Daño medular intraoperatorio en cirugía de escoliosis idiopática: reinstrumentar y cuándo / Spinal cord injury as a complication of idiopathic scoliosis surgery: when to plan a new surgical procedure

La lesión medular es una complicación temida y devastadora del tratamiento quirúrgico de la escoliosis idiopática. Su prevención requiere una técnica quirúrgica y anestésica depurada y potenciales evocados somatosensoriales (PESS) y motores (PEM) intraoperatorios para poder instaurar rápidamente medidas neuroprotectoras, entre ellas la desinstrumentación vertebral. Es esperable que la escoliosis continúe progresando posteriormente. Presentamos el caso de una niña de 12 años, con una escoliosis juvenil grave y progresiva, que en la cirugía correctora sufre una lesión medular espinal. A pesar de la buena evolución neurológica, la deformidad vertebral sigue progresando y se replantea la reinstrumentación vertebral, con su inherente riesgo de relesión medular y secuelas finales más graves. En conclusión, el manejo de las escoliosis progresivas graves que coexisten con daño medular debe individualizarse. La gravedad de las secuelas neurológicas parece aumentar la progresión de la deformidad ortopédica y hacer más incierta la respuesta a cualquier tratamiento posterior (AU)

Spinal cord injury (SCI) is a dreaded and devastating complication of the surgical treatment of idiopathic scoliosis. Its prevention requires meticulous surgical and anaesthetic procedures and combined monitoring of somatosensory (SEPs) and motor (PEM) evoked potentials to quickly establish neuroprotective measures such as pedicle screw removal. It is expected that the scoliosis keeps progressing afterwards. We report the case of a 12-year-old girl who had a spinal cord injury during scoliosis corrective surgery. Despite the good neurological outcome, the spinal deformity continues to progress and a new surgical procedure might be considered, with its inherent risk of a second SCI and more serious neural damage. In conclusion, the management of progressive scoliosis that coexists with an SCI must be individualized. The severity of neurologic sequelae seems to worsen the progression of spinal deformity and make the response to any subsequent treatment more uncertain (AU)

Distinct ERG rearrangement prevalence in prostate cancer: higher frequency in young age and in low PSA prostate cancer.

BACKGROUND: The TMPRSS2-ERG gene fusion resulting in ERG overexpression has been found in around 50% of prostate cancers (PCa) and is a very early event in tumorigenesis. Most studies have reported on selected surgical cohorts with inconsistent results. We hypothesized that ERG gene rearrangements impact tumor development and investigated the frequency of ERG overexpression in the context of clinicopathological tumor characteristics. METHODS: ERG overexpression (ERG+ or ERG-) was determined by immunohistochemistry (IHC) in 1039 radical prostatectomy (RP) tumors and association with PSA, D'Amico risk score, histopathology, biochemical recurrence, body mass index and age of PCa cases was analyzed. RESULTS: ERG+ was associated with younger age at diagnosis (P<0.0001), lower serum PSA (P=0.002) and lower prostate volume (PV) (P=0.001). It was most frequent in the youngest age quartile (≤55 years, 63.9% ERG+) and decreased constantly with increasing age to 40.8% in the oldest age quartile (≥67 years, P<0.0001). In the PSA range <4 ng ml(-1) the frequency of ERG positivity was 60.2% compared with 47.5 and 49.1% in the PSA ranges 4-10 and ≥10 ng ml(-1), respectively. In the first age quartile, ERG+ patients had lower median serum PSA and fPSA% and smaller PV. In the highest age quartile tumor volume (TV) was increased. Similar differences were observed in the low PSA range. Multivariate analysis identified the first age quartile as a predictor for ERG status (odds ratios (OR) 2.05, P=0.007). No association was found with the D'Amico progression risk score and with biochemical tumor recurrence. CONCLUSIONS: ERG+ tumors manifest clinically at lower PSA levels and their prevalence is age dependent. This suggests acceleration of tumor development by ERG overexpression that results in earlier tumor detection in young patients. Long-term results are warranted to determine the impact of ERG overexpression on disease outcome.

Nódulos eritematovioláceos y telangiectasias en muslos / Erythematous violaceous nodules and telangiectasis on the thighs

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Morphological features of TMPRSS2-ERG gene fusion prostate cancer.

The TMPRSS2-ETS fusion prostate cancers comprise 50-70% of the prostate-specific antigen (PSA)-screened hospital-based prostate cancers examined to date, making it perhaps the most common genetic rearrangement in human cancer. The most common variant involves androgen-regulated TMPRSS2 and ERG, both located on chromosome 21. Emerging data from our group and others suggests that TMPRSS2-ERG fusion prostate cancer is associated with higher tumour stage and prostate cancer-specific death. The goal of this study was to determine if this common somatic alteration is associated with a morphological phenotype. We assessed 253 prostate cancer cases for TMPRSS2-ERG fusion status using an ERG break-apart FISH assay. Blinded to gene fusion status, two reviewers assessed each tumour for presence or absence of eight morphological features. Statistical analysis was performed to look for significant associations between morphological features and TMPRSS2-ERG fusion status. Five morphological features were associated with TMPRSS2-ERG fusion prostate cancer: blue-tinged mucin, cribriform growth pattern, macronucleoli, intraductal tumour spread, and signet-ring cell features, all with p-values < 0.05. Only 24% (n=30/125) of tumours without any of these features displayed the TMPRSS2-ERG fusion. By comparison, 55% (n=38/69) of cases with one feature (RR=3.88), 86% (n=38/44) of cases with two features (RR=20.06), and 93% (n=14/15) of cases with three or more features (RR=44.33) were fusion positive (p<0.001). To our knowledge, this is the first study that demonstrates a significant link between a molecular alteration in prostate cancer and distinct phenotypic features. The strength of these findings is similar to microsatellite unstable colon cancer and breast cancer involving BRCA1 and BRCA2 mutations. The biological effect of TMPRSS2-ERG overexpression may drive pathways that favour these common morphological features that pathologists observe daily. These features may also be helpful in diagnosing TMPRSS2-ERG fusion prostate cancer, which may have both prognostic and therapeutic implications.

TMPRSS2:ERG gene fusion associated with lethal prostate cancer in a watchful waiting cohort.

The identification of the TMPRSS2:ERG fusion in prostate cancer suggests that distinct molecular subtypes may define risk for disease progression. In surgical series, TMPRSS2:ERG fusion was identified in 50% of the tumors. Here, we report on a population-based cohort of men with localized prostate cancers followed by expectant (watchful waiting) therapy with 15% (17/111) TMPRSS2:ERG fusion. We identified a statistically significant association between TMPRSS2:ERG fusion and prostate cancer specific death (cumulative incidence ratio=2.7, P<0.01, 95% confidence interval=1.3-5.8). Quantitative reverse-transcription-polymerase chain reaction demonstrated high ets-related [corrected] gene (ERG) expression to be associated with TMPRSS2:ERG fusion (P<0.005). These data suggest that TMPRSS2:ERG fusion prostate cancers may have a more aggressive phenotype, possibly mediated through increased ERG expression.

Dos enfermedades relacionadas con el VHH-8 en un paciente VIH-negativo: sarcoma de Kaposi y enfermedad de Castleman multicéntrica. Respuesta a tratamiento con rituximab y CHOP / Two HHV8-related illnesses in a HIV-negative patient: Kaposi’s sarcoma and multicentric castleman’s disease. Response to treatment with Rituximab and CHOP

El virus herpes humano tipo 8 (VHH-8) se descubrió en 1994 a partir de la biopsia de un sarcoma de Kaposi en un paciente con sida. Desde entonces se ha identificado en todas las variantes de sarcoma de Kaposi y en otros dos raros procesos: la enfermedad de Castleman multicéntrica y el linfoma primario de cavidades. Se presenta el caso de un paciente varón de 68 años de edad VIH-negativo con sarcoma de Kaposi de un año de evolución en seguimiento por Dermatología que consultó por astenia, anorexia y fiebre. A la exploración se detectaron múltiples adenopatías laterocervicales, axilares e inguinales. En la biopsia de una de dichas adenopatías se apreciaron hallazgos característicos de la variante de células plasmáticas de la enfermedad de Castleman. Se realizaron serologías para el VHH-8 y el VIH que resultaron positiva y negativa respectivamente (IgG anti-VHH-8 positivo, título 1/640, inmunofluorescencia indirecta). Mediante reacción en cadena de la polimerasa se amplificó VHH-8 en sangre periférica. El paciente recibió tratamiento con 8 ciclos de CHOP y rituximab con una completa resolución de las adenopatías y la sintomatología general sin observarse empeoramiento de su sarcoma de Kaposi. El paciente permanece en remisión completa 10 meses después de finalizado el tratamiento. Se presenta el caso de un paciente VIH-negativo y VHH-8-positivo, diagnosticado de sarcoma de Kaposi clásico que desarrolló una enfermedad de Castleman multicéntrica variante de células plasmáticas. La coincidencia de dos o más enfermedades relacionadas con el VHH-8 en un paciente VIH-negativo es un hecho descrito raramente en la literatura. El tratamiento con rituximab combinado con quimioterapia tipo CHOP ha demostrado ser eficaz en este caso sin que haya podido observarse un agravamiento de su SK

Human herpes virus 8 (HHV8) was discovered in 1994 in the biopsy of a Kaposi's sarcoma in a patient with AIDS. Since then it has been identified in all variants of Kaposi's sarcoma and in another two rare disorders: multicentric Castleman's disease and primary body-cavity based lymphomas. The case discusses a 68 year old, HIV-negative male patient, presenting Kaposi's sarcoma for one year and being monitored by dermatology, who presented for weakness, anorexia and fever. On examination, he was found to have adenitis of the lymph nodes in his neck, underarm and groin. A biopsy on one of the swellings led to findings characteristic of multicentric plasma cell variant Castleman's disease. Blood tests for HHV8 and HIV were carried out, resulting positive and negative respectively (IgG anti-HHV8 positive, title 1/640, indirect immunofluorescence). PCR amplification showed HHV8 in peripheral blood. Patient received 8 cycles of CHOP and rituximab, leading to complete disappearance of the adenitis and general symptoms, with no worsening of his Kaposi's sarcoma. Patient remained in complete remission for 10 months after treatment. This paper discusses the case of a HIV­, HHV8+ patient, diagnosed with classic Kaposi's sarcoma, who developed multicentric plasma cell variant Castleman's disease. The coincidence of two or more HHV8-related illnesses in a HIV-negative patient has rarely been described in medical literature. Treatment with rituximab combined with CHOP chemotherapy was effective in this case, and no worsening of the patient's KS was observed