A case of endobronchial paraganglioma.

Paragangliomas are rare lung tumours; endobronchial localisation is even more rare. This report describes the case of a 59-year-old patient with a symptomatic endobronchial paraganglioma successfully resected by means of pulmonary lobectomy. Recognition of this uncommon tumour can lead to a correct diagnosis and therapeutic strategy.

New positron emission tomography derived parameters as predictive factors for recurrence in resected stage I non-small cell lung cancer.

BACKGROUND: The recurrence rate for stage I non-small cell lung cancer is high, with 20-40% of patients that relapse after surgery. The aim of this study was to evaluate new F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) derived parameters, such as standardized uptake value index (SUVindex), metabolic tumor volume (MTV) and total lesion glycolysis (TLG), as predictive factors for recurrence in resected stage I non-small cell lung cancer. METHODS: We retrospectively reviewed 99 resected stage I non-small cell lung cancer patients that were grouped by SUVindex, TLG and MTV above or below their median value. Disease free survival was evaluated as primary end point. RESULTS: The 5-year overall survival and the 5-year disease free survival rates were 62% and 73%, respectively. The median SUVindex, MTL and TLG were 2.73, 2.95 and 9.61, respectively. Patients with low SUVindex, MTV and TLG were more likely to have smaller tumors (p ≤ 0.001). Univariate analysis demonstrated that SUVindex (p = 0.027), MTV (p = 0.014) and TLG (p = 0.006) were significantly related to recurrence showing a better predictive performance than SUVmax (p = 0.031). The 5-year disease free survival rates in patients with low and high SUVindex, MTV and TLG were 84% and 59%, 86% and 62% and 88% and 60%, respectively. The multivariate analysis showed that only TLG was an independent prognostic factor (p = 0.014) with a hazard ratio of 4.782. CONCLUSION: Of the three PET-derived parameters evaluated, TLG seems to be the most accurate in stratifying surgically treated stage I non-small cell lung cancer patients according to their risk of recurrence.

Pulmonary vein stenosis requiring lobectomy after radiofrequency catheter ablation for atrial fibrillation.

Pulmonary vein stenosis is one of the potential complications of radiofrequency catheter ablation for atrial fibrillation. This complication is generally treated by transcatheter techniques using balloon angioplasty or stent implantation. We report a case of a 17-year-old boy with acquired left inferior pulmonary vein stenosis following radiofrequency catheter ablation for atrial fibrillation, conditioning recurrent pneumonia. Despite three attempts of vein dilation by means of angioplasty, the stenosis always recurred with worsening of symptoms. A left inferior lobectomy was then performed and after 33 months the patient is well and with no symptoms.

The surgical treatment of lung cancer in patients with previous lymphoproliferative disorders: a historical cohort study.

OBJECTIVES: We undertook a historical cohort study to compare, in terms of morbidity, mortality and long-term survival associated with lung cancer resection, a group of patients with previous lymphoproliferative disorders and a group without a hematological history. METHODS: We identified 29 patients with a previous lymphoproliferative disorder who underwent lung cancer resection. These subjects (Group-A) were matched with 87 patients without a hematological history who underwent pulmonary resection during the same period (Group-B). RESULTS: We found no significant difference between the two groups in length of hospitalization, comorbidities, spirometric parameters, type of surgery, histology, neoadjuvant chemotherapy, morbidity, mortality, median survival (Group-A = 37 months; Group-B = 52 months) and 5-year survival (Group-A = 37%; Group-B = 42%). The mean age of Group-A patients was significantly lower than that of Group-B patients (62 vs 66 years; p = 0.024). Group-A patients had a well differentiated lung cancer more frequently than Group-B patients (p = 0.001). Group-A patients had transitory bacteraemies more frequently than Group-B patients (p = 0.005). Multivariate Cox regression analysis showed that age (p = 0.01) and lung cancer stage (p = 0.04) were significantly associated with survival. CONCLUSIONS: Patients with lymphoproliferative disorders had a lower age and more differentiated lung cancers than those without lymphoproliferative disorders. Patients with lymphoproliferative disorders and those without a hematological history had similar morbidity, mortality and long-term survival after pulmonary resection. Distinguishing patients with and without a lymphoproliferative disorder seems to be of limited value in the decision-making process of evaluating the indications for surgical treatment of lung cancer.

First analytical evidences of precious colourants on Mediterranean illuminated manuscripts.

Two Byzantine VI century manuscripts known as Vienna Dioskurides and Vienna Genesis, held in the Austrian National Library at Vienna, were analysed with in situ non-invasive techniques. Raman spectroscopy, UV-Vis diffuse reflectance spectrophotometry with optic fibres and X-ray fluorescence spectrometry were used to characterise the palette of these early Middle Ages manuscripts. The analytical study was performed to have a better knowledge on the colourants used by ancient miniature painters, a subject known more on the basis of traditional sources (i.e. medieval treatises) than of analytical evidences. Indeed these illuminated manuscripts are, to the authors' knowledge, among the oldest ever being analysed, so that the colourants found in them can be considered among the oldest evidences of their use. The main feature of Vienna Dioskurides and Vienna Genesis palettes is their richness, exemplified by the simultaneous presence of gold and ultramarine blue; in Vienna Dioskurides cinnabar is also present. Information regarding ultramarine blue is surprising, being the analytical evidence of the use of this precious pigment at least three centuries before its use in Western manuscripts, a feature justified by the fact that the Byzantine Empire was the dominant culture in early Middle Ages in the Mediterranean World. Other colourants include azurite and indigo, red lead, orpiment, red and yellow ochres, while a mixture of blue and yellow colourants, known as vergaut, was used to render green hues. Organic colourants were also used, such as madder and Tyrian purple, the latter employed to dye the parchment of Vienna Genesis.

Combined treatment of non-small cell lung cancer with synchronous brain metastases: a single center experience.

AIM: The aim of this study was to analyze our experience with combined treatment of non-small cell lung cancer with synchronous brain metastases. METHODS: Between 1992 and 2008, 31 patients were treated by performing neurosurgery (or stereotactic radiosurgery) and lung surgery. Patients were divided into two groups according to their preoperative mediastinal work-up: group A (CT scan) and group B (FDG-PET scan). RESULTS: Twenty-six patients had one brain metastasis and five had two. Neurosurgery was performed in 10 patients, stereotactic radiosurgery in 20 and both approaches in 1. Seven patients underwent chemotherapy after cerebral procedure. Pulmonary resection was complete in 27 cases and incomplete in 4. Histological findings showed: adenocarcinoma in 19 cases, squamous cell carcinoma in 8 and large cell carcinoma in 4. All patients underwent adjuvant chemotherapy. Overall 1, 2 and 5-year survival rates were 83%, 47% and 21%, respectively. The median survival was 22 months. Univariate analysis showed a better prognosis for complete resection (P=0.008), adenocarcinomas (P=0.015), N0 disease (P=0.038), and Group B (P=0.045). Multivariate analysis showed that only the radicality of the resection (P=0.027) and Group B (P=0.047) were independent prognostic factors. CONCLUSION: Our experience confirms that selected patients with non-small cell lung cancer and synchronous brain metastases may be effectively treated by combined therapy. Complete resection, adenocarcinoma histology and N0 disease were prognostic factors. The incorporation of FDG-PET scan into the preoperative work-up may translate into a survival benefit.

Surgical treatment of multiple primary adenocarcinomas of the lung.

INTRODUCTION: The incidence of lung adenocarcinomas has steadily increased over the last decades. The aim of this study was to assess the results of surgical treatment of multiple primary adenocarcinomas of the lung (MPAL) analyzing the radiological and histological features. METHODS: From 1988 to 2005, 26 patients underwent surgical treatment for MPAL at our department, for a total of 52 tumors. Three patients had synchronous and 23 had metachronous tumors. RESULTS: Thirty-seven tumors were classified as solid, two as ground-glass opacities (GGO) and 13 as mixed solid/GGO tumors on the basis of CT scan evaluation. Histology revealed 26 adenocarcinomas, five adenocarcinomas with a bronchioloalveolar (BAC) pattern and 21 BAC. There was no postoperative mortality. Five-year survival of patients with synchronous tumors was 66 %. Survival of patients with metachronous tumors was 95 % and 70 % from the first and second operation. Patients with stage II and III a tumors had significantly reduced survival rates ( P < 0.05). Survival was 60 % after lobectomy and 78 % after wedge resection. CONCLUSIONS: Surgical treatment of MPAL is associated with favorable results. Sublobar resections, when technically feasible, provide adequate oncological management.

Preoperative assessment in patients with postintubation tracheal stenosis : Rigid and flexible bronchoscopy versus spiral CT scan with multiplanar reconstructions.

BACKGROUND: Postintubation stenosis remains the most frequent indication for tracheal surgery. Rigid bronchoscopy has traditionally been considered the technique of choice for the preoperative diagnostic assessment. However, this technique is not routinely available, and new techniques such as flexible videobronchoscopy and spiral computed tomography (CT) scan with multiplanar reconstructions have been proposed as alternatives to rigid bronchoscopy. The aim of this study was to compare these techniques in the diagnostic assessment of patients with tracheal stenosis submitted to surgical treatment. METHODS: Twelve patients who underwent airway resection and reconstruction for postintubation tracheal and laryngotracheal stenosis were preoperatively evaluated with rigid and flexible bronchoscopy and with spiral CT scan with multiplanar reconstructions. The following parameters were examined: involvement of subglottic larynx, length of the stenosis, and associated lesions. The results were compared with the intraoperative findings. RESULTS: The accuracy of rigid bronchoscopy, flexible bronchoscopy, and CT scan in the evaluation of the involvement of subglottic larynx was, respectively, 92%, 83%, and 83%. The evaluation of the length of the stenosis was correct in 83%, 92%, and 25% of the patients, respectively, with rigid bronchoscopy, flexible bronchoscopy, and CT scan. A significant correlation was observed between the length of the stenosis measured intraoperatively and preoperatively with rigid (p < 0.001) and flexible bronchoscopy (p < 0.05) but not with CT scan (p = 0.08). The three techniques correctly showed the presence of an associated tracheoesophageal fistula in two patients, but CT scan did not correctly show the exact location of the fistula in relation to the airway. Flexible bronchoscopy was the only effective technique in the assessment of laryngeal function. CONCLUSIONS: Rigid bronchoscopy remains the procedure of choice in the evaluation of candidates for tracheal resection and reconstruction for postintubation stenosis, and it should be available in centers that perform surgery of the airway. Flexible bronchoscopy and CT scan have to be considered complementary techniques in the evaluation of laryngeal function and during follow-up.

Neoadjuvant chemotherapy and pneumonectomy in a lung cancer patient on hemodialysis.

Major pulmonary resections are rarely performed in non-small cell lung cancer patients on hemodialysis. To date only two cases of pneumonectomy performed in such patients are reported in the literature. Moreover, chemotherapy, as a treatment for advanced non-small cell lung cancer, is not routinely administered to patients with end-stage renal disease requiring hemodialysis. We present the case of a stage IIIB non-small cell lung cancer patient on hemodialysis who successfully underwent neoadjuvant chemotherapy followed by pneumonectomy. To our knowledge, this is the first case of non-small cell lung cancer patient on hemodialysis reported in the literature who successfully underwent this type of combined therapy.

Thoracoscopic treatment of a pericardial diverticulum.

A 35-year-old female patient presented with a history of recurrent chest pain. On chest x-ray, a regularly shaped lesion at the right cardiophrenic angle was observed. The lesion appeared smaller on a subsequent x-ray. Magnetic resonance imaging showed a cystic lesion that could be differentiated from the pericardium only in its lower part. Thoracoscopy revealed a pericardial diverticulum. Resection of the lesion was performed thoracoscopically, with complete remission of the symptoms.

Surgical tracheostomy versus percutaneous dilatational tracheostomy. A prospective-randomized study with long-term follow-up.

BACKGROUND: To compare surgical tracheostomy (ST) versus percutaneous dilatational tracheostomy (PDT) in terms of complication rates. In particular we specifically studied the late tracheal complications of both methods by means of endoscopic controls of patients up to 6 months after the procedures. DESIGN: prospective-randomized clinical study. SETTING: University-affiliated tertiary care referral hospital. PATIENTS: 50 consecutive translaryngeally intubated patients with respiratory failure were randomized to undergo either ST (25 patients) or endoscopic guided PDT (25 patients). RESULTS: ST was performed in 41+/-14 min versus 14+/-6 min for PDT (p<0.0001). There was no procedure-related death. In the ST group there were no intraoperative complications. In the PDT group 2 intraoperative complications (minor hemorrhages) were observed. In the ST group 9 early postoperative complications occurred: one minor bleeding, 7 stomal infections and one accidental decannulation. In the PDT group only one early postoperative complication (minor bleeding) occurred. Early postoperative complication rates were 36% for ST and 4% for PDT. In the ST group there were no late tracheal complications. In the PDT group 2 late tracheal complications (one segmental malacia and one stenosis at the level of the stoma) were observed. CONCLUSIONS: This study confirms that PDT is a simpler and quicker procedure than ST and that it has a lower rate of early postoperative complications. Late tracheal complications were more frequent, although the difference was not statistically-significant, in the PDT group. Further investigations of long-term outcome following PDT are therefore necessary.

Therapeutic outcome according to histologic subtype in 121 patients with malignant pleural mesothelioma.

One-hundred and twenty-one cases of malignant pleural mesothelioma (MPM) seen between 1986 and 1999 at the authors' Institution were reviewed. Histotype was epithelial in 88 patients (73%), sarcomatous in 21 (17%) and mixed in 12 (10%). Ninety-one patients received a treatment (38 palliative pleurectomy and no further therapy, 16 palliative pleurectomy followed by chemotherapy, 37 chemotherapy alone), while 30 were referred to supportive care only. Median survival of the whole population was 10.5 months. The 1-, 2- and 3-year survival were 40, 17 and 8%, respectively. Univariate analysis of subgroups showed that poor performance status (PS), non-epithelial histotype, Butchart stage>I and International Mesothelioma Interest Group (IMIG) stage>I were individually associated with lower survival. Patients receiving any therapy survived longer than patients treated with supportive care only (P=0.0004). Treatment modality had an independent prognostic value (P=0.00005), with a survival advantage for patients receiving surgery and adjuvant chemotherapy. Multivariate analysis confirmed the independent prognostic value of PS (P=0.001; HR=2.48) and treatment modality (P=0.003; HR=1.38). The prognostic role of PS (P=0.02) and treatment modality (P=0.01) was confirmed in the subset of patients with epithelial histology. On the contrary, therapy had no impact on survival in patients with sarcomatoid MPM (P=0.74). Despite the predicted bias of a retrospective non-randomized evaluation of treatment-related factors, patients with good PS and epithelial histology seemed to have a survival benefit from surgery or multimodality therapy, as opposite to patients with poor PS or non-epithelial histotype. However, these results must be confirmed in a larger prospective trial with uniform treatment.

Thoracoscopic techniques in the management of benign mediastinal dumbbell tumors.

Mediastinal dumbbell tumors can be resected with a variety of open surgical approaches. Recently, thoracoscopic techniques have been suggested for the treatment of benign neurogenic lesions. Over a 5-year period, three patients with a benign mediastinal dumbbell tumor were treated via a combined microneurosurgical and thoracoscopic approach. The neurosurgical phase consisted of a posterior laminectomy to free the tumor from the spinal cord, followed by an intervertebral foraminotomy. Thoracoscopic resection of the lesion was then performed in the same setting. The operative times were 240, 260, and 280 min, and there were no operative complications. The postoperative stays were 6, 7, and 7 days; the postoperative period was uneventful in all three patients. Pathologic examination revealed a benign schwannoma in two cases and an angiolipoma in one case. One patient reported the onset of paraesthesia in the left hypocondrium on the distribution area of the transected T10 and T11 intercostal nerves; slight paraesthesia still remains 15 months from surgery. We conclude that the combined posterior neurosurgical and thoracoscopic approach is a safe and effective method for the removal of benign mediastinal dumbbell tumors, whether neurogenic or nonneurogenic in origin.

Evaluation of radiological and pathological prognostic factors in surgically-treated patients with bronchoalveolar carcinoma.

OBJECTIVE: The incidence of adenocarcinoma and bronchoalveolar carcinoma has increased in recent years. The aim of this study was to retrospectively evaluate radiological and pathological factors affecting survival in patients with bronchoalveolar carcinoma (BAC) or BAC associated with adenocarcinoma who underwent surgical treatment. METHODS: From May 1988 to September 1999, 49 patients with BAC or BAC and adenocarcinoma underwent surgical treatment. Complete resection was performed in 42 patients. In these patients the impact of the following factors on survival was evaluated: stage, TNM status, radiological and pathological findings (percentage of bronchoalveolar carcinoma in the tumour, presence or absence of sclerosing and mucinous patterns, vascular invasion and lymphocytic infiltration). RESULTS: Twenty-nine patients were male and 20 female. Mean age was 63 years. Five-year survival was 54%. Univariate analysis of the patients who underwent complete resection demonstrated a favourable impact on survival in stages Ia and Ib (P = 0.01) and in the absence of nodal involvement (P = 0.02) and mucinous patterns (P = 0.02). Mucinous pattern was also prognostically relevant at multivariate analysis (P = 0.02). In the 27 patients with stage Ia and Ib disease, univariate analysis demonstrated that the absence of mucinous pattern (P = 0.006) and a higher percentage of BAC (P = 0.01) favourably influenced survival. The latter data were also confirmed by multivariate analysis (P = 0.01). CONCLUSION: Surgical treatment of early-stage BAC and combined BAC and adenocarcinoma is associated with favourable results. However, the definition of prognostic factors is of utmost importance to improve the results of the treatment. In our series tumours of the mucinous subtype and with a lower percentage of BAC had a worse prognosis.

[Treatment of malignant pleural mesothelioma]. / Il trattamento del mesotelioma pleurico maligno.

BACKGROUND: In this study all patients observed between January 1993 and October 1997 with malignant pleural mesothelioma (MPM) have been analyzed in order to describe the impact of treatment modality on survival. METHODS: Medical records of 56 patients with MPM (44 male, 12 female, median age = 59 yrs) were reviewed. In 34 cases the histotype was epithelial, in 4 sarcomatoid, in 4 mixed, in 3 desmoplastic, and in 11 not specified. Four treatment modalities were identified: 1) Surgery (subtotal pleurectomy) = 20 patients; 2) Chemotherapy = 19 patients; 3) Surgery+Chemo-therapy = 8 patients; 4) Supportive care = 9 patients. RESULTS: The median survival was: 1) Surgery = 12.4 months; 2) Chemotherapy = 7.5 months; 3) Surgery+Chemotherapy = 12 months; 4) Supportive care = 11.4 months. Using univariate analysis, 8 prognostic factors were studied (age, sex, asbestos exposure, side, histotype, performance status, stage, treatment). Among these, only the stage and the performance status had shown a prognostic value on survival (p<0.05), while the treatment modality had not significantly influenced the prognosis. Using multivariate analysis only performance status showed to be significatively associated with survival (p=0.01 and odds ratio = 1.9, I.C. 1.2-3.2). CONCLUSIONS: Despite the limits of a retrospective study, personal experience confirms the ineffectiveness of current therapeutical approaches to MPM. A better understanding of MPM is required to develop new therapeutical approaches and alter the dismal prognosis of this disease.

Leukotriene D4-induced activation of smooth-muscle cells from human bronchi is partly Ca2+-independent.

Cysteine-containing leukotrienes (cysteinyl-LTs) are potent bronchoconstrictors and play a key role in asthma. We found that histamine and LTD4 markedly constrict strips of human bronchi (HB) with similar efficacy. However, in human airway smooth-muscle (HASM) cells, LTD4, at variance with histamine, elicited only a small, transient change in intracellular calcium ion concentration. HASM cells express both Ca2+-dependent and -independent isoforms of protein kinase C (PKC) (i.e., PKC-alpha and PKC-alpha ). Western blot analysis showed that PKC-alpha is activated by histamine and, to a lesser extent, by LTD4, whereas only LTD4 translocates PKC-alpha. This translocation was specifically inhibited by the LTD4 antagonist pobilukast. Phorbol-dibutyrate ester (PDBu) (a PKC activator) contracted HB strips to the same extent in the presence as in the absence of extra- and intracellular Ca2+. In the absence of Ca2+, LTD4 contracted HB strips to the same extent as did PDBu, suggesting the involvement of a Ca2+-independent PKC in LTD4-mediated signal transduction. PDBu-induced desensitization and the PKC inhibitor H7 abolished the slow and sustained LTD4-triggered contraction of HB strips in the absence of Ca2+, although H7 did not greatly affect the response in the presence of the ion. Thus, in human airways, we identified a novel LTD4 transduction mechanism linked to bronchial smooth-muscle contraction, which is partly independent of Ca2+ and involves the activation of PKC-alpha.

Effect of endogenous and exogenous prostaglandin E(2) on interleukin-1 beta-induced cyclooxygenase-2 expression in human airway smooth-muscle cells.

We studied the effect of endogenous and exogenous prostaglandin E(2) (PGE(2)), a metabolite of arachidonic acid through the cyclooxygenase (COX) pathway, on interleukin (IL)-1 beta-induced COX-2 expression, using primary cultures of human bronchial smooth-muscle cells (HBSMC). Treatment with exogenous PGE(2) resulted in enhanced expression of IL-1 beta-induced COX-2 protein and messenger RNA (mRNA) as compared with the effect of the cytokine per se. Inhibition of PGE(2) production with a nonselective COX inhibitor (flurbiprofen, 10 microM) resulted in a significant reduction in IL-1 beta- induced COX-2 expression, supporting a role of endogenous COX metabolites in the modulation of COX-2 expression. None of the experimental conditions used in the study affected the expression of constitutive cyclooxygenase (COX-1). Treatment with cycloheximide to inhibit translation, and with dexamethasone or actinomycin D to inhibit transcription, linked the effect of PGE(2) to the transcriptional level of COX-2 mRNA rather than to a potential effect on protein and/or mRNA stabilization. PGE(2) increased adenylate cyclase activity in a concentration dependent manner, and forskolin, a direct activator of adenylate cyclase, caused a marked increase in IL-1 beta-dependent COX-2, suggesting the existence of a causal relationship between the two events. The same results were observed with salbutamol, a bronchodilator that acts by increasing cyclic adenosine monophosphate. The effect of PGE(2) on COX-2 expression may contribute to the hypothesized antiinflammatory role of PGE(2) in human airways, providing a self-amplifying loop leading to increased biosynthesis of PGE(2) during an inflammatory event.

Mediastinal dumbbell angiolipoma.

Nonneurogenic dumbbell tumors are rare. This report describes the case of a 46-year-old woman with a symptomatic mediastinal dumbbell angiolipoma. The tumor was successfully resected using a single-stage procedure, combining a posterior microneurosurgical and thoracoscopic approach. The patient made an uneventful recovery and the neurologic symptoms improved immediately.

Diagnostic and therapeutic management of neuroendocrine lung tumors: a clinical study of 44 cases.

Neuroendocrine tumors of the lung (NTL) are a distinct subset of tumors with a wide range of histological patterns and clinical behavior. Controversy still exists as to the ideal diagnostic and therapeutic approach to these neoplasms. A series of 44 consecutive NTL patients operated on at our Institution was retrospectively reviewed in order to critically analyze the diagnostic and therapeutic management. A preoperative diagnosis was obtained in 11 patients (25%). All patients underwent an anatomical surgical resection with lymphoadenectomy. Pathological diagnosis was typical carcinoid (TC) tumor in 36 cases, atypical carcinoid (AC) in three and large-cell neuroendocrine carcinoma (LCNEC) in five. One patient had preoperative chemotherapy. Node-positive patients received postoperative radiotherapy on the mediastinal area. Median follow-up time was 40 months for TC and 51.5 months for AC/LCNEC. Recurrence of disease was observed in three patients with TC and in two with AC/LCNEC. Actuarial 5-year survival was 93% for TC and 70% for AC/LCNEC. Survival was not influenced by tumor size, while lymph node metastases were associated with a worse prognosis. However, due to the limited number of patients, no statistical significance was observed. In conclusion, our study confirms findings in the literature showing that TC and AC/LCNEC are clinically different, and that a differential preoperative diagnosis and treatment is necessary. Although the results of new diagnostic techniques such as octreotide scintigraphy are encouraging, they need to be validated in a larger number of patients. Surgery, with anatomical resection and lymphoadenectomy, remains the treatment of choice in all these tumors. Laser treatment should be considered only as a palliative procedure or as a complementary technique to surgery. The role of adjuvant treatments in AC and LCNEC is uncertain and should be evaluated in larger trials. The prognostic role of biological factors such as cytometry and genetic markers requires further investigation before any definitive conclusions can be drawn.

[Congenital lobar emphysema in adults]. / Enfisema lobare congenito nell'adulto.

Due to the severity of respiratory symptoms, congenital lobar emphysema often requires surgical treatment in the early stages of life. Diagnosis of congenital lobar emphysema in adult life is therefore unusual, often presenting with mild symptoms. Diagnostic assessment is therefore of great importance in the treatment of these patients. A case of congenital lobar emphysema in an adult is reported and the diagnostic and therapeutical approach are discussed. The use of new diagnostic techniques such as dynamic RMN and SPET V/Q lung scan may improve the accuracy of the diagnostic evaluation. Pulmonary lobectomy led to marked improvement in respiratory function. Accurate diagnosis and appropriate management of congenital lobar emphysema in adult patients can lead to favourable results.