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.

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.

[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.

Counts and areas of S-100-positive epidermal dendritic cells in atypical molluscum contagiosum affecting HIV+ patients.

Molluscum contagiosum is a common and self-limiting viral infection, that in HIV+ patients courses as an opportunist affection with atypical clinical features. Impaired cell-mediated immune response could be involved in such atypical growth. We evaluated the density and area of Langerhans cells (LC) using S-100 immunohistochemistry in seven atypical molluscum contagiosum. LC density was quantified by three different methods using computer-assisted morphometry as well as estimating the relative area of LC with respect to epidermal area. Results were compared with two control groups (normal skin specimens and molluscum contagiosum affecting non-AIDS healthy patients). We found a virtual absence of LC in areas of molluscum lesions affecting both HIV+ and non-AIDS patients. Likewise we observed an evident decrease in LC density in perilesional epidermis of atypical molluscum with respect to both control groups. Upon comparing the counts and areas, we observed that this reduction in LC count was statistically significant only when considering LC related to length of basement membrane in atypical molluscum with respect to normal skin specimens. Our finding of a reduced number of LC in the perilesional epidermis of HIV+ patients with atypical molluscum could explain the high frequency and clinical challenge of molluscum contagiosum in immunocompromised people. In spite of these results, further studies of LC kinetics and functions are required to precisely elucidate their role in the course of molluscum contagiosum in HIV+ patients.

[Simple thyroid cyst]. / Quiste simple de tiroides.

Simple or true thyroid cysts are very rare. We only have found one case in which colorless and acellular transparent fluid has been found by means of the fine needle aspiration puncture (FNAP). We report the case of a 33 year old female who first presented with a rapidly growing thyroid nodule. The thyroid scintigraphy revealed the existence of a cold nodule and the ultrasonography showed a lesion having a cystic appearance. The FNAP was performed and was both diagnostic and therapeutic in this case.

Analysis of EBV latency by EBER in situ hybridization in nasopharyngeal carcinoma Spanish patients.

BACKGROUND: The aim of this study was to analyse the Epstein-Barr virus (EBV) latency by detecting the EBV-associated latent small nuclear RNAs (EBER), in a group of biopsies from Spanish patients with diagnosed nasopharyngeal carcinoma (NPC). MATERIALS AND METHODS: NPC paraffin samples with the presence of EBV demonstrated by non-isotopic in situ hybridization (NISH) and nested-PCR, were analysed for EBV latency using EBER in situ hybridization (EBER-ISH). RESULTS: We detected EBER in 83.3% of samples (10 out of 12 cases), demonstrating the relationships between EBV genome presence with the latent viral infection. We correlated these results of EBV-DNA and -RNA presence with the immunoexpression of latent membrane protein-1 (LMP-1), a viral oncogenic protein (8 out of 12 cases or 66.6%). CONCLUSION: These results indicate that all the types of NPC are variants of an EBV-associated malignancy and that viral latency is a critical phenomenon in the development of this neoplasia.

Immunohistochemical absence of CD21 membrane receptor in nasopharyngeal carcinoma cells infected by Epstein-Barr virus in Spanish patients.

OBJECTIVES/HYPOTHESIS: The aim of this study was to analyze the relevance of the CD21 membrane receptor in nasopharyngeal carcinoma (NPC). CD21 is implicated in the introduction of the Epstein-Barr virus (EBV) genome into epithelial cells and B lymphocytes. STUDY DESIGN: Immunohistochemical analysis of CD21 in NPC. METHODS: Paraffin-embedded samples of NPC of different histological types with demonstrated presence of EBV were analyzed for CD21 expression using immunohistochemistry. RESULTS: We detected EBV by non-isotopic in situ hybridization (NISH) and nested polymerase chain reaction (PCR) in 100% of samples, regardless of histological type, supporting the previous view that all the types of NPC are variants of an EBV-associated malignancy. CD21 was not detected in NPC, and this absence was a typical feature in our data group. CONCLUSIONS: The loss of the CD21 membrane receptor could be one of the immunophenotypical changes of the neoplastic cells that occur in the evolution of the NPC malignancy.