Renal cell carcinoma with rhabdoid features: an aggressive neoplasm

AIMS: Only a few reports on renal cell carcinoma with rhabdoid features have been published. This study was performed to investigate the clinicopathological characteristics of renal cell carcinomas with rhabdoid features. METHODS AND RESULTS: Among 253 cases of renal cell carcinoma in adults, eight cases with rhabdoid features were detected. Rhabdoid areas ranged from 10% to 90% of each of the cases. Seven of the eight cases were TNM stage III or IV, and four of the eight cases died within 8 months of surgery. Immunohistochemically, the rhabdoid areas were positive for CAM 5.2 (4/8), AE1/AE3 (6/8), epithelial membrane antigen (6/8) and vimentin (8/8), and negative for myogenetic markers (0/8). The mean MIB-1 labelling index in the rhabdoid areas was higher than that in the definite carcinomatous areas. Ultrastructurally, perinuclear whorls of intermediate filaments were demonstrated in three of the eight cases using paraffin-embedded blocks. CONCLUSIONS: The rhabdoid areas in renal cell carcinoma have histological, immunohistochemical and ultrastructural similarities to malignant rhabdoid tumours. Renal cell carcinoma with rhabdoid features is a highly aggressive neoplasm and its malignant behaviour may be due to the high cell-proliferative activity of the rhabdoid areas. Rhabdoid features in renal cell carcinoma may represent the endpoint of clonal evolution of renal cell carcinoma (especially in clear cell type cases).     

Two cases of B cell lymphoma associated with hemophagocytic syndrome

B cell lymphoma-associated hemophagocytic syndrome (B-LAHS) is clinically characterized by hepatosplenomegaly and bone marrow invasion without lymphadenopathy and skin lesions. Several cases of B-LAHS have been reported to demonstrate histopathologic findings of intravascular lymphomatosis (IVL), which in Western countries is characterized by a high rate of skin involvement and, rarely, bone marrow involvement and hemophagocytosis. Here we describe two interesting cases of B-LAHS. One patient was a 52-year-old woman whose bone marrow showed proliferation of large CD20-positive cells and hemophagocytosis at presentation. Combination chemotherapy was not effective, and the patient died of progressive disease. At autopsy, the lymphoma cells showed extravascular proliferation in many organs such as the bone marrow and liver, whereas in the adrenal glands, the lymphoma cells showed intravascular proliferation. The other patient was a 50-year-old man who had swellings of the bilateral kidneys and adrenal glands at presentation. Skin involvement by large lymphoma cells, a rare complication of B-LAHS, was observed. At autopsy, there was no evidence of IVL. Both of these patients showed high fever and cytopenia, and the disease took an aggressive clinical course, as in other reported cases of B-LAHS.     

Response of plasma ACTH and adrenocortical hormones to potassium loading in essential hypertension

The effect of potassium loading on plasma adrenocortical hormones concentrations in 9 patients with essential hypertension (EH) was investigated. The plasma renin activity (PRA), plasma concentrations of growth hormone (GH), ACTH, cortisol, deoxycorticosterone (DOC), 18-hydroxy-deoxycorticosterone (18-OH-DOC) and aldosterone, and serum electrolytes were measured before and after potassium chloride (KC1) infusion (0.33 mEq/kg/h, for one hour). The KC1 infusion caused significant increases in serum potassium levels and plasma levels of GH, ACTH, cortisol, DOC, 18-OH-DOC and aldosterone, while PRA remained unchanged. Regression analysis at 30 min revealed significant positive correlations between delta ACTH and delta cortisol, between delta ACTH and delta DOC, between delta ACTH and delta 18-OH-DOC. However, the relationship between delta ACTH and delta aldosterone was not statistically significant. These results suggest that (1) acute potassium loading causes a significant increase in the plasma ACTH level and increased levels of adrenocortical hormones may be produced by increased ACTH secretion, and (2) it may be considered that a part of the increased level of plasma aldosterone following acute potassium loading may arise from increased ACTH secretion in EH.     

Effects of N-(3-[3-(1-piperidinylmethyl)phenoxy]propyl)-acetoxyacetamide hydrochloride (TZU-0460), a histamine H2-receptor antagonist, on gastric acid secretion and ulcer formation

N-(3-[3-(1-Piperidinylmethyl)phenoxy]propyl)-acetoxyacetamide++ + hydrochloride (TZU-0460) was compared with cimetidine for the effects on gastric acid secretion in dog and rat and on ulcer formation in rat. TZU-0460 as well as cimetidine, given i.v. or p.o., produced a dose-dependent inhibition of acid secretion stimulated by histamine, pentagastrin or carbachol in Heidenhain gastric pouch dogs and gastric lumen-perfused rats. In dog, the relative potencies of TZU-0460 to cimetidine, given p.o. and i.v., were 6.2 and 5.1, respectively, in acid secretion stimulated by histamine, and those gained by i.v. route were 3.5 by pentagastrin and 4.2 by carbachol. In rat, however, relative potencies of TZU-0460 to cimetidine, given i.v., were 2.8, 2.2 and 1.6 in acid secretion stimulated by histamine, pentagastrin and carbachol, respectively. TZU-0460, given p.o., prevented the formation of gastric ulcers induced by exposure to stress, pylorus-ligation, both pylorus-ligation and acetylsalicyclic acid, indometacin or reserpine in rats. TZU-0460 was about twice as active as cimetidine on these experimental models of gastric ulcers. TZU-0460, given p.o., prevented the formation of duodenal ulcer induced by cysteamine in rats, whereas cimetidine failed to prevent it significantly.     

Serial water changes in human skeletal muscles on exercise studied with proton magnetic resonance spectroscopy and imaging

In vivo ¹H-magnetic resonance imaging (MRI) enabled us to study the distribution of water in living tissues and to document changes in human skeletal muscles during physical exercise. The purpose of the present study was to determine the total muscle water changes after exercise using water in¹H-MR spectroscopy and to compare these changes to the signal intensity change on T₂*-weighted images and/or to the T₂ value change. Seven young male volunteers were positioned in a 1.5 T Philips MR imaging system. They were then asked to dorsiflex their ankle joint against a 2 kg weight once every 2 seconds for 2 minutes. The peak height of water declined according to the clearance curve after exercise in all seven cases with the¹H-MRS similar to the signal intensity. The increasing rate at peak height of total muscle water exceeded both the signal intensity and the T₂ value because the water peak height on the¹H-MRS included the extracellular water. In addition, we measured the changes in signal intensity in both calf muscles after walking race exercise. The time intensity curves were used to draw a clearance curve for each muscle group after exercise. It was possible to discern which muscle was used most from the T₂*-weighted image that was obtained once after exercise.     

PPD-specific IgG1 antibody subclass upregulate tumour necrosis factor expression in PPD-stimulated monocytes: possible link with disease pathogenesis in tuberculosis

Cachexia is a prominent feature of advanced tuberculosis, in association with increased expression of the monokine tumour necrosis factor (TNF)-alpha. Monocytes, have high affinity receptors (mannose, complement and Fc gamma1 and gamma111) which mediate antigen uptake and subsequent cytokine activation. Several mycobacterial proteins, including PPD, can stimulate TNF-alpha secretion from monocytes. However, the role of various receptors in stimulating or regulating TNF-alpha secretion is still unclear. We have previously shown selective augmentation of opsonic antibodies (IgG1 and IgG3) in tuberculosis patients with advanced pulmonary disease. We now analyse the role of opsonizing antibodies in modulating TNF-alpha expression in antigen stimulated monocytes. PPD was used as the prototypic mycobacterial antigen to stimulate monocytes from PPD skin test negative donors (n = 7) in the presence of plasma from tuberculosis patients (n = 8), containing known amounts of IgG1 and IgG3 anti-PPD antibodies. TNF-alpha secretion was enhanced in the presence of TB plasma (4/8) but not in the presence of control plasma. Using Spearman Rank analysis (two-tailed Fisher exact test), a significant correlation (rho = 0.762; P = 0. 04) was observed between IgG1 antibodies and enhancement of TNF-alpha secretion. No significant association was observed with IgG2 (rho = 0.310; P = 0.41), IgG3 (rho = 0.089; P = 0.81) or IgG4 (rho = - 0.357; P = 0.347) subclass antibodies. Absorption of IgG1 with protein 'A' removed the enhancement of TNF-alpha secretion activity from the plasma samples. Our results therefore indicate that IgG1 antibodies may enhance the chronic release of TNF-alpha in TB patients with progressive disease and, for the first time, show a direct link between disease pathogenesis and raised antibody levels.     

Hip dysfunction‐related urinary incontinence: A prospective analysis of 189 female patients undergoing total hip arthroplasty

Patients reporting that their symptoms of urinary incontinence have decreased after total hip arthroplasty can be encountered in orthopedic practice. In this questionnaire‐based study, we prospectively evaluated the symptoms of urinary incontinence before and after total hip arthroplasty. The International Consultation on Incontinence Questionnaire‐Short Form was used to prospectively evaluate these symptoms in 189 consecutive female patients undergoing total hip arthroplasty. The patients were asked to fill in a questionnaire before and 3 months after surgery. A decrease in the International Consultation on Incontinence Questionnaire‐Short Form score of ≥1 point was defined as an improvement, whereas an increase of ≥1 point was defined as worsening. A total of 81 (43%) patients reported urinary incontinence before total hip arthroplasty. At 3 months after surgery, symptoms of urinary incontinence were improved in 64% of these patients, remained unchanged in 32% and worsened in 4%. Mean International Consultation on Incontinence Questionnaire‐Short Form score in patients with urinary incontinence before surgery significantly improved from 6.0 to 3.5 (P = 0.0002). These findings suggest a relationship between hip joint function and pelvic floor function, and consequently a hip dysfunction‐related urinary incontinence.     

Orthognathic surgery case of metal allergy applied orthodontic appliances made of organic polymer

This case report presents a case that underwent orthognathic treatment with non-metallic appliances made of organic polymer. The patient was a 19-year-old female with mandibular protrusion and maxillary anterior crowding. The overbite was +3.0 mm, and the overjet ?3.0 mm. She was allergic to nickel (Ni³⁺), cobalt (Co³⁺) and cobalt–chrome (Co–Cr³⁺). The pre-surgical orthodontic treatment included the extraction of the maxillary right-and-left second premolars and mandibular right third molar. An orthodontic appliance made of organic polymer was employed. Orthognathic surgery with bilateral sagittal split ramus osteotomy was carried out. After 32 months pre-surgical orthodontic treatment, the left and right sides were set back 9 and 7 mm, respectively. Rigid fixation of the splitted mandible was employed using two absorbency screws in each side, and the inter-maxillary fixation was performed with elastic threads for 8 days. Total treatment period was 42 months. For retention, a non-metal retainer was employed for the upper and lower dentition. The consistent non-metal treatment on orthognathic case of metal allergy was attained successfully. However, the maxillary molars and the mandibular anterior teeth appeared elongated. This was considered to be caused by the low rigidity of a plastic material.     

Rhabdoid features in leiomyosarcoma of soft tissue: with special reference to aggressive behavior.

The presence of rhabdoid cells has been reported in various types of malignant neoplasms and has been determined to be a predictor of aggressive behavior of neoplasms regardless of tumor histogenesis. One hundred and thirteen cases of leiomyosarcoma, selected from 1800 soft tissue sarcomas, were reviewed on hematoxylin and eosin sections, and immunohistochemical staining when available, and seven cases with rhabdoid features were retrieved. Clinicopathologic differences were analyzed to compare between cases with rhabdoid features and those without rhabdoid features. In the seven cases with rhabdoid features, two were intra-abdominal, and the others arose in external soft tissues including muscle, subcutis, and cutis. Patient age ranged from 33 to 84 years, three were female, and four were male. Tumor size ranged from 3 to 22 cm. Clinical evidence showed no differences from those cases without rhabdoid features. Histologically, one of the abdominal cases was epithelioid leiomyosarcoma. Two of the 7 cases were better subclassified as pleomorphic leiomyosarcoma, in which rhabdoid cells are diffusely scattered. In cases other than those with pleomorphic leiomyosarcomas, foci of anaplastic areas were observed, and collections of rhabdoid cells were present in those areas. Immunohistochemical examination of the cases confirmed myogenic differentiation, and showed rhabdoid cells being positive for vimentin and desmin in the inclusion bodies, and diffusely so for muscle actin in the cytoplasm. After dividing all the cases of leiomyosarcoma by their location, prognostic analysis was performed. Leiomyosarcoma of external soft tissue with rhabdoid cells showed a tendency for poorer prognoses than cases without rhabdoid features. On the contrary, retroperitoneal cases did not. This study indicates that rhabdoid features are associated with aggressive biological behavior in leiomyosarcoma of the external soft tissue.     

Depressive episodes of bipolar disorder in early teenage years: changes with increasing age and the significance of IQ

BACKGROUND: Depressive (or depression-like) episodes are the most common manifestations of bipolar affective disorder in early teenage years. The present paper analyses the clinical features and their changes over time in these episodes. METHODS: By a prospective study on children who had their first affective or psychotic episodes between the ages of ten and fifteen, those who eventually met the ICD 10 diagnostic criteria for bipolar disorder were selected and followed up. RESULTS: There were three boys and nine girls. Their early depressive episodes were characterised by psychotic features and clinging to the mother in most cases, and in some by brief episodes and/or a good response to sulpiride. However, these characteristics tended to disappear with increasing age. Five children (42%) had an IQ of 61-75. LIMITATIONS: Generalisability of the results is limited because of the small number of patients and the lack of control groups. CONCLUSIONS: Bipolar disorder in early teenage years may show clinical features and a drug response that are different from those in adulthood. Low IQ may expedite the onset of bipolar disorder.