Juvenile cellular adenofibroma of the breast is a unique neoplasm of the breast that should be differentiated from other important benign and malignant lesions of the juvenile breasts. We report a case with it's characteristic clinical, gross and histological features. The tumor was in the right breast with the size of 20 cm in maximum extent. This patient was also associated with hemihypertrophy of the right side. Microscopically the masses were characterized by prominent stromal cellularity associated with pericanalicular duct proliferation.
Child ; Male ; Female ; Humans

Cystic hygroma is a congenital malformation of the lymphatic system appearing single or multiloculated fluid-filled cavities, most often around the neck. They often progress to hydrops and cause fetal death, and frequently associated with chromosomal abnormalities and other congenital malformations. The purpose of our study is to delineate the nature of cystic hygroma and determine the relationship between cystic hygroma and associated anomalies including fetal hydrops. We used data from 26 cases of cervical cystic hygroma in autopsy files of SNU Children's Hospital. Most of cystic hygroma were found in stillborn or premature infants. The fetal cases had been dead for a quite a long period since there was discrepancy between the true gestational age and the developmental age estimated from the body length. Of 26 fetuses only 2 were studied chromosomally and both of them showed 45X. Of 26 cystic hygromas 23 occurred in the posterior neck and 3 in the anterior neck. They ranged from 2.5 to 14 cm (mean: 7.9 cm). The cystic hygroma of the posterior neck consisted of two symmetric sacs on both sides and in the nape and extended to the occipital region. The cystic hygromas of the anterior neck were unilateral or bilateral, and multiloculated and extended into the adjacent cheek. Cystic hygromas of posterior neck were always associated with hydrops, while no recognizable hydrops was found in cystic hygromas of anterior neck. The cystic hygromas were larger in patients with severe hydrops than in patients with less severe hydrops. Associated abnormalities, found in 88%, included hydrops fetalis(88%), growth retardation(80%), cardiovascular anomalies(27%), horseshoe kidney(23%), skeletal anomalies(12%) and hypoxic changes(31%) in visceral organs. In summary, when a hygroma is detected during fetal life, careful sonographic examination for associated congenital anomalies, fetal karyotyping and consideration of artificial abortion are indicated.
Autopsy* ; Cheek ; Chromosome Aberrations ; Edema ; Fetal Death ; Fetus ; Gestational Age ; Humans ; Hydrops Fetalis ; Infant, Newborn ; Infant, Premature ; Karyotyping ; Lymphangioma, Cystic* ; Lymphatic System ; Neck* ; Turner Syndrome ; Ultrasonography

To evaluate the prenatal development of human femur, a histologic study was made based on normal femora of 24 embryos and 122 fetuses ranging in age from 5 to 41 weeks of gestation. Following observations were made. 1) The femur starts to be formed as mesenchymal condensation in the lower limb bud at 5 weeks of gestation. 2) By the end of the embryonic period (8 weeks), most of the skeleton of lower limb bud consisted of cartilage model defined by perichondrium. 3) At 9 weeks of gestation, endochondral ossification and primary bony trabeculae were prominent in the central portion of cartilage model. 4) At 11 weeks of gestation, along with pregression of endochondral ossification proximally endochondral growth zone was established near the proximal chondro-osseous junction. 5) At 12 weeks of gestation, most centrally located trabeculae were removed, and the marrow cavity and hematopoietic cells were recognized. The cartilage canal was also recognized at this time in the proximal epiphyseal cartilage. 6) In the middle of prenatal life (15-30 weeks), the shape of the femur was reconstructed by remodeling process. 7) The lamellar bone began to form by 31 weeks of gestation. 8) At 15 weeks of gestation, proximal end of ossified shaft showed convexity, and at 29 weeks this convexity was altered to form a rather distinct angulation. 9) As the femoral shaft elongated, primary bony collar continued to develop near the growth plate and remained at the level of the hypertrophied cartilage.
Humans

Sequential development pattern of the intrahepatic bile ducts is analyzed histologically and immunohistochemically using 50 embryonal and fetal liver specimens. Serial sections are made to reconstruct the intrahepatic biliary system, and monoclonal antibodies were used for differential antigenic expression of the biliary system. By 9 weeks of gestation a layer of small round cells, namely plate cells, became first noticed around large portal vein branches close to the hepatic hilum. These plate cells extended subsequently to more distal branches. The ductal plates became duplicated to contain cleft-like spaces and tubular structures. These tubules gradually became incorporated into surrounding connective tissue around the protal vein, and gave the appearance of the individualized bile duct. At term(40 weeks of gestation) the smallest branches of the portal vein were still surrounded by a discontinuous ductal plate. The ductal plate cells showed strong positive reaction for CAM 5.2 and AE1/AE3. They also expressed CK 19 and AE1 from 9 weeks of gestation on. The immunoreactivity of bile duct cells for cytokeratins persisted throughout the whole gestational period. Carcinoembryonic antigen was expressed along the luminal border of the bile duct, duplicated ductal plate and intrahepatic bile canaliculi. Laminin was demonstrated along the basement membrane of the bile duct cells from 9 weeks of gestation. Few duplicated ductal plates were composed of two different types of cells, duct-like cells and hepatocyte-like cells. The duct-like cells and hepatocyte-like cells showed same imrhunoreactivity with the hepatocytes and the bile duct cells, respectively, suggesting that the intrahepatic bile duct cells are actually coming from the hepatocytes around the branches of the portal vein.
Humans

A total of 322 fetuses and 29 embryos were examined light microscopically to evaluate the morphological development of the human gastrointestinal tract with increasing gestational age. The human embryos were analysed by reconstruction of serial section slides. One hundred and forty fetuses ranging from 15 to 41 weeks of gestation were used for correlating the gestational age and the body weight with the measurement of the small and large intestines. 1) The esophagus develps from the distal part of the foregut through a partition of the tracheoesophageal septum. Initially the esophagus is short, but it elongates rapidly and reaches its final relative length and position by the seventh week. The epithelium of the esophagus proliferates and completely obliterates the lumen by the seventh week, but recanalization occurs by the ninth week. The esophageal epithelia consist of three different groups of cells; stratified squamoid cells, ciliated columnar cells and tall columnar cells by the 21th week. 2) The stomach appears as a fusiform enlargement of the caudal part of the esophagus at the fourth week. During next two weeks the stomach acquires its adult shape and final position. The primordial gastric pits and the parietal cells appear at the eighth and eleventh weeks, respectively. 3) As the midgut elongates, it forms a ventral U-shaped loop, called primitive intestinal loop by the fifth week. At this stage the cecum appears as a swelling from the caudal limb of the intestinal loop. By the seventh week the bulks of the caudal limb of the intestine herniate into the umbilical cord, in which the loop rotates 90 degrees counterclockwise around the axis of the superior mesenteric artery. During the tenth week, the intestine returns to the abdomen and undergoes a further 180 degrees counterclockwise rotatation. 4) The cloaca is divided into two parts by a urorectal septum at the fifth week. By the seventh week the urorectal septum has fused with the cloacal membrane, dividing it into a dorsal anal membrane and ventral urogenital membrane. The anal membrane ruptures at the eighth week. 5) During the sixth week the duodenal lumen becomes completely filled with proliferating epithelium. The villi project from the mucosa of the small and large intestines at the eighth and eleventh weeks, respectively. The villi of large intestine become resorbed again after the 21th week. At the fifth week Paneth cells appear through the entire length of the intestine including the rectum, but disappear in the colon and the rectum after the 36th week. 6) The developing Auerbach's plexuses are well recognized along the outside of muscle coat throughout the gastrointestinal tract, but demonstrate no immunoreactivity for the anti-neuron specific enolase antibody. The neuroblasts in the myenteric plexus reveal strong positivity for the anti-neuron specific enolase antibody at the eleventh week, but the ganglion cells differentiate by the fourteenth week. 7) Differentiation of the gastrointestinal wall and development of the myenteric plexus begin form the esophagus and progress caudally down to the colon. But the anorectal wall is differentiated from the cloaca more earlier than the esophagus, stomach and colon are. 8) The small and the large intestines elongate progressively with the increasing gestational age and body weight and increase approximately 5.6 and 5.4 folds, respectively during the 25 weeks from the 15th to 41th week of gestation. At the 40th gestational week the small intestine is 5.68 times the length of the colon. 9) The correlation between the body weight (BW), crown-rump length (CR) and intestianl length (small intestine (SI), large itestine (LI) is presented as: SI (cm)= -33.67 + 4.14CR + 11.62 (BW)(1/3), LI (cm)= 5.56 + 0.76CR + 0.007BW (gm), CR (cm)= 7.82 + 0.015 SI + 0.41 (BW)(1/2).
Adult ; Male ; Female ; Humans

We reviewed surgical or autopsy specimens of the gastrointestinal tract from 14 patients with necrotizing enterocolitis(NEC) to define the specific pathologic findings and the pathologic changes of the bowel in the premature and fullterm infants, and then to see how these findings are related each other and to clinical features. In two-thirds of patients two or more continuous segments of the gastrointestinal tract were affected, and the ileum and colon were by far the most common sites of involvement. Pathologically, coagulation necrosis, inflammatory reaction, ulceration, microthrombi, overgrowth of microorganism, and perforation were the leading changes of NEC. On the basis of these histopathologic findings of the involving intestine, we could classify the pathology of NEC into two different histologic types. Type 1 and II are thought to represent pathologic changes of the intestine in acute and chronic stages of disease process. The symptom duration of the patients was significantly different between two types. The pathologic changes of the involved bowel were similar in both premature and fullterm infants, although the premature tended to have type 1 and the fullterm babies had more cases of type II. These pathologic changes could be associated with various clinical manifestation of NEC.
Infant ; Male ; Female ; Infant, Newborn ; Humans

Congenital cystic adenomatoid malformation of the lung(CCAML) is a rare developmental anomaly characterized by an "adenomatoid" hyperplasia of terminal respiratory structures with formation of the cysts of varying sizes. CCAML is separated into three major types based on the gross and microscopic findings. We have analyzed 22 cases of CCAML, those consisted of 6 autopsy cases and 16 surgical specimens. Out of 22 cases, 5 cases were composed of large cysts(type I) and 9 cases had multiple small cysts(type II). Remaining one case revealed features of solid type(type III), and 7 cases were mixed form. There were 16 boys and 6 girls. All cases were below the age of 14 years. There was no clear-cut age difference between different types of CCAML. However, inflammation, fibrosis and pseudostratification of epithelium were often found in older age. All fetal autopsy cases of CCAML had hydrops fetalis and were associated with maternal hydramnios. One case of type III showed definite mucinogenic cells in the cysts unexpectedly, and one case of the mixed form(typeI+II+III) was found in a fetus of 22 weeks of gestational age. Above findings contradicted the classical description of the CCAML, and suggested that arbitrary classification into three types may not be the best way in understanding this condition.
Cysts

Of all the primary central nervous system tumors, the medulloblastoma, glioblastoma multiforme, ependymoma and pineal germinoma tend to exfoliate in the cerebrospinal space. With all other types of the tumor, abnormal cells may seldom be definitely identified in the cerebrospinal fluid. Up to now the tumor cells have been rarely found in CSF cases of retinoblastoma. We have experienced a case of advanced retinoblastoma that showed exfoliated cells in spinal fluid.

BACKGROUND: Along with the recent greater number of medical devices for the elderly in the market, there has been a parallel increase in the number of clinical trials for these devices. It is uncertain, though, whether the target population has the awareness and understanding of these clinical trials and the possible adverse events of the devices. METHODS: A total of 147 elderly subjects (mean age, 65.8+/-8.3 years; 39 males) responded to the questionnaire. A pilot study during the development of the questionnaire was conducted in 2009 with the actual survey taking place from June to November 2011. The questionnaire included 22 items-10 items on awareness and understanding of the clinical trial; 4 items on the understanding of adverse events; and 8 items on demographical characteristics. RESULTS: Of the responders, 62.5% had participated in at least one clinical trial and had heard about the clinical trials through a doctor, newspaper, or television. And 63.3% of the responders understood that the purpose of the trial was to show efficacy and safety for the medical device. There were significant differences on the awareness of the trial by gender and life level. However, there was no statistically significant difference in adverse events awareness. CONCLUSION: Although elderly subjects participate in clinical trials, it seems they do not receive enough information when involved in clinical trials of medical devices. In the future, before these trials are carried out, medical device companies need to ensure that they provide their elderly subjects with better education and information.
Aged ; Dietary Sucrose ; Health Services Needs and Demand ; Humans ; Hypogonadism ; Mitochondrial Diseases ; Periodicals ; Ophthalmoplegia ; Pilot Projects ; Surveys and Questionnaires ; Television

We have studied morphologic characteristics and apoptosis on the fetal brain of the trisomy 16 mouse, a model for human trisomy 21 syndrome. This study was based on serial sections of the whole brain from a sample of sixteen trisomy 16 mice and forty-six age-matched control littermates from embryonic day (ED) 12 to ED 18. Trisomy 16 brains showed a reduction of telencephalic size and abnormal cortical development. At ED 13 trisomy 16 and control brains appeared similar. By ED 14 difference in the cortical thickness and telencephalic growth became evident, and by ED 16 a marked size difference had developed between the trisomy 16 and control brains. By ED 18, however, the thickness of the trisomy 16 cortex had increased considerably and was not significantly different with respect to the thickness and cross-sectional areas of the pallium and its constituent cortical layers. The cell density of the trisomy 16 cortex had persistently decreased before ED 17, when the cell density of control and trisomy 16 corteces was similar within each layer. At ED 18 cell density of trisomy 16 cortex in each layer increased. There was inverse relationship between a number of TUNEL positive apoptotic cells and cell density in the trisomy 16 brains. Our results suggest that developmental abnormalities of the trisomy 16 brain indicated developmental delay of the telencephalon growth, which may be caused by apoptosis rather than by a proliferation defect.
Animals ; Apoptosis* ; Brain* ; Cell Count ; Down Syndrome ; Humans ; In Situ Nick-End Labeling ; Mice* ; Telencephalon ; Trisomy*