Bladder Fetal Rhabdomyoma Intermediate Type.

INTRODUCTION: Rhabdomyomas are benign tumors of striated muscle, the bladder localization is very rare. CLINICAL CASE: We present an 87-year-old male consulting for gross hematuria. Cystoscopy was done with evidence of bulged bladder mucosa in right side wall and dome. Post-transurethral resection of the bladder (TURB) pathological anatomy was negative for malignancy. As extension study abdominopelvic computed tomography was performed identifying a bladder thickening of right posterior sidewall and an increased density of the adjacent fat. Second TURB was performed and a fetal bladder rhabdomyoma intermediate type was obtained. We performed another biopsy to confirm this rare pathology, with the same diagnosis. Subsequently, the patient continues with hematuria deciding on hemostatic radiotherapy (not candidate for cystectomy or arterial embolization). Currently, the patient is asymptomatic. DISCUSSION: Bladder rhabdomyomas are rare tumors, and, in fact, there have been only 5 papers published. Some cases are only isolation cited in the bladder mesenchymal tumors, and other polemic cases in which clinical and macroscopic characteristics remembered a rhabdomyosarcoma. The importance of this publication case is the macro- and microscopic images that can corroborate the final diagnosis, helping us to differentiate between rhabdomyoma, rhabdomyofibroma, or the malignant rhabdomyosarcoma, and shows the treatment possibilities of these tumors.

Choroid plexus papilloma with stromal deposition of amyloid and elastic material.

Congophilic birefringent amyloid deposits, with immunostaining for transthyretin (TTR) and amyloid P, associated with numerous coarse, enlarged and thick elastic fibres, are reported in the stroma of two choroid plexus papillomas, a finding not previously described in choroid plexus tumours. TTR was expressed as aggregates of 'doughnut-shaped' bodies, in which the TTR-positive peripheral area encircled the elastic fibre (TTR-negative core). Ultrastructurally, the amyloid microfibrils surrounded the elastic fibres and appeared to continue into the microfibrillar mantle of the latter. The stromal TTR-amyloid deposits associated with abundant elastic fibres in tumours that occur in the choroid plexus may be related to the alteration (production/accumulation, insufficient breakdown and/or extracellular matrix modifications) of some of the choroid plexus functions (removal, target and source of polypeptides, including TTR synthesis) and may be of interest for future studies on choroid plexus polypeptide activity and on protein development into elastomeric and amyloidogenic microfibrils.

Relation between body fat and liver fat accumulation and cytokine pattern in non-alcoholic patients with chronic HCV infection.

BACKGROUND: Liver steatosis in chronic hepatitis C virus (HCV) infection is multifactorial. Therefore, there is not necessarily a relation between obesity and liver fat.On the other hand, body fat secretes cytokines, and cytokines and oxidative damage play important roles on progression of liver disease. METHODS: We analyzed the relationships between liver fat (assessed by histomorphometry) and trunk and subcutaneous fat (waist perimeter, triceps skinfold, BMI); the relationships between liver and body fat and cytokines (IL-6, TNF-alpha, IL-8, IFN-gamma, IL-4), adipokines (adiponectin and TIMP-1), and serum malondiladehyde and antioxidants (glutathione peroxidase and superoxide dismutase (SOD) activities); and the relationships of these data with histological changes in 40 HCV-infected non-alcoholic patients. RESULTS: Significant correlations were found between liver fat and waist perimeter and BMI, and between serum TIMP-1 and liver fat. Serum TIMP-1 was significantly related to body fat stores; serum IL-6 and IFN-gamma were related to histological inflammation. Patients with waist perimeter >102 cm (men) or 88 cm (women) showed increased liver fat. In 38.8% of non-obese patients, liver fat accumulation was intense. CONCLUSIONS: There is a relationship between visceral fat, serum TIMP-1 and liver steatosis. However, at least in some patients, factors different from mere adiposity play a role in liver steatosis.

Progressive renal failure as the first manifestation of monoclonal light-chain deposition disease with rapid multiple organ involvement.

Light-chain deposition disease (LCDD) is an uncommon condition in which monoclonal light chains are deposited in different organs causing varying degrees of tissue damage. We report the case of a 56-year-old male with progressive renal failure as the first manifestation of LCDD without initial evidence of monoclonal immunoglobulin protein in either serum or urine. The patient later developed severe systemic disease with rapid multiple organ involvement. Finally, he died 2 months after diagnosis, despite steroid and cyclophosphamide therapy.

Histogenesis and morphofunctional characteristics of chromaffin cells.

This article reviews the current status of research about the histogenesis and morphofunctional characteristics of chromaffin cells in the adrenal medulla. First, this study reports the selective migration, transcription and activation factors, and the morphological events of the chromaffin cell precursors during adrenal medulla development. Subsequently, the morphofunctional characteristics of adrenergic and non-adrenergic cells are considered, with particular reference to the characteristics of chromaffin granules and their biological steps, including their formation, traffic (storage, targeting and docking), exocytosis in the strict sense and recapture. Moreover, the relationship of chromaffin cells with other tissue components of the adrenal medulla is also revised, comprising the ganglion cells, sustentacular cells, nerves and connective-vascular tissue.

Granular cell tumors of the esophagus: report of five cases and review of diagnostic and therapeutic techniques.

Granular cell tumors (GCT) of the esophagus are stromal lesions originating from the Schwann cells of the submucosal neuronal plexus. Although they are very infrequent, they constitute the second largest cause of non-epithelial tumors in the esophagus after leiomyomas. These tumors are generally benign, although a certain number of malignant, aggressive cases have been reported. Diagnosis requires that this possibility be ruled out before deciding on which course of therapeutic action to take as well as familiarization with the relevant indicators. GCT linked synchronically or metachronically to other malignant neoplasias of the esophagus have also been described, but the actual extent of this association is uncertain. This report describes five cases of GCT recently diagnosed as incidental findings following endoscopic exploration. All of these were benign and were treated conservatively. The article discusses new aspects relating to the diagnosis of these lesions and the role carried out by endoscopic ultrasonography in their characterization, both at preliminary diagnosis and monitoring levels. No standard therapeutic guidelines exist for the management of GCT, but endoscopic treatment without invading the muscularis propria layer would be used for symptomatic patients, creating histopathological doubts requiring research on the entire organ. Endoscopic therapeutic techniques are analyzed (resection with forceps or diathermy handles, yttrium-aluminum-garnet laser ablation, alcohol injection) in esophageal GCT, which have overtaken surgery in most cases due to their efficiency, greater safety and fewer complications.

Adult stem and transit-amplifying cell location.

Adult stem cells (ASC)--able to self renew and to intervene in maintaining the structural and functional integrity of their original tissue--can express greater plasticity than traditionally attributed to them, adopting functional phenotypes and expression profiles of cells from other tissues. Therefore, they could be useful to regenerative medicine and tissue engineering. Transit-amplifying cells (TAC) are committed progenitors among the ASC and their terminally differentiated daughter cells. The ASC reside in a specialized physical location named niche, which constitutes a three-dimensional microenviroment where ASC and TAC are protected and controlled in their self-renewing capacity and differentiation. The niche can be located near or far from the recruitment point, requiring a short or long-distance cellular migration, respectively. This paper briefly reviews the current status of research about ASC plasticity, transdifferentiation, fusion and functional adaptation mechanisms. Subsequently, ASC and TAC occurrence, characteristics and location have been considered in the skin, cornea, respiratory tract, teeth, gastrointestinal tract, liver, pancreas, salivary glands, kidney, breast, prostate, endometrium, mesenchyma, bone marrow, skeletal and cardiac muscle, nervous system and pituitary gland. Moreover, the role of cancer ASC has also been revised.

Case report. Pulmonary involvement in an adult male affected by type B Niemann-Pick disease.

We report the case of a 39-year-old male patient affected by type B Niemann-Pick disease, in whom pulmonary involvement became evident 15 years after the initial diagnosis. Pulmonary involvement was discovered incidentally during the evaluation of a dry cough and exertional dyspnoea which occurred in the context of an acute febrile, self-limiting illness. In this case, the pulmonary involvement is clinically mild, with minimal alteration of the diffusing capacity for carbon monoxide (DL(CO)), despite moderate fibrosis and widespread infiltration of both alveoli and interstitium by sea blue histiocytes.

Prognostic significance of Ki-67 nuclear proliferative antigen, bcl-2 protein, and p53 expression in follicular and diffuse large B-cell lymphoma.

We analyzed 104 patients with non-Hodgkin's lymphoma, follicular or diffuse large-B-cell-type lymphoma, in order to evaluate the correlation between clinical characteristics and immunohistochemical parameters. Immunostaining was performed by means of monoclonal antibodies against Ki-67, bcl-2, and p53 expression. Forty-nine of the patients showed follicular lymphoma. A high expression of bcl-2 was found in 93%, high expression of p53 in 57%, and low expression of Ki-67 in 96%. Follicular lymphoma grade III showed a p53 expression (p = 0.07) slightly higher than follicular lymphoma grades I and II, not reaching statistical significance. Follicular lymphoma grades I and II tended to express lower Ki-67 and higher levels of bcl-2 expression than grade III (p = 0.06). Fifty-five cases showed diffuse large-B-cell lymphoma. Among them, bcl-2 was absent in 39%, whereas p53 and Ki-67 expression were high in 38%. In the diffuse large-B-cell lymphomas, a high bcl-2 expression correlated with stages III and IV (p = 0.03) and involvement of more than one extranodal area (p = 0.03). High Ki-67 expression was also associated to extranodal involvement of more than one area (p = 0.03). Overall survival of patients did not show statistically significant differences regarding Ki-67, bcl-2, and p53 tumoral expression. Prognostic factors for overall survival in the multivariate analysis were age (p = 0.02) and LDH (p = 0.003). Time to progression was worse among follicular lymphoma with high p53 expression than with mild/moderate p53 expression (p = 0.009).

[Inflammatory pseudotumor of the maxillary sinus: report of a new case and review of the literature]. / Pseudotumor inflamatorio de seno maxilar: presentación de un nuevo caso y revisión de la literatura.

A new case of inflammatory pseudotumor (IPT) of the maxillary sinus is presented. The clinical and histological features of this rare entity are discussed. Immunohistochemical studies support a possible myofibroblastic origin. Early diagnosis is important to prevent IPT, classified as a benign disease, from becoming a progressive destructive lesion.

Pseudotumor inflamatorio de seno maxilar: presentación de un nuevo caso y revisión de la literatura / Inflammatory pseudotumor of the maxillary sinus: report of a new case and review of the literature

Presentamos un nuevo caso de pseudotumor inflamatorio de seno maxilar. Revisamos las características clínicas y patológicas de esta rara entidad y realizamos estudios inmunohistoquímicos que aportan nuevos datos para el diagnóstico y nos orientan hacia un origen miofibroblástico del tumor. Destacamos la importancia de un diagnóstico precoz, para evitar que esta lesión clasificada como benigna se cronifique y adquiera un carácter destructivo (AU)

A new case of inflammatory pseudotumor (IPT) of the maxillary sinus is presented. The clinical and histological features of this rare entity are discussed. Immunohistochemical studies support a possible myofibroblastic origin. Early diagnosis is important to prevent IPT, classified as a benign disease, from becoming a progressive destructive lesion (AU)

Hepatocarcinoma con metástasis intestinales polipoides: hallazgo en un caso de necropsia / Hepatocarcinoma with polypoid intestinal metastasis. Autopsy findings

No disponible

Malignant rhabdoid tumor beside benign skin mesenchymal neoplasm with myofibromatous features.

Only a few reports of primary cutaneous rhabdoid tumors have been published. We describe the case of a 3-month-old female patient who developed a rhabdoid type cutaneous sarcomatoid neoplasm in her upper back, close to a benign myofibromatous proliferation of infancy. The lesion was studied both by light microscopy and immunohistochemically. Flow cytometry was performed showing a DNA diploid profile of the malignant tumor. The pathological findings suggest a mesenchymal origin (hemangiopericytic or myofibroblastic type) for both tumors. The patient was surgically treated, but she died nine months later.

Sodium butyrate inhibits carcinoma development in a 1,2-dimethylhydrazine-induced rat colon cancer.

BACKGROUND: Butyric acid is one of the most important by-products of dietary fiber degradation. It is an important trophic agent for the intestinal mucosa under different experimental conditions. Data obtained from several in vitro studies strongly suggest that butyrate can be a potential therapeutic agent in controlling the growth of some cancer cells. However to date in vivo animal studies have failed to show conclusive results. METHODS: We evaluated the effects of intracecal administration of butyrate in an experimental model of colonic carcinogenesis induced by 1,2-dimethylhydrazine (DMH), administered at the site where it is naturally produced, the cecum. We studied the incidence of colon tumors and their main histologic features. RESULTS: Direct application of sodium butyrate significantly decreased the total number of tumors and the incidence of malignancies and carcinoma in the colon. CONCLUSIONS: Butyrate may inhibit the growth of tumors induced by DMH.

Perineurial cell tumor (perineurioma) with granular cells.

A form of benign cutaneous tumor with perineurioma findings and with the presence of associated granular cells is described. The two cases studied consisted of whorls made up of a high number of circumferentially arranged flattened cells, with perineurial characteristics, including bipolar cell processes, pinocytotic vesicles, a basal lamina, a positive immunoreactivity for EMA, and absence of immunostaining for S-100 protein. The granular cells, enclosed within the whorls, contained densely packed vesicles, particles with an apparently solid core, as well as membrane-limited vacuoles with disintegrating cellular organelles and electron-dense amorphous material. While failing to demonstrate any immunoreactivity for EMA, the granular cells showed positivity for S-100 protein, which supports their Schwann-cell origin. Due to its morphological and immunohistochemical characteristics, this peculiar form of tumor can be considered as a perineurioma with perineurial cell whorls and granular cell changes occurring in associated Schwann cells at the center of the whorls.

[Caroli's disease, congenital hepatic fibrosis and renal polycystosis: a little frequent association]. / Enfermedad de Caroli, fibrosis hepática congénita y poliquistosis renal: una asociación poco frecuente.

We report the case of a woman with Caroli's syndrome (Caroli's disease and congenital hepatic fibrosis) and adult polycystic kidney disease. Whereas the association of these two entities with autosomal-recessive-polycystic kidney disease has been widely reported, its association with adult-polycystic kidney disease is very infrequent.