[Postoperative course in 7 cases of primary sarcoma of the lung]. / Evolución posquirúrgica de 7 sarcomas pulmonares primitivos.

Unlike lung metastases of extrapulmonary sarcomas, primary sarcoma of the lung is very rare. We analyzed 7 primary sarcomas treated surgically in Hospital Universitario La Paz, Madrid, Spain between 1985 and 2001. Preoperative histologic diagnosis was correct for 2 patients. Surgery was performed in all 7 patients. Resection was extended to the left atrium in 1 patient, to the chest wall in another, and the parietal pleura were removed from 2 others. Surgical resection was considered complete in 6 cases. Histology revealed 4 cases of malignant fibrous histiocytoma, 1 angiosarcoma, 1 osteogenic sarcoma, and 1 undifferentiated sarcoma. Enlarged lymph nodes removed during surgery were tumor free. Three patients received complementary treatment. The 3 longest-surviving patients were treated with surgery alone; at the end of the study, these patients remained alive 16, 9, and 4 years after surgery. One patient has a recurrent lymph node tumor in a single lung. Three patients died within 1 year and another died 24 months after surgery. In conclusion, surgical treatment of primary sarcoma of the lung can achieve good survival.

Evolución posquirúrgica de 7 sarcomas pulmonares primitivos / Postoperative course in 7 cases of primary sarcoma of the lung

Los sarcomas primitivos pulmonares son muy raros, pero no los metastásicos. Se analizaron 7 sarcomas primarios operados en nuestro servicio entre 1985 y 2001. El diagnóstico histológico preoperatorio fue correcto en 2 pacientes. Se realizó cirugía en todos. En uno se amplió la resección a aurícula izquierda, en otro a pared torácica y en otros 2 se extirpó pleura parietal. Se consideró cirugía completa en 6 casos. En el estudio histológico 4 fueron fibrohistiocitomas malignos, uno angiosarcoma, uno sarcoma osteogénico y uno sarcoma indiferenciado. Las adenopatías extirpadas estaban libres de tumor. Se hizo tratamiento complementario en 3 pacientes. En los 3 de mayor supervivencia se realizó sólo cirugía, con un seguimiento de 16, 9 y 4 años, respectivamente. Uno tiene actualmente una recidiva tumoral nodular en pulmón único. En cuanto a los fallecidos, 3 murieron antes de un año y otro después de 24 meses de evolución. En conclusión, la cirugía en los sarcomas pulmonares primitivos puede conseguir una elevada supervivencia

Unlike lung metastases of extrapulmonary sarcomas, primary sarcoma of the lung is very rare. We analyzed 7 primary sarcomas treated surgically in Hospital Universitario La Paz, Madrid, Spain between 1985 and 2001. Preoperative histologic diagnosis was correct for 2 patients. Surgery was performed in all 7 patients. Resection was extended to the left atrium in 1 patient, to the chest wall in another, and the parietal pleura were removed from 2 others. Surgical resection was considered complete in 6 cases. Histology revealed 4 cases of malignant fibrous histiocytoma, 1 angiosarcoma, 1 osteogenic sarcoma, and 1 undifferentiated sarcoma. Enlarged lymph nodes removed during surgery were tumor free. Three patients received complementary treatment. The 3 longest-surviving patients were treated with surgery alone; at the end of the study, these patients remained alive 16, 9, and 4 years after surgery. One patient has a recurrent lymph node tumor in a single lung. Three patients died within 1 year and another died 24 months after surgery. In conclusion, surgical treatment of primary sarcoma of the lung can achieve good survival

[Analysis of logistical and organizational aspects of a cruise for patients with chronic respiratory insufficiency. The RESpIRA Expedition and the COPD Cruise]. / Análisis de los aspectos logísticos y organizativos de un crucero para pacientes con insuficiencia respiratoria crónica. La Expedición RESpIRA y el Crucero de la EPOC.

Patients with chronic respiratory insufficiency who are receiving domiciliary oxygen therapy and mechanical ventilation report great difficulty in taking complex trips involving several destinations and prolonged stays away from home. Such patients share a common need for home equipment whose technology is relatively sophisticated, a condition that limits their freedom of movement. We are referring to systems for delivering oxygen therapy and mechanical respirators. Given that such patients have problems traveling by air, we hypothesized that a cruise would be an ideal alternative, given that travel would take place in the hotel itself. A cruise would facilitate the logistics of the journey, given that the equipment would have to be set up at only one setting. Working with these assumptions, we have thus far organized two cruises for chronic respiratory insufficiency patients: the "RESpIRA Expedition" and the "COPD Cruise". Our experience shows that the organizational problems to be coped with are patient recruitment, financing and choice of itinerary. With those aspects clear, organizers must then obtain the authorization of the cruise operator, including the approval of the medical and safety personnel on board. After obtaining permission for the cruise and as soon as the organizers know how many patients will travel, a list of oxygen therapy equipment (respirators and disposable supplies) must be compiled. Finally, equipment suppliers must be found. Afterwards, all that remains is to enjoy the trip. The participation of physicians responsible for domiciliary oxygen therapy and mechanical ventilation programs is essential for making patients feel safe and for assuring solutions for technical and medical problems that might arise.

Análisis de los aspectos logísticos y organizativosde un crucero para pacientes con insuficiencia respiratoriacrónica. La Expedición RESpIRA y el Crucero de la EPOC / Analysis of logistical and organizational aspects of a cruise for patients with chronic respiratory insufficiency. The RESpIRA Expedition and the COPD Cruise

Los pacientes con insuficiencia respiratoria crónica incluidos en programas de oxigenoterapia y ventilación mecánica domiciliaria refieren tener habitualmente grandes dificultades para realizar viajes complejos que impliquen diferentes destinos y permanencias prolongadas fuera de su domicilio habitual. Todos ellos tienen en común la necesidad de disponer de un equipamiento tecnológico más o menos sofisticado en casa que limita su libertad de movimientos. Hablamos de los sistemas de oxigenoterapia y respiradores mecánicos. Dadas las dificultades para viajar en avión que tienen este tipo de pacientes, nos planteamos que un crucero sería una alternativa ideal al ser el hotel el que se desplaza. Ello facilitaría la logística del viaje, pues sólo tendríamos que realizar la instalación de los equipos en un único emplazamiento. Con estas premisas hemos organizado hasta la fecha 2 cruceros para enfermos respiratorios crónicos, la Expedición RESpIRA y el Crucero de la EPOC. En nuestra experiencia, los problemas a los que debemos enfrentarnos a la hora de organizar un crucero para pacientes con insuficiencia respiratoria crónica se relacionan con el reclutamiento de pacientes, la financiación del viaje y la elección del itinerario que vamos a realizar. Una vez aclarados estos aspectos, hay que conseguir la autorización de la compañía naviera correspondiente, con el visto bueno de los servicios médicos y de seguridad de a bordo. Tras obtener el permiso para poder realizar el crucero y una vez que sepamos cuántos pacientes van a viajar, es necesario conocer qué material vamos a necesitar en términos de número de equipos de oxigenoterapia, respiradores y material fungible y, finalmente, dónde lo vamos a conseguir. Después sólo quedará disfrutar de la excursión. La participación de los médicos responsables de los programas de oxigenoterapia y ventilación mecánica domiciliaria es fundamental para transmitir seguridad a los pacientes y garantizar la solución de los problemas técnicos y médicos que pueden presentarse durante el viaje (AU)

[Prognostic factors and outcome of thymectomy in 80 cases of myasthenia gravis]. / Análisis de factores pronósticos y resultados de la timectomía en 80 casos de miastenia gravis.

OBJECTIVES: To analyze the results of thymectomy in our series of patients with myasthenia gravis (MG) and to study the influence of the most common prognostic factors. MATERIAL AND METHODS: Eighty MG patients over a period of 23 years underwent thymectomy consecutively in our hospital. Preoperative assessment included clinical evaluation of muscle weakness, edrophonium testing, electromyography, lung function testing, chest X-rays and CAT scans. Symptoms were assessed by the Osserman scale. The surgical approach was amplified transsternal thymectomy. The prognostic factors studied were sex, age, clinical stage, duration of disease before surgery and histology of the thymus. Clinical outcome was assessed using Millichap and Dodge's criteria. Follow-up was by the chest surgery and neurology departments. RESULTS: Complete remission was observed in 29 cases (36.2%) and significant improvement in 42 (52.5%). Complications developed in 9 patients (11.2%). Most patients were women (53/27) and outcomes for men and women were not statistically different. Mean age was 36 years (range 11-79), with no significant difference in outcome for patients who were older or younger than 60 years of age. Nor were differences evident related to presurgical clinical stage or levels of severity (I + IIa/IIb + III). Differences in outcome were highly significantly related to duration of disease (< 24 / > 24 months) (p = 0.0022), such that outcome was more satisfactory when the pre-surgical course of disease was shorter, provided that no thymoma was present. CONCLUSIONS: Amplified transsternal thymectomy was safe and effective for those patients with MG. When disease had been present for less than two years, the prognosis was better.

Análisis de factores pronósticos y resultados de la timectomía en 80 casos de miastenia gravis / Prognostic factors and outcome of thymectomy in 80 cases of myasthenia gravis

OBJETIVOS: Analizar los resultados de la timectomía en pacientes con miastenia gravis (MG) en nuestra serie y estudiar la influencia de los factores pronósticos más comúnmente investigados. MATERIAL Y MÉTODOS: Durante 23 años 80 pacientes con MG fueron timectomizados consecutivamente en nuestro hospital. El estudio preoperatorio se hizo mediante evaluación clínica de la fatigabilidad muscular, test de edrofonio, electromiografía, pruebas funcionales respiratorias (PFR), radiografía de tórax y TAC. La distribución clínica se hizo según la escala de Osserman. La técnica quirúrgica fue la timectomía trans-esternal ampliada. Los factores pronósticos estudiados son: sexo, edad, estadio clínico, tiempo de evolución prequirúrgico e histología del timo. La respuesta clínica se evaluó según los criterios de Millichap y Dodge. El seguimiento fue realizado por los servicios de cirugía torácica y neurología. RESULTADOS: En 29 casos (36,2 por ciento) se obtuvo la remisión completa y en 42 (52,5 por ciento), una mejoría clínica importante. Hubo complicaciones en 9 casos (11,2 por ciento). Existe un predominio femenino (53/27), sin diferencias significativas en las respuestas respecto a los varones. La edad media es de 36 años (rango, 11-79), sin diferencias significativas entre pacientes mayores o menores de 60 años. No hubo diferencias por el estadio clínico prequirúrgico, ni aun agrupándolos en estadios leves/graves (I + IIa/IIb + III). Comparando las respuestas en los pacientes según el tiempo de evolución (< 24 meses/evolución superior), encontramos diferencias muy significativas (p = 0,0022), favorables a los pacientes con menos tiempo de evolución, cuando no existía un timoma asociado. El estudio no pudo demostrar diferencias significativas en la respuesta según la histología del timo. CONCLUSIONES: La timectomía transesternal ampliada resultó ser un procedimiento seguro, con un alto índice de respuestas clínicas satisfactorias en pacientes con MG. Una evolución inferior a 2 años se reveló como un indicador de buen pronóstico (AU)

[Cystic adenoid carcinoma. Apropos of a case with very prolonged survival]. / Carcinoma adenoide quístico. A propósito de un caso con supervivencia muy prolongada.

Adenoid cystic carcinoma is an infrequent tumor, but not a rare one. Its primary location is most often the salivary glands, although it can be found in many other organs, including the lung, which is the most frequent site of remote metastasis. The tumor's two main features, which indicate a need for long-term monitoring, are 1) its high level of local aggressivity and rate of recidivism in spite of radical excision, with recurrence sometimes taking place long after the primary tumor appears, and 2) its slow natural history even when disseminated disease is present. Type of tumoral tissue is an important prognostic factor.

[Bronchoalveolar lavage in respiratory pathology]. / El lavado broncoalveolar en patología respiratoria.

Bronchoalveolar lavage (BAL) offers great opportunities in the study of pulmonary diseases. It is safe and noninvasive technique, with similar possibilities than other invasive diagnostic methods and allo-wind repetitions. With the obtained sample, we can assess what happens at the bronchial level as well as the alveolar structures. The introduction of new study methods has increased its profitability in several respiratory pathologies: analysis of tumoral markers in lung cancer, monoclonal antibodies in histiocytosis X, and so on. The BAL can be considered a technique of diagnosis, follow-up, prognosis and evaluation in pulmonary diseases.