Inflammatory myofibroblastic tumor of the esophagus in childhood: a case report and a review of the literature.

Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal disease of low malignancy consisting of inflammatory cells inside a mesenchymal stroma comprising myofibroblasts. Biological behavior is variable, recurrence is uncommon, metastatic disease is rare. Treatment strategy is based on the status of low-grade malignant tumor.

Metronomic chemotherapy with the COMBAT regimen in advanced pediatric malignancies: a multicenter experience.

Background: The outcome of children with refractory/relapsed malignancies remains poor and novel therapies are urgently required. One of the promising approaches is metronomic chemotherapy. We present the clinical results of 74 children with advanced solid tumors treated according to treatment recommendation with data registry in three European pediatric centers.

[More than 10 years of complete remission of monoclonal gammopathy of undetermined significance and cessation of light chain deposition disease-associated nephrotic syndrome following treatment with vincristine, adriamycin and high-dose dexamethasone (VAD)].

Light chain deposits in the form of amorphous material (light chain deposition disease) damage most frequently kidneys and, less frequently, they affect other organs. The incidence of light chain deposition disease is much lower than that of AL-amyloidosis. Symmetrical swelling of both legs, swelling of the eye lids, erythrocyturia and nephrotic proteinuria were the first signs of light chain deposition disease in our patient.

[Fibrillary glomerulonephritis--a rare cause of nephrotic syndrome].

Fibrillary glomerulonephritis (FGN) is a rarely diagnosed disease with clinical manifestations such as proteinuria, microscopic hematuria, nephrotic syndrome or decreased kidney function. Around one half of patients develop chronic renal failure in the course of several years. The diagnosis of fibrillary glomerulonephritis is to be established only basing on the results of renal biopsy.

[Amplification of 2p13-16 in a case of extranodal large cell diffuse B-cell lymphoma].

Comparative genomic hybridisation (CGH) allows to detect genetic abnormalities associated with poor prognosis in subset of patients with diffuse large B-cell lymphoma (DLBCL). Amplification of 2p13-16 represents an adverse genetic sign especially in extranodal DLBCL. In the present case, 2p13-16 amplification was revealed by CGH in extranodal DLBCL.

[Primary bone hyperparathyroidism].

The authors report the case of a 19-year-old girl with osteolytic bone lesions in whom primary hyperparathyreoidism caused by parathyroid gland adenoma was diagnosed. They emphasize necessity to be aware of a relationship of this rare condition to bone lesions that may be benign in appearance and may show ambiguous histological findings. The patient underwent excision of the adenoma and subsequent substitution therapy, which resulted in the healing of bone lesions.

[Fibrillary immunotactoid glomerulonephritis--an unusual cause of irreversible kidney failure].

Fibrillary-immunotactoid glomerulopathy (FITGP) is a rare cause of nephrotic syndrome. In this patient, male 65-years-old, respectively, fibrillary glomerulonefritis (FGN) was diagnosed by percutaneous renal biopsy. Clinically, the disease manifested with long term proteinuria and nephrotic syndrome without any associated systemic disorder.

Epithelioid hemangioendothelioma of the mandible.

The authors describe a locally recurrent tumor of the mandible whose microscopic structure and immunohistochemical findings corresponded to an epithelioid hemangioendothelioma. The tumorous cells had an epithelioid character, they created rudimentary vascular lumina, and they focally expressed vascular markers CD 34, CD 31, and F VIII. Locally the tumor grew aggressively.

[Fibrillary glomerulonephritis].

Fibrillary glomerulonephritis (FGN) is a rare cause of nephrotic syndrome. In three patients (65-year-old male; 57- and 38-year-old females) FGN was diagnosed by percutaneous renal biopsy. Clinically, the disease manifested itself with long term proteinuria and nephrotic syndrome without any associated systemic disorder.

Extraorbital giant cell angiofibroma.

We describe a case of giant cell angiofibroma (GCA) in extraorbital location. A 47-year-old man developed a subcutaneous tumor on the forearm, which measured 6 cm in diameter and was well demarcated. Histologically, small-sized vessels similar to arterioles, sometimes with a hyalinized wall, were regularly distributed throughout the tumor tissue.

[Lymphoproliferative disease after transplantation].

Herein we describe 7 cases of posttransplantation lymphoproliferative disease (PTLD), 5 in men and 2 in women (aged from 25 to 62 years), occurring from 4 months to 12 years (mean, 7 years) after transplantation. Our patients were recipients of kidney, kidney and pancreas, heart, and autologous peripheral haematopoetic stem cells. Four cases were diagnosed as monomorphic and three as polymorphic type of PTLD according to the WHO classification.

[Incidence of carcinomas in goiters and their combination with Hashimoto's thyroiditis].

In the investigated group 300 patients were operated for a thyropathy mostly struma nodosa, tumours were proved in 69 cases; 38 of them were carcinomas of various extent and 3 of them were combined with Hashinoto's lymphomatous goitre. The overwhelming majority of the carcinomas were papillary carcinomas (22 cases). The incidence of papillary carcinoma in Hashimoto's goitre was about 8%; this fact calls for more attention to autoaggressive thyroiditis both in examination of surgical material, and during autopsies in middle-aged women, where this thyroiditis is often clinically silent.

Pleomorphic hyalinizing angiectatic tumor.

Authors present an additional case report of pleomorphic hyalinizing angiectatic tumor (PHAT), only recently recognized entity amongst the soft tissue tumors. 63-year-old man underwent surgery for subcutaneous tumor growing on the forearm. Grossly, ovoid well demarcated tumor 4.

[Sclerosing epithelioid fibrosarcoma].

Sclerosing epithelioid fibrosarcoma (SEF) was first described in 1995 and since then 39 cases have been reported. Here we describe 6 cases of SEF (3 in women and 3 in men). The patients aged from 22 to 79 years.

[Diagnosis of malacoplakia in a transplanted kidney by needle biopsy].

Malakoplakia is an uncommon inflammatory condition rarely involving parenchyma of transplanted kidney. We report a 44-year-old female recipient of a cadaveric renal allograft treated with cyclosporin A and prednisone. After transplantation, E.

[Leiomyosarcoma of the bone.].

The case history presents a patient with a rare malignant bone tumor - leiomyosarcoma in the region of distal femur. A large resection of the tumor was made with a specially tailored distal femur replacement. The authors emphasize the necessity of a detailed histological examination including immuno-histochemistry which proved the diagnosis of leiomyosarcoma as opposed to the original diagnosis of fibrosarcoma.

[Low grade malignant fibromyxoid sarcoma].

Two cases of low-grade fibromyxoid sarcoma are presented. In a 31-year-old male the tumour arouse in the scapular soft tissues and local recurrence occurred. In a female of 45, the neoplasm was located in the thigh.

[Effects of carvedilol, a sympatholytic, in experimental alloxan diabetes in laboratory rats].

A possible effect of the sympatholytic carvedilol on alloxan-induced diabetes mellitus in the laboratory rat was examined in experiments. The animals were divided into a group treated with carvedilol in a single daily dose of 10mg/kg in 1 ml of diluting solution i.p and the control group which received only diluting solution in the pertinent amount.

[The effects of Carvedilol, a beta-blocker, in experimental ischemia-reperfusion kidney injury].

Carvedilol is a recently introduced drug with multiple action with a non-selective beta-antiadrenergic and selective alpha1-antiadrenergic action used for treatment of mild to medium severe hypertension. The authors investigated in their experiments the protective effect of carvedilol under conditions of ischaemia-reperfusion of the kidney in the laboratory rat. The animals were divided into four groups 1.

[Aggressive angiomyxoma of the pelvic-peritoneal region (case report)].

The authors describe the case-history of a 30-year-old woman with an aggressive angiomyxoma. This rare tumour is usually found in the pelvic and perineal area and reaches a big size. In the presented case the weight of the tumour was 1020 g.

[Protective effect of carvedilol on renal circulation and tissue injury in reperfusion of ischemic kidneys in rats].

In one-kidney rats the resting blood flow in the renal artery was recorded for 10 minutes then the artery was clipped for one hour and the following reperfusion blood flow was registered again for 10 minutes. The animals were divided into three groups: Series A, untreated control animals. Series B, two weeks before renal ischemia the rats were fed a diet containing 0.

Aggressive angiomyxoma of the pelvioperineal region. Immunohistological and ultrastructural study of seven cases.

We describe seven cases of aggressive angiomyxoma of the pelvioperineal region. In three cases, we observed immunohistochemical signs of myoid differentiation in the tumor cells. In one of these cases, we could confirm the myoid differentiation at the ultrastructural level.

[Secondary adenocarcinoma of the ovary and problems in diagnosis].

In a group of 10 secondary ovarian adenocarcinomas primary tumours were verified by biopsy in colorectal region (8 cases), once in gall bladder and once in appendix. The age of patients varied between 36 up to 60 years. The lesion was unilateral in 8 cases, bilateral in two.

[Liver diseases in children with alpha 1-antitrypsin deficiency in infancy].

The authors evaluate the health status of children with alpha-1-antitrypsin deficiency, focused on liver disease in infant age. The children were selected by neonatal screening. Of 21 children one had severe neonatal hepatitis with progression to cirrhosis, 2 children had clinically apparent jaundice to the age of two months, 6 children had elevated total bilirubin and transaminase levels without clinical signs of the disease, 12 of the remaining children had no clinical and laboratory signs of liver disease.