Granulocytic sarcoma as the cause of giant abdominal mass: diagnosis by fine needle aspiration and review of the literature.

Granulocytic sarcoma (GS) or chloroma is a neoplasia consisting of myeloid precursors in an extramedullary site. It is generally associated with myeloproliferative disorders especially with myeloid neoplasias. A young woman with huge abdominal mass due to GS associated with chronic myelocytic leukemia (CML) has been reported and literature is reviewed.

MUC1 expression in plasmacytoma.

Plasmacytoma (Pm) is a plasma cell (PC) neoplasia consisting of PCs and some of them show progression to multiple myeloma. But there is no clear indicators predicting this progression. In this study, MUC1 expression was evaluated in Pm cases to determine the predictive value and was compared with histopathologic grade which is known to be a prognostic indicator in Pm. Nine of 31 cases with Pm showed MUC1 expression. Only two of the 18 cases with mature morphology showed MUC1 expression while seven of 13 cases with immature morphology showed MUC1 expression and this was statistically significant (P<0.006). Additionally, four of 11 cases with BM involvement showed MUC1 expression while five of 20 cases without BM involvement showed MUC1 expression. There was a trend MUC1 expression with BM involvement but there was not statistically significant association between MUC1 expression and BM involvement. We found that MUC1 expression is associated with immature morphology which is an important prognostic indicator in Pm and by analogy MUC1 expression may be an additional prognostic indicator in patients with Pm.

Postmastectomy malignant lymphoma.

Postmastectomy lymphedema (PML) is a morbid condition occurring in patients with breast carcinoma treated with radical/modified radical mastectomy. Postmastectomy angiosarcoma (PMA) is the most common neoplasia seen in these patients. Primary malignant lymphoma arising in PML is a rare neoplasia and 3 cases have been reported until now. In this report a patient with diffuse large cell lymphoma (DLCL) arising in PML is reported and the other three cases are reviewed.

Lymphomatous skin infiltration at the site of previous varicella zoster virus infection in a patient with T cell lymphoma.

Cutaneous infiltrations in hemopoietic neoplasias are not uncommon. They are generally localized on the legs, arms, back, anterior chest, scalp and face. In rare cases specific infiltration of neoplastic cells is localized in the site of herpes zoster and herpes simplex scars. In this report a case with T cell lymphoma in leukemic phase with skin infiltration in the previous Varicella Zoster Virus (VZV) site of infection is reported and literature is reviewed.

The expression of cytoskeletal proteins (alpha-SMA, vimentin, desmin) in kidney tissue: a comparison of fetal, normal kidneys, and glomerulonephritis.

BACKGROUND: The aim of the study is a comparison of the expression of cytoskeletal proteins, alpha smooth muscle actin (alpha-SMA), vimentin, and desmin in fetal, normal kidney and proliferative (diffuse proliferative and membranoproliferative glomerulonephritis) and nonproliferative (membranous glomerulonephritis) glomerulonephritis. METHODS: We have studied the expression of cytoskeletal proteins (alpha-SMA, vimentin, desmin) in the paraffin embedded tissue sections from the kidneys of 10 normal kidney (adults and infants), 13 fetal kidney, 12 membranous glomerulonephritis (MGN), 8 membranoproliferative glomerulonephritis (MPGN), 8 diffuse proliferative glomerulonephritis (DPGN). Interstitial and glomerular positive stainings were evaluated. RESULTS: Vimentin expression was similar in normal infant and adult kidneys with positive staining in glomeruli and negative staining in interstitium. In fetal kidneys, glomerular mesangial and epithelial cells and blastematous areas showed positive reactivity with vimentin. Alpha-SMA staining was different among the groups. In fetal kidney, alpha-SMA expression was found in glomerular mesangial cells and blastematous areas. Alpha-SMA staining was positive in peritubular area and glomerular mesangial cells in infant kidney. In adult kidneys, glomerular staining with alpha-SMA disappeared but peritubular positivity continued. Interstitial staining with alpha-SMA was positive in fibrotic areas of proliferative (MPGN, DPGN) and non-proliferative (MGN) glomerulonephritis, but positive glomerular staining with alpha-SMA was found only proliferative glomerulonephritis. Desmin expression was negative in all groups. CONCLUSIONS: Desmin is not expressed in early stages of kidney growth, infant and adult kidneys, and proliferative and nonproliferative glomerulonephritis. Interstitial staining of vimentin in the diseased kidney tissues revealed increased fibrosis. Alpha-SMA revealed important differences in different stages of nephrogenesis. Glomerular mesangial staining with alpha-SMA in developing (fetal and infant kidneys) and proliferative glomerulonephritis suggest that it may be a marker of proliferation. In addition, it shows myofibroblastic differentiation in interstitium in diseased kidneys.

Primitive neuroectodermal tumor of the kidney: a rare entity.

Primitive neuroectodermal tumor (PNET) is usually aggressive and rapidly progressing and metastasizing tumor. Occurrence of the this type of tumor in the kidney is considered as unusual, and few cases have been reported so far. We present a PNET arising from the kidney in a 23-year-old female patient.

Cholestasis and renal failure in a patient with secondary amyloidosis.

Amyloid, was first described in the 19th century by Virchow, which means starch or cellulose. Extracellular deposition of this unique protein fibrils in tissues, often leading organ dysfunction is known as amyloidosis. In systemic amyloidosis, amyloid fibrils may deposit in different organs including kidneys, heart and liver. Although liver is effected frequently, clinical liver disease is rare. There are a few cases that secondary amyloidosis presented with hepatic involvement and cholestasis which seems to be limited to primary amyloidosis. Hepatic amyloidosis with cholestasis may be a predictor of involvement of other organs and thus of poor prognosis. We report a case of a 67 year old man with renal failure and prominent cholestasis due to AA amyloid deposition in the liver.

Lipoid proteinosis: Urbach-Wiethe disease.

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal recessive disorder in which hyalinized material is deposited in the skin, mucous membranes and brain. Laryngeal changes resulting in hoarseness may be present at birth or in early infancy, often being the first sign of the disease. A typical case is presented.

The frequencies of hepatitis B virus markers and hepatitis C virus antibody in patients with glomerulonephritis.

Hepatitis B virus markers and hepatitis C virus (HCV) antibody were assayed in 72 patients with glomerulonephritis (GN) at the time of biopsy. The patients, 41 males and 31 females, were aged between 16 and 68 years. The frequency of positivity for HbsAg, anti-Hbs, and anti-Hbe, anti-Hbc and anti-HCV antibody was 5.5, 20.8, 2.8, 16.6 and 1.4% respectively. At the same time, HbsAg and anti-HCV positivity were 8.5 and 0.95% in blood donors. We can say that morbidity and prognosis may be affected by hepatitis infection. Infections of hepatitis B and C have to be evaluated in patients with GN, especially in endemic areas. In our study group the frequencies of HbsAg positivity and anti-HCV positivity were not higher in patients with GN than in blood donors.

Combined occurrence of primary cerebral lymphoma and meningioma.

A unique combination of primary cerebral lymphoma and meningioma was encountered in a 38-year-old woman who recently presented clinically with headache and papilledema. The both tumors were identified by CT scan and diagnosed histopathologically after the surgery. The association of a highly malignant primary cerebral lymphoma and a slowly growing meningioma is extremely rare. The possibility of an unknown underlying pathologic mechanism predisposing to multiple tumors should be considered.

Influence of enalapril on experimental cyclosporin A nephrotoxicity.

The purpose of this study was to investigate if enalapril could be administered with cyclosporin A (CyA) to reduce its nephrotoxicity. Sixty rats were divided into five groups: group I, Control group; group II, rat treated with oral enalapril; group III, rats treated with CyA: group IV, rats treated with CyA and enalapril; group V, rats treated with enalapril before the CyA therapy. At the end of the therapy mean serum creatinine concentrations were not statistically different between the groups (P > 0.05), in groups treated with CyA there were no statistically significant differences between mean CyA levels (P > 0.05), and mean blood urea nitrogen levels of the groups treated with CyA were significantly elevated (P < 0.05) compared with groups not treated with CyA. Morphologically acute CyA nephrotoxicity was evaluated by the following features: (1) tubular vacuolization, (2) tubular necrosis, (3) tubular microcalcification, and (4) peritubular capillary congestion. These lesions were scored semiquantitatively on a scale from 0 to 4+. The most common tubular pathology was tubular vacuolization, which was more severe in groups III and IV. Tubular necrosis was most severe in group III. In conclusion, enalapril seems to suppress the severest form of CyA nephrotoxicity, namely tubular necrosis, if administered prior to CyA treatment.

Two cases of nephrotic syndrome and tertian malaria in south-eastern Anatolia.

Tertian malaria is endemic in south-eastern Anatolia. As in Europe and America, in south-eastern Anatolia, an etiological agent is seldom identified in nephrotic syndrome. Two patients with Plasmodium vivax and nephrotic syndrome are described here. The possible relationship between Plasmodium vivax and nephrotic syndrome should be explored in children in endemic malarial regions.

AA-amyloidosis presenting with chronic diarrhea and cardiac manifestations.

In secondary amyloidosis (AA type), clinically significant cardiac and gastrointestinal involvement are uncommon, in contrast to the primary type. We report a case presenting with chronic diarrhea and cardiac manifestations who was diagnosed as having AA-amyloidosis with unknown predisposing illness based on endomyocardial, rectal and subcutaneous fat tissue biopsies.

Surgical treatment of symptomatic subependymoma of the nervous system. Report of five cases.

Subependymomas are uncommon and relatively benign tumors with a distinctive histological appearance. They are generally asymptomatic and most of them are found incidentally at postmortem examination. These tumors are usually located in the fourth and lateral ventricle, and sometimes in the spinal cord. The authors present a series of 5 pure subependymomas of the central nervous system all of which were symptomatic and all of which were treated by microsurgical removal. The clinico-pathological findings and the results of microsurgical treatment in this series are presented.

Efficacy and side effects of cyclosporin A in nephrotic syndrome of childhood.

The efficacy and side effects of the oral administration of cyclosporin A (Cyc-A) were investigated in 13 children with the nephrotic syndrome. Two of these children were steroid-dependent, 9 were resistant to steroids, and 2 displayed frequent relapses. The diagnosis of 6 patients was mesangioproliferative glomerulonephritis (MezPGN), of another 6 mesangiocapillary glomerulonephritis, and of 1 minimal change disease. Cyc-A was administered for 6 months at a dosage of 7 mg/kg/day with continual monitoring of the serum levels. At the end of the treatment period, 7 of the patients (53.8%) had a total remission, 5 (38.4%) a partial remission, and 1 (7.8%) did not respond to treatment. While total remission was achieved in 4 patients who had responded well to steroids, this was true for only 3 (33.3%) of the 9 patients who had not benefited from steroids. The effects and side effects of Cyc-A on histopathological morphology were also investigated by means of posttreatment biopsies. While histopathological improvement was seen in 3 patients with MezPGN, there was evidence of deterioration in 1 patient with MLH.

Sweet's syndrome associated with G-CSF.

Sweet's syndrome (SS) developed in two patients with acute myeloid leukaemia (AML) treated with granulocyte colony stimulating factor (G-CSF) for febrile neutropenia due to AML chemotherapy. Fever, painful skin and conjunctival lesions developed and neutrophilic infiltration was detected at biopsy specimens. Neutrophilia was not detected. Skin lesions regressed within 1-2 weeks and conjunctival lesions within 4 weeks following the cessation of G-CSF. We conclude that SS may be a complication of G-CSF therapy and tender skin and/or conjunctival lesions developing during G-CSF therapy should suggest the possibility of SS.