PULMONARY HYPERTENSION ASSOCIATED WITH PORTAL HYPERTENSION IN CHILDHOOD. Case Report of a 6-Year-Old Child and Review of the Literature

Pulmonary hypertension is a rare complication of portal hypertension. Reports of childhood cases are especially rare. This report describes an autopsy case of a 6-year-old boy with congenital biliary atresia followed by liver cirrhosis in whom severe hypertensive pulmonary arterial changes, including medial hypertrophy, intimal fibrosis and plexiform lesions were demonstrated. Fresh and organizing fibrin-platelet thrombi as well as probable organized thrombi with recanalization were occasionally found in the pulmonary vasculature, but it was thought that they had probably been formed locally as a late complication rather than being of thromboembolic origin. Retrospectively, the chest roentgenograms had revealed abnormalities suggestive of pulmonary hypertension since infancy, but the patient showed no apparent symptoms of it during life. Previously reported childhood cases of pulmonary hypertension associated with portal hypertension were briefly reviewed. Although the mechanism is presently not known, it is suggested that patients with portal hypertension, even in early childhood, are at risk of developing this unusual complication. ACTA PATHOL JPN 38: 897∼907, 1988.     

Peculiar axonal debris with subsequent astrocytic response (foamy spheroid body)

Summary Foamy spheroid bodies (FSBs) are described, as newly identified pathological structures occurring in human brain. FSBs favoured the substantia nigra pars reticulata (SNPR) and/or globus pallidus (GP) in degenerative conditions especially postencephalitic parkinsonism, progressive supranuclear palsy, pallido-nigro-luysial atrophy and multiple system atrophy. No FSBs were observed anywhere in the presence of substantia nigra pars compacta (SNPC) degeneration, such as occurs in idiopathic Parkinson's disease, or luysio-pallidal system degeneration, such as found in dentato-rubro-pallidoluysial atrophy or Joseph's disease. FSBs were also occasionally identified in the substantia nigra (SN) and/or GP of aged persons. In addition to SN and GP lesions, FSBs were seen in diffuse axonal lesions of long fibre tracts (the corpus callosum, the superior cerebellar peduncle) after non-missile head injuries, and in peri-infarct lesions. Under the light microscope, FSBs appear as slightly eosinophilic, foamy and nearly round objects with vague outlines, measuring approximately 10–50 m in diameter. Some FSBs contain coarse, eosinophilic clusters at their periphery. FSB stained black when stained by the Gallyas silver method. Some FSBs were immunohistochemically positive for synaptophysin and 68 kDa neurofilament. Glial fibrillary acidic proteinpositive fibres were observed alongside and/or inside some FSBs. Electron microscopically, FSBs were found to consist of collections of neuritic debris containing a variety of dense bodies and a small number of both mitochondria and neurofilaments. Some such collections were surrounded by astrocytic processes. These findings strongly suggest that FSBs are collections of small axonal debris destined for removal by astrocytes in due course. A variety of factors (degeneration of the SNPR and/or the GP, injury, infarction, ageing) seemed to be responsible for the histogenesis of FSBs.     

Ras p21 expression in nephroblastoma group tumors

Ras p21 expression in 30 cases of nephroblastoma and related tumors was evaluated by the immunoperoxidase method using monoclonal anti-ras p21 antibody (RAP-5) raised against a synthetic polypeptide fragment of ras p21. In normal renal tissue, the epithelial cells of convoluted and collecting tubules and arterial wall leiomyocytes were positively stained. In nephroblastoma tumors, some of the epithelial cells forming primitive tubular and glomerular-like structures in the nephroblastic-type tumors and the cells with rhabdomyocytic features were positive. However, the epithelial cells in more differentiated glandular structures of epithelial-type tumors, the rhabdoid cells in rhabdoid tumors and the cells in congenital mesoblastic nephroma failed to show positive staining. Nephroblastic cells, stromal cells and sarcomatous cells were also negative.     

Expression of matrilysin in vascular endothelial cells adjacent to matrilysin-producing tumors

Matrilysin is believed to play important roles in tumor progression and metastasis. In the present study, we analyzed matrilysin-producing cells in various human cancer tissues by immunohistochemistry and in situ hybridization. Tumor cells in colorectal carcinomas, pancreatic carcinomas, transitional-cell carcinomas of the kidney and small-cell lung carcinomas were frequently positive for matrilysin. In addition, we found that endothelial cells of arterioles and venules adjacent to matrilysin-positive tumors expressed matrilysin mRNA and protein. The endothelial cells adjacent to matrilysin-negative tumors and those in normal tissues were negative for matrilysin. Furthermore, analyses by casein zymography, Western blotting and RT-PCR showed that matrilysin was weakly expressed by cultured human umbilical vein endothelial cells. Our results suggest that the expression of matrilysin in vascular endothelial cells and in tumor cells may be regulated by common soluble factors, and that endothelial cell-derived matrilysin may contribute to tumor angiogenesis and tumor metastasis. Int. J. Cancer 72:441–445, 1997. © 1997 Wiley-Liss, Inc.     

Primary carcinoid of the testis: a case report

A 66-year-old male was hospitalized with right femur fracture and injury of the popliteal artery sustained in a traffic accident. The patient underwent external fixation and repair of popliteal artery. At operation, the orthopedists noticed a mass in the scrotum and requested our consultation. A right orchiectomy was done under the diagnosis of a testicular tumor. The tumor measured 9 x 6 x 5 cm in size. Histological diagnosis was pure carcinoid of the testis. Computerized tomography and other studies showed no tumorous lesions elsewhere. Symptoms of a carcinoid syndrome were not noted. No evidence of metastasis has been seen for 12 months after surgery.     

Ossifying fibromyxoid tumor of soft parts in a child: a case report.

Ossifying fibromyxoid tumor (OFMT) is a relatively rare tumor, most of which occur in adults with preferential localization in subcutis or muscles of the extremeties. Although Schwannian or cartilage origin has been suggested, histogenesis of this tumor still is unclear. The authors present a 6-year-old girl with retroperitoneal OFMT showing paraspinal extension, who is alive and tumor free 9 years after excision. The current case is the youngest reported patient showing unusual deep trunk site with surgically identified association with the spinal nerve. Both the clinical and histopathologic features strongly suggested Schwannian origin of this tumor.     

Pancreatic carcinoma in childhood. Report of an autopsy case and a review of the literature

Clinical and histological findings of pancreatic carcinoma in a 6-year-old boy are reported. Gradual change of histological appearance of the tumor during his course of 3 years and elevation of serum alpha-1-fetoprotein (AFP) are documented. Two biopsy specimens showed immature histological appearance compatible with pancreatoblastoma, and autopsy material showed well-differentiated adenocarcinoma with distinct ductal and acinar differentiation. Electron microscopy demonstrated zymogen-like granules in the apical portion of the neoplastic cells. Immunoperoxidase method demonstrated AFP in the neoplastic cells in addition to alpha-1-antitrypsin. Literature of pancreatic tumor in the young was reviewed, and characteristics of this case were discussed.