Renal venin düşük dereceli leiyomiyosarkomu: Olgu sunumu

Renal venden kaynaklanan leyomiyosarkomlar oldukça nadir görülen malign tümörlerdir. Bugüne kadar literatürde bildirilmiş 30 kadar olgu vardır. Klinik semptomlar renal kitleye bağlıdır ve genellikle hastalar preoperatif olarak böbrek karsinomu ön tanısıyla opere edilirler. Doğru tanı sıklıkla ameliyat materyallerinde konulur. Düşük dereceli leiyomiyosarkomlar leiyomiyomlardan ayrılmalıdırlar. Artmış mitotik aktivite ve nekroz ayırıcı tanıda önemli kriterlerdir. Sol böbreğinde düşük dereceli leiyomiyosarkom tanısı alan 62 yaşındaki kadın hasta, klinik ve patolojik özellikleriyle birlikte literatür eşliğinde sunulmaktadır.Leiomyosarcomas originating from renal vein are quite rare malignant tumors since only 30 cases have been reported in the literature. Clinical symptoms which are due to a renal mass along with radiological findings, usually mislead to a preoperative diagnosis of renal cell carcinoma. The correct diagnosis is frequently made on nephrectomy specimens. Low-grade leiomyosarcomas need to be differentiated from leiomyomas. Increased mitotic activity and necrosis are known to be gold standards for differential diagnosis. In this report, we present a case of a 62 year old woman whose tumor in the left kidney diagnosed as low-grade leiomyosarcoma together with a brief review of the literature

Pleural giant solitary fibrous tumor and immunohistochemical profile

Plevranın soliter fibröz tümörü nadir görülen ve yavaş büyüyen intratorasik bir neoplazmdır. Parietal ya da daha büyük oranda viseral plevranın submezenkimal hücrelerinden köken alır. Olguların çok büyük kısmı benign klinik seyir göstermekle birlikte %7,5-37 arasında belirtilen oranlarda malign seyreden olgular bildirilmiştir. Sunulan olgu malignite potansiyelli plevral soliter fibröz tümör olarak değerlendirilmiştir. Solunum sıkıntısı şikayetiyle hastanemize başvuran 74 yaşındaki kadın hasta çekilen bilgisayarlı tomografisinde plevra ile ilişkili mediastinal kitle saptanması nedeniyle opere edildi. Makroskopik incelemede tümoral kitle 754 gr, 17x12x5,5 cm ölçülerde, lastik kıvamlı, iyi sınırlı, lobuler konturluydu. Kesit yüzü lifsi görünümde olup bir alanda geniş nekroz alanı yanı sıra miksoid dejenerasyon gösteren alanlar görüldü. Mikroskopik değerlendirmede kollajenden zengin stromada üniform iğsi hücre topluluları ile karakterli hiposellüler alanlar ve daha sellüler alanlar dikkati çekti. Dallanma gösteren damar ağı belirgin bir özellikti. Mitoz belirgin ölçüde nadir olmakla birlikte nekroz yaygın olarak izlendi. İmmünohistokimyasal incelemede tümör CD34, bcl-2 ve vimentin güçlü ve yaygın pozitif, sitokeratin, aktin, S100, desmin negatifti. Ki-67 indeksi en yoğun olduğu alanda %10-15 olarak saptandı. Olgu mitoz ve pleomorfizm göstermemekle birlikte, selüler alanların ve nekrozun varlığı nedeniyle malignite potansiyeli olan soliter fibröz tümör olarak değerlendirildi. Operasyon sonrası 15. ayda hastada nüks ya da metastaz saptanmadı.Pleural solitary fibrous tumor is a rare and slow growing intrathoracic neoplasm. It originates from submesenchymal cells of parietal or, more commonly, visceral pleura. Although most cases follow a benign clinical course, it has been reported that a malignant outcome is seen in 7,5-37% of cases. We present here a case considered as pleural solitary fibrous tumor with malignant potential. A 74-yearold woman presented with dyspnea and computerized tomography showed a mediastinal mass connected to the pleura. The tumor was removed by surgery. Grossly, the tumor was lobulated, welldemarcated and mostly encapsulated. Its weight was 754 gr. and it measured 17x12x5.5 cm. The cut surface had a whorled appearance. Focal necrosis and myxoid degenerative areas were noted. Histopathologically, hypocellular areas characterized by uniform, small spindle cells in a collagen rich stroma and hypercellular areas were seen. The tumor had a prominent branching vascular network. Although mitosis was infrequent, necrosis was common. Tumoral cells were strongly immunoreactive for CD34, bcl-2 and vimentin by immunohistochemistry. Cytokeratin, actin, S100, CD117 and desmin were negative. The ratio of Ki-67 positive cells was 10%. The patient was diagnosed as pleural solitary fibrous tumor with malignant potential because of hypercellularity and presence of large necrotic areas although increased mitosis and pleomorphism were almost absent. There was no recurrence or metastasis 15 months after the surgery

Systemic Mastocytosis Presenting with a Prominent B Lymphocyte Proliferation in the Bone Marrow and Extensive Fibrosis of the Spleen

Systemic mastocytosis is a disease characterized by multifocal mast cell proliferation in the bone marrow or other extracutaneous organs. Because of loosely scattered and hypo-/agranular mast cells, the diagnosis is sometimes very difficult. In the bone marrow, mast cell infiltration may be associated with prominent lymphoid infiltration leading to a misdiagnosis of a low grade non-Hodgkin lymphoma

Systemic mastocytosis presenting with a prominent B lymphocyte proliferation in the bone marrow and extensive fibrosis of the spleen

Abstract. Systemic mastocytosis is a disease characterized by multifocal mast cell proliferation in the bone marrow or other extracutaneous organs. Because of loosely scattered and hypo-/agranular mast cells, the diagnosis is sometimes very difficult. In the bone marrow, mast cell infiltration may be associated with prominent lymphoid infiltration leading to a misdiagnosis of a low grade non-Hodgkin lymphoma. A 49-year-old woman presented with right arm and leg pain, psychiatric symptoms, and diarrhea for four years. Physical examination and laboratory investigation revealed hepatosplenomegaly, anemia, mild thrombocytosis, mild leucocytosis and lymphocytosis. In the bone marrow biopsy, there was a prominent B lymphocyte proliferation reminiscent of a low grade non-Hodgkin lymphoma/leukemia and there were some spindle cells aggregates in paratrabecular location. The consecutive bone marrow biopsies were similar to the first. The subsequent splenectomy specimen exhibited striking fibrosis. In the lymph node sections, there was marginal zone hyperplasia. Multifocal accumulations of mast cells were strongly positive with mast cell tryptase and CD117 on immunohistochemical staining, though no metachromasia was identified in Giemsa and Toluidine Blue stained aspirates and tissue sections, probably due to hypo-/agranulation of mast cells. The case was presented to emphasize the importance of the antibody to mast cell tryptase in the diagnosis of mastocytosis and to discuss problems of differential diagnosis of systemic mastocytosis

Investigation of neuraminidase 1 gene association in Henoch-Schönlein Purpura (HSP) with renal involvement

Objectives: HSP is a common small vessel vasculitis. It is the most common cause of non-thrombocytopenic purpura in childhood. The role of genes in etiopathogenesis of the disease, which has not yet been clearly elucidated, is being emphasized. Many genes called sialidases are being studied and is thought that the NEU1 gene may be particularly important in the etiopathogenesis of HSP. The aim of this study is to investigate the role of the NEU1 gene in the etiopathogenesis of HSP and its relation to renal involvement.Materials and methods: Fifty patients followed in the Celal Bayar University Hafsa Sultan Hospital Pediatric Nephrology Department, with the diagnosis of HSP renal involvement were included into the study. For the control group, age and gender matched 50 cases were accepted among the outpatients admitted to Pediatric Department without any chronic diseases. NEU1 gene mutation analysis was performed in blood samples of both patient and control groups by using the Sanger DNA sequencing method.Results: NEU1 genetic mutation was not detected in any HSP patient with renal involvement and control group. Conclusion: In our study, the NEU 1 gene was not found to be associated with HSP nephritis. No changes were detected in the investigated regions of the NEU1 gene.

Selenium, nickel, and calcium levels in cancerous and non-cancerous prostate tissue samples and their relation with some parameters

In the present study, tissue samples of patients with cancerous and non-cancerous prostate were analyzed for their Se, Ni, and Ca contents. Possible relationship between Se, Ni, and Ca concentrations and some parameters including preoperative prostate-specific antigen (PSA) levels, histopathological neurovascular invasion, extra-capsular extension, seminal vesicle invasion, positive surgical margins, PSA relapse after radical prostatectomy, and total Gleason scores were obtained. Inductively coupled plasma (ICP) optical emission spectrometry and ICP-mass spectrometry instruments were used for the determination of analytes interested. All the system parameters in digestion and measurement steps were optimized to obtain efficient digestion and sensitive measurements. There was no statistically meaningful difference observed in the concentration of selenium in cancerous and benign prostatic tissues (p = 0.347) while nickel levels in cancerous tissues were observed as significantly lower than benign tissues (p < 0.001). In addition, calcium concentration in cancerous tissue samples were found to be statistically lower than those in benign tissues (p < 0.001) with mean values of 657 and 1,431 mg/kg, respectively