Postirradiation angiosarcoma of the chest wall and breast: issues of radiogenic origin, diagnosis and treatment in two cases.

The authors report two cases of postradiation angiosarcoma (AS) among 5,100 breast cancer patients treated in the period 1980-1994 at the National Institute of Oncology, Budapest. Relevant data in the literature is also reviewed to analyze the questions of radiogenic origin, diagnosis and treatment. Secondary AS occurred in these cases in a previously irradiated field after a 6- and 8-year latency period, respectively. Detailed histopathological and immunohistochemical examinations from the biopsy specimens confirmed the diagnosis as AS. The first patient was treated successfully with radical surgery. The second patient, with unresectable AS, died of rapid local progression within 4 months. The incidence of chest wall and breast AS after radiotherapy was found to be 0.46 per 1,000 in our patient population, which means an estimated odds ratio of 2.9 for secondary AS. Patients treated with radiotherapy for primary breast cancer are at higher risk for developing secondary AS compared to the healthy population. An etiological relationship between radiotherapy and subsequent AS of the chest wall and breast is likely, but still controversial. Initial radical surgery is the only effective treatment for achieving long-term survival. These very rare cases deserve special attention due to the atypical clinical appearance, difficulties of differential diagnosis and poor prognosis.

[Bcl-2 expression in testicular cancer in relation to tumor progression]. / Bcl-2 expresszió vizsgálata hererákokban, összefüggésben a tumorprogresszióval.

Bcl-2 expression has been studied extensively in a variety of human tumors. However, there are lack of clinical data in regard to its expression in germ cell testicular tumors (GCTTs). In this study we screened bcl-2 expression in 70 patient with GCTTs using the immunohistochemistry (IHC) and streptavidin biotin alkaline phosphatase method. Furthermore, we correlated this expression with metastatic behaviour and clinical stage. Overall, 41 (58%) carcinomas stained with anti-bcl-2 (DAKO-124) monoclonal antibody, By histologic type, these lesions included 11 (42.3%) of 26 seminomas (S) and 30 (68.18%) of 44 non seminomatous germ cell testicular tumors (NSGCT). The incidence of bcl-2 immunostaining was higher (P = 0.05, two-tailed, Fisher's test) in NSGCT than in seminomas. Bcl-2 expression was higher in tumors from metastatic patients than in tumors from metastatic-free patient (p = 0). There was a significant difference between the three stages of disease as to the expression of bcl-2 (chi 2 = 0). High level of bcl-2 was clearly dominant in tumors of advanced stages. The present finding revealed that bcl-2 expression occurs in GCTTs. Further, they suggested that bcl-2 is associated with a more progressed malignant phenotype in these tumors.

Drug resistance and sensitivity of germ cell testicular tumors: evaluation of clinical relevance of MDR1/Pgp, p53, and metallothionein (MT) proteins.

BACKGROUND: Although in vitro and clinical studies indicate that overexpression of P-glycoprotein (Pgp), p53, or metallothionein (MT) is involved in modulating drug resistance/sensitivity of cancer cells, the clinical relevance of the overexpression remains to be elucidated. MATERIALS AND METHODS: In this paper the expression and clinical value of Pgp, p53, and MT were evaluated immunohistochemically in 77 specimens of germ cell testicular tumors (GCT). We also studied the interrelationship(s) between the investigated markers. RESULTS: Pgp positivity correlated with cancers of advanced stages (P = 0.000). p53 and MT immunostaining does not predict a poor response to chemotherapy, but rather is correlated to a favorable clinical outcome (P = 0.001, P = 0.00006 respectively). We obtained an inverse association between Pgp and p53 (P = 0.0005), and positive strong association between p53 and MT immunoreactivity (P = 0.0002). CONCLUSIONS: Based on our results in patients with germ cell testicular tumors we assume that the poor clinical outcome seen in certain Pgp positive tumors is the consequence of Pgp association with a more progressive malignant phenotype, rather than its role in multidrug resistance (MDR). p53 and MT immunoreactivity predicts a better response rate to chemotherapy, wheres tumors lacking or demonstrating low MT and or p53 expression show a worse prognosis.

[Amidaron-induced dermatopathy resulting from unnecessary Cordarone therapy of ventricular parasystole]. / Kamrai parasystolia felesleges Cordarone-kezelése kapacsán jelentkezö amiodaron-dermatopathia.

The case history of a patient is reported who was treated with a variety of antiarrhythmics over a period of years because of refractory ventricular "bigeminy". As the arrhythmia did not respond to any kind of therapy, amiodarone treatment was started, which the patient received in a maintenance dose of 600-400 mg/day for 4 years. More recently, a bluish-grey hyperpigmentation of the face and other areas of the skin exposed to sunlight developed. A cutaneous biopsy of the hand revealed pigment deposits and lamellated lysosomal inclusions characteristic for amiodarone dermatopathy. The interactive, computer-assisted analysis of the ventricular ectopic activity has clearly demonstrated its innocent, parasystolic nature. The differentiation between ventricular extrasystolic and parasystolic activity is essential, because the latter arrhythmia does not require specific antiarrhythmic pharmacotherapy.

[Secondary membranous glomerulonephritis]. / Secunder membranosus glomerulonephritis.

Following 689 percutaneous renal biopsies, membranous glomerulonephritis was proved in 68 patients. In 16 (23.5%) an underlying primary disease was verified, and thus the glomerulonephritis the secondary form. The primary disease was SLE in 5 cases, diabetes mellitus in 5 cases, rheumatoid arthritis in 3 cases, chronic active hepatitis in 2 cases, an ulcerative colitis and eosinophilic angiolymphoid hyperplasia in 1 patient. As initial sign, nephrotic syndrome emerged in 87.5% of the 16 cases. Microscopic haematuria was observed in half of the patients, as was hypertension, while acute renal failure presented in only 1 case. Histologically, in 13 cases the predominance of early glomerular alterations was characteristic, while in 9 cases the picture proved to be equivocal and accompanied by some degree of interstitial alterations. During combined treatment, remission was achieved in 75%. Two patients with SLE died, but not as a consequence of renal failure. Transient side-effects of the treatment were registered in 5 cases. The principal pathogenetic and clinical differences between the individual secondary nephritis forms, and the difficulty of their differentiation from the idiopathic cases, even on repeated examination, are emphasized. In 3 patients the possibility of secondary renal processes was suggested by the histological picture, and this was proved by the detailed clinical findings.

[Glomerulonephritis in diabetics]. / Glomerulonephritis diabeteses betegekben.

Diagnosis of glomerulonephritis (GN) is rare among diabetics and few data relevant to this issue can be found in literature. In Institute of Pathology of "Szent-Györgyi Albert" University of Medicine the presence of GN was found in cases during the examination of renal biopsy material of 36 diabetics. All patients have suffered from diabetes mellitus of 2nd type and of less than 10 year existence, requiring no insulin treatment. In 2 cases diffuse diabetic glomerulosclerosis associated with GN. It is emphasized that kidney biopsy and its complex--light and electronmicroscopic and immunhistological--examination are essential to the diagnosis of GN of diabetics.

Long-term effects of steroid and cytostatic treatment on the clinical course of idiopathic membranous glomerulonephritis (retrospective study).

The clinical course of 30 patients with idiopathic membranous glomerulonephritis has been followed up for 8.1 +/- 3.4 (4-17) years. First, in each case a long-term prednisone treatment was administered. During this therapy 12 (40%) patients improved and 9 had remission. In the 18 (60%) steroid resistant cases the treatment was combined with cytostatic drugs. After a combined regime for 1.9 +/- 0.8 years, 8 patients have improved, 2 of whom remitted. It has been suggested that due to a long-term immunomodulator therapy the level of urinary protein excretion might be reduced in 77% of patients. There was no effect of the applied treatment in 7% of cases, still in 16% a gradual deterioration was observed. Relapsing nephrotic syndrome has occurred in 3 (10%) of the total cases. Side effects requiring cessation of treatment were not observed.

Incidence of nephrotic syndrome in patients with glomerulonephritis.

The occurrence of nephrotic syndrome was studied in different types of glomerulonephritis. In 284 consecutive adult cases the diagnosis was confirmed by the histological evaluation of percutaneous renal biopsy. Nephrotic syndrome was more frequent in minimal change glomerulonephritis, in focal sclerosis and in membranous nephropathy. The incidence of glomerulonephritis decreases, while the relative incidence of nephrotic syndrome increases with age. Glomerulonephritis is more frequent in men whereas the relative incidence of nephrotic syndrome is higher in women.

Arteriolar lesions in human renal biopsy material with special regard to the ultrastructural changes in the basal lamina network of the vascular wall.

The arteriolar changes in renal biopsy samples were studied by light and electron microscopy and immunohistologic observations. Arteriolar hyaline thickening was found to occur in virtually all renal diseases, regardless of whether these were accompanied by hypertension or not. Only amyloidosis and dense deposit glomerulonephritis were accompanied by specific ultrastructural arteriolar changes. The nonspecific "hyalin" was shown ultrastructurally to contain various components: accumulated basement membrane material, fine granular deposit (with filamentous or lipid details), and granulovesicular and threadlike membrane structures. Presumably the material constituting these structures originates partly from the blood and partly from elements of the vascular wall itself.

Inhibiting effect of foetal renal antigen on leukocyte migration in human glomerulonephritis.

In 141 cases of glomerulonephritis confirmed by renal biopsy it was demonstrated that foetal glomerular basement membrane antigen caused a migration inhibition most frequently in minimal change glomerulonephritis. Cellular hypersensitivity was less common in membranous nephropathy, membranoproliferative (I-III) glomerulonephritis, IgA nephropathy and lupus nephritis. The correlation between LMT positivity and the occurrence of renal tissue IF activity was a linear one, but in one type, minimal change glomerulonephritis, there was no such correlation. The occurrence of LMT positivity does not show any considerable difference in glomerulonephritis with and without nephrosis.

[Panarteritis nodosa with fatal outcome]. / Panarteriitis nodosa mit tödlichem Verlauf.

A case of panarteritis nodosa with lethal outcome is reported. Initially, the disease involved only the skin, later on it became systemic and lethal due to damage of pancreas and kidney. The atypical course and the therapy resistance of the disease are emphasized.

Concretions in renal basement membranes.

A study of 196 renal biopsies was carried out by electron microscopy. In 78 cases concretions were detected, which x-ray microanalysis (EDAX) proved to be calcium phosphate deposits. Concretions always occurred in damaged basement membranes, especially in the basement membranes of Bowman's capsules and of proximal convoluted tubules. They were frequently also found in sclerotic foci. Both nephrotic syndrome (through hypercholesterolaemia) and progressive sclerotic processes seem to play a role in the pathogenesis of concretion formation. Concretions are considered to represent early dystrophic calcification.

[Disease of dense basement membranes]. / Bolezn' plotnykh bazal'nykh membran.

In the disease of dense basal membranes, typical membrane lesions of the immune complex origin were found both in the kidneys and the spleen. On this basis the disease of dense basal membranes is considered to be a distinct entity of systemic nature.