the tumors in the scalp and ear was done. The largest one measured 13 9 6 cm, and had homogeneous, grayish yellow cut surface with calcification. Light microscopic examination showed abundant eosinophilic hyaline material with extensive calcification and metaplastic bone formation. Spindle cells were rarely observed at the periphery of the tumor. Hyaline matrix was PAS positive, diastase resistant, and alcian blue negative.
Scattered spindle cells were positive for vimentin but negative for S-100 protein and smooth muscle actin. There were many reports regarding early lesions of juvenile hyaline fibromatosis; however in this patient, tumor existed for more than 20 years and the histology was somewhat different from the early lesions reported in the literature.
- Placental Metastais of Maternal Gastric Adenocarcinoma: A case report.
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Hye Seung Lee, Young Ah Kim, Chong Jai Kim, Je Geun Chi
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Korean J Pathol. 1999;33(3):214-216.
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- Metastasis of a maternal neoplasm to the products of conception is extremely rare. About 50% of the reported cases were not examined grossly or had no grossly visible tumor deposit. A case of placental micrometastasis of gastric adenocarcinoma in a 28-year-old woman is presented.
Artificial termination was performed in 22 weeks of gestation and a female fetus weighing 440 gm was delivered.
The placenta weighed 220 gm and was grossly normal.
Microscopic examination revealed a small number of micrometastasis scattered in the intervillous space.
- The Current Practice of the Autopsy Services and the Autopsy Records at the Seoul National University Hospital.
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Jeong Wook Seo, Yoon Sung Lee, Je Geun Chi, Ghee Young Choe, Soong Deok Lee, Chong Jai Kim, In Ae Park, Woo Ho Kim, Ja June Jang, Chul Woo Kim, Seong Hoe Park, Jung Bin Lee, Hyun Soon Lee, Yong Il Kim, Eui Keun Ham, Sang Kook Lee
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Korean J Pathol. 1998;32(6):453-459.
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- This study outlines the current status of the autopsy practice and the medical records for autopsies at the Department of Pathology, Seoul National University Hospital.
Total number of autopsy cases from 1954 to 1995 was 3,131.
Adults aged over 17 were 371 cases and children were 2,515 cases. The demographic data in 245 cases was not available.
The number of adult autopsies and its proportion among total number of autopsies during 10-year periods decreased from 144 cases (40%) during the 10-year-period from 1956 to 52 cases (3%) during the 10-year-period from 1986. The number of children cases during the same period groups increased slightly from 210 cases (58%) to 393 cases (25%). But the number of fetal cases increased rapidly from 7 cases (2%) to 1,146 cases (72%). Among fetal autopsies the proportion of fetuses died earlier than 24 weeks of gestation increased and this figure exceeds that of fetuses that died later than 24 weeks of gestation from 1992. Forty percent of the cases were submitted from the clinical departments of the Seoul National University Hospital but the remainders were referred from 73 hospitals. Final autopsy diagnoses were analysed according to the Korean Standard Classification of Disease (KCD)-3 coding system and by searching key words for all cases. Common diagnoses as coded among cases from 1990 were P9, P0, P2, Q2 and Q0. Common diseases by key words for adult cases were liver disease, tuberculosis and pneumonia.
Common diseases for children cases were pneumonia, hyaline membrane disease, meningitis and tuberculosis. Through this study we could show the importance of autopsy services for fetuses. We could also establish a regular registration system for autopsies at general hospitals.
- Congenital Cystic Disease of the Kidney overview and a classification.
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Mee Joo, Yeon Mee Kim, Chong Jai Kim, Yeon Lim Suh, Jeong Wook Seo, Je Geun Chi
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Korean J Pathol. 1997;31(3):233-243.
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- The congenital renal cystic disease encompasses a complex group of pathologic and clinical entities. We retrospectively reviewed 42 cases of congenital renal cystic lesions classified into four Potter types in a series of 2,063 consecutive autopsies from 1981 to 1996. According to our study based on morphologic, clinical, genetic features and associated anomalies, type I and III are relatively compatible with Potter's original definition. However, it was reasonable that type II and IV are classified to the same group because of: 1) very similar histologic findings representing dysplastic kidney, 2) many associated anomalies, 3) no evidence of inheritance, and 4) presence of a combined type. Syndrome associated cysts, such as Meckel-Gruber syndrome, were also separately classified. If the dysplastic evidence was insufficient for diagnosis to the dysplastic kidney in type II and IV, then these cases would be better classified into a cystic disease associated with congenital hydronephrosis. We propose a classification of the congenital cystic disease of the kidney to be: 1) dysplastic kidney, 2) cystic disease associated with congenital hydronephrosis, 3) polycystic kidney, and 4) syndromic cystic disease.
- Congenital Anomalies Observed by Autopsies at the Seoul National University Children's Hospital.
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Jin Haeng Chung, Jeong Wook Seo, Chong Jai Kim, Chul Woo Kim, Je G Chi
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Korean J Pathol. 1997;31(2):93-99.
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- A retrospective analysis was performed on the 968 cases of fetal or pediatric autopsies over five year period (1990-1994), at the Seoul National University Children's Hospital. Age/mode distribution of cases were artificial abortus(30.6%), spontaneous abortus(12.0%), stillbirth(21.9%), neonates(29%), infants(2.8%) and children(0.9%). Male/female ratio was 1.21. Overall incidence of congenital anomalies was 60.8% and 34.0% of all cases had anomalies involving multiple organ systems.
Percentage of cases with any anomaly was 71.6% in artificial abortus, 35.3% in spontaneous abortus, 59% in still births, 65.5% in neonates and 38.9% in infant and children. Common organ systems involved were the cardiovascular system (39.0%), musculoskeletal system (23.6%), nervous system (22.6%), gastrointestinal system (19.9%), and urinary system (14.6%). From these results, we found that the congenital anomalies were most significant diseases of the perinatal period and the cardiovascular anomalies were the most common anomalies of them.
- Incidence of Acute Placental Inflammation through Histopathological Analysis: One year experience in 1995 at Seoul National University Hospital.
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Hyun Ju Yoo, Yun Kyung Kang, Chong Jai Kim, Jung Sun Kim, Tae Sook Kim, Kyung Cheun Jung, Kyo Hoon Park, Jong Kwan Jun, Bo Hyun Yoon
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Korean J Pathol. 1996;30(12):1123-1128.
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- The diagnosis of acute inflammation of the placenta, represented as acute chorioamnionitis, is important in that it is associated with a poor clinical outcome for both the mother and the fetus, including major perinatal morbidities such as sepsis, respiratory distress syndrome, and CNS damage. However, current medical trends in Korea seem to overlook the significance of a histopathological diagnosis of acute placental inflammation, mainly due to the indifferences of clinicians and pathologists. Since late 1993, histopathological examinations have been performed on preterm placentas at Seoul National University. These examinations have demonstrated acute placental inflammation in a significant number of cases. In the present study the incidence of acute placental inflammation was analyzed in 521 placentas which were submitted for pathological examinations in 1995. Examinations were performed to provide basic information on the incidence and profile of acute placental inflammation in this hospital and, thereby, to emphasize the significance of histopathological examinations of the placenta in the routine surgical pathology service.
Among the 521 placentas, acute inflammation was found in 194 cases (37.2%). In preterm placentas acute inflammation was found in 39.6% of the cases (67/169), while 36.1% (127/352) of term placentas showed acute inflammation. Taking the delivery mode into account, 26.3% (49/186) of the placentas delivered by cesarean section showed acute inflammation, while 43.3% (145/335) of the transvaginally delivered placentas showed inflammation. The present analysis demonstrates the existence of acute inflammation in a significant proportion of placentas with different clinical settings. The importance of a histopathological examination in routine hospital practice should be emphasized.
- Application of the Revised Case Matrix Format to Tutorial in Pathology Teaching: An Interim Approach toward Problem-Based Learning under Traditional Curricular Structure.
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Yong Il Kim, Chong Jai Kim, Gee Young Kim, Chul Woo Kim, Woo Ho Kim, Ja June Jang, Je Geun Chi, Gyeong Hoon Kang, Myeong Cherl Kook, Jung Sun Kim, Tae Sook Kim, Gee Young Kwon, So Dug Lim
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Korean J Pathol. 1996;30(8):570-661.
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Abstract
- This paper describes a student-centered case study program concerning the tumor pathology course for first year students in medical school under the traditional curricular structure. A traditional, discipline-oriented, lecture-laboratory approach was partly modified by introducing a tutuorial session using a modified case matrix format during the laboratory hours without altering the general scheme of the existing system. Small group tutorial sessions were set with the development of learning objectives emphasizing clinicopathologic reasoning and early exposure to future practical presentation which was followed by the large class session; each tutorial was supplied with a short clinical history, gross kodachrome slides, and microslides. The session for problem identification was replaced by proving a series of instructor-designed questions for both pathology and interdisciplinary correlation during which pedagogical implication was stressed the most. Student's active participation, development of self learning skill and vigorous teaching-learning process among students, and motivation/relevance for forthcoming pathology study were among the benefits conferred by this modification. We conclude that this approach is an interim step to meet the advantages of problem-based learning even in a traditional curricular structure.
- Early Gastric Carcinoma with Hepatoid Differentiation: Report of a case with histotopographic analysis.
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Gyeong Hoon Kang, Chong Jai Kim, Yong Il Kim
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Korean J Pathol. 1991;25(6):594-600.
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- A 56-year-old man received subtotal gastrectomy for an early gastric carcinoma type IIa+IIc with submucosal invasion. The tumor was made up of mixed papillo-tubular adenocarcinoma and solid carcinomatous portion, the latter comprising approximately four-fifths of the total tumor mass. The solid portion was confined within the submucosa and revealed a mixture of trabecular, compact and pelioid patterns of large polyhedra cells, resembling hepatocellular carcinoma of the liver(Edmondson-Steiner grade 2). Sinusoid-like vascular stroma of classical trabecular hepatocellular carcinoma intervened the tumor cell nests but was not associated with endothelial-cell lining. Immunohistochemical stainings with alpha-fetoprotein and alpha1-antitrypsin gave a strong reactivity in those areas of hepatoid differentiation and in the adjacent minute portion of adenocarcinoma. The findings suggest that a portion of gastric carcinoma may transdifferentiate into cells with hepatoid features along the line of endodermal lineage.
- Juvenile Granulosa Cell Tumor of the Ovary: Report of a Case of Malignant Form with Unusual Pleomorphism.
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Chong Jai Kim, Jin Suk Suh, Sung Hye Park, Je G Chi
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Korean J Pathol. 1990;24(3):316-320.
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- A case of Juvenile Granulosa Cell Tumor (JGCT) of the ovary with unusual pleomorphic histologic and malignant biologic behaviour is described. The tumor occurred in a 10-year-old girl and was associated with clinical features of isosexual pseudoprecosity and a marked elevation of serum estradiol.
The mass manifested initially in the right ovary and subsequently involved the contralateral ovary. A multi-organ metastasis developed during a 6-month-interval despite chemotherapy. She received two operations at 6-month interval, and tissues were obtained from the tumor mass. A marked histologic difference was observed between these two samples. The second biopsy showed profound cellular pleomorphism with numerous multinucleated tumor giant cell formation and hyaline bodies. The differential diagnosis from germ cell tumor and the possible factors for the pleomorphism are discussed.
- Holoacardius Hemisomus Acephalus: A case report.
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Tae Jin Kim, Chong Jai Kim, Sung Hye Park, Suk Keun Lee, Je G Chi
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Korean J Pathol. 1989;23(4):487-489.
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- An autopsy case of holoacardius hemisomus acephalus is reported. She weighed 2,190 gm and the height was 38 cm. The head and upper extremities were absent, while the vertebrae and lower extremities were relatively well developed, but severely edematous. The heart, lungs, stomach, liver, spleen, and pancreas were missing, but the lower abdominal organs including kidneys, adrenal, urinary bladder, and genital organs were present. The intestine was blind-ended at jejunal level but opened into a normal anus. The umbilical cord had two arteries and one vein.
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