Sarcomas of the female genital tract in general are rare and ovarian sarcomas comprise less than 1% of ovarian malignancies. In the literature there are 15 reported angiosarcomas of patients 21 year old and younger with no one originated in the ovary. We report a case of ovarian angiosarcoma in an 11 year old girl, presented with left side hip pain. MRI of abdomen and pelvis confirmed expansive solid and cystic mass occupied both ovaries. Imunohistochemistry staining was performed, CD34, Factor VIII, CD31, in order to confirm the diagnosis. Final diagnosis was angiosarcoma. The patient received 6 cycles of chemotherapy, according to the CWS-2002P protocol. 8 months after the diagnosis was established, there were no signs of any tumors according to the ultrasound, CT scan, and MRI. Although, extremely rare, angiosarcoma can also affect children and this diagnosis should be considered carefully in tumor with rich vascular network, necrosis and brisk mitotic activity.
Background:Nephrotic syndrome (NS) is one of the most frequent glomerular diseases among children. While most of the children with primary NS respond to steroid treatment, 10 to 20% of the patients are steroid-resistant, and the best therapy for such cases has never been defined.Objective:The present study aimed to evaluate steroid-resistant nephrotic syndrome (SRNS) patients.Materials and methods:Our research included 50 children (56% female and 44% male) with NS. NS was defined as the presence of edema, massive proteinuria, hypoalbuminemia and hyperlipidemia. Patients with NS were treated according to international protocol. SRNS was diagnosed in patients with idiopathic NS based on lack of complete remission despite treatment with steroids. Renal biopsy was performed in 22 patients with SRNS at the Pediatric Clinic II of the University Clinical Center in Sarajevo (UCCS). Histopathologic analyzes of renal biopsy were performed at the Department of Pathology, University Clinical Center in Tuzla (UCCT). Patients with SRNS, after kidney biopsy were treated with nonsteroidal immunosuppressant’s.Results:Eight (36.4%) of the 22 patients who had undergone renal biopsies had minimal change disease (MCNS) and seven (31.8%) had focal segmental glomerulosclerosis (FSGS). The immunosuppressive drugs used in SRNS were Cyclosporine (CsA), Cyclophosphamide (CYC), Mycophenolat mofetil (MMF) and Rituximab (RTX). Three patients (13.6%) did not respond to any treatment and had developed end - stage renal disease (ESRD).Conclusion:With current treatments, some children will ultimately achieve a sustained remission with one of the second line or third line of the proposed drugs. Patients with refractory NS will go to progression towards ESRD. The rapid development of molecular genetics will give a new contribution to the pathogenesis and treatment of this disease.
Low-grade squamous intraepithelial lesion (SIL) is a cytologic diagnosis etiologically related to human papilloma virus (HPV) infection that leads to the release of inflammation mediators, the formation of reactive oxygen species (ROS) and decreased levels of antioxidants in tissues, which is why antioxidants might be considered effective against SIL progression. This randomized double-blind placebo-controlled study aimed to investigate the effectiveness of alpha-lipoic acid (ALA) supplementation (600 mg/day) on the regression of low-grade SIL in 100 patients. Low-grade SIL was determined after the cytological screening, colposcopic examination and targeted biopsy and histological confirmation of cytological–colposcopic diagnosis. Inflammation parameters and the presence of HPV were determined by standard laboratory methods. Dietary and lifestyle habits were investigated using a standardized and validated semi-quantitative food questionnaire (FFQ). ALA supplementation significantly reduced the proportion of patients with low-grade cytological abnormalities, in comparison to placebo. Given the obtained level of significance (p < 0.001), the presented results indicate that short-term ALA supplementation shows a clinically significant effect on cervical cytology. Future studies should focus on the use of innovative formulations of ALA that might induce bioavailability and therapeutic efficiency against HPV infection and the investigation of synergistic effects of concurrent dietary/lifestyle modification and ALA supplementation in both low-grade and high-grade SIL.
The role of Epstein Barr virus (EBV) in the onset of Hodgkin's lymphoma has been a subject of ongoing research. However, confirmation of EBV oncogenic involvement was not possible due to the small number of neoplastic cells characteristic for this type of tumor. Presence of EBV infection in neoplastic and non-neoplastic cells was analyzed in 81 cases of Hodgkin's lymphoma. In neoplastic cells, using an immunohistochemical method, latent membrane protein 1 (LMP1) was found in 33,3% of cases, while in situ hybridization results demonstrated the presence of EBER RNA in 48,1% of the cases. EBER RNA was found in non-neoplastic lymphocytes in 38,3% of cases. EBV is most frequently associated with Hodgkin's lymphoma in the first and seventh decade of life, specifically the nodular sclerosis subtype. No apparent difference was observed in the association of Hodgkin's lymphoma with EBV between genders, or in relation to clinical stage of the disease and average age of the patient. However, association with childhood age is significantly greater in comparison to adults. EBV associated disease shows a significantly greater prevalence in T lymphocytes. Slightly more abundant are cytotoxic T lymphocytes, which are also more frequently in contact with Reed-Sternberg cells, although there is no difference in number and positioning of histiocytes. Variations between the data on the association of EBV with Hodgkin's lymphoma among studies from different parts of the world suggest that factors of age, gender, ethnic background and social status might present biological modifiers of EBV influence on the pathogenesis of this neoplasm. The differences in non-neoplastic infiltrate EBV+ and EBV- lymphoma indicate the effect of the virus on the immune interaction of tumor and host in this disease.
We present the case of a 67-year-old female patient with microscopic polyangiitis presented with polyneuropathy of lower extremities and rapidly progressive glomerulonephritis. Disease had started as a pain and weakening of muscular strength first in the left and than in the right leg. Electromiography has shown that a mainly dominant neurological affection was paresis of peroneal nerve in both lower extremities. In laboratory examination the titer of anti-myeloperoxidase anti-neutrophilic cytoplasmic antibodies (p-ANCA) was elevated. Due to renal involvement presented as a microscopic haematuria and decreasing of renal function, patient undergone kidney biopsy. It confirmed the immune vasculitis microscopic polyangiitis type with ANCA-associated glomerulonephritis. This is one of rare case of microscopic polyangiitis without lung simptomatology, first presented with asymmetrical polineuropathy of lower extremities. The patient was treated with methylprednisolone and cyclophosphamide in dosis adjusted to the level of disease severity and the renal function (methylprednisolone 1 mg/kg of body weight for two months with gradually tapering to the minimum effective dose and cyclophosphamide 1 mg/kg of body weight). This treatment lead to the partial remission of disease. In maintenance therapy azathioprin was introduced instead of cyclophosphamide.
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