[Translated article] Vertebral metastases. En bloc treatment.

En bloc resection of vertebral metastases has been the subject of study in medical literature due to its impact on patients' quality of life and effectiveness in local disease control. This bibliographic analysis examines the findings and perspectives of published studies concerning en bloc resection of oligometastases in the spine. The technique, which involves the complete removal of the tumour along with a portion of the surrounding bone, has been shown to improve local tumour control, reduce recurrence, and potentially prolong patient survival compared to conventional decompression and stabilisation techniques. However, en bloc resection also presents risks and complications, such as surgical morbidity and extended recovery time. Appropriate patient selection, preoperative planning, and a multidisciplinary approach are essential to optimise outcomes. As new techniques and advances in adjuvant treatment develop, en bloc resection of oligometastases in the spine remains an area of interest in oncological research.

Vertebral metastases. En bloc treatment. / Metástasis vertebrales. Tratamiento en bloque.

En bloc resection of vertebral metastases has been the subject of study in medical literature due to its impact on patients' quality of life and effectiveness in local disease control. This bibliographic analysis examines the findings and perspectives of published studies concerning en bloc resection of oligometastases in the spine. The technique, which involves the complete removal of the tumour along with a portion of the surrounding bone, has been shown to improve local tumour control, reduce recurrence, and potentially prolong patient survival compared to conventional decompression and stabilization techniques. However, en bloc resection also presents risks and complications, such as surgical morbidity and extended recovery time. Appropriate patient selection, preoperative planning, and a multidisciplinary approach are essential to optimize outcomes. As new techniques and advances in adjuvant treatment develop, en bloc resection of oligometastases in the spine remains an area of interest in oncological research.

Simplified risk-prediction for benchmarking and quality improvement in emergency general surgery. Prospective, multicenter, observational cohort study.

BACKGROUND AND AIMS: Emergency General Surgery (EGS) conditions account for millions of deaths worldwide, yet it is practiced without benchmarking-based quality improvement programs. The aim of this observational, prospective, multicenter, nationwide study was to determine the best benchmark cutoff points in EGS, as a reference to guide improvement measures. METHODS: Over a 6-month period, 38 centers (5% of all public hospitals) attending EGS patients on a 24-h, 7-days a week basis, enrolled consecutive patients requiring an emergent/urgent surgical procedure. Patients were stratified into cohorts of low (i.e., expected morbidity risk <33%), middle and high risk using the novel m-LUCENTUM calculator. RESULTS: A total of 7258 patients were included; age (mean ± SD) was 51.1 ± 21.5 years, 43.2% were female. Benchmark cutoffs in the low-risk cohort (5639 patients, 77.7% of total) were: use of laparoscopy ≥40.9%, length of hospital stays ≤3 days, any complication within 30 days ≤ 17.7%, and 30-day mortality ≤1.1%. The variables with the greatest impact were septicemia on length of hospital stay (21 days; adjusted beta coefficient 16.8; 95% CI: 15.3 to 18.3; P < .001), and respiratory failure on mortality (risk-adjusted population attributable fraction 44.6%, 95% CI 29.6 to 59.6, P < .001). Use of laparoscopy (odds ratio 0.764, 95% CI 0.678 to 0.861; P < .001), and intraoperative blood loss (101-500 mL: odds ratio 2.699, 95% CI 2.152 to 3.380; P < .001; and 500-1000 mL: odds ratio 2.875, 95% CI 1.403 to 5.858; P = .013) were associated with increased morbidity. CONCLUSIONS: This study offers, for the first time, clinically-based benchmark values in EGS and identifies measures for improvement.

Genome-wide profiling of non-smoking-related lung cancer cells reveals common RB1 rearrangements associated with histopathologic transformation in EGFR-mutant tumors.

BACKGROUND: The etiology and the molecular basis of lung adenocarcinomas (LuADs) in nonsmokers are currently unknown. Furthermore, the scarcity of available primary cultures continues to hamper our biological understanding of non-smoking-related lung adenocarcinomas (NSK-LuADs). PATIENTS AND METHODS: We established patient-derived cancer cell (PDC) cultures from metastatic NSK-LuADs, including two pairs of matched EGFR-mutant PDCs before and after resistance to tyrosine kinase inhibitors (TKIs), and then performed whole-exome and RNA sequencing to delineate their genomic architecture. For validation, we analyzed independent cohorts of primary LuADs. RESULTS: In addition to known non-smoker-associated alterations (e.g. RET, ALK, EGFR, and ERBB2), we discovered novel fusions and recurrently mutated genes, including ATF7IP, a regulator of gene expression, that was inactivated in 5% of primary LuAD cases. We also found germline mutations at dominant familiar-cancer genes, highlighting the importance of genetic predisposition in the origin of a subset of NSK-LuADs. Furthermore, there was an over-representation of inactivating alterations at RB1, mostly through complex intragenic rearrangements, in treatment-naive EGFR-mutant LuADs. Three EGFR-mutant and one EGFR-wild-type tumors acquired resistance to EGFR-TKIs and chemotherapy, respectively, and histology on re-biopsies revealed the development of small-cell lung cancer/squamous cell carcinoma (SCLC/LuSCC) transformation. These features were consistent with RB1 inactivation and acquired EGFR-T790M mutation or FGFR3-TACC3 fusion in EGFR-mutant tumors. CONCLUSIONS: We found recurrent alterations in LuADs that deserve further exploration. Our work also demonstrates that a subset of NSK-LuADs arises within cancer-predisposition syndromes. The preferential occurrence of RB1 inactivation, via complex rearrangements, found in EGFR-mutant tumors appears to favor SCLC/LuSCC transformation under growth-inhibition pressures. Thus RB1 inactivation may predict the risk of LuAD transformation to a more aggressive type of lung cancer, and may need to be considered as a part of the clinical management of NSK-LuADs patients.

Nasopharyngeal carcinoma: 30-year experience of a single institution in a non-endemic area.

PURPOSE: Over the past years, radiotherapy techniques have changed significantly. The impact of these changes in the management of nasopharyngeal carcinoma (NPC) has not been fully evaluated. METHODS/PATIENTS: Between 1984 and 2014, 223 NPC were diagnosed in our hospital. Prior to 2000, patients were treated with 2D treatment plan (RT2D) that evolved to 3D schemes thereafter (RT3D). RESULTS: Tumors in the RT3D period showed significantly lower stages than those in the RT2D period. 5-year cause-specific survival improved from 55.7% (95% CI: 46.7-64.7%) in the RT2D period to 78.7% (95% CI: 68.7-88.7%) in the RT3D period (P = 0.006). This difference was greater for non-keratinizing NPC, where specific survival went from 63.2% (95% CI: 52.2-74.2%) to 84.4% (95% CI: 74.4-94.4%) (P = 0.014). CONCLUSION: Recent changes in treatment strategies including concurrent chemoradiation and 3D radiotherapy may have impacted in better survival for NPC. Improved imaging techniques may have contributed by earlier detection and better treatment planning.

Primary pleomorphic liposarcoma of the spine. Case report and review of the literature.

INTRODUCTION: To describe a single case, the fourth ever reported, of pleomorphic liposarcoma of the spine and to undertake a review of the literature. PRESENTATION OF CASE: A 60 year old male patient had a bilateral lumbosciatica over a 3 month period. Imaging tests revealed a tumor mass in L1-L3 and a fracture in L2. Also, he had a mural thrombus both in the inferior vena cava and the left renal vein. The biopsy revealed a well-differentiated liposarcoma. En bloc resection of the lesion and stabilization was carried out. Due to the condition of the patient (hemodynamic instability, wound dehiscence and infection, and hypoproteinemia), a decision was made not to subject the patient to either radiation therapy or chemotherapy. The patient was subsequently found to suffer from myopathic paraparesis and a surgical wound infection. At three months, liver metastases were evident, as well as a recurrence of the lesion. A venous thrombosis that extended from the lower iliac vein to the right atrium was observed. The patient died from type I hepatorenal syndrome. DISCUSSION: Pleomorphic liposarcoma of the spine is a rare occurrence. En bloc resection with wide margins is the treatment of choice. The use of radiotherapy in the spine is controversial. The role that should be played by chemotherapy is still unclear, although it has been employed in treatments. CONCLUSION: In spite of treatment, these tumors lead to a poor prognosis, with high rates of recurrence, metastasis, and mortality.

Impact of a multidisciplinary team training programme on rectal cancer outcomes in Spain.

AIM: The Spanish Rectal Cancer Project was established in 2006, inspired by the Norwegian Rectal Cancer Project. It consisted of an educational project aiming to introduce mesorectal excision surgery to surgeons, pathologists and radiologists. Its effect on local recurrence (LR) was compared with the Norwegian Project. METHOD: An observational cohort study was carried out including all patients (4700) with rectal cancer from a population of 19 329 992 inhabitants operated on in 51 Spanish hospitals between March 2006 and June 2010. Curative resection was defined as a resection with an uninvolved circumferential margin in patients without distant metastases and without intra-operative rectal perforation. The effectiveness of the programme was measured by a central registry with feedback to participating institutions of their own results compared with the national average. The main outcome measures were LR and adverse effects in curative resections. RESULTS: Of the 4700 patients, 3213 had a resection considered to be curative. LR rates were 4.7% (95% CI 0.03-0.59), metastasis rate was 16% (95% CI 0.14-0.17) and overall survival was 87.8% (95% CI 0.86-0.89). Multivariate analysis showed that advanced TNM stage and decreasing distance of the tumour from the anal verge had a negative influence on LR. CONCLUSION: This study shows that the results obtained in Norway have been reproduced in a larger population in Spain applying a similar methodology.

Lymphocytic hypophysitis successfully treated with azathioprine: first case report.

An aggressive case of lymphocytic hypophysitis is described which was successfully treated with azathioprine after failure of corticosteroids. The patient, aged 53, had frontal headache, diplopia, and diabetes insipidus. Cranial magnetic resonance imaging (MRI) showed an intrasellar and suprasellar contrast enhancing mass with involvement of the left cavernous sinus and an enlarged pituitary stalk. A putative diagnosis of lymphocytic hypophysitis was made and prednisone was prescribed. Symptoms improved but recurred after the dose was reduced. Trans-sphenoidal surgery was attempted but the suprasellar portion of the mass could not be pulled through the pituitary fossa. Histological examination confirmed the diagnosis of lymphocytic hypophysitis. Two months later he developed aseptic meningoencephalitis which was treated with high dose methylprednisolone pulse therapy. MRI revealed a progression of suprasellar mass. At this stage azathioprine treatment was begun. Four weeks later MRI shown no evidence of residual lesion and no pituitary stalk enlargement. After follow up of 18 months without azathioprine there was no clinical or radiological evidence of the disease. This is the first evidence of the efficacy of azathioprine treatment in a patient with lymphocytic hypophysitis.

Adult-onset subacute sclerosing panencephalitis: clinico-pathological findings in 2 new cases.

Subacute sclerosing panencephalitis (SSPE), an uncommon disease usually affecting children and adolescents, is caused by persistent measles infection that progresses to chronic infection with fatal outcome. The debut of this disease in adults is rare, with a small number of cases in the medical literature. This article presents the clinical, radiologic and post-mortem neuropathologic findings in 2 new cases of women with SSPE (1 of them during pregnancy), which showed very atypical clinical characteristics, presentation and evolution. The influence of pregnancy on the course of the disease was unfavorable, in keeping with earlier reports. Our patients showed a very prolonged biphasal clinical course, with a period of disease-free remission that lasted several years. Histological study disclosed features of inflammatory disease associated with others of a neurodegenerative nature, such as the formation of neurofibrillary tangles, which would relate SSPE with other tauopathies.

[Posterior spinal artery infarction and nonbacterial thrombotic endocarditis]. / Infarto de la arteria espinal posterior y endocarditis trombótica no bacteriana.

Nonbacterial thrombotic endocarditis (NBTE) has been described as an important complication in patients affected by different kind of illnesses, above all underlying malignancies. Fifty % of the patients affected by NBTE will have embolic events in different organs. The poverty of specific symptomatology appears to difficult seriously its diagnosis. In fact, systemic embolic infarctions are usually asymptomatic, except when central nervous system is involved. We present a patient affected by NBTE with an asymptomatic cervical spinal cord infarction, which diagnosis was made by pathologic study. The difficulty to explore typical sings of spinal lesion and the absence of previous reported cases of NBTE with posterior spinal artery infarction, make this one fairly interesting. We think that fibrinoplatelet embolism from NBTE should be included in the etiology of the posterior spinal artery infarction.

Location of disulfide bonds in mature alpha-L-fucosidase from pea.

Fuc-9 is the mature form of a vacuolar alpha-L-fucosidase enzyme which seems to play an important role in plant growth regulation. Fuc-9 is a 202-residue protein containing five Cys residues located at positions 64, 109, 127, 162 and 169. In this study, the disulfide structure of Fuc-9 was determined by MALDI-TOF mass spectrometry (MS), with minimal clean-up of the samples and at a nanomolar scale. Two strategies, based on a specific chemical cleavage (with 2-nitro-5-thiocyanobenzoic acid and alkaline conditions) at the Cys residues and modification of Cys residues by acrylamide/deuterium labeled acrylamide alkylation, were used. Using these methods, the disulfide pairings Cys64-Cys109 and Cys162-Cys169 could be established. The advantages and limitations of our experimental approach are discussed.

Transient ischaemic attack: a common initial manifestation of cardiac myxomas.

BACKGROUND AND AIMS: Cardiac myxomas may present clinically with many different features. Since highly effective treatments exist, it is important that they are diagnosed quickly in order to avoid further complications. Our aim was to determine the influence of neurological presentation in diagnosis and prognosis of cardiac myxomas. METHODS: We have reviewed the clinical charts of 28 patients diagnosed with cardiac myxomas seen at our centre in the last 20 years. RESULTS: Mean age at diagnosis in patients with neurological events was 49.22 years and 60.84 years in those without neurological manifestations (p = 0.0325). Most frequent presentations were: cardiac manifestations (92.8%), general manifestations (71.4%) and embolic events (39.3%). Nine patients (32.1%) presented with cerebral embolism; 7 of whom presented with transient ischaemic attacks (TIA), which was the first manifestation in 6 of them; 3 of them later suffered complete cerebral infarction with sequelae. Echocardiography confirmed diagnosis in 26 out of 27 patients in which it was performed. None of the patients presented neurological symptoms after surgery. CONCLUSION: The most frequent initial neurological manifestation in our series was TIA. Nevertheless, none of the patients were diagnosed after the first neurological symptom. Although the contribution of cardiac myxomas to the total amount of TIA is low, since surgery is highly effective and of low risk, and patients with neurological manifestations are younger, it is vital to consider the possibility of cardiac myxoma after a TIA of unknown origin.

Mitral papillary fibroelastoma as a cause of cardiogenic embolic stroke: report of two cases and review of the literature.

UNLABELLED: Papillary fibroelastoma (PFE) is a rare benign tumour that attaches to the endocardial surface, mostly on cardiac valves. Though usually asymptomatic, it can be the source of several complications. To date, 49 cases have been reported of embolic stroke with a PFE as the probable origin. CASE REPORTS: (i) a 39-year-old male presented with ischemic embolic stroke; the presence of a PFE was assessed by means of transoesophageal echocardiography and confirmed by pathological findings; (ii) a 32-year-old woman presented with sudden onset of left hemiparesis; a cardiogenic embolic stroke was suspected, and a diagnosis of PFE was made based on echocardiographic and pathological findings. In both cases, surgical excision of the tumours was performed with no recurrences at follow-up. Two mechanisms can explain the formation of emboli in PFE: dislodgement of the tumour leaves or fibrin-platelet aggregation on the endocardial surface of these leaves. Transthoracic echocardiography may lead to the suspicion of a PFE, but transoesophageal echocardiography is required for confirmation. Prompt surgical excision is indicated in most cases. Anticoagulation is only recommended in situations of high surgical risk and during the wait for surgery.

Encephalopathy associated to autoimmune thyroid disease: a more appropriate term for an underestimated condition?

Hashimoto's encephalopathy is a severe and rather infrequent clinical condition initially described in patients suffering from chronic lymphocytic thyroiditis. Its origin is still controversial but it can be agreed to have an autoimmune etiology. In fact, its most characteristic finding is the high titre of antithyroid antibodies, especially antimicrosomal. We describe three cases of Hashimoto's encephalopathy and establish a relationship between the clinical status, the antithyroid antibody levels and its response to corticosteroid treatment. There was an excellent response to corticosteroid treatment in all three cases. Interestingly, one case was associated with Graves' disease. Given this, and after the review of the literature, we believe that the term 'encephalopathy associated to autoimmune thyroid disease' could be more appropriate to define this entity. Finally, we suggest that autoimmune thyroid encephalopathy must be suspected in the face of unaccounted acute or subacute encephalopathy with high levels of antithyroid antibodies.

[Calcified cerebral metastases. Study of two cases and review of literature]. / Metástasis cerebrales calcificadas. Estudio de dos casos y revisión de la bibliografía.

Although the most frequent cause of intracranial neoplasms are metastases, they very rarely calcify; classic histopathological series have shown the incidence to be approximately 1%. We have found 54 reported cases of intracranial calcified metastasis, the most frequent primary tumour reported being pulmonary adenocarcinoma (13 cases) and adenocarcinoma of gastrointestinal tract and adenocarcinoma of breast (8 cases each). Some reports have shown a longer survival in patients with this kind of image on cerebral scans. We present two patients with intracranial calcified metastasis due to dissemination in both cases of pulmonary adenocarcinomas. The survival under treatment in both patients was similar to that of patients with no calcified metastasis on brain scans. So, facing calcified intracranial lesions, metastatic origin should be considered in the differential diagnosis, pulmonary tumours being the first to be ruled out.

[Abdominal compartmental syndrome in non-injured patients]. / Síndrome compartimental abdominal en pacientes no traumáticos.

Abdominal compartment syndrome (ACS) is characterized by increased intraabdominal pressure and a set of secondary pathophysiological changes in the abdominal. ACS has reappeared in the literature recently in relation to the surgical concept to damage control, applied particularity in contexts of severe abdominal injury polytraumatized patients. We report two cases of ACS that appeared after scheduled abdominal surgery: one after repair of a large eventration and the other in the context of septic shock due to fecaloid peritonitis. Both patients died of multisystem organ failure in spite of surgical decompression. We wish to emphasize that ACS can appear in contexts other than surgery for damage control, and we stress the need to measure intravesical pressure as a reflection of intraabdominal pressure, particularly in certain high risk patients in the postoperative recovery ward. Finally, we review the pathophysiology of ACS and its management, which is based on early treatment to prevent multisystem organ failure with an associated high risk of death.

[Hypertrophic pachymeningitis. A review of the histories of two cases and pathological relationship with the Tolosa-Hunt syndrome and the orbital pseudotumor]. / Paquimeningitis hipertrófica. Revisión histórica a propósito de dos casos y relación patogénica con el síndrome Tolosa-Hunt y el pseudotumor orbitario.

INTRODUCTION: Hypertrophic pachymeningitis is an infrequent condition which starts with a thickening of the dura mater and whose pathogenesis is unknown. We present two new cases of unknown aetiology. CLINICAL CASE: Case 1. A 53 year old man complained of occipital headache, tinnitus and deafness since February 1981. In October 1981 he was admitted to hospital with a worse headache, perio-orbital pain, dysgeusia and ipsilateral peripheral facial palsy. In December he had generalized tonic-clonic seizures and paralysis of the VII and XI right cranial nerves and IX, X and XII left cranial nerves. In February 1982 he developed right trigeminal neuralgia. He was readmitted in November 1983 with continuous headache, vomiting and a behavior disorder. On CT there was marked attenuation of the posterior dura mater, which the neurosurgical department considered unsuitable for biopsy. He died in March 1985. On necropsy there was hypertrophic pachymeningitis. Case 2. A 62 year old patient consulted in November 1995 complaining of right hypoacusia for the past six months, progressively accompanied by ipsilateral paralysis of the II, IV, VI, VII and VIII cranial nerves but with no other alterations on physical examination. Analytical and serological investigations were normal. Cranial MR showed an extraparenchymatous infiltrating lesion in the middle cranial fossa. Biopsy was decided on when no clinical improvement was seen with corticosteroid treatment. The pathologist reported hypertrophic pachymeningitis. Treatment was started with cyclophosphamide in monthly doses and the condition has remained stable to date. CONCLUSION: With these two cases we wish to establish a pathogenic relation between the Tolosa-Hunt syndrome and orbital pseudotumor and show the role played by immunosuppressive treatment in the control of hypertrophic pachymeningitis.