Orbital involvement by non-Hodgkin lymphoma NK T cells.

CLINICAL CASE: The case is presented of 37 year-old male with a history of nasal obstruction with right rhinorrhea, headache, hearing loss and right exophthalmos of 4 months progression. The MRI revealed that the ethmoidal and maxillary sinuses contained inflammatory tissue extending into the orbital region. The biopsy confirmed a non-Hodgkin lymphoma of natural killer (NK) T cells. DISCUSSION: Non-Hodgkin's T NK lymphoma is a rare tumor in the orbital area that requires an early detection and multi-disciplinary care to ensure appropriate monitoring and treatment.

Synovial cysts of the temporomandibular joint: an immunohistochemical characterization and literature review.

Synovial cysts of the temporomandibular joint (TMJ) are very rare, and to date, only 12 cases of a synovial cyst in the TMJ region have been reported in the literature. In this paper, we present the clinicopathological and immunohistochemical characteristics of one such lesion affecting a 48-year-old woman, presented with a mass in the left preauricular region. We describe the usefulness of immunohistochemical analysis for recognizing the synovial lining, which allowed for clear differentiation between ganglion and synovial cysts. Immunohistochemical analyses can be used to diagnose synovial cysts with certainty; however, using at least two markers is advisable to distinguish the two existing synovial cell subtypes. Our findings indicate that synovial cysts of TMJ possess an internal lining dominated by type B (fibroblast-like) synoviocytes.

Obstructive uropathy in a HIV+ infant under indinavir treatment.

The case is presented of a 10-year-old HIV+ male with renoureteral pain, who developed an obstructive uropathy with renal function impairment and required endoscopic placement of a ureteral stent. Certain aspects of the epidemiology, clinical presentation, diagnosis, treatment and prevention are discussed.

Renal donor implication in the origin of BK infection: analysis of genomic viral subtypes.

UNLABELLED: BK virus (BKV) reactivation in immunocompromised kidney transplant patients can produce a tubulointerstitial nephropathy (BKVN). Molecular tools that test for DNA-BKV provide early detection and assist in management, but some aspects of the pathogenesis of this infection, such as donor causality, remain unclear. MATERIALS AND METHODS: Between November 2004 and January 2006, 55 Spanish kidney donors were studied for BK infection. A quantitative PCR assay was performed on urine and serum to detect BKV. To determine the origin of the viral infection, a transcription control region of the BK polymorphism sequence was designed to identify the viral subtype. RESULTS: Fifteen of 55 (27%) donors were BK-PCR positive: 13 in urine and 2 in serum and urine. Moreover, monitoring of recipient pairs detected BK-PCR positivity in 14 of 73 recipients. We studied eight BK-PCR positive recipients (corresponding to four pairs) and their respective donors. The same viral genome was observed in the four pairs, namely, the A250-1-a, WW-like, AS, and JL genotypes. Interestingly, one of the four pairs showed the donor and the two recipients to display exactly the same JL genotype. CONCLUSION: On the basis of our preliminary results analyzing the molecular fingerprints of donor and recipient pairs, we have presented new data implicating the donor, in at least some cases, as the source of BK infection.

[Small cell carcinoma of the urinary bladder in a young man. A case report and literature review]. / Carcinoma vesical de células pequeñas en un varón joven. Presentación de un caso y revisión de la literatura.

Small cell carcinoma of the bladder is a rare entity characterized by an aggressive clinical behaviour with a high incidence of systemic metastases. We report a case of small cell carcinoma of the bladder in a young man. The primary local tumour was treated by radical surgery, pelvic radiation therapy and polychemotherapy according CDDP protocol. The patient died six months after surgery because disease progression. We also review and update the literature concerning this infrequently tumour.

[Predictive value of progression of muscularis mucosae and percentage G3 cells in vesical TIG3 tumours]. / Valor predictivo de progresin de la muscularis mucosae y del porcentaje de células G3 en tumores vesicales T1G3.

INTRODUCTION AND OBJECTIVE: Vesical tumor T1G3 constitutes the border between the superficial tumor and the infiltrante tumor. Some of these tumors do not respond to BCG and progress, with cystectomy that present poor results, patients who would benefit from a precocious and aggressive treatment if we could identify them in an preinvasive stage. New predictive factors try to select to these tumors, being little the works that consider anatomo-pathological meticulous study (substanding of the T1 in T1a and T1b and percentage of present G3 cells in the tumor). Our objective is to analyze the value of these anatomo-pathological considerations like predictive factors of progression. MATERIAL AND METHODS: Retrospective study of a series of 91 patient affection of vesical tumor T1G3 with initial treatment by means of RTU and BCG. We analyzed 12 variables. The new predictive factors: the level of invasion respect to muscularis mucosae and the percentage of G3 cells. By means of logistic regression analisys we establish the independent pronostic factors for tumoral progression. RESULTS: A total of 31 patients presented infiltration of detrusor, passing away 17 of tumoral cause, after an average time of pursuit of 57.8 +/- 28.2 months. In 8 cases (9%) the substanding could not be determined. The rate of progression for T1a tumors was of 20% (8/40) and for T1b 53% (23/43). Presented independent predictive value of progression the multiplicity (odds: 7.26), the size (odds: 2.14), the presence of Cis (odds: 1.42) and the subestanding (odds: 6.81). CONCLUSION: The substanding is a predictive factor of progression clinically useful in vesical tumors T1G3, reason why we considered habitual clinical introduction.

[Prognostic value for progression of the regulating proteins of the cellular cycle in PT1G3 bladder tumours]. / Valor pronóstico de progresión de las moléculas reguladoras del ciclo celular en tumores vesicales T1G3.

INTRODUCTION AND OBJECTIVE: Bladder tumor T1G3 constitutes the group of superficial tumors more aggressive. New prognostic factors in the field of the cytogenetics and molecular biology have been analyzed, with often contradictory results, being little the specific works in tumors T1G3. Our objective is to determine if in this group of tumors the immunohistochemical markers present predictive value of clinically useful progression, and therefore with validity to indicate more suitable a precocious therapeutic attitude. MATERIAL AND METHODS: Retrospective study of a series of 83 patients affected of bladder tumor T1G3, on which we analyzed a total of 14 variables; between the new predictive factors: the immunohistochemical determination of regulating proteins of the cellular cycle: p53, p21 and bcl-2, as well as the Ki-67 protein like marker of cellular proliferation. By means of logistic regression analysis we establish the independent prognostic variables for tumorlike progression. RESULTS: The cut point established for Ki67 and p53 was 40% of inmmunomarked cells, 20% for p21 and 10% for Bcl-2. The univariant analysis showed different rates from progression and free times of progression based on the immunohistochemistry of Ki67 and p53: nevertheless, the logistic regression demonstrated that single the immunohistochemistry of p53 presented independent predictive value. CONCLUSIONS: The determination of p53 presents predictive value of clinically useful progression in bledder tumors T1G3, so that its determination can constitute a essential factor in the strategies of treatment of these tumors.

Ameloblastoma. Diagnosis by means of FNAB. Report of two cases.

INTRODUCTION: Ameloblastomas are the most frequent odontogenic tumors of the maxilla. In spite of their benign cytohistological appearance, they behave as invasive recurring tumors, with the possibility of metastasis. FNAB is a rapid, bloodless test that provides a pre-surgical diagnosis, thus, on occasions avoiding the need for diagnostic biopsies. We present the cytological characteristics of two cases of jugal recurrences of mandibular ameloblastomas diagnosed by FNAB, as well as their cytohistological correlation. CLINICAL CASES: Two patients, a 36-year-old woman, and a 62-year-old male who both attended with mandibular swelling of a few months evolution. In both cases the first diagnostic approximation was the histological study of the tumoral mass, together with the radiological studies. Following therapeutic extirpation both cases recurred. The diagnosis of the recurrences was established cytologically by means of FNAB. The cytologic smears revealed a granular background with isolated macrophages and giant multinucleate cells and an abundant epithelial cellularity of basaloid appearance arranged in cohesive groups forming images of peripheral palidasing, as well as small groups of squamous metaplastic cells. DISCUSSION: FNAB is considered to be a rapid, bloodless and reliable method for the diagnosis of ameloblastoma. The cytology of these tumors reveals components of the lesion that, in general, are sufficient for the diagnosis of ameloblastoma, especially in cases of recurrence.

A risk model for non-small cell lung cancer using clinicopathological variables, angiogenesis and oncoprotein expression.

BACKGROUND: The aim of this study was to investigate new prognostic factors, by using a prognostic model, that could help to identify the patient group with the greatest probability of death. PATIENTS AND METHODS: First, the clinicopathological variables were analyzed. Second, microvessels were immunohistochemically (IHC) stained with factor VIII-related antibody and then counted in the most intense vascularization area or hotspot, using an automatic image analyzer. In addition, biological angiogenesis-related factors such as vascular endothelial growth factor (VEGF) and inducible nitric oxide synthase expression (iNOS) were also studied. Finally, we evaluated the IHC expression of p53 and p21WAF1 tumor supressor proteins. RESULTS: The significant independent predictors were: tumor size (p=0.0063), angiogenesis (p=0.0271) and p21WAF1 (p=0.0478). Thus, the most important factor was tumor size 2.7462 [95% CI=1.3307-5.6673]. Finally, these variables were included in a risk model, in order to identify the group with the highest associated probability of death. CONCLUSION: The analysis of several prognostic factors could establish a more accurate patient risk profile.

Polymerase chain reaction detection of BK virus and monitoring of BK nephropathy in renal transplant recipients at the University Hospital La Fe.

INTRODUCTION: Reactivation of BK infection occurs in immunocompromised hosts causing tubulointerstitial nephropathy (BKVN). Approximately 5% of kidney transplant recipients (KTR) develop BKVN, special half of whom lose their grafts. However, BKVN morphologic diagnosis on a renal biopsy is complicated, because the cytopathic changes can sometimes mimic rejection. Thus, BKV DNA-polymerase chain reaction (PCR) assay on serum, urine, and renal tissue is useful for early detection and monitoring of BKV. MATERIALS AND METHODS: We performed routine monthly urine cytologies looking for decoy cells as a marker of virus replication. Then, we performed a qualitative PCR on urine and serum in all recipients (independently of positive or negative cytology). We amplified 3 BK viral genome regions, LT (early transcription region) and VP1 (late transcription region) seeking a more accurate virus detection, and the TCR (control transcription region) region to perform a polymorphism sequence analysis to identify the BK genomic variant. Finally, the BKVN diagnosis was confirmed using renal biopsy. RESULTS: At present, 132 patients have been monitored. Thirteen of 40 (33%) were PCR-urine-positive cases (5 LT+/VP1- and 8 LT+/VP1+), and 10 of 132 (7.5%) were PCR-serum-positive cases (7 LT+/VP1- and 3 LT+/VP1+). When we compared PCR-urine and cytology results, 11 of 40 (27.5%) patients showed a positive cytology, 6 of whom were PCR- urine-positive (1 LT+/VP1- and 5 LT+/VP1+); whereas, 29 patients showed a negative cytology, 7 of whom were PCR-urine-positive(3 LT+/VP1- and 4 LT+/VP1+). Thus, comparison of PCR- urine and cytology results revealed false-positive and false-negative cases. Finally, TCR sequence analysis was performed in 9 patients to identify the BK genomic variants. CONCLUSION: Testing for BKV DNA in urine and serum is a noninvasive early detection assay and monitoring tool.

[Study of the angiogenesis as prognostic factor of pT1G3 bladder tumours]. / Estudio de la angiogénesis como factor pronóstico de los tumores vesicales pT1G3.

INTRODUCTION AND OBJECTIVES: Angiogenic activity has been considered like prognostic factor in several solid tumors. This activity can be analysed by two ways: immunohistochemical determination of molecules that activate/inhibit angiogenesis or quantitive measure of microvascular density (MD). Our objective is to determine the prognostic value of Vascular Endothelial Growth Factor (VEGF) and Microvascular Density (MD) in pT1G3 bladder tumours. MATERIAL AND METHODS: We have studied retrospectively 83 patients with pT1G3 tumors treated by TUR + endovesical instillations with a follow up of 3 years at least. We analysed VEGF expression monoclonal antibody No. 360P. To determine MD we have marked vessels with FVIII antibody and detected "hot spots" areas. The number of microvessels is quantited by a digital image analyser excluding those that have more than 50 micras of diameter. We established the correlation of these findings with recurrence, progression and survival by using Chi-square test and Kaplan-Meier curves (log-rank). RESULTS: Average follow up was 58 +/- 28 months. We have established like cut-off 50% of tumor cells (VEGF) and 30 microvessels/fields (MD). Chi-square test did not show correlation with survival neither recurrence but it was positive for progression p(VEGF) 0.048 and p(DM) 0.021. Kaplan Meier curves determined significative differences only for free of progression time respect to MD (p 0.038). CONCLUSIONS: We did not find statistically significant value for recurrence nor survival. Just MD reached prognostic value for progression. More studies and multivariant analysis are required to determine the clinical utility of MD, specially in order to make more aggressive therapeutic options in this kind of patients.

[Cytomegalovirus nephrotic syndrome]. / Síndrome nefrótico infantil por citomegalovirus.

The case of a 5 months old infant with a nephrotic syndrome after neonatal cytomegalovirus infection is reported. Genomic amplification nested-PCR for CMV was positive in renal biopsy. Treatment with gancyclovir was effective to maintain nephrotic syndrome remission. We stresses the importance to discharge an infections cause of the nephrotic syndrome of newborns and infants due to the possibility on curative treatment.

[Bilateral testicular epidermoid cyst in a pediatric patient with Klinefelter syndrome]. / Quiste epidermoide testicular bilateral en un paciente pediátrico con síndrome de Klinefelter.

Epidermoid cysts of the testis are rare in children (3% of all the testicular tumors). Bilateral appearance has only been described in the pediatric age in 2 cases and none associated to Klinefelter's syndrome. We present, for our knowledge, the first case of bilateral epidermoid testicular cyst associated to klinefelter's syndrome in a boy, highlighting its management and therapeutic approach. We analyze the different kinds of treatment.

[Retrorbital metastasis an initial manifestation of prostatic cancer]. / Metástasis retrorbitaria como manifestación inicial del cáncer de próstata.

Orbital metastasis is an unusual localization within tumoral dissemination of prostatic cancer. Similarly, it is rare that orbital metastasis might be responsible for the clinic manifestations that determine the initial diagnosis of neoplasia. We illustrate the case of a patient suffering from prostatic adernocarcinoma that displayed alterations of facial sensitivity and right eye exophthalmos. We describe how the final diagnosis was reached and the patient's response to the suppressive hormonal treatment. The patient's rate of survival has proved to be longer than the rest of cases documented, with over 30-month follow-up.

[Mucinous adenocarcinoma of the bladder]. / Adenocarcinoma mucinoso de vejiga.

Mucinous adenocarcinoma is a rare entity within the group of primary adenocarcinoma of the bladder which represent 0.5-2% of all malignant epithelial bladder tumours. In spite of the rarity of this tumoral type; it is a poor prognosis entity mainly due to its diagnosis especially in advanced stage of the disease. There is no general agreement on the treatment of adenocarcinoma of bladder. Not withstanding surgery would be the only curative treatment, although unfortunately, it is curative in just a few cases. We report six cases with mucinous adenocarcinoma of the bladder attended in our Department in the last ten years (january 1991-december 2001). In one of them a radical cystectomy was performed, while transurethral resection with or without adjuvant treatment was practiced in the other one. Only one patient is alive today, namely, the one where the tumour not invade the muscular tissue. These findings show the discouraging results of this entity closely intertwined with the pathologic stage.

[Small cell carcinoma of the prostate]. / Carcinoma prostático de células pequeñas.

Pure small cell carcinoma of the prostate is rare (less than 1% of all prostatic neoplasm). As a result there are few reports in the literature that describe the salient features and appropriate management of this cancer (less than 200 cases reported). Small cell carcinomas of the prostate are a heterogeneous group of tumors, a number of them have neuroendocrine differentiation and are highly aggressive, commonly with visceral metastases at time of diagnosis. Complete temporary remission has been reported with chemotherapy but this tumor has a poor prognosis. The median overall survival from the time of diagnosis is between 5-17.5 months. We report 2 new cases of small cell carcinoma of the prostate and a review of the literature.

[Foam cell nephropathy in heterozygous female with Fabry's disease]. / Nefropatía de células espumosas en mujer heterozigota portadora de la enfermedad de Fabry.

We describe the clinical and pathological characteristics of a 49 year-old heterozygous female carrier of Anderson-Fabry's disease. Light microscopy and ultrastructural study of a renal biopsy showed the presence of foam cell nephropathy and galactosylceramide deposits affecting podocytes, the parietal epithelium of Bowman's capsule and the distal tubular cells, endothelial cells and medullary interstitial elements. Retrospectively, the presence of storage disease was confirmed in a hysterectomy specimen obtained two years previously. Our observation shows that heterozygotes for this disorder can not only carry and transmit the disease but may also develop pathological deposits in various organs. A renal biopsy from these carriers allows precise identification of the disease, facilitates adequate genetic counseling and gives the option of enzyme replacement therapy in patients who have pathological deposits.

Expression of p53 protein and tumor angiogenesis as prognostic factors in nasopharyngeal carcinoma patients.

The objective of this study was to evaluate the possible prognostic significance of p53 protein overexpression and tumor angiogenesis (TA) in nasopharyngeal carcinoma (NPC) patients, together with other clinicopathological variables. Forty-two NPC patients were evaluated in relation to survival. Nuclear p53 overexpression in neoplastic and endothelial cells was detected by immunohistochemistry (IHC) with the monoclonal antibody DO-7 and the polyclonal antibody against factor VIII-related antigen, respectively. Thereafter, we evaluated p53 cases in order to determine their nuclear immunoreactivity from negative (-) to positive (+, ++, +++). In addition, microvessels were counted in the most active areas of tumor neovascularization or hotspots using an image computer analyzer (MicroImage). A Cox multiple regression survival analysis was used to determine the best prognostic indicators in NPC patients. As a result, tumor microvessel count, considered as a continuous variable, was the most important independent prognostic indicator in relation to survival (p = 0.0273), with a relative risk of death of 2,4399 [95% confidence interval = 1.1051 ; 5.3871] associated with the highest microvessel counts. Moreover, the only clinicopathological variable that demonstrated prognostic value in a Cox multiple regression survival analysis was histological type (p = 0.05). In addition, we did not observe any statistical association between intratumoral microvessel density (IMD), clinicopathological variables and p53 protein expression.

[Cholesterol crystals embolization. A case report]. / Embolismo por cristales de colesterol. A propósito de un caso.

Cholesterol Crystals Embolization is not a very frequent entity that happens in people with atheromatous disease of spontaneous for or after the realization of vascular manipulation (angiographic, angioplasty procedures, cardiovascular surgery) or more rarely with the treatment with oral anticoagulants and after thrombolytic therapy. The clinical presentation is according to the arterial affected territory and so from diagnosed cases in autopsy reports to florid clinic forms exist. The more frequent are the cutaneous manifestations with apparition of livedo reticularis and purple toes that could go accompanied of necrosis of lower limbs, consecutive from the renal manifestations and the gastrointestinal tract one. The diagnosis is based on the biopsy of organ affected lesions to level of arterioles, that revealed characteristic cholesterol clefts, above all the cutaneous biopsy, muscular and in any case renal. As for the treatment is symptomatic with analgesics, antiplatelet agents, anti-inflammatory, etc ... but it doesn't after the presage and the evolution of the illness. A clear contraindication exists to the use of oral anticoagulants and heparin.