Relapse of colon cancer followed by polymyositis: Report of a case and review of the literature

The direct causal relationship between dermatomyositis-polymyositis (PM) and malignancy remains controversial. We describe herein the case of a patient who underwent surgical treatment for colon cancer, which had preceded the onset of PM with tumor relapse. The PM markedly improved following the initiation of steroid therapy, and has remained under control, probably as a result of chemotherapy. The current concepts of variable clinical courses and the possible mechanism for the association of PM with malignancy are discussed following this case report.     

Primary malignant lymphoma of the rectum with distant metastasis to the stomach: Report of a case

Primary extranodal malignant lymphoma frequently occurs in the gastrointestinal tract; however, it is rarely encountered in the rectum. We report herein the case of an 85-year-old man who underwent abdominoperineal resection for primary malignant lymphoma of the rectum, and 1 year later, developed hematemesis caused by gastric metastasis. To our knowledge this is the first case report of such distant metastasis developing from malignant lymphoma of the rectum.     

Evidence of Delayed Mesangial Transport of Human IgA in Glomeruli of ddY Mice Pretreated with Sheep Anti-type IV Collagen Serum

In order to investigate whether mesangial transport by glomeruli is delayed in ddY mice pretreated with sheep anti type IV collagen serum, the mice were administered an overload of human IgA myeloma serum. Non pretreated ddY mice used as controls and both experimental and control BALB/c mice were also processed in a similar manner. The intensities of mesangial deposition of human IgA were examined periodically and were found to correlate well with deposition of mouse IgA. Both mouse and human IgAs showed a gradual increase for up to 8 experimental weeks. In the control young ddY mice, however, the overloaded mesangial human IgA quickly disappeared, presenting no appreciable mesangial deposition of autologous IgA. In sharp contrast, both the experimental and control BALB/c mice showed an initially prolonged and rather heavy mesangial deposition of human IgA, followed by a gradual decrease and somewhat light mesangial deposition of autologous mouse IgA. These results obtained using experimental ddY mice appear to confirm the possibility that non immunological local trapping, due to retardation of mesangial transport function, causes mesangial deposition of autologous mouse IgA in this particular strain. Acta Pathol Jpn 39: 289 295, 1989.     

EXPERIMENTAL MESANGIOPROLIFERATIVE GLOMERULONEPHRITIS IN RATS INDUCED BY INTRAVENOUS ADMINISTRATION OF ANTI-THYMOCYTE SERUM

Focal glomerulonephritis was induced in rats, by a single intravenous injection of anti-Thy-1.1 antibody (ATS). One hour after the administration, the glomeruli of affected rats developed necrotic changes of the mesangial cells while after two hours, mesangiolytic changes appeared. From six days onwards, focal segmental mesangial proliferation which persisted until 30 days, occurred. This is thought to be the first report of experimental nephritis induced by pure anti-mesangial antibody.     

RETARDED MESANGIAL TRANSPORT AND ITS PATHOMORPHOLOGIC SEQUELAE IN HUMAN AND EXPERIMENTAL RENAL DISEASES

Human renal biopsy specimens (472 cases) from varied kidney diseases, especially minimal glomerular change group and other idiopathic glomerular diseases having nephrotic manifestation of mainly juvenile individuals, showed morphologic evidence of paraarterial deposits of afferent arterioles at the glomerular entrances in more than 50% of examined cases. Because these deposits were often accompanied with concomitant mesangial, intraarterial and subendothelial deposits of afferent arterioles, it was felt that retarded mesangial transport which is ordinarily associated with certain glomerular diseases might be an important factor to produce these particular paraarterial deposits. The referred deposits of minimal glomerular change group cases were thought to predispose the occurrence of focal sclerotic capillary lesions at the vascular poles of glomeruli. The experimental chronic nephrotic rats produced by daily administration of aminonucleoside of puromycin revealed mesangial dysfunction with increased uptake and retarded disposal of secondarily overloaded aggregated human gamma globulin at mesangial areas in glomeruli. Besides, the increased deposits of autologous serum proteins in mesangial areas and arteriolar walls were common findings in those rats, and these deposits were observed to be always preceded to the occurrence of segmental sclerotic changes of glomeruli, which were often associated in the later stage of this experiment. ACTA PATHOL. JPN. 33: 219∼236, 1983.     

Surgical treatments for 7 pituitary adenomas in multiple endocrine neoplasia type 1

We analyzed 7 pituitary adenomas in patients with multiple endocrine neoplasia type 1 (MEN1). Their incidence was 1.6% among 450 patients with pituitary adenomas which had been treated surgically in our department between 1978 and 1999. The age, gender, symptoms, type of hormone secretion, pathological and operative findings in the MEN1 patients were not apparently different from those in the non-MEN1 patients. Incidence of non-functioning pituitary adenomas, however, was more frequently encountered in our series than that in previous reports among Caucasian people. One patient who had 5 previous operations for the MEN tumors died following postoperative subarachnoid hemorrhage. Four of the seven patients presented initial symptoms related to pituitary adenomas and increased serum Ca level was retrospectively recognized in three of the four at the time of treatment for the pituitary adenomas.     

The Histology of the Lung in Neonates with Tricuspid Valve Disease and Gross Cardiomegaly Due to Severe Regurgitation

Lung autopsies from four neonates with Ebstein's anomaly or tricuspid valve dysplasia and gross cardiomegaly were examined. The mean cardiothoracic ratio was 92 ± 0.5%. The degrees of pulmonary hypoplasia and pulmonary artery muscularization were assessed and were compared with those in lungs from four controls and four patients with diaphragmatic hernias. Lung and body weight ratios and radial alveolar counts, which reflect pulmonary hypoplasia and immaturity, were significantly decreased only in patients with diaphragmatic hernia. The thickness of the medial muscle layer in small pulmonary arteries was greater in patients with diaphragmatic hernia; however, in patients with tricuspid valve disease, it was relatively small. Abnormal vascular muscle extension was seen only in patients with diaphragmatic hernia. In the cases of tricuspid valve disease, although cardiomegaly and lung compression were severe, lung hypoplasia and immaturity were not, and neither abnormal medial thickening nor extension were found. Our results suggest that, at least in full-term infants with tricuspid valve disease, surgical relief of lung compression may improve respiratory function, even if the cardiomegaly is severe.     

Peculiar respiratory response observed during sleep-onset REM sleep of an infant with Ondine's curse

We treated an infant with congenital central hypoventilation syndrome ("Ondine's curse"). She was cyanotic and given ventilatory support at the first hour after birth. An investigation of sleep state and respiration performed at the age of 3 months led to this diagnosis. Hypoventilation persisted in all sleep stages, with the most severely reduced tidal volumes occurring during delta-wave sleep (stages 3 and 4). In addition, severe secondary reduction in tidal volumes occurred in sleep-onset REM sleep. This phenomenon was absent in non sleep-onset REM sleep. At 4 months of age, her respiratory treatment was successfully converted to positive-pressure ventilation via a nasal mask, thus avoiding tracheotomy. This transition to noninvasive ventilatory support dramatically improved her quality of life during wakefulness. This report may be a clue to discuss the function of sleep-onset REM sleep seen in the early stage of life and suggests that nasal mask ventilation is a viable option in selected cases with congenital central hypoventilation syndrome (CCHS).     

An anaplastic pleomorphic xanthoastrocytoma with periventricular extension: An autopsy case report and review of the literature

Pleomorphic xanthoastrocytomas (PXAs) are rare low-grade astrocytic tumors that typically present as superficial nodular cystic tumors of the cerebrum attached to the leptomeninx. Histologically, they are pleomorphic, hypercellular glial neoplasms. Despite the presence of microscopic pleomorphism, patients’ postoperative prognosis is generally good. Anaplastic PXAs (APXAs) have a high mitotic index and patients with APXAs have a worse prognosis than patients with PXAs. Here, we report an autopsy case of APXA initially diagnosed as PXA. After gross total resection, the tumor recurred and was diagnosed as an APXA; thereafter, the patient died. An autopsy revealed that the tumor had relapsed at the primary site and had spread to the leptomeningeal space while concurrently invading the cerebrum including the periventricular area forming multifocal lesions. The histological findings of the autopsy were similar to those for epithelioid glioblastoma (EGBM) and small cell glioblastoma (SCGBM). In particular, the periventricular area with multifocal lesions was composed of SCGBM-like cells. It has been shown that multifocal lesions are frequently identified in patients with SCGBM. This is the first histopathologically confirmed case of APXA-related tumor presenting with periventricular extension and multifocal lesion formation. The periventricular extension might be a feature of PXAs and APXAs. However, suspected periventricular spread on imaging in past cases of PXAs and APXAs might instead represent the malignant transformation of these tumors to glioblastoma-like high-grade tumors, which often show SCGBM-like histological patterns.