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

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

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

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

Soft tissue myoepithelial tumors of the head and neck region are very rare, and only one case of soft tissue myoepithelial tumor occurring in the masticator space has been reported in the world literature. A case of soft tissue myoepithelial tumor with benign histomorphology, but with an invasive growth pattern, occurred in the masticator space of a 46-year- old male patient. Magnetic resonance imaging of paranasal sinus/nasopharynx revealed a well-defined, lobulated heterogeneous mass with high signal intensity and dense calcification in the masticator space between the left mandible ramus and pterygoid process. Grossly, the tumor was a well- circumscribed ovoid solid mass and consisted of yellowish gray glistening firm tissue. Histologically, the tumor showed a multinodular growth pattern and consisted of epithelioid cells in chondromyxoid stroma and of spindle-shaped to ovoid cells in the hyaline stroma. The tumor cells appeared bland, and no mitosis or necrosis was found within the tumor. The tumor focally invaded to adhered bone tissue. Immunohistochemically, the tumor cells were diffusely positive for epithelial membrane antigen, smooth muscle actin, but negative for other epithelial markers. Ultrastructurally, the cytoplasm of the tumor cells contained sparse microfilaments and subplasmalemmal densities. Attenuated desmosomes were commonly seen between the tumor cells.
Stomatognathic Diseases/*pathology ; Soft Tissue Neoplasms/*pathology/ultrastructure ; Myoepithelioma/*pathology/ultrastructure ; Middle Aged ; Microscopy, Electron ; Male ; Immunohistochemistry ; 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

A rare case of functioning oncocytic parathyroid adenoma associated with primary hyperparathyroidism was found in a 45-year-old woman. The preoperative serum calcium level was 13.1 mg/dL, the phosphate level was 2.44 mg/dL and the parathyroid hormone level was 153 pg/mL. Neck CT revealed a 2.5x1x1 cm, well enhanced mass behind the left thyroid gland, which was compatible with parathyroid adenoma. The removed parathyroid gland showed a well circumscribed, ovoid, brown colored, soft, solid mass. Histologically, this mass was composed of broad sheets of uniform cells having round dense nuclei and abundant eosinophilic granular cytoplasms. The adjacent rim of the normal parathyroid tissue was identified in the periphery of the mass. After operation, all hormone levels were normalized.
Adenoma ; Calcium ; Cytoplasm ; Eosinophils ; Female ; Humans ; Hyperparathyroidism, Primary* ; Middle Aged ; Neck ; Parathyroid Glands ; Parathyroid Hormone ; Parathyroid Neoplasms* ; Thyroid Gland

The present lesion was the first reported case of a benign intracystic phyllodes tumor in the inguinal region. We report the case of a 51-year-old female patient who presented with an inguinal mass. A clinical diagnosis of malignant lymphoma was considered in this case. The resected tumor was well-circumscribed and showed numerous papillary nodular protrusions into a central cystic cavity (3.5x2.5 cm). The microscopic findings showed hyperplastic epithelium-lined cysts with leaf-like intraluminal epithelium-lined bland stromal projections. The epithelial cell linings were strongly positive for estrogen and progesterone receptors.
Diagnosis ; Epithelial Cells ; Estrogens ; Female ; Groin ; Humans ; Lymphoma ; Middle Aged ; Phyllodes Tumor* ; Receptors, Progesterone

Pure choriocarcinoma is very rare in the testes, and host immune responses including tumor infiltrating lymphocytes are unusual in choriocarcinoma. This study reports a case of pure testicular choriocarcinoma with extensive lymphocytic infiltrate and granulomatous inflammation. Scrotal ultrasonography revealed a heterogeneous, hyperechoic intratesticular mass. -human chorionic gonadotropin levels were elevated in a radioimmunoassay. The hemorrhagic and necrotic solid mass was composed of two cell populations - mononuclear pleomorphic cells and intimately admixed multinucleated smudged cells. The tumor cells were positive for cytokeratin 7, epidermal growth factor receptors, human placental lactogen and p57. Many inflammatory cells were present within the tumor. The majority of infiltrating cells were CD8-positive cytotoxic cells, which also expressed granzyme-B and TIA-1. The tumor cells were positive for FasL, but negative for Fas. Therefore, this case seemed to escape the host defense response to the tumor due to the loss of Fas, although the cellular host immune response was still active.
Tumor Markers, Biological/analysis ; Testicular Neoplasms/*pathology/ultrasonography ; Male ; Lymphocytes, Tumor-Infiltrating/*pathology ; Humans ; Granuloma/*pathology/ultrasonography ; Chorionic Gonadotropin, beta Subunit, Human/metabolism ; Choriocarcinoma/*pathology/ultrasonography ; Adult