Localized constriction and torsion of the umbilical cord are rare cord abnormalities. The seg- mental absence of Whartion's jelly in the involved area of the cord is believed to be an etiologic factor of the constriction and subsequent torsion. In the past, constriction and torsion were thought to occur after the death of the fetus as a result of maceration. However, recently it has been suggested that the torsion of the constricted area might cause fetal death. We report an autopsy case of a localized constriction and torsion of the umbilical cord in a stillborn baby. The baby was delivered to a 27-year-old primiparous woman after 28 weeks gestation. The fetus was of 24 gestational weeks in size and weight. And the placenta weight was 259 gm. A grossly macerated fetus showed a cystic hygroma in the posterior neck. There were two areas of constriction in the umbilical cord, one at the squamoamniotic junction and the other, 15 cm from the fetal end. Torsions were noted in both constricted areas, more severe than in the mid portion of the cord. Microscopically, the Whartion's jelly was deficient and partly replaced by fibrosis in the constricted areas. It is believed that these two areas of constrictions and torsion are causally related to the intrauterine fetal death in this case.
Female ; Humans

This study is aimed to observe the expressions of different genes, including the extracellular matrix proteins, growth factors, and transcription factors during different developmental stages of mouse submandibular gland. Reverse transcription-polymerase chain reaction (RT-PCR) and the antisense inhibition in organ culture system were performed using mouse embryos and newborns. Total 140 mouse embryos (E14(80), E15(20), E16(20), E18(20)) and 30 newborn mice (D2(10), D3(10), D6(10)) obtained from 60 pregnant mice and 3 adult mice (3 weeks old) were used for the cDNA production and the salivary gland organ culture. Syndecan, perlecan, laminin alpha1 chain, TGF beta1, beta 3, and sonic hedgehog mRNAs were expressed in the early stage (E14~E16) of the submandibular gland development, whereas transglutaminase C (TGase C), E-cadherin, epimorphin, laminin beta2 and gamma1 chains, and HGF mRNAs were expressed in the middle and late stages (E16~E18, D2~D6). Antisense inhibition of different genes in the organ culture of E14 mouse embryos of submandibular gland showed specific growth retardation in the development of ductal and acinar cells. Especially, the antisense inhibition of perlecan, E-cadherin, laminin alpha1 chain, laminin beta2 chain, and syndecan mRNA arrested the growth of ductal and acinar cells. While the antisense inhibition of integrin beta5 greatly affected the acinar cell differentiation and also produced cystic dilatation of salivary ducts, the antisense inhibition of fibronectin showed aberrant growth of ectomesenchymal tissues of the mouse submandibular gland.
Acinar Cells ; Adult ; Animals ; Cadherins ; Dilatation ; DNA, Complementary ; Embryonic Structures ; Extracellular Matrix Proteins ; Fibronectins ; Gene Expression* ; Hedgehogs ; Humans ; Infant, Newborn ; Intercellular Signaling Peptides and Proteins ; Laminin ; Mice* ; Organ Culture Techniques* ; RNA, Messenger ; Salivary Ducts ; Salivary Glands ; Submandibular Gland* ; Syndecans ; Transcription Factors

Atresia of larynx is a rare fatal anomaly that should bring an immediate medical attention for proper managenent. We reported a case who died in neonatal period because of respiratory difficulty. His first problem was difficulty of inserting tracheal tube through the larynx. It was of interest in this case that he was presented with generalized edema and also massive lung edema. The lung was characterized by total absence of squamous and amniotic debris in the alveolar spaces and massive inflation of the alveoli by clear fluid that was thought to be amniotic fouid produced by the lung per se. Because there was no connection between oral cavity and the lungs, there would be no way the amniotic fluid outside the fetus. The laryngeal atresia was of infraglottic type and was complete with dispalced cricoid cartilage. Associated anomalies were left persistent supperior vena cava, perimembranous ventricular septal defect, spina bifida and focal cerebellar heterotopia.
Infant, Newborn ; Humans

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.

Sarcomatoid renal cell carcinoma is an uncommon tumor that has to be distinguished from renal carcinosarcoma. We have described three cases of sarcomatoid renal cell carcinoma showing different clinical and light microscopic features. An ultrastructural study of the tumor cells from the sarcomatoid area revealed frequent desmosomal junction, confirming the epithelial nature of the neoplasm. All three cases showed an aggressive clinical course and tended to invade adjacent organs or tissues. We believe that an histological and immunohistochemical examination in conjunction with an electron microscopic examination are necessary to diagnose sarcomatoid renal cell carcinoma.
Carcinoma, Renal Cell* ; Carcinosarcoma* ; Desmosomes ; Microscopy, Electron

During the past two decades, silicone (polydimethylsiloxane) has become one of the most extensively applied biomaterials. Although pure silicone is relatively inert and usually causes only minimal tissue reactions, it has been reported to evoke a definite foreign body reaction. We studied five cases of silicone-induced granulomas in various sites; two in the breast, one in the breast and axillary lymph nodes, one in the subcutis of the abdomen, back and extremities and one in the eyeball, to illustrate the salient histopathologic features of reactions to silicone with particular emphasis to its differences from paraffin granuloma. For this, 17 paraffinomas were also studied. Tissue reaction to silicone liquid and gel was characterized by numerous round to oval empty cystic vacuoles, mild to moderate fat necrosis, foreign body reaction, a variable degree of mononuclear inflammatory cell infiltration and mild focal fibrosis. The cystic spaces were relatively uniform and showed a snow-man like appearance. In contrast to the silicone granulomas, the paraffinomas, also refered to as sclerosing lipogranulomas showed diffuse sclerosis and frequent calcification around the cystic vacuoles. The cystic spaces in paraffinomas were swiss cheese-like configuration, and the content of the cystic spaces was dirty and frequently calcified. However, there were certain similarities between these two types of granulomas particularly in the early phases of the reaction, therefore, the history of silicone injection or implant, is sometimes critical to the diagnosis of silicone granuloma. Despite great technologic advances in the manufacturing of prostheses and medical equipment, droplets and/or particles of silicone still escape into the body tissues in a variety of ways; therefores, the pathologist should always wonder whether the histologic reaction observed is due to silicone or to some other foreign material including paraffin.

Congenital obstuction of pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veinsor total pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which accompanied other cardiac anomalies. Right pulmonary veins were involved in all seven cases including one bilateral case. Pulmonary veins were occluded totally in five and partially in three lungs. Pumonary catheterization and angiography were done for diagnosis. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural thickening, which were quite specific, whereas pulmonry venous dilatation was dominant findings in partial obstruction cases. Pulmonary perfusion scans (n=3) showed total perfusion defects in the cases of total occlusion of veins. MR imaging (n=2) demonstrated total occlusion of pulmonary veins in the venoatrial junction in two, and membranous focal obstruction in one lung. Two patients had pneumonectomy and histological confirmation, Although catheterization and angiography are essential for the diagnosis, MR imaging is thought to be useful for the diagnosis of pulmonary vein obstruction.
Angiography ; Catheterization ; Catheters ; Constriction, Pathologic ; Diagnosis ; Dilatation ; Drainage ; Estrogens, Conjugated (USP) ; Humans ; Hypertension ; Lung ; Magnetic Resonance Imaging ; Perfusion ; Pneumonectomy ; Pulmonary Edema ; Pulmonary Veins* ; Radiography, Thoracic ; Veins

The congenital renal cystic disease encompasses a complex group of pathologic and clinical entities. We retrospectively reviewed 42 cases of congenital renal cystic lesions classified into four Potter types in a series of 2,063 consecutive autopsies from 1981 to 1996. According to our study based on morphologic, clinical, genetic features and associated anomalies, type I and III are relatively compatible with Potter's original definition. However, it was reasonable that type II and IV are classified to the same group because of: 1) very similar histologic findings representing dysplastic kidney, 2) many associated anomalies, 3) no evidence of inheritance, and 4) presence of a combined type. Syndrome associated cysts, such as Meckel-Gruber syndrome, were also separately classified. If the dysplastic evidence was insufficient for diagnosis to the dysplastic kidney in type II and IV, then these cases would be better classified into a cystic disease associated with congenital hydronephrosis. We propose a classification of the congenital cystic disease of the kidney to be: 1) dysplastic kidney, 2) cystic disease associated with congenital hydronephrosis, 3) polycystic kidney, and 4) syndromic cystic disease.
Autopsy ; Classification* ; Diagnosis ; Hydronephrosis ; Kidney* ; Polycystic Kidney Diseases ; Retrospective Studies ; Wills

Kinky hair syndrome is a sex-linked recessively inherited copper metabolic disorder with severeneurodegenerative change and infant death. In 1962, Menkes and associates described five boys of a relatedpedigree with severe psychomotor retadation, seizures and widespread cerebral and cerebellar degeneration. In1969, Wesenberg and associated specified the radiological characterization of the syndrom. Symmetrical metaphsealspurring and diaphyseal periosteal reaction fo the long bones, anterior rib flaring, a malformed cerebral arterialsystem and subdural effusion. In 1972, Danks and associates found the disease to be associated with a defect ofcopper metabolism, confirmed by studies with labelled Cu. Authors experienced a case with characteristic clinicalpicture, and report cebral and abdominal arteriographic changes and plain radiographic findings with brain CT, DSAand post-mortem angiography.
Angiography ; Brain ; Copper ; Infant Death ; Menkes Kinky Hair Syndrome ; Metabolism ; Ribs ; Seizures ; Subdural Effusion

Kimura's disease is a chronic inflammatory disorder of unknown etiology, presenting usually as a painless subcutaneous swelling in the head and neck region or in the lymph nodes. We experienced a case of Kimura's lymphadenitis with characteristic Warthin-Finkeldey type polykaryocytes by fine needle aspiration cytology. The patient was a 10 - year old male, with two enlarged lymph nodes in the postauricular area. Fine needle aspiration cytology from the lymph nodes disclosed hypercellular smears with some scattered eosinophils and polykaryocytes in a polymorphous lymphoid background. There were also fragmented vessel walls and activated endothelial cell clusters in the slightly necrotic background. The Warthin-Finkeldey type polykaryocytes had three to thirty nuclei and prominent nucleoli with ill defined cytoplasmic borders. Their nuclei were arranged in grapevine or ring shaped clusters. As these polykaryocytes could also be found in lymph nodes and extranodal tissues of both reactive and neoplastic lymphoid disorders, polykaryocytes themselves are clinically nonspecific. However, the morphologic features of the Warthin-Finkeldey type giant cells are quite different from the foreign body type or Langhans' type giant cells. When the characteristic cytologic features of Kimura's disease such as significant number of eosinophils in a background of lymphoid cells asd proliferation of vessels and endothelial cells are also observed in the smear, it is possible to suggest this diagnosis in the appropriate clinical setting.
Biopsy, Fine-Needle* ; Cytoplasm ; Diagnosis ; Endothelial Cells ; Eosinophils ; Foreign Bodies ; Giant Cells* ; Head ; Humans ; Lymph Nodes ; Lymphadenitis* ; Lymphocytes ; Male ; Neck