Ectopic liver in the umbilicus is very rare, and a few cases have been reported in the world. It is thought that an increased intraabdominal pressure resulting in entrapment of liver cell nests causes the presence of liver in the umbilicus. It relates to neonatal problem such as infection. We report a case of ectopic umbilical liver in a stillborn male of 28 gestational weeks. His mother discovered loss of fetal movement 2 days before admission, and intrauterine fetal death was diagnosed by ultrasonography. Grossly, the umbilical cord was markedly swollen. On cut section, a well circumscribed, oval round, tan-colored soft mass was noted within the cord. Histologically, it consisted of hepatic cords without bile ducts. The umbilical vessels were distorted by the ectopic liver, and contained thrombi. These findings suggest that ectopic umbilical liver results in the intrauterine fetal death
Male ; Infant, Newborn ; Humans

Hereditary sensory and autonomic neuropathies (HSAN) are disorders of hereditary neuropathy mainly affecting sensation and also accompanying autonomic nervous system dysfunction. They are divided into five subtypes based on inheritance pattern and clinical manifestation. Among HSAN, type II is characterized by autosomal recessive inheritance, presentation at later stage of life, slow progression and mainly sensation abnormalities. The main pathology of the peripheral nerve is the absence of myelinated nerve fibers. Type IV is very rare disorder and only a few cases have been reported. It is characterized by autosomal recessive inheritance, presentation at birth as failure to thrive, retarded motor development, unexplained pyrexia and rapidly progressive and severe clinical course. The main pathology of the peripheral nerve is a loss of unmyelinated and small myelinated nerve fibers. We report two cases of type IV and one case of type II especially focusing on ultrastructural findings, which are characteristic of and diagnostic for HSAN.
Autonomic Nervous System ; Failure to Thrive ; Fever ; Hereditary Sensory and Autonomic Neuropathies* ; Inheritance Patterns ; Nerve Fibers, Myelinated ; Parturition ; Pathology ; Peripheral Nerves ; Sensation ; Wills

The choroid plexus papilloma is a rare tumor. Its incidence is 0.4-0.6% of all intracranial tumors. Most cases of this tumor are found in the young subject, especially infant and childhood but its congenital form is very rare. The clinical and pathologic findings of congenital choroid plexus papilloma are similar to that of any age. But the cilia on the cell surface are common in the former and very rare in the latter. We present a case of congenital choroid plexus papilloma of the lateral ventricle in a 2 month-old male baby. He had suffered from a sudden onset of convulsions and a drowsy mental state for 2 days. The CT scan revealed a large intraventricular tumor in the left lateral ventricle with hydrocephalus. After ventriculo-peritoneal shunt(V-P shunt), his symptoms were improved. But the hydrocephalus was aggravated due to malfunction of V-P shunt, and he recieved the operation after 4 months. The gross examination revealed a large ovoid papillary tumor(4x3x3cm). On light microscopic examination, the tumor showed papillary structure lined by columnar eosinophilic cells. Some cells had cilia. The electron microscopic finding showed intercellular junction, microvilli and cilia. The tumor cells were positive for cytokeratin, vimentin and S-100 protein.
Infant ; Child ; Male ; Female ; Humans ; Incidence

We report a case of aggressive angiomyxoma in ischiorectal fossa of a 39-year-old women. The tumor is characterized by relatively large size(13 x 11 cm), grossly gelatinous appearance and locally infiltrative nature. Microscopically, it consists of many variable sized blood vessels and spindle or stellate cells widely separated in myxoid or collagenous stroma. Immunohistochemical stains reveal that the tumor cells are strongly positive for actin and desmin. Electron microscopic findings are that of a few cells dispersed in abundant intercellular substance and collagen bundles. These cells form irregular cytoplasmic process without basal lamina and contain endoplasmic reticulum having cistern.
Female ; Humans

Metanephric adenoma is a rare renal epithelial tumor. Its light microscopic features are very characteristic, and immunohistochemical and electron microscopic studies are not critical to the diagnosis. The literature indicate that, to date, the tumor has behaved in a benign fashion, and predominantly but not exclusively occurred in middle-aged women. It occurs in a wide range up to 11 cm and is usually an incidental finding but may be symptomatic with hematuria or flank pain. Recently, we have experienced a case of renal tumor showing distinctive adenomatous features, which is incidentally found in a 52-year-old female. This tumor is confined to the renal cortex and is well-circumscribed with a characteristic uniform and orderly proliferation of compact well-differentiated small tubules lined by bland oval cells with a very low level of mitotic activity. The term metanephric adenoma is appropriate for this tumor because it accurately describes its bland proliferation of tubules and reflects the embryonic architectural and cytological appearance of this proliferation. The pattern of the tumor, with its occasional papillary glomeruloid- like bodies and foci of elongated tubules, is reminiscent of the fetal metanephric kidney.
Adenoma* ; Diagnosis ; Female ; Flank Pain ; Hematuria ; Humans ; Incidental Findings ; Kidney* ; Middle Aged

PURPOSE: To investigate the expression pattern of p27 protein in primary gastric lymphomas. MATERIALS AND METHODS: Immunostaining for the p27 protein was performed in 16 cases of low grade mucosa-associated lymphoid tissue (MALT) lymphomas and 16 cases of high grade B-cell lymphomas of the stomach. RESULTS: All low grade MALT lymphomas were positive, however all high grade lymphomas were negative forp27 protein. Most of the monocytoid cells in the low grade lymphoma were unstained. CONCLUSION: Loss of p27 protein expression is well correlated with histologic grade and appears to be associated with the high grade transformation seen in primary gastric lymphomas.
Lymphoid Tissue ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, B-Cell, Marginal Zone ; Stomach

The simultaneous occurrence of an adenocarcinoma and schwannoma is extremely rare in the stomach, and only one such case has been previously reported, which presented as two separate masses. Indeed, the collision of these tumors has never been reported. We report the case of a 61-year-old male patient who was diagnosed with the synchronous development of a schwannoma and advanced mucinous adenocarcinoma of the stomach, in which the carcinoma cells focally invaded the schwannoma.
Adenocarcinoma ; Adenocarcinoma, Mucinous ; Humans ; Male ; Middle Aged ; Neurilemmoma ; Stomach

There is a wide array of designation for cutaneous vascular disorders based on clinical characteristics, histology, embryology, cellular biology, and hemodynamics. The cutaneous vascular disorders can be divided into hemangioma and vascular malformation according to the biologic classification based on cell kinetics i.e. endothelial hyperplasia. There are clinical and histologic differences between them. In this study, clinical, histologic and im-munohistochemical evaluations were attempted on 40 cases of cutaneous vascular disorders diagnosed the period between 1985 and 1993. The results are as follows: 1) Twenty-three out of forty cases were immunoreacive for proliferating cell nuclear antigen(PCNA). The lesions composed of capillary-sized blood vessels with endothelial hyperplasia were diffusely reactive, whereas those composed largely of dilated blood vessels with or without focal endothelial hyperplasia were only focally reactive. 2) Each groups of the classic classification contained both reactive and nonreactive cases except nevus flammeus and juvenfle hemangioma. 3) In contrast to the cases nonreactive for PCNA, those reactive for PCNA contained areas of proliferating small vessels, which showed reactivity for PCNA. In conclusion, the cutaneous vascular disorders diagnosed by the classic classification are heterogeneous in the pattern of the endothelial hyperplasia and the PCNA staining. Therefore it should be classified by the clinical and the histologic characteristics.
Hemangioma

Malignant tumors of the breast which contain stromal osteoclast-like, multinucleated giant cells are a rare entity of yet unknown clinical significance. These benign multinucleated giant cells are known to occur mostly in areas of prominent angiogenesis and in close association with tumor cells. Supplementary immunohistochemical and electronmicroscopic examinations indicate that the multinucleated giant cells are of histiocytic origin. We report on a case of infiltrating ductal carcinoma with stromal osteoclast-like multinucleated giant cells occurring in the right breast of a 37 year-old woman. Grossly, the tumor was characterized by a well-circumscribed dark brown, solid firm mass. Microscopically, multinucleated giant cells were found in the stroma intermingled with malignant tumor cells. The stroma showed only a small amount of mononuclear cell infiltration and a moderate degree of vascular proliferation. Immunohistochemical stains revealed the tumor cells to be positive for carcinoembryonic antigen, epithelial membrane antigen and cytokeratin while the multinucleated giant cells were positive for vimentin, CD68 and negative for all other stains tested. Ultrastructurally the multinucleated giant cells differed from tumor cells by having abundant cytoplasmic organelles such as rough endoplasmic reticulum, lysosomes, ribosomes, and vesicles but lacking desmosomes or other types of intercellular junctions. Other characteristic features of multinucleated giant cells included, indented nuclei and prominent cytoplasmic process.
Female ; Humans

PURPOSE: Smad proteins mediate cellular signaling through the transforming growth factor-beta family (TGF-beta s). Smads 2 and 3 transmit signals from TGF-beta, and Smad4 is a common mediator, as well. However, little is known concerning the expression patterns of Smads in lymphoid tissue. MATERIALS AND METHODS: Immunohistochemistry for Smad3 and Smad4 was performed on paraffin-embedded tissue sections collected from 26 T- or NK-cell lymphomas. RESULTS: Nearly all cells in germinal centers were positive for Smad3, and more than 50% of paracortical cells were positive for Smad3 in reactive lymphoid tissue. When Smad4 immunostaining was conducted, nearly all the cells in the germinal centers showed diffuse cytoplasmic staining, and most of them exhibited nuclear positivity, as well. In addition, more than 50% of the cells in the paracortex were positive for Smad4. Furthermore, the Smad3 staining pattern was preserved in all malignant lymphomas, but four of these cases (15%) exhibited decreased expression of Smad4. All lymphoblastic lymphomas showed strong positivity in most of tumor cells, but one unspecified peripheral lymphoma, two nasal NK/T cell lymphomas, and one anaplastic large cell lymphoma were negative for Smad4. CONCLUSIONS: These results suggest that TGF-beta-specific Smads may be actively involved in signal transduction in lymphoid organs and that Smad-mediated TGF-beta signaling pathways are operative in malignant lymphoma. In addition, loss of Smad4 expression might be associated with development of some T-cell lymphomas.
Cytoplasm ; Germinal Center ; Humans ; Immunohistochemistry ; Lymphoid Tissue ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Signal Transduction ; Smad Proteins ; T-Lymphocytes ; Transforming Growth Factor beta