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Simultaneous Bilateral Spontaneous Pneumothorax Revealed Birt-Hogg-Dubè Syndrome
No full textSimultaneous bilateral spontaneous pneumothorax is a very rare clinical event, comprising approximately 1% of all spontaneous pneumothoraces. Clinical signs and symptoms may vary from mild chest pain and dyspnea to severe respiratory failure; nevertheless immediate treatment is mandatory as this condition can deteriorate and progress to tension pneumothorax. An underlying lung disease has been commonly described; in most istances primary or secondary tumors, interstitial diseases, and infectious diseases. Birt-Hogg-Dubè syndrome is a rare inherited disorder clinically characterized by multiple fibrofolliculomas, renal tumors, lung cysts, and, in ~24% of the patients, occurrence of spontaneous pneumothorax. In this case, we firstly report the concurrence of these rare conditions, as a patient presenting a simultaneous bilateral spontaneous pneumothorax was diagnosed with Birt-Hogg-Dubè syndrome based on the typical radiological findings and genetic testing of the folliculin gene located on chromosome 17
Impatto del grading istologico sulla sopravvivenza nei pazienti sottoposti a resezione radicale per cancro del polmone
No full textTrattamento multimodale del timoma invasivo stadio IV A
Full text linkScopo dello studio. Valutare la applicabilità e l’efficacia di un protocollo multimodale nel trattamento del timoma allo stadio IVA alla diagnosi.
Materiali e metodi. Dal 1990 al 2007, 27 pazienti affetti da timoma allo stadio IVA sono stati sottoposti ad un protocollo di chemioterapia preoperatoria, chirurgia e radioterapia postoperatoria sul mediastino. Il trattamento preoperatorio è stato caratterizzato da 4 cicli di chemioterapia a base di derivati del platino; la radioterapia postoperatoria è stata somministrata in 45-60 Gy al mediastino e pregresso letto tumorale mediante un acceleratore lineare da 10MeV.
Risultati. Sono stati trattati 14 maschi e 13 femmine con età media di 48.5 anni (intervallo 29-68). Sintomi aspecifici erano presenti in 20 casi alla diagnosi, mentre in 6 pazienti si sono riportate sindromi autoimmuni timo-correlate (4 miastenia gravis, 1 LES e 1 aplasia pura della serie rossa). La conferma istologica è stata ottenuta prima dello inserimento nel protocollo: sono stati riportati 1 timoma AB, 3 B1, 5 B2, 8 B3, 4 timomi misti B1-B2, 3 B1-B3 e 3 B2-B3. La chemioterapia neoadjuvante è stata ben tollerata da tutti i pazienti con una tossicità in media di grado lieve-moderato; in 25 casi su 27 si è avuta una risposta clinica e radiologica al trattamento di induzione e i pazienti sono stati successivamente sottoposti a chirurgia con intento macroscopicamente radicale. Il timoma, i residui del timo e gli organi direttamente invasi dalla neoplasia sono stati asportati contemporaneamente alle disseminazioni pleuriche di malattia: sono state effettuate 1 pneumonectomia extrapleurica, 1 pneumonectomia, 3 lobectomie polmonari, 12 resezioni atipiche polmonari, 25 pleurectomie più o meno estese, 18 pericardiectomie, 5 resezioni di vena anonima o cava superiore, 5 resezioni diaframmatiche, 3 resezioni limitate di parete toracica e 2 sezioni di nervo frenico. In 9 casi si è dovuto procedere a resezione incompleta per residuo macroscopico sui grossi vasi (7 casi) o nervi frenici bilateralmente (2 casi) mentre in 16 casi (64%) si è proceduto a resezione macroscopica completa. La mortalità e morbilità postoperatorie sono state rispettivamente nulla e del 24%. La radioterapia postoperatoria è stata somministrata a tutti i pazienti (media 57 Gy, intervallo 55-61) con una minima tossicità correlata.
Il follow-up medio è stato di 87 mesi (intervallo 29-147) e la sopravvivenza malattia-correlata e libera da malattia a 10 anni dal trattamento è stata rispettivamente del 68% e 39%.
La sopravvivenza malattia-correlata a 10 anni è stata del 80% in caso di resezione completa e del 41% in caso di resezione incompleta (p=0.03), mentre a 8 anni è stata del 66% nei timomi non-B3 e del 19% nei timomi B3 (p=0.016).
Conclusioni. Il protocollo multimodale basato su chemioterapia di induzione, chirurgia e radioterapia postoperatoria consente un buon tasso di resezioni chirurgiche complete ed è dimostrato essere un trattamento con un basso tasso di complicanze ed efficace nel garantire una buona sopravvivenza a lungo termine nei pazienti affetti da timoma allo stadio IVA alla diagnosi.Objective. To evaluate the feasibility and the efficacy of a multimodal treatment in the management of stage IVA thymoma at first diagnosis.
Material and methods. From 1990 to 2007, 27 patients affected by stage IVA thymoma underwent neoadjuvant chemotherapy, surgery and subsequent mediastinal radiation therapy. Neoadjuvant chemotherapy consisted of 4 courses of platinum-based chemotherapy administered every 3 weeks; adjuvant radiation therapy consisted of 45 to 60 Gy administered by a 10MeV linear accelerator to the whole mediastinum and previous tumour bed.
Results. There were 14 males and 13 females, mean age was 48.5 years (range 29-68). Not specific symptoms were present in 20 cases and thymus-related syndromes were reported in 6 (4 myasthenia gravis, 1 LES, 1 pure red aplasia). Histological subtypes were confirmed before patients’ enrolment: there were 1 AB, 3 B1, 5 B2, 8 B3, 4 mixed B1-B2, 3 mixed B1-B3 and 3 mixed B2-B3 thymomas. Neoadjuvant chemotherapy was well tolerated in all cases with a mild to moderate related toxicity; 25 out of 27 patients manifested a clinical and radiological response to the neoadjuvant chemotherapy and received subsequent surgery. Thymomas, residual thymic tissue and tumour involved organs were resected together with the pleural implants: 1 extrapleural pneumonectomy, 1 pneumonectomy, 3 pulmonary lobectomy, 12 wedge lung resections, 25 limited to complete parietal pleurectomy, 18 pericardiectomy, 5 innominate and vena cava resection, 5 diaphragmatic resection, 3 limited chest wall resection and 2 phrenic nerve resection were carried out. Gross residual disease was left behind because of massive involvement of great vessels in 7 cases and bilateral phrenic nerve in 2, whereas 16 patients received complete macroscopic resection (64%). Postoperative mortality and morbidity were null and 24%, respectively. Adjuvant radiation therapy was administered to all patients (mean 57 Gy, range 55-61) with a minimal related toxicity. Mean follow-up was 87 months (range 29-147) and 10-years disease-related and disease-free survival were 68% and 39%, respectively. Disease-related survival at 10-years was 80% and 41% when complete and incomplete resection were compared (p=0.03); disease-related survival at 8-years was 66% and 19% when non-B3 and B3 thymoma were compared (p=0.016).
Conclusions. Multimodal management based on induction chemotherapy, subsequent surgery and postoperative mediastinal radiation allows a good complete resection rate and it is demonstrated to be a safe and effective treatment to warrant a good long-term survival in stage IVA thymoma patients
Thoracoscopic approach to lung metastases
No full textSurgical resection of lung metastases has become routine and the introduction of video assisted thoracic surgery (VATS) has provided a mini-invasive option to this treatment. However, oncological radicality of VATS is criticized especially in the event of deeply located lesions requiring manual palpation and lymph node dissection. At today, VATS lung metastasectomy is performed with diagnostic purposes or with curative intent as initial resection in patients with one to a few, peripherally located lesions. New perspectives are represented by the transxiphoid port, which may allow hand palpation during VATS, and awake lung metastasectomy in epidural anesthesia, which can permit a reduction of global operating room time. In conclusion, VATS metastasectomy is valid in selected but increasing number of patients, without compromising oncologic radicality
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