Seudotumor inflamatorio y tumor miofibroblástico inflamatorio. Criterios diagnósticos y diferencias pronósticas / Inflammatory pseudotumor and myofibroblastic inflammatory tumor. Diagnostic criteria and prognostic differences

Introducción El seudotumor inflamatorio (PTI) y el tumor miofibroblástico inflamatorio (TMI) son 2 entidades muy poco frecuentes que se incluían antiguamente en la misma categoría; sin embargo, en la actualidad se consideran 2 enfermedades diferentes debido al origen neoplásico del TMI. Nuestro objetivo es compartir nuestra experiencia en el manejo de estos 2 tipos de lesiones que debemos tener en cuenta en el diagnóstico diferencial de masas o nódulos pulmonares. Métodos Fueron estudiados retrospectivamente 13 pacientes con diagnóstico anatomopatológico de PTI o TMI, intervenidos entre los años 2008 y 2019. Registramos la información pre y postoperatoria de cada uno, así como el análisis de supervivencia. Resultados De los 13 pacientes, 8 eran varones y 5 mujeres. La media de edad de presentación fue de 53,5 años. En 6 pacientes se practicó una segmentectomía atípica, en 6 fue necesario realizar una lobectomía y en un caso una neumonectomía. En todos los casos se consiguió una resección completa. El diagnóstico fue posible gracias a la histología, técnicas de inmunohistoquímica (IHQ) y de hibridación fluorescente in situ (FISH) determinando la expresión de IgG4 y el reordenamiento de ALK, respectivamente. Tras una mediana de seguimiento de 49 meses no se observaron datos de recidiva locorregional ni a distancia en los pacientes estudiados. Conclusión El PTI y el TMI son tumores poco frecuentes con muy buen pronóstico. El diagnóstico de ambas entidades se basa principalmente en técnicas anatomopatológicas específicas. La cirugía tiene, en la mayor parte de las ocasiones, un papel tanto diagnóstico como terapéutico (AU)

Introduction Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) are two very rare entities that were formerly included in the same category; however, today they are considered two different diseases due to the neoplastic origin of the IMT. Our objective is to share our experience in the management of these two types of tumors that we must take into account in the differential diagnosis of pulmonary masses or nodules. Methods Thirteen patients with a pathological diagnosis of IPT and IMT who underwent surgery between 2008 and 2019 were retrospectively studied. We recorded the pre and postoperative information of each one, as well as the survival analysis. Results Of the 13 patients, 8 were men and 5 women. The mean age of presentation was 53,5 years. An atypical segmentectomy was performed in 6 patients; a lobectomy was necessary in 6 and a pneumonectomy in 1 case. In all cases a complete resection was achieved. Diagnosis was possible thanks to histology, immunohistochemical (IHQ) and fluorescent in situ hybridization (FISH) techniques determining the expression of IgG4 and the rearrangement of ALK, respectively. After a median follow up of 49 months, we didńt find any loco-regional or distant recurrence in the patients studied. Conclusion IPT and IMT are rare tumors with a very good prognostic. The diagnosis of both entities is based mainly on specific anatomopathological techniques. Surgery has, in most cases, both a diagnostic and therapeutic role (AU)

Amiodarone-induced Ataxia: A Case Report of Severe Cerebellar Dysfunction and Review of Literature.

Amiodarone therapy is widely prescribed in patients with atrial fibrillation. The higher prevalence of this arrhythmic heart disease, and the specific age-related issues of homeostasis in the elderly population, makes this group particularly exposed to its adverse effects. Among the many described side-effects, neurological impairments are the less documented and studied. Because amiodarone can be responsible for severe complications, as described in the case below, a close monitoring is necessary throughout its prescription. Awareness should be brought on the amiodarone-induced neurological side-effects as they could be overlooked.

Atypical carcinoid tumours of the lung: prognostic factors and patterns of recurrence.

BACKGROUND: Atypical carcinoids (AC) of the lung are rare intermediate-grade neuroendocrine neoplasms. Prognostic factors for these tumours are undefined. METHODS: Our cooperative group retrieved data on 127 patients operated between 1980 and 2009 because of an AC. Several clinical and pathological features were studied. RESULTS: In a univariable analysis, T-status (p=0.005), N-status (p=0.021), preoperative M-status (previously treated) (p=0.04), and distant recurrence developed during the outcome (p<0.001) presented statistically significant differences related to survival of these patients. In a multivariable analysis, only distant recurrence was demonstrated to be an independent risk factor for survival (p<0.001; HR: 13.1). During the monitoring, 25.2% of the patients presented some kind of recurrence. When we studied recurrence factors in a univariable manner, sublobar resections presented significant relationship with locoregional recurrence (p<0.001). In the case of distant recurrence, T and N status presented significant differences. Patients with preoperative M1 status presented higher frequencies of locoregional and distant recurrence (p=0.004 and p<0.001, respectively). In a multivariable analysis, sublobar resection was an independent prognostic factor to predict locoregional recurrence (p=0.002; HR: 18.1). CONCLUSIONS: Complete standard surgical resection with radical lymphadenectomy is essential for AC. Sublobar resections are related to locoregional recurrence, so they should be avoided except for carefully selected patients. Nodal status is an important prognostic factor to predict survival and recurrence. Distant recurrence is related to poor outcome.

Síndrome del seno silente. Caso clínico / Silent sinus syndrome. Clinical case

CASO CLÍNICO: Varón de 53 años que acude a la consulta por enoftalmos en el ojo derecho progresivo sin sintomatología asociada. Ausencia de antecedente traumático. En la exploración se observa un enoftalmos y leve desplazamiento inferior del globo derecho. En la TC se aprecia una ocupación total del antro maxilar derecho y del seno frontal, así como una desviación del tabique nasal hacia la derecha, lo que confirma el diagnóstico de síndrome del seno silente. DISCUSIÓN: Se comentan las características más importantes del síndrome del seno silente, una enfermedad poco conocida que no debe confundirse con la sinusitis maxilar

CLINICAL CASE: A 53 year-old man presented with a progressive enophthalmos without any sinus or nasal symptoms. There was no history of a trauma. The ophthalmology examination showed enophtalmos and hypoglobus. The computerized tomography (CT) showed a collapsed maxillary and frontal sinus and a lateral deviation of the nasal septum that led us to the diagnosis. DISCUSSION: The clinical features of silent sinus syndrome are described, as well as the need to distinguish it from maxillary sinusitis

[Silent sinus syndrome. Clinical case]. / Síndrome del seno silente. Caso clínico.

CLINICAL CASE: A 53 year-old man presented with a progressive enophthalmos without any sinus or nasal symptoms. There was no history of a trauma. The ophthalmology examination showed enophtalmos and hypoglobus. The computerized tomography (CT) showed a collapsed maxillary and frontal sinus and a lateral deviation of the nasal septum that led us to the diagnosis. DISCUSSION: The clinical features of silent sinus syndrome are described, as well as the need to distinguish it from maxillary sinusitis.

Aortic thrombus and multiple embolisms during a Mycoplasma pneumoniae infection.

We report the case of a patient who presented with a thrombus of the aortic arch complicated with splenic, renal and peroneal artery embolisms, associated with transient lupus anticoagulant, during a Mycoplasma pneumoniae infection. The outcome was good under antibiotic and anticoagulant treatment. We also review the medical literature on M. pneumoniae-related thromboses.

Surgical lung biopsy for diffuse lung disease. Our experience in the last 15 years.

INTRODUCTION: Surgical lung biopsy is a technique that presents a morbi-mortality rate of considerable importance. We analyze our experience with surgical lung biopsies for the diagnosis of diffuse lung disease and the effect produced on the indications for surgical biopsy in these pathologies after the publication of the consensus of the ATS (American Thoracic Society) and ERS (European Respiratory Society) for Idiopathic Pulmonary Fibrosis (IPF). PATIENTS AND METHODS: We performed a retrospective review of 171 patients operated between January 1997 and December 2011. We divided the series into 2 groups: group 1 (operated between 1997 and 2002) and group 2 (operated between 2003 and 2011). Suspected preoperative diagnosis, respiratory status, pathological postoperative diagnoses, percentage of thoracotomies, mean postoperative stay and perioperative morbidity and mortality were analyzed. RESULTS: Group 1 consisted of 99 patients and group two 72. The most frequent postoperative diagnoses were: usual interstitial pneumonia and extrinsic allergic alveolitis. There were ten (5.84%) deaths. Death was caused by progressive respiratory failure that was related to interstitial lung disease in 7 (70%) of 10 cases, alveolar haemorrhage in 2 (20%) and heart failure in 1 (10%). CONCLUSIONS: Since the publication of the ATS and ERS consensus on the IPF, we have observed a noticeable decrease in the number of indications for surgical lung biopsy. This technique, though simple, has a considerable morbidity and mortality.

Cross-industry standard process for data mining is applicable to the lung cancer surgery domain, improving decision making as well as knowledge and quality management

OBJECTIVES: The aim of this study was to assess the applicability of knowledge discovery in database methodology, based upon data mining techniques, to the investigation of lung cancer surgery. METHODS: According to CRISP 1.0 methodology, a data mining (DM) project was developed on a data warehouse containing records for 501 patients operated on for lung cancer with curative intention. The modelling technique was logistic regression. RESULTS: The finally selected model presented the following values: sensitivity 9.68%, specificity 100%, global precision 94.02%, positive predictive value 100% and negative predictive value 93.98% for a cut-off point set at 0.5. A receiver operating characteristic (ROC) curve was constructed. The area under the curve (CI 95%) was 0.817 (0.740- 0.893) (p < 0.05). Statistical association with perioperative mortality was found for the following variables [odds ratio (CI 95%)]: age over 70 [2.3822 (1.0338-5.4891)], heart disease [2.4875 (1.0089-6.1334)], peripheral arterial disease [5.7705 (1.9296-17.2570)], pneumonectomy [3.6199 (1.4939-8.7715)] and length of surgery (min) [1.0067 (1.0008-1.0126)]. CONCLUSIONS: The CRISP-DM process model is very suitable for lung cancer surgery analysis, improving decision making as well as knowledge and quality management (AU)

Pulmonar collision tumor: metastatic adenoid cystic carcinoma and lung adenocarcinoma.

We report an extraordinary case of collision tumor consisting of a lung adenocarcinoma and a metastatic adenoid cystic carcinoma in a 56 year-old man. He was diagnosed with a pulmonary nodule 11 years after treatment of an adenoid cystic carcinoma of the right maxillary sinus. A non-small cell carcinoma was observed when a transbronchial biopsy was performed. The other component of the nodule was only diagnosed with pathological examination of the resection specimen.

[Propylthiouracil-induced multispecific antineutrophil cytoplasmic antibodies]. / Anticorps anticytoplasme des polynucléaires neutrophiles multispécifiques induits par le propylthiouracile.

We report the case of a 28-year-old woman who presented with arthralgia during a treatment with propylthiouracil (PTU) for hyperthyroidism. Detection of antineutrophil cytoplasmic antibodies (ANCA) against myeloperoxidase, proteinase 3 and elastase led to suspect PTU induced vasculitis.

[Value of assaying adenosine deaminase level in patients with neuromeningeal tuberculosis]. / Intérêt du dosage de l'adénosine désaminase dans les infections tuberculeuses neuro-méningées.

Neuromeningeal tuberculosis is a rare extrapulmonary location in France. Delayed diagnosis can lead to therapeutic failure and severe sequels. However early diagnosis is a major challenge that requires the proper epidemiological, clinical, radiological and biological resources. Problems related to diagnosis of mycobacteria infection and to shortcomings in certain healthcare systems can hinder early diagnosis. The purpose of this review was to describe the diagnostic value of assaying adenosine deaminase activity in cerebrospinal fluid from patients with neuromeningeal tuberculosis. Evidence from studies published over the last 25 years indicate that the sensitivity and specificity of measuring adenosine deaminase activity range from 36 to 92% and 71 to 100% respectively depending of cutoff values used. Before performing this assay, it is necessary to rule out obvious or frequent etiologies such as purulent bacterial meningitis or cryptococcosis in HIV patients. Taken together these studies show that this simple, inexpensive technique is a valuable tool for early diagnosis and management of tuberculosis patients and that it can be easily implemented in hospital labs regardless of technical or financial resources.

NEMO specifically recognizes K63-linked poly-ubiquitin chains through a new bipartite ubiquitin-binding domain.

An important property of NEMO, the core element of the IKK complex involved in NF-kappaB activation, resides in its ability to specifically recognize poly-ubiquitin chains. A small domain called NOA/UBAN has been suggested to be responsible for this property. We recently demonstrated that the C-terminal Zinc Finger (ZF) of NEMO is also able to bind ubiquitin. We show here by ZF swapping and mutagenesis that this represents its only function. While neither NOA nor ZF shows any preference for K63-linked chains, we demonstrate that together they form a bipartite high-affinity K63-specific ubiquitin-binding domain. A similar domain can be found in two other proteins, Optineurin and ABIN2, and can be freely exchanged with that of NEMO without interfering with its activity. This suggests that the main function of the C-terminal half of NEMO is to specifically bind K63-linked poly-ubiquitin chains. We also demonstrate that the recently described binding of NEMO to linear poly-ubiquitin chains is dependent on the NOA alone and does not require the presence of the ZF.

Bronchogenic carcinoma in patients under 50 years old.

INTRODUCTION: Lung cancer in young patients is increasing in frequency. Its clinical course seems to be more aggressive than in the elderly. Our objective is to assess the clinicopathologic characteristics and survival of patients with bronchogenic carcinoma who underwent surgery at our department, comparing people younger than 50 years to older patients. MATERIALS AND METHODS: We present a retrospective study of 610 patients diagnosed with non-small-cell lung cancer operated on between 1997 and 2006. They were classified into two groups: under 50 (n=60) and equal to or over 50 (n=550). RESULTS: The proportion of women, smokers and adenocarcinoma were significantly higher in young patients. There were no significant differences in survival rate between the two groups. CONCLUSIONS: In our series, despite the differences in sex, smoking history and histology, the behaviour of the disease is similar in both age groups.

Bronchogenic carcinoma in patients under 50 years old

INTRODUCTION: Lung cancer in young patients is increasing in frequency. Its clinical course seems to be more aggressive than in the elderly. Our objective is to assess the clinicopathologic characteristics and survival of patients with bronchogenic carcinoma who underwent surgery at our department, comparing people younger than 50 years to older patients. MATERIALS AND METHODS: We present a retrospective study of 610 patients diagnosed with non-small-cell lung cancer operated on between 1997 and 2006. They were classified into two groups: under 50 (n=60) and equal to or over 50 (n=550). RESULTS: The proportion of women, smokers and adenocarcinoma were significantly higher in young patients. There were no significant differences in survival rate between the two groups. CONCLUSIONS: In our series, despite the differences in sex, smoking history and histology, the behaviour of the disease is similar in both age groups (AU)

[Role of pharmacogenetics in chemotherapy of colorectal cancers]. / Implication de la pharmacogénétique dans les traitements des cancers colorectaux.

SCOPE: Clinical implications associated with polymorphisms in drug-metabolizing genes involved in the chemotherapy of colorectal cancers (5-flurorouracil, oxaliplatin and irinotecan) are reviewed. CURRENT SITUATION AND SALIENT POINTS: Treatments of colorectal cancers have been greatly improved last years but patients respond differently to identical medication. Genetic polymorphisms are one of the major causes of these individual responses to drugs associated with sometimes severe adverse effects. Pharmacogenetics is based on all polymorphisms that determine genetic human diversity associated with variable response to anticancer drugs. PERSPECTIVES: Morbidity and mortality related to toxicity or inefficacy of these drugs could be reduced by analyzing the pharmacogenetic profile of patients before treatment. Results should be integrated in protocols for monitoring and assessment the dosage of drugs.

[Pericardial effusion as the only first clinical manifestation of systemic lupus erythematosus]. / Derrame pericárdico como manifestación clínica única de lupus eritematoso sistémico.

Pericarditis is the most frequent cardiac manifestation of systemic lupus erythematosus (SLE), but pericardial effusion rarely occurs as the only first clinical manifestation of SLE. We report the case of a male patient whose initial manifestation of lupus was a pericardial effusion. We comment on its clinical course and we reviewed in the medical literature different presenting features in SLE.

Derrame pericárdico como manifestación clínica única de lupus eritematoso sistémico / Pericardial effusion as the only first clinical manifestation of systemic lupus erythematosus

La afectación del pericardio es la manifestación cardíaca más frecuente del lupus eritematoso sistémico (LES). Sin embargo, es infrecuente que el derrame pericárdico sea la forma de presentación del LES. Describimos el caso de un paciente varón con LES que debutó clínicamente con derrame pericárdico, comentamos su curso clínico y revisamos en la bibliografía médica distintas formas de presentación del LES

Pericarditis is the most frequent cardiac manifestation of systemic lupus erythematosus (SLE), but pericardial effusion rarely occurs as the only first clinical manifestation of SLE. We report the case of a male patient whose initial manifestation of lupus was a pericardial effusion. We comment on its clinical course and we reviewed in the medical literature different presenting features in SLE

[Severe hypofibrinegenemia after rattlesnake envenomation in France]. / Hypofibrinogénémie sévère après morsure de crotale en France.

A 22-year-old man, who maintains illegally numerous exotic snakes at home (suburbs of Paris), was bitten by one of his Bresilian rattlesnakes, the lance-headed viper Bothrops moojeni, with grade III envenomation. The fibrinogen was less than 0,5 g/L, the prothombin time was 22%, the activated partial thromboplastin time was 94 seconds. The authors discuss the biological and clinical management of this defibrination, due to defibrinogenating proteases (thrombin-like enzymes), present in Bothrops moojeni venom. The patient received 7 vials of an antivenom directed to another crotal, Bothrops lanceolatus. Despite the importance of defibrinogenation, there was only a few clinical evidence of bleeding, according to the literature. The normalization of coagulation studies occured only after day 11. This case-report outlines the danger of the increase of exotic snakes maintained as pet in France and the difficulties to obtain specific antivenoms.