Squamous cell carcinoma over pilonidal chronic disease. A new therapeutic approach.

INTRODUCTION: Pilonidal sinus is a very common disease. Malignant transformation occurs in 0,1% of patients. We present a case of squamous cell carcinoma arised from recurrent pilonidal disease, managed by multimodal treatment. PRESENTATION OF CASE: We present a 70-year-old man with chronic pilonidal sinus. Inflammation had worsened in previous months and exploration revealed a large ulcerative mass which biopsy showed a squamous cell carcinoma. CT scan and MRI imaging showed tumoral invasion of the coccyx and both gluteus major muscles. Neoadjuvant radiotherapy, chemotherapy as radiosensitizer and surgery with intraoperative radiotherapy was decided in the multidisciplinary tumor committee. Post neoadjuvant therapy MRI showed partial response with a decrease of the mass but persistence of the coccyx infiltration. Surgery consisted in en-bloc resection of the tumor with presacral tissues, coccyx and partial gluteal resection. Intraoperative radiotherapy was administered over the sacrum and in the bed of the coccyx resection. One week later, reconstructive surgery was practiced using a latissimus dorsi free flap, advancement of gluteal flaps and skin graft. Histological examination showed no residual tumor. The patient is currently asymptomatic and he has a satisfactory quality of life. DISCUSSION: Although squamous cell carcinoma is rare, it must be suspected in patients with recurrent pilonidal disease. Diagnosis is done by histological examination of biopsies. This type of tumors have a high local recurrence rate. CONCLUSION: We propose a multimodal treatment that includes neoadjuvant radiotherapy and chemotherapy as radiosensitizer and surgery plus intraoperative radiotherapy with the aim to decrease local recurrence rate.

Rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation (ROHHAD syndrome): A case report and literature review. / Síndrome ROHHAD (obesidad de rápida progresión, disfunción hipotalámica, hipoventilación y disregulación autonómica). Presentación de un caso y revisión de la literatura.

INTRODUCTION: ROHHAD syndrome (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation) is a rare and complex disease, presenting in previously healthy children at the age of 2-4 years. Up to 40% of cases are associated with neural crest tumours. DEVELOPMENT: We present the case of a 2-year-old girl with symptoms of rapidly progressing obesity, who a few months later developed hypothalamic dysfunction with severe electrolyte imbalance, behaviour disorder, hypoventilation, and severe autonomic dysregulation, among other symptoms. Although the pathophysiology of this syndrome remains unclear, an autoimmune hypothesis has been proposed for ROHHAD. Therefore, after obtaining a limited response to intravenous immunoglobulins, we decided to test the response to a high dose cyclophosphamide (low dose was not effective either). Unfortunately our patient experienced many severe complications (among them central pontine myelinolysis, from which the patient recovered, and failure to wean from the ventilator requiring tracheostomy and long term ventilation) that required a prolonged ICU stay. Although her behaviour improved, our patient unfortunately died suddenly at home at the age of 5 due to respiratory pathology. CONCLUSIONS: ROHHAD syndrome is a rare and little-known disease which requires a multidisciplinary approach because it involves complex symptoms and multiple organ system involvement. Alveolar hypoventilation should be identified early and appropriate treatment should be started promptly for the best possible outcome. Immunomodulatory treatment with immunoglobulins, cyclophosphamide, or rituximab has previously resulted in symptom improvement in some cases. Because of the low incidence of the syndrome, multi-centre studies must be carried out in order to gather more accurate information about ROHHAD pathophysiology and design an appropriate therapeutic approach.

Active and reactive behaviour in human mobility: the influence of attraction points on pedestrians.

Human mobility is becoming an accessible field of study, thanks to the progress and availability of tracking technologies as a common feature of smart phones. We describe an example of a scalable experiment exploiting these circumstances at a public, outdoor fair in Barcelona (Spain). Participants were tracked while wandering through an open space with activity stands attracting their attention. We develop a general modelling framework based on Langevin dynamics, which allows us to test the influence of two distinct types of ingredients on mobility: reactive or context-dependent factors, modelled by means of a force field generated by attraction points in a given spatial configuration and active or inherent factors, modelled from intrinsic movement patterns of the subjects. The additive and constructive framework model accounts for some observed features. Starting with the simplest model (purely random walkers) as a reference, we progressively introduce different ingredients such as persistence, memory and perceptual landscape, aiming to untangle active and reactive contributions and quantify their respective relevance. The proposed approach may help in anticipating the spatial distribution of citizens in alternative scenarios and in improving the design of public events based on a facts-based approach.

Use of GlideScope for double lumen endotracheal tube insertion in an awake patient with difficult airway / Uso del Glidescope para la inserción de un tubo de doble luz en paciente despierto con vía aérea difícil

There is scientific evidence that an anticipated difficult airway must be managed with the patient being awake. The GlideScope has been proven to be a useful device to intubate the trachea in some instances when difficult airway is present, and particularly in the awake patient. It has also been used for double lumen tube (DLT) in the anaesthetized patient, but its use with DLT in both circumstances, awake patients with difficult airway has not been described. GlideScope enabled us to achieve accurate local anesthetic spraying and a successful endotracheal intubation with a double lumen tube (DLT) in an awake patient with predicted difficult airway and bronchoaspiration risk. Different ways to resolve cases like this can be found in the anesthetic literature, but we think this could be another option to bear in mind. We also describe a new variation in the maneuver of introducing a DLT into the trachea under GlideScope view as DLT presents with some difficulties when introduced under normal circumstances. This option could add some risk for the patients when used in inexperienced hands and there is not sufficient scientific evidence in the literature to recommend it for all cases (AU)

Existe evidencia científica de que la vía aérea difícil anticipada debe manipularse con el paciente despierto. Se ha demostrado la utilidad del GlideScope ante una vía aérea difícil y específicamente en el paciente despierto y también ha sido utilizado para introducir tubos de doble luz (TDL) en pacientes anestesiados, pero su uso con TDL en paciente despierto con vía aérea difícil todavía no ha sido descrito. GlideScope nos permitió conseguir una adecuada anestesia tópica de la vía aérea e intubación endotraqueal con un tubo de doble luz en un paciente despierto con vía aérea difícil prevista y riesgo de broncoaspiración. En la bibliografía se halla diferentes maneras de resolver casos similares, pero pensamos que la descrita puede ser otra opción a tener en cuenta. También se describe una nueva variación en la maniobra de introducción del TDL en la tráquea con GlideScope. La maniobra descrita podría suponer algún riesgo para el paciente en manos inexpertas y no hay suficiente evidencia científica para generalizar su recomendación

Use of GlideScope for double lumen endotracheal tube insertion in an awake patient with difficult airway.

There is scientific evidence that an anticipated difficult airway must be managed with the patient being awake. The GlideScope has been proven to be a useful device to intubate the trachea in some instances when difficult airway is present, and particularly in the awake patient. It has also been used for double lumen tube (DLT) in the anaesthetized patient, but its use with DLT in both circumstances, awake patients with difficult airway has not been described. GlideScope enabled us to achieve accurate local anesthetic spraying and a successful endotracheal intubation with a double lumen tube (DLT) in an awake patient with predicted difficult airway and bronchoaspiration risk. Different ways to resolve cases like this can be found in the anesthetic literature, but we think this could be another option to bear in mind. We also describe a new variation in the maneuver of introducing a DLT into the trachea under GlideScope view as DLT presents with some difficulties when introduced under normal circumstances. This option could add some risk for the patients when used in inexperienced hands and there is not sufficient scientific evidence in the literature to recommend it for all cases.

Instilación de anestesia tópica en la vía aérea con la sonda MADgic para la intubación del paciente despierto con videolaringoscopio Glidescope / No disponible

No disponible

Hemorragia digestiva como presentación de un tumor germinal testicular / Gastrointestinal bleeding as presentation of a testicular germ cell tumor

No disponible

Epirubicin-cyclophosphamide adjuvant chemotherapy plus tamoxifen administered concurrently versus sequentially: randomized phase III trial in postmenopausal node-positive breast cancer patients. A GEICAM 9401 study.

BACKGROUND: A prospective randomized clinical trial was implemented to assess whether the concomitant or the sequential addition of tamoxifen to chemotherapy provides improved clinical benefit in the adjuvant treatment of breast cancer in postmenopausal patients. PATIENTS AND METHODS: Four-hundred and eighty-five patients with node-positive operable disease were randomized to receive tamoxifen (20 mg/day) concomitantly (CON) or sequentially (SEQ) to EC chemotherapy (epirubicin 75 mg/m(2) + cyclophosphamide 600 mg/m(2) on day 1, every 21 days for four cycles). RESULTS: In the 474 fully evaluable patients there were 96 events; eight being second neoplasms and 88 being related to the breast cancer. Of these, 48 of 88 occurred in the CON arm and 40 of 88 in the SEQ arm. The Kaplan-Meier estimation of disease-free survival (DFS) at 5 years was 70% in the CON and 75% in the SEQ group (log-rank test, P = 0.43). Adjusted hazard ratio for treatment was 1.11 (95% confidence interval 0.71-1.73; P = 0.64). CONCLUSION: This study fails to show an advantage of one treatment arm over the other, but a trend, albeit non-significant, appears to favor the sequential addition of tamoxifen to epirubicin + cyclophosphamide and, as such, warrants further investigation.

Gemcitabine and low dose carboplatin in the treatment of elderly patients with advanced non-small cell lung cancer.

BACKGROUND: Fifty percent of lung cancers arise in patients over 65 years old and 30% in those over 70. The aim of this study was to evaluate response, survival and tolerability of the combination carboplatin-gemcitabine in elderly patients with advanced non-small cell lung cancer (NSCLC). METHODS: Between May 1998 and December 2000, 88 patients were included. Median age was 74 (range 65-83). Treatment consisted of gemcitabine 1250 mg/m(2) (1000 mg/m(2) in the first six patients) on days 1 and 8, and carboplatin AUC=4 on day 1, every 21 days. Prognostic factors for survival were analysed. Performance status (PS) and symptoms were evaluated before and after three and six courses. RESULTS: A total of 400 cycles were administered (median of four per patient). WHO grades 3-4 toxicities were: neutropenia in 13% of the cycles, thrombocytopenia and anaemia in 4.5 and 14.7% of patients in any cycle. There was one treatment-related death. According to the intent-to-treat analysis, 33 patients achieved objective response, 33 had stable disease, and 22 had treatment failure (progression in 18 patients). Median and 1 year survival were 9 months and 34%, respectively. Median time to progression was 8 months. Only disease stage and PS showed independent prognostic value. Comorbidity and PS displayed no close correlation. Symptom improvement was seen as follows: pain (61.7%), dyspnea (50%), haemoptysis (80%), anorexia (62.5%) and asthenia (61.5%). CONCLUSIONS: The combination carboplatin-gemcitabine at these doses is feasible in elderly patients with advanced non-small cell lung cancer. Tolerability and toxicity are acceptable. Response rate and survival stand in the range of the most active regimens. Comorbidity and PS showed prognostic independence.

Oxidative stress induces the expression of the major histocompatibility complex in murine tumor cells.

The effect of t-butyl hydroperoxide (t-BOOH) on the induction of the Major Histocompatibility Complex (MHC) class I genes has been studied in two cell clones (B9 and G2) of the methylcholanthrene-induced murine fibrosarcoma GR9. These two clones were selected based on their different biological and biochemical behavior specially related to their tumor induction capability when injected into a BALB/c mouse. t-BOOH (0.125 mM) induced the expression of H-2 molecules in both cell clones. In B9 cell clone, in which MHC basal expression is very low or absent, t-BOOH significantly induced H-2Kd, H-2Dd and H-2Ld molecules. In G2 cell clone the expression of MHC class I genes was also enhanced by the xenobiotic, the effect being especially significant on the H-2Ld molecule which is not expressed under basal conditions. H-2 molecules expression was accompanied by the activation of the transactivator factor NF kappa B. These results suggest that oxidative stress may modulate the antigen expression of tumor cells and thus the immune response of the host organism. Basal levels of oxidative parameters, such as anti-oxidant enzymes, malondialdehyde (MDA) and the DNA damaged base 8-hydroxy-2'-deoxyguanosine (8-OHdG), showed differences between the two fibrosarcoma cell clones.

[Epidemiology of urinary lithiasis]. / Epidemiología de la litiasis urinaria.

An epidemiological study of urinary stone disease has been carried out in the districts of La Sierra and Hellín (Albacete, Spain). Between January 1992 and December 1999 a total of 887 patients with urinary lithiasis have been studied, analysing the number of patients discharged from hospital with the primary diagnosis of lithiasis, the distribution of lithiasis by area, age, gender, family history of lithiasis, diet, occupation and concurrent pathology. The geographical, geological, and climatic conditions, the composition of the water supply and the clinical characteristics are described. The incidence of urinary lithiasis in the whole area in 2.66 per thousand inhabitants/year, showing a male predominance with a male:female ratio of 1.26:1. The mean age was 51.34 years (bimodal distribution with peaks in the 4th and 7th decades). 20.41% of the patients had a family history, with the father being the most frequently affected relation. Those occupations associated with a sedentary life style or with a hot, dry workplace show a higher incidence of lithiasis. A hot, dry climate favours the formation of urinary lithiasis and the highest incidence of lithiasis is in the summer, during the months of July and August. No statistically significant influence was shown between at the hardness of the water and the incidence of urinary lithiasis in the study population.

Antioxidant enzyme activities and the production of MDA and 8-oxo-dG in chronic lymphocytic leukemia.

Chronic lymphocytic leukemia (CLL) is a neoplastic disease susceptible to antioxidant enzyme alterations and oxidative stress. We have examined the activities of superoxide dismutase (SOD), catalase (CAT), and glutathione peroxidase (GPx), and the oxidized/reduced glutathione (GSSG/GSH) ratio together with the levels of malondialdehyde (MDA) and 8-oxo-2'-deoxyguanosine (8-oxo-dG) in lymphocytes of CLL patients and compared them with those of normal subjects of the same age. SOD and CAT activity decreased in CLL lymphocytes while GPx activity increased. GSH content of CLL lymphocytes also increased, and GSSG concentration remained constant. Thus, a reduced GSSG/GSH ratio was obtained. The oxidation product MDA, and the damaged DNA base 8-oxo-dG were also increased in CLL. The observed changes in enzyme activities, GSSG/GSH ratio, and MDA were significantly enhanced as the duration of the disease increased in years. The results support a predominant oxidative stress status in CLL lymphocytes and emphasize the role of the examined parameters as markers of the disease evolution.

Epidemiología de la litiasis urinaria / The epidemiology of urinary lithiasis

Se realiza un estudio epidemiológico de la enfermedad litiásica en las comarcas de la Sierra y de Hellín (Albacete). Entre Enero de 1992 y Diciembre de 1999, se han recogido 887 pacientes con litiasis urinaria, analizando la incidencia hospitalaria, distribución de litiásicos por localidad, edad, sexo, antecedentes familiares de litiasis, dieta, profesión y patología concomitante, se describen las características geográficas, geológicas, climáticas, composición de aguas, así como las características clínicas.La incidencia de urolitiasis en esta área es de 2,66 por mil habitantes/año, de predominio en varones con una relación de hombre/mujer de 1,26, la edad media es de 51,34 años (comportamiento bimodal con picos en la cuarta y séptima décadas).El 20,41 por ciento de los pacientes tienen antecedentes familiares, siendo el padre el parentesco más afectado. Las profesiones que comportan una vida sedentaria o se relacionan en ambientes calurosos tienen una mayor incidencia de urolitiasis. Los hábitats con clima caluroso y seco favorecen la enfermedad litiásica, produciéndose la mayor incidencia de litiasis en verano durante los meses de Julio y Agosto. No apreciamos una influencia estadísticamente significativa entre la dureza de las aguas y la incidencia de litiasis urinaria en la población estudiada (AU)

ESHAP is an active regimen for relapsing Hodgkin's disease.

BACKGROUND: Refractory or relapsing Hodgkin's disease is associated with a poor prognosis. There is no widely accepted salvage chemotherapy regimen for these patients. However, the addition of high-dose chemotherapy followed by autologous hematopoietic transplantation (AHT) has proven of benefit to them. A prospective clinical trial was carried out to evaluate the efficacy and toxicity of ESHAP (etoposide, methylprednisolone, high-dose cytarabine, and cisplatin). PATIENTS AND METHODS: Twenty-two patients with refractory (5) or relapsing Hodgkin's disease (17) were entered and scheduled to receive three courses of ESHAP. Patients suitable for AHT were then given high-dose chemotherapy with CBV (cyclophosphamide, carmustine, and etoposide) plus AHT, whereas responding, non-AHT-suitable patients completed six ESHAP courses. RESULTS: Nine patients achieved complete responses and seven partial responses (overall response rate 73%) with ESHAP. Grade 3-4 myelotoxicity was seen in 13 patients (59%). Nine patients received CBV plus AHT. At a median follow-up time of 50 months (range 6-96), seven patients (32%) are alive and disease-free. Three patients died of toxic effects of ESHAP (1) or CBV (2). Actuarial overall survival and disease-free survival were 35% and 27% at three years. CONCLUSIONS: ESHAP is an active regimen for relapsing Hodgkin's disease, with myelosuppression as its dose-limiting toxicity. An increased risk of treatment-related mortality when it is combined with high-dose chemotherapy can not be ruled out.

Metastatic follicular thyroid carcinoma to the kidney: a case report.

Clinically detectable well-differentiated metastatic thyroid cancer to the kidney is rare, with only 12 cases reported in the medical literature. The authors report a case of metastatic thyroid carcinoma to the kidney in a patient with widespread dissemination. She underwent total thyroidectomy, radical left nephrectomy, radioactive ablation with I-131, radiotherapy, and thyroid suppression therapy. Well-differentiated thyroid metastatic cancer can be amenable to treatment with successful long-term results.