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Biomedical subjects

J G Chi

Publications and source records attributed to J G Chi.

At least 91 records · Page 5Linked to original sources

Choroid plexus carcinoma in an infant.

Choroid plexus carcinoma is a rare tumor and has a strong tendency to spread along the cerebrospinal fluid pathway. The tumor frequently occurs in infants. Radiation therapy is not indicated in infants and the response of this tumor to chemotherapy is variable. Therefore, surgical removal plays a major role in the management of this tumor, especially in infants. A 2-month-old girl with an acute communicating hydrocephalus was presented. Through the left posterior parietal transcortical approach, a choroid plexus carcinoma which had poor demarcation from the posterior thalamus and the medial wall of the lateral ventricle was subtotally (> 95%) removed. Postoperatively a ventriculoperitoneal shunt was inserted. Chemotherapy was refused. Magnetic resonance imaging taken at 11 months after surgery showed multiple intracranial seeding of the tumor. She was in a bed-ridden state. This case revealed the aggressive behaviour of choroid plexus carcinoma in an infant and the dismal result of subtotal removal alone, though it is rather radical.

Choroid Plexus Neoplasms↗

Correlation of histopathologic classification with proliferative activity and DNA ploidy in 120 intracranial meningiomas, with special reference to atypical meningioma.

Histologic classification of 120 meningiomas was correlated with their proliferative fraction and DNA ploidy using immunohistochemistry and flow cytometry to differentiate histologically atypical meningiomas from benign ones. Histologically, the 120 meningiomas included 101 benign (43 meningotheliomatous, 40 transitional, 11 fibroblastic, 2 secretory, 2 microcystic, 2 angiomatous, and 1 psammomatous), 15 atypical, and 4 malignant meningiomas. As a histologic spectrum between the benign and malignant meningiomas, atypical meningiomas were defined by the presence of two of the following criteria; high cellularity, focal necrosis, uninterrupted growth pattern, and certain cytologic findings i.e., high nuclear/cytoplasmic ratio, coarse chromatin, and prominent nucleoli. In 56 cases, immunostaining for proliferating cell nuclear antigen showed higher proliferating cell fraction in atypical and malignant meningiomas than that in benign meningiomas (p < 0.05). In the flow cytometric analysis, aneuploidy was more often seen in atypical meningiomas compared to benign meningiomas (p < 0.05). We found that benign, atypical, and malignant meningiomas could be histologically classified and correlated with proliferative activity and DNA ploidy pattern. Therefore, atypical meningiomas should be distinguished from benign meningiomas by histopathologic examination and confirmed by studies on their proliferation fractions and DNA ploidies.

Aneuploidy↗

Adrenal carcinosarcoma--a case report.

Carcinosarcoma of the adrenal gland is an extremely rare variant of adrenocortical carcinoma, characterized by a biphasic pattern of carcinoma and sarcoma-like components. We report a case of adrenal carcinosarcoma occurring in a 61-year-old Korean man who presented with right flank pain of one month duration radiating to the back and right iliac crest. The tumor measured 12 x 12 x 7 cm and adhered to the liver and right upper pole of the kidney. The carcinomatous component of the tumor showed polygonal cells in a broad anastomosing trabecular pattern with delicate slit-like vascular channels; the sarcomatous component showed uniform spindled cells in a fascicular pattern. Both the carcinomatous and sarcomatous portions of the tumor were positive for pan-cytokeratin, and vimentin stained only the sarcomatous areas.

Adrenal Gland Neoplasms↗

Cerebral leiomyoma in a child.

A case of primary cerebral leiomyoma in a 12 year-old boy with unique clinical features is described. He presented with fever, and the magnetic resonance imaging demonstrated a well demarcated mass in the subcortical white matter of the right temporal lobe. The mass showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images with a strong homogeneous gadolinium-DTPA enhancement. The mass was removed in toto and was composed of fasciculating, monotonous oval to spindle cells which had both immunohistochemical and ultrastructural features of primitive smooth muscle differentiation. The patient is free of recurrence during the follow-up period of 56 months. Detailed clinicopathologic findings are discussed.

Brain Neoplasms↗

Intracranial metastasis from clear cell sarcoma of the kidney--a case report.

Childhood kidney tumors seldom metastasize into the cranial cavity unless it is a special histological variant. We report a 4-year-old boy with multiple intracranial metastases in the left parietotemporal and right cerebellar area from primary clear cell sarcoma of the kidney without evidence of bony metastases. Metastatic tumor revealed nests of uniformly polygonal cells with clear cytoplasm demarcated by delicate fibrovascular arcades. Tumor cells were positive for vimentin and negative for cytokeratin, S-100 protein, desmin, and myoglobin. Cellular proliferation rate measured by PCNA, and Ki-67 was not significantly different between primary tumor mass and metastatic brain lesion. Expression of p53 oncoprotein was not evident in both lesions. These findings suggested that the relapse and metastasis of clear cell sarcoma of the kidney was probably due to regrowth of micro-metastases which were present at an early stage of disease.

Brain↗

Idiopathic retroperitoneal fibrosis presented as an abdominal mass and nephrotic syndrome.

We present a 30-year-old male patient who was initially diagnosed as minimal change nephrotic syndrome, 5 years later, the patient developed a localized form of idiopathic retroperitoneal fibrosis (IRF). An elevated ESR and concomitant nephrotic syndrome in the patient suggested the immunologic nature of IRF, IRF has been reported in association with collagen diseases and rarely with proliferative and nonproliferative glomerulopathies. To our knowledge, the association between minimal change lesion (MC) and IRF has not been reported. Furthermore, the fact that IRF presented itself as an abdominal mass and lacked systemic symptoms was also unusual.

Adult↗

Prenatal development of cranial base in normal Korean fetuses.

BACKGROUND: The cranial base is not only an end result of complex segmental growth of rostral end of axial skeleton but also has a tremendous impact on the development of the brain and facial structure. However, little is known about the method for the systematic analysis of the shape of the cranial base in the developing human fetus. We used roentgenograms of the cranial base and eviscerated bones for the assessment of the development of the cranial bony complex. METHODS: The cranial base was removed from 64 normal human fetuses after 18-40 weeks of gestation. Roentgenograms were taken perpendicular to the cranial base. Major anatomical landmarks are defined as follows: the center of pituitary fossa of sphenoid bone (point S), the growth center of zygomatic bone, the anterior point of nasal septum, otic cartilage, and anterior and posterior growth centers of maxilla. The anterior cranial base angle, the middle cranial base angle, the posterior cranial base angle, the maxillary trapezoid area, the horizontal middle face area, and the occipital cranial base triangle area are defined from the landmarks, and the growth of each parameter was analyzed by gestational age. RESULTS: The proportional growth of the anterior, middle, and posterior cranial fossae could be assessed by the angles around the center of the pituitary fossa (point S) and by the anterior, middle, and posterior cranial base angles. The anterior cranial base angle was relatively constant during the fetal period at 107.4-112.5 degrees, whereas the middle cranial base angle gradually increased, and the posterior cranial base angle decreased. With increasing gestational age, the horizontal middle face area increased rapidly, in contrast to those of the maxillary trapezoid area, occipital cranial base triangle area, or foramen magnum area. CONCLUSIONS: The important keys in the structural development of the normal human cranial base are anterior, middle, and posterior cranial base angles. We could define the developmental pattern of human cranial base, which in turn provides a model of a standard growth pattern applicable to the study of the different malformations in the craniofacial structure.

Cephalometry↗

Primary intramedullary spinal cord primitive neuroectodermal tumor with intracranial seeding in an infant.

Primary spinal cord primitive neuroectodermal tumor (PNET) is a rare entity. In all 13 cases have been reported in the literature, including 3 with intracranial seeding. A 3-month-old girl with involvement of the spinal cord below the mid-thoracic level is described. The brain MRI revealed findings indicative of seeding along the intracranial subarachnoid space. Biopsy, duraplasty and removal of laminotomy flap were done. In spite of a good response to the first cycle of postoperative "8-drugs-in-a-day' chemotherapy, further treatment was refused. She died 21 days after the onset of leg weakness, which reveals the rapid progression of untreated cases. To our knowledge, this is the first case of spinal cord PNET with parenchymal involvement that has been described in an infant.

Fatal Outcome↗

Immunohistochemical evaluation of transglutaminase C in tumours of salivary glands.

Transglutaminase C (TGase C), a family of Ca(2+)-dependent enzymes and an essential component in the cross-linking of peptide bonds, has been found to be a marker of epithelial differentiation with a possible role in cellular apoptosis, extracellular matrix stabilisation and Ca2+ binding, thereby having a potential role in tumour growth, differentiation and invasive behaviour. The expression of TGase C was evaluated in normal human salivary glands and their neoplastic lesions which included pleomorphic adenoma (n = 30), Warthin's tumour (n = 5), adenoid cystic carcinoma (n = 10), acinic cell carcinoma (n = 5), mucoepidermoid carcinoma (n = 5) and control tissue specimens of normal oral mucosa and squamous cell carcinoma, using polyclonal antibody, the specificity of which was determined by Western blotting, generated by immunising rabbits with purified transglutaminase. The TGase C was observed in the epithelial cells in the control tissue specimens examined. Pleiomorphic adenoma revealed reaction products in luminal tumour cells, the non-luminal or modified myoepithelial cells and their plasmacytoid variants, squamous metaplastic cells and chondroid cells. Adenoid cystic carcinomas had tumour cells in the luminal cells of tubular and cribriform structures and the acinic cell carcinoma had from low to moderate immunoreactivity in the tumour cell component and a diffuse immunoreactivity in the stroma for TGase C. Mucoepidermoid carcinoma showed no reaction products in the mucous-producing cells, while intermediate and epidermoid cells had immunoreactivity in the cell cytoplasm. As the presence of TGase C in salivary gland tumours was confined to those tumour cells which form the predominant histomorphology in each tumour subtype, it may be suggested that these enzymes may have a potential role in the regulation of cellular function in neoplastic salivary tissues affecting tumour growth, differentiation and neoplastic behaviour.

Adenolymphoma↗

Tri-amelia and phocomelia with multiple malformations resembling Roberts syndrome in a fetus: is it a variant or a new syndrome?

Roberts-SC phocomelia syndrome is a rare autosomal recessive disorder characterized by morphological anomalies such as limb defects and midfacial clefting, and by premature centromeric division in chromosomal study. Although it has been regarded as a single genetic entity and includes various morphologic defects, babies are being reported nowadays with severe facial defects, tetra-amelia, and pulmonary abnormality, yet with normal chromosomal findings. We have added a case resembling Roberts syndrome with various congenital anomalies. A gestation with a fetus was terminated at 24 weeks of gestational age because of multiple fetal anomalies. Postmortem examination revealed a severe mid-facial cleft, tri-amelia and phocomelia, multiple encephaloceles, protruding and hypoteloric eyes, low-set ears, atrial septal defect of ostium secundum type, patent ductus arteriosus, bilateral two-lobed lungs with incomplete lobation, multiple visceral anomalies, a penis without scrotum, abnormal dermoglyphics, and absence of nipples.

Abnormalities, Multiple↗

Proliferating cell nuclear antigen expression in Wilms' tumor: a comparative study of pre- and post-chemotherapy specimens.

A total of 36 Wilms' tumors were immunohistochemically analyzed for proliferative (proliferating cell nuclear antigen-labeling) index (PI) with special reference to the effect of preoperative chemotherapy on the proliferative potential of tumors and differences in PI according to the histologic components (epithelial, blastemal and stromal). The cases were divided into two groups according to the status of chemotherapy at the time of surgical removal of the tumors. In group I (n = 12, cases with pre-operative chemotherapy), the mean PI +/- s.d. values of epithelial, blastemal and stromal types were 19.5 (n = 1), 10.1 +/- 5.28 (n = 8), and 16.2 +/- 13.49 (n = 3), respectively, and in group II (n = 24, cases without preoperative chemotherapy), 45.2 +/- 15.41 (n = 3), 28.5 +/- 16.92 (n = 10), and 23.2 +/- 4.45 (n = 2), respectively. The cases with mixed histology in group II (n = 9) had comparable PI values for each histologic component. The mean PI +/- s.d. was 12.1 +/- 7.76 in group I and 31.9 +/- 15.95 in group II. The PI value of group II was significantly higher than that of group I (P < 0.001). Alterations in the PI value following chemotherapy implied that the interpretation of tumor histology following chemotherapy should be more prudent because alteration of the PI, which reflects the proliferative potential of tumor cells, suggests modified biology of viable cells in pretreated cases as a consequence of chemotherapy.

Adolescent↗

Central neurocytoma: proliferative potential and biological behavior.

The authors analyzed 13 central neurocytomas diagnosed at Seoul National University Hospital between January 1982 and December 1993 to clarify the proliferative potential and biological behavior of these tumors. The tumor was confined to the lateral and third ventricles in 12 cases and in one case extended from the posterior thalamus to the body and trigone area of the lateral ventricle. In all 13 cases, typical clinical and radiological findings were observed, and histological diagnosis was performed via craniotomy. The diagnosis was made using light microscopic examination, immunohistochemical staining for neuronal markers, and electron microscopic findings of neuronal differentiation. One patient died due to tumor progression with recurrence 26 months after subtotal removal plus radiation therapy. Another patient had a recurrence 18 months after total tumor removal. The remaining 11 patients are free of recurrent tumor after a follow-up period that ranged from 14 to 109 months (median 50 months). To predict the proliferative potential, immunoreactivity to proliferating cell nuclear antigen (PCNA), silver colloid staining for nucleolar organizing regions (AgNORs), and DNA flow cytometry were performed in 10 of the 13 cases. The proportion of PCNA-positive cells was less than 1% in all cases and the AgNORs score ranged from 1.11 to 2.0 (mean 1.67). The DNA flow cytometry revealed diploidy in all cases and the calculated proliferation index ranged from 5.1% to 9.6% (mean 7.8%). The one case of tumor recurrence, in which the authors performed the study of proliferative potential, and another case that demonstrated mild nuclear pleomorphism also showed low percentages of PCNA-positive cells, low AgNORs scores, and diploidy in DNA flow cytometry. It is suggested that most central neurocytomas follow a benign clinical course with low proliferative potential assessed by PCNA, AgNORs, and DNA flow cytometry; however, recurrence is possible within a relatively short time period.

Adolescent↗

Cerebral sparganosis: clinical manifestations, treatment, and outcome.

Cerebral sparganosis is a rare parasitic disease caused by infestation by the plerocercoid larva of Spirometra mansoni. The authors retrospectively analyzed 17 cases of cerebral sparganosis treated at Seoul National University Hospital between 1986 and 1994. The patients' ages at diagnosis ranged from 6 to 57 years (median 32 years) and the male/female ratio was 13:4. Diagnosis was based on radiological findings, serological test results, operative findings, and histopathological examinations. Characteristic magnetic resonance (MR) findings consisted of widespread white matter degeneration and cortical atrophy, mixed-signal lesion (low in the central and high in the peripheral regions on T2-weighted images) with irregular dense enhancement of central foci and changes in the location and shape of the enhancing lesion in follow-up studies. Ten patients underwent surgical removal of the parasitic lesion, six received medical treatment alone (five with praziquantel and one with antiepileptic drugs), and one underwent insertion of a ventriculoperitoneal shunt and a course of praziquantel. Follow-up periods ranged from 13 to 111 months (mean 49 months). Seven patients who underwent complete removal of the lesion, live worm, or degenerative worm with surrounding granuloma showed a favorable course. Patients who received medical treatment alone or incomplete removal exhibited progression in their neurological deficits and their seizures could not be controlled. Medication with praziquantel seemed to have no killing effect on live worms. The authors conclude that MR imaging is the most valuable modality for the early detection of cerebral sparganosis and that complete surgical removal of granuloma together with worms, whether they are alive or degenerative, is the treatment of choice.

Adolescent↗

Expression of bFGF and VEGF in brain astrocytoma.

Neovascularization is an important factor in the prognosis of brain tumor and many angiogenetic factors have been evaluated for prognostic significance. Among them, basic fibroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF) are known as potent angiogentic factors and mitogens. We evaluated seven cases of grade II brain astrocytoma. Four, group A, was diagnosed as anaplastic progression at their second operation, and three, group B, did not. Using monoclonal antibodies to bFGF and VEGF in paraffin embedded tissue from first operation, their immunoreactivity and differences between two groups were examined. The growth fractions of these tumor were also measured by Ki-67 monoclonal antibodies (MIB1). Immunostaining for bFGF in tumor cells were observed in both nuclei and cytoplasm, and for VEGF, mainly observed in the cytoplasm. Mean cell count number +/- standard deviation per high power field in each were as follows: 1) for bFGF, 20.08 +/- 6.38 in group A and 0.87 +/- 0.90 in group B (P < 0.01), 2) for VEGF, 43.75 +/- 17.09 in group A, and 0.8 +/- 1.06 in group B (P < 0.05) and 3) for the proliferation index with Ki-67 antibodies, 3.20 +/- 0.81 in group A and 0.77 +/- 1.03 in group B (P < 0.05). This data supports the assertion that angiogenetic factor such as bFGF and VEGF may contribute to progressive change of astrocytoma by tumor angiogenesis.

Adolescent↗

Papillary ependymoma: its differential diagnosis from choroid plexus papilloma.

Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. Here, we report two cases of papillary ependymomas regarding their unique clinicopathologic features and differential points from choroid plexus papilloma. Brain MRI revealed a large mass in the left lateral ventricle in one case and a 3cm sized mass in the pineal area and the 3rd ventricle in the other. Microscopically, the tumor was characterized by papillary and tubular structures. Immunohistochemically, the tumor cells in both cases expressed cytokeratins(CK22 and CAM 5.2) but did not express glial fibrillary acidic protein(GFAP), vimentin, epithelial membrane antigen, and S100 protein. This is a very unusual immunohistochemical feature for papillary ependymoma. Ultrastructurally, the tumor showed a mosaic pattern of tumor cells with frequent intercellular microrosettes having a few stubby microvilli, a few cilia and zonulae adherentes. The cytoplasmic processes were markedly reduced compared to conventional ependymoma. The cytoplasm did not contain intermediate filaments. Interestingly, the mitochondria showed abnormal features with a pleomorphic shape and abnormal cristae in both cases. These ultrastructural features enabled differentiation between papillary ependymoma and choroid plexus papilloma in addition to the light microscopic findings.

Adult↗

Adamantinoma of tibia with predominant features of fibrous dysplasia--a case report.

We report a case of adamantinoma of the tibia resembling fibrous dysplasia. The patient was a 55-year-old male, and complained of pain in the right lower leg. Roentgenographs showed a well demarcated osteolytic lesion with small foci of calcification and septation within the diaphysis of the distal tibia. The cortex was partially disrupted. Histologically, initial biopsy specimen showed fibrous connective tissue and trabeculae of immature woven bone, strongly suggestive of fibrous dysplasia. The lesion recurred and the second biopsy revealed nests of spindle cells and tubular epithelial structures embedded in granulation type-fibrous tissue. Immunohistochemically, both the nests of spindle cells and the tubular structures gave a positive reaction for cytokeratin. The present case emphasizes once again that histological diagnosis of fibrous dysplasia of the tibia should be made carefully with exclusion of the possibility of adamantinoma.

Ameloblastoma↗

Congenital quadrigeminal lipoma with osteocartilagenous element. A case report.

A case of congenital tectal lipoma found incidentally at an autopsy is reported. This female fetus was a product of pregnancy termination at 24 weeks of gestational age after sonographic detection of hydrocephalus. Autopsy revealed a small mass in the periaqueductal portion. The mass was composed of adipose tissue, cartilage, and mature bony tissue with hematopoiesis. The resultant diagnosis was tectal lipoma with osteocartilagenous element. It is uncertain whether the lesion represents a teratoma or hamartoma or mesenchymal metaplasia. The osteocartilagenous component suggests the latter.

Adult↗