Because early embryonic development of the tracheobronchial tree and foregut are closely associated, there is a wide spectrum of congenital anomalies involving either one or both organ systems. We analysed a total of 89 surgical and autopsy cases that are assumed to belong to congenital bronchopulmonary foregut malformation from the files of Seoul National University Hospital and Children's Hospital during the periord of 1961~1990. We also reviewed the serial sections of the embryos and fetuses from 3 weeks to fifteen weeks fertilization age for the observation of tracheobronchial and esophageal trees. Intralobar sequestrations(25 cases) and extralobar pulmonary sequestrations(4 cases) with patent, involuted-partial or complete-communication with the alimentary tract, tracheoesophageal fistula(30 cases) with or without esophageal atresia, esophageal atresia, esophageal stenosis due to tracheobroncheal remnant(4 cases), foregut duplication cysts(3 cases), esophageal or gastric diverticulum(1 cases), and bronchogenic cysts(22 cases) are included in this analysis(Table 1). Through this study, we confirmed the unifying concept of "bronchopulmonary forgut malformations". We believe a common embryologic pathogenesis leads to the formation of a previously described spectrum of malformations.

BACKGROUND: Hydrops fetalis is defined as abnormal accumulation of serous fluid in two or more fetal compartments, and this malady is known to be associated with various pathologic conditions. METHODS: We collected 149 cases of hydrops fetalis out of 2,312 autopsies, and we tried to elaborate the underlying causes of hydrops fetalis. The diagnosis was based on the material from either antenatal termination or intrauterine death. RESULTS: The relative incidence of hydrops fetalis was 6.44% of all the pediatric autopsies we performed. The gestational age was evenly distributed from 18 to 33 weeks, except for 30 to 31 weeks. There was no sex difference in the incidence of hydrops fetalis. The main causes were cardiovascular diseases (30.9%), cystic hygroma (13.4%), chromosomal anomaly (8.05%), thoracic conditions (7.38%), followed by urinary tract malformation (4.03%), infection (4.03%) and anemia (3.36%). The most common chromosomal anomaly was Turner syndrome and the second one was Down syndrome. CONCLUSION: Since various conditions can be the cause of hydrops fetalis, pathologists should pay attention to elaborate the underlying causes in every single autopsy.
Anemia ; Autopsy* ; Cardiovascular Diseases ; Diagnosis ; Down Syndrome ; Edema* ; Fetus ; Gestational Age ; Hydrops Fetalis* ; Incidence ; Lymphangioma, Cystic ; Sex Characteristics ; Turner Syndrome ; Urinary Tract

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 ; Carcinoma, Papillary/*pathology/surgery ; Case Report ; Diagnosis, Differential ; Ependymoma/*pathology/surgery ; Fatal Outcome ; Female ; Follow-Up Studies ; Glioma/*pathology ; Human ; Magnetic Resonance Imaging ; Middle Age

No abstract available.
Dinoprost* ; Female ; Laparoscopes* ; Pregnancy ; Pregnancy, Tubal*

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.
Diagnosis, Differential ; Biopsy ; Neoplasm Metastasis

We have reviewed malignant lymphomas in children (15 years and less) that were diagnosed at the Department of Pathology, Seoul National University Hospital and Seoul National University Children's Hospital over the last 10 years, 1979~1989, trying to find any significant histological difference between childhood and adult lymphomas. After critical review by 3 pathologists, the lymphomas are classified according to Rappaport, Lukes-Collins and NCI (working formulation) classifications for non-Hodgkin's lymphomas and Reye classification for Hodgkin's disease. Fifty mine non-Hodgkin's lymphoma (NHL) and 18 Hodgkin's disease (HD) was the material that this study was based. NHL vs HD was 3 : 1, this HD being 23.4% of all malignant lymphomas in children.
Child ; Adult ; Male ; Female ; Humans

No abstract available.
MELAS Syndrome*

Epithelial-myoepithelial carcinoma of intercalated duct(origin) is a recently described tumor characterized by its typical biphasic pattern of central duct like cell and peripheral clear cell. We described a case of epithelial-myoepithelial carcinoma in a 10-year-old boy. Microscopically, the tumor showed typical biphasic pattern, diffuse proliferation of clear cells and linining epithelial cells of tubular structures. Immunohistochemically, the clear cell showed positive reaction to S-100 protein, and the epithelial cells expressed cytokeratin indicating myoepithelial and epithelial differentiation respectively. Biphasic differentiation of the tumor cells could be also proved by electronmicroscopic study.

We report a case of epidermolysis bullosa simplex occurred in a 12-year-old girl, who presented with intractable bullous lesions of the hands and feet after minor traumas ever since her early neonatal period. The bullous lesions were noted on the 2nd and 4th webs of the fingers and dorsum of the hands as well as on the skin of the ankle. The lesions were healed without scar formation. Family history was not contributary and seasonal pattern was not noted. Histologic sections revealed intraepidermal bullae just above the well preserved basement membrane. Electron microscopic findings revealed cytolysis of basal cells with preservation of the basement membrane, indicating the epidermal type of epidermolysis bullosa simplex.
Infant, Newborn ; Humans

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.