Primary retroperitoneal mucinous cystadenoma. A rare case with two cysts and review of the literature.

Background: Primary retroperitoneal mucinous cystadenoma is a rare neoplasm, with benign biological behavior. Delay in diagnosis and treatment of this tumor may be fatal for the patient, because of complications, such as rupture, infection and malignant transformation.

Case Presentation: We present a 23-year-old woman, who was admitted to the hospital because of a palpable abdominal mass and discomfort since 4 months.

Mandibulofacial dysostosis (Treacher-Collins syndrome) in the fetus: novel association with Pectus carinatum in a molecularly confirmed case and review of the fetal phenotype.

Background: Treacher Collins syndrome is the most common mandibulofacial dysostosis of autosomal dominant or, rarely, recessive inheritance. Affected fetuses may be identified by prenatal ultrasound or diagnosed at autopsy in case of perinatal death or pregnancy termination.

Methods: We describe the ultrasonographic, autopsy, and molecular findings in a 25-week-gestation affected fetus, and review the clinical, prenatal, and postmortem findings in 15 previously reported fetal and perinatal cases.

Hemolytic-uremic syndrome, malignant hypertension and IgA nephropathy: successful treatment with plasma exchange therapy.

A young patient with hemolytic-uremic syndrome and malignant hypertension with serious deterioration of renal function is described whose biopsy specimen showed additional IgA mesangial deposits. The patient responded to steroid treatment and to plasma exchange therapy without the need of hemodialysis sessions. In the following years, he achieved clinical remission and his blood pressure was in normal ranges without any further complications.

Immunohistochemical evaluation of podocalyxin expression in glomerulopathies associated with nephrotic syndrome.

It is now well established that morphological change of podocytes is closely correlated to the development of proteinuria. The aim of this study was to investigate the role of podocalyxin, a major podocyte protein, in the pathogenesis of glomerulopathies primarily associated with the nephrotic syndrome. Immunohistochemical expression of podocalyxin has been evaluated in 51 renal samples, including healthy controls, patients with podocytopathies (minimal change disease [MCD], focal segmental glomerulosclerosis [FSGS]) and membranous glomerulopathy (MG).

IgA nephropathy in association with Crohn's disease: a case report and brief review of the literature.

A case of immunoglobulin A nephropathy (IgAN) complicating a 10-year history of biopsy-proven Crohn's disease in a 31-year-old man is described. The patient presented with mild proteinuria and impaired renal function in the setting of an exacerbation of Crohn's disease. Renal biopsy showed IgAN.

Membranoproliferative glomerulonephritis in the setting of multicentric angiofollicular lymph node hyperplasia (Castleman's disease) complicated by Evan's syndrome.

Systemic Castleman's disease is a lymphoproliferative disorder with various clinical presentations and incompletely understood aetiology. The authors report on a rare case of the plasma cell variant of Castleman's disease associated with autoimmune haemolytic anaemia and autoimmune thrombocytopenia (Evan's syndrome) and complicated by mixed nephrotic-nephritic syndrome and acute renal failure due to an underlying glomerulopathy with microscopic and immunofluorescence findings suggestive of membranoproliferative glomerulonephritis (MPGN) type I. Immunocomplexed glomerulonephritis is rare in Castleman's disease, while, to the best of our knowledge, constellation of all these autoimmune phenomena is reported for the first time suggesting that apart from the putative role of VEGF and IL-6 in the pathogenesis of the disease, a more generalised immunological disturbance occurs, probably through autoantibodies induced by active polyclonal B cells raised from Castleman's disease tumour.

Piriformis syndrome--an attempt to understand its pathology.

Objective: Gross anatomy of the hip rotators and histology of the sciatic nerves in adult cadavers were studied, aiming to the identification of possible pathologic changes related to the piriformis syndrome (PS).

Material: 50 cadavers were dissected; in 17 cases with macroscopical findings the sciatic nerves were harvested (34 sciatic nerves; 17 cadavers). History of low back or leg pain was not available.

Membranous nephritis associated with acquired cytomegalovirus infection in a 19-month-old baby.

Membranous nephritis (MN) is a rare form of glomerulonephritis in childhood, with an incidence of 0.8 to 6.7% based on renal biopsy specimens.

Association between microvessel density and histologic grade in renal cell carcinomas.

Angiogenesis seems to contribute to tumor growth and the development of metastases. There may be an association between the vascular density of individual tumors and their prognosis. In the present survey we studied 53 cases of renal cell carcinoma investigating possible relationship between histologic grade and microvessel density (MVD) measured by an image analysis system.

Ichthyosis follicularis with alopecia and photophobia in a girl with cataract: histological and electron microscopy findings.

A rare congenital ectodermal disorder characterized by ichthyosis follicularis, alopecia and photophobia has been designated the acronym IFAP. An X-linked recessive mode of inheritance was initially proposed but a few recent reports in girls suggested genetic heterogeneity of this syndrome. We herein describe a 3-year-old girl with clinical and histological features typical of IFAP.

Epidermolysis bullosa acquisita: treatment with intravenous immunoglobulins.

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune bullous disorder that is often difficult to treat. Few cases have been reported and therapy consists mainly of combinations of systemic steroids, immunosuppressants and, recently, administration of intravenous human immunoglobulin (IVIg). We describe a case of EBA in which our therapeutic choices were limited due to the patient's poor general condition, including extensive infection of the lesions and a history of pulmonary tuberculosis.

Congenital epulis: an ultrastructural and immunohistochemical case study.

We report a case of an otherwise healthy, two days old, female with a a mass derived from the mandible. Light microscopy findings were compatible with the diagnosis of congenital epulis. The lesion showed negative staining for S-100 protein, which is a characteristic finding in cases of epulis and distinguishes it from granular cell tumor.

Localization of the glucocorticoid receptor in rat brain mitochondria.

The distribution of glucocorticoid receptor in subcellular fractions of brain cortex and hippocampus, two regions rich in glucocorticoid receptor, has revealed its presence in nuclei, cytosol, mitochondria, synaptosomes, and synaptosomal mitochondria. The identification of glucocorticoid receptor has been accomplished both by Western blotting using antibodies recognizing the carboxy and the amino terminus of the glucocorticoid receptor and by immunogold electron microscopy using the same anti-glucocorticoid receptor antibodies. Antibody-glucocorticoid receptor interaction is abolished by preincubation of each antibody with its competing peptide.

Lectin histochemistry as a predictor of dysplasia grade in colorectal adenomas.

Lectins are sugar-binding proteins that bind to specific cellular carbohydrates, commonly affecting cellular physiology. Phaseolus vulgaris leucoagglutinin (PHA), ulex europaeus isoagglutinin-I (UEA-I), wheat germ agglutinin (WGA) and peanut agglutinin (PNA) are among the most well studied lectins in various tissues. The purpose of this study was to detect the above lectins binding sites and so examine alterations in glycoconjugate expression in neoplastic cells of 52 colorectal adenomas with various clinicopathologic characteristics and proliferation rates.

Histological diagnosis of cutaneous "warty" carcinoma on a pre-existing HPV lesion.

A middle-aged man with an 8-year history of a fungating tumour mass on his thigh was histologically diagnosed as having an invasive "warty" carcinoma at the location of a pre-existing human papillomavirus (HPV) lesion. The tumour surface had a verruciform appearance with papillae containing fibrovascular cores. Many of the malignant cells displayed changes consistent with koilocytotic atypia.

Computerized morphometric study on fine needle aspirates of cellular follicular lesions of the thyroid.

Objective: To investigate the value of computerized nuclear morphometry in the differential diagnosis of cellular follicular lesions of the thyroid cytologically diagnosed on fine needle aspiration (FNA) smears.

Study Design: Sixty cases of FNA thyroid smears were cytologically diagnosed and classified as follows: 30 cases of follicular carcinoma, 20 cases of cellular hyperplastic nodules and 10 cases of follicular adenoma. Using an image analysis system, two morphometric variables, nuclear area and major axis length of the nucleus, were measured for each case.

Membranous glomerulonephritis: a morphometric study.

Archival material from 45 renal biopsies with a diagnosis of idiopathic membranous glomerulonephritis (MGN) were studied by computer-aided image analysis in order to evaluate the prognostic significance of glomerular and interstitial morphometry in MGN. The control group consisted of thirty seven normal renal biopsy specimens. The surface area, the perimeter, the major axis length and the shape factor of renal glomeruli as well as the percentage of the interstitial fibrosis were measured.

Spontaneous interbody fusion after controlled injuries to the spine: an experimental study in rabbits.

To evaluate the rationale of spontaneous spine fusion after a spinal injury, the authors conducted an experimental study that consisted of three types of controlled injuries to a rabbit spine model. The first was injury to the intervertebral disk (type I injury). The second was injury of the intervertebral disk along with injury to one of the adjacent vertebral end plates (type II).

A solitary cutaneous tumor with distinct areas of verruca and seborrheic keratosis-like lesion.

A single, exophytic, cutaneous tumor on the thigh of a 52-year-old man was examined by light microscopy, in situ hybridization and immunohistochemistry. It demonstrated distinct areas of verruca and of seborrheic keratosis-like morphology simultaneously. Focally, architectural abnormalities were noted in some deeper parts of the tumor, but there was no morphological evidence of malignancy.

In situ expression of E-selectin and intercellular adhesion molecule-1 in chronic inflammatory diseases of the gastrointestinal tract.

Aim: The study of cell adhesion molecules contributes to our understanding of the inflammatory mechanisms which include the endothelial activation of newly formed or pre-existing vessels, the increase of inflammatory cells' adhesive capability and their migration into perivascular tissues. The aim of the present study was to investigate the local presence and the extent of expression of E-selectin and intercellular adhesion molecule-1 (ICAM-1) in the mucosa of patients with chronic gastritis, chronic inflammatory bowel disease, and controls, as well as to identify possible correlations between in situ expression of the above adhesion molecules and degree of inflammatory activity or therapeutic response.

Design: In cryostat tissue sections we examined the immunohistochemical expression and localization of E-selectin and the intercellular adhesion molecule-1 (ICAM-1).

Middle ear adenomatous tumor with a predominant neuroendocrine component.

A primary adenomatous tumor of the middle ear was examined by light microscopy, ultrastructural and immunohistochemical techniques. In support of its extensive neuroendocrine differentiation, was the diffuse detection of neuron-specific enolase (NSE) and positive immunoreaction with antibodies to chromogranin and synaptophysin. The great majority of tumor cells contained neurosecretory granules and intraluminal mucin production could be focally detected.

Morphological-histochemical study of intestinal carcinoids and K-ras mutation analysis in appendiceal carcinoids.

Intestinal carcinoids are potentially malignant neoplasms. Their histogenesis and pathogenesis are currently uncertain. The morphological and histochemical characteristics of twenty intestinal carcinoids are studied.

Neuroendocrine differentiation in follicle-cell thyroid carcinoma: correlation to prognostic factors in papillary carcinoma.

The presence of NE-differentiation in the follicle-cell thyroid carcinoma has been investigated by immunohistochemical detection of chromogranin A in the neoplastic cells. It has been found that NE-differentiation was present in a significant percentage in the papillary Ca alone (46.60%) whereas all Huerthle-cell neoplasms and the large majority of follicular and undifferentiated carcinomas showed non reactivity for Chromogranin A.

Lipoid proteinosis of the oral mucosa: case report and review of the literature.

We describe a 37-year-old woman who presented with progressive mouth dryness. Physical examination revealed long-standing plaques on the face and upper limbs, papular lesions of the oral cavity and tongue firmness. A lower lip biopsy was performed.