A Rare Case of Coexisting Breast Cancer and Refractory Acute Myeloid Leukemia.

The occurrence of acute myeloid leukemia (AML) within six months from a diagnosis of breast cancer (BC) is rarely reported in the literature, and it is associated with a poor prognosis. We report herein the case of a 40-year-old woman referred to our centre affected by BC and simultaneous AML. The patient proved refractory to first line therapy and achieved complete remission (CR) with a clofarabine-based regimen followed by allogeneic stem cell transplantation (ASCT). Both during salvage chemotherapy and after ASCT, the patient presented severe infectious complications ( acute cholecistytis and Nocardia pneumonia, respectively) treated with surgery, and currently she is alive in CR for both diseases after 29 months of follow-up. The case highlights the importance of a diagnostic assessment of any unexplained cytopenia in association with solid neoplasia under treatment, underlining the feasibility and priority of a timely treatment of the haematological neoplasm in order to achieve long-term survival.

Colistin-based Treatment of Multidrug-resistant Gram-negative Bacterial Pulmonary Infections After Lung Transplantation.

BACKGROUND: Lung transplantation (LT) is a viable option for a select group of patients with end-stage lung disease. However, infections are a major complication after LT, accounting for significant morbidity and mortality. Several germs may be responsible; multidrug-resistant Gram-negative (MDR-GN) bacteria are emerging. Colistin is widely used in the treatment of these infections and is administered by inhalation and/or parenterally. At our institution, in patients with tracheostomy, colistin is administered by direct instillation in the airway during bronchoscopy. We reviewed a series of patients who underwent LT complicated by postoperative MDR-GN bacterial pulmonary infection. METHODS: From January 2015 to May 2017, 26 lung transplants were performed. In the postoperative course, 14 (54%) developed MDR-GN bacterial infection; respiratory specimen culture, blood tests, and chest X-ray were considered. Colistin was the only antibiotic usable. Thirteen patients received intravenous (IV) colistin; in the subgroup of patients with tracheostomy, colistin was instilled directly in the airway, and 6 patients received inhaled colistin. RESULTS: Seven patients needed tracheostomy. Pseudomonas aeruginosa was the predominant infection (86%), with Acinetobacter baumanii seen in 2 cases (14%). An early clinical-laboratory response was observed in 9 patients (64%). White blood cell count and C-reactive protein values improved (P = .02 and P = .001, respectively). A significant reduction in bacterial load was observed on microbiologic bronchoalveolar lavage specimens. CONCLUSION: Colistin instilled directly in the airway did not show side effects. The combination of IV and inhaled/instilled colistin could be a useful treatment option for MDR-GN infections after LT.

Efficacy of Extracorporeal Photopheresis in Patients With Bronchiolitis Obliterans Syndrome After Lung Transplantation.

BACKGROUND: Lung transplantation (LT) is only therapeutic option for patients affected by chronic respiratory failure. Chronic rejection, also known as bronchiolitis obliterans syndrome (BOS), is still the main cause of death and the most important factor that influences post-transplantation quality of life. Currently available therapies have not been proven to result in significant benefit in the prevention or treatment of BOS. Extracorporeal photopheresis (ECP) seems to reduce the rate of lung function decline in transplant recipients with progressive BOS. METHODS: From 1991 until now, 239 LTs were performed at our center. Fifty-four patients (22.5%) developed BOS; 15 of these (27.7%) were treated with ECP. At the beginning of the treatment, all patients showed a mean decline of forced expiratory volume in 1 second (FEV1) from baseline values of 45.8% ± 17.2%; 2 patients were in long-term oxygen therapy. RESULTS: Mean follow-up from November 2013 to June 2016 was 11.6 ± 7 months. Twelve patients (80%) showed lung function stabilization with an FEV1 range after treatment between -6% to +8% from the pre-treatment values. We did not report any adverse effects or increase of infections incidence. DISCUSSION: ECP seems to be an effective and well-tolerated therapeutic option for LT patients with BOS in terms of stabilization of lung function and increased survival.

Evaluation of Renal Function in Patients Undergoing Lung Transplantation.

BACKGROUND: Acute kidney injury and chronic kidney failure are serious complications after lung transplantation. Glomerular filtration rate (GFR) is the primary indicator of renal function. Several equations have been proposed to evaluate the estimated GFR (eGFR). We compared three different equations to determine which has the better correlation with the development of acute and chronic renal failure in lung recipients. METHODS: Twenty-two patients with a mean age of 54.4 ± 8.5 years underwent lung transplantation from 2010 to 2015. Thirteen (59%) had pulmonary fibrosis, 7 (32%) emphysema, 1 (4.5%) bronchiectasis, and 1 (4.5%) lymphangioleiomyomatosis. In all patients, eGFR was measured preoperatively using Cockcroft-Gault (CG), Modification of Diet in Renal Disease (MDRD), and Levey's Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equations. In 20 patients (90%) eGFR was calculated at 1, 3, and 6 months. RESULTS: According to CKD-EPI and MDRD, eight patients (36.3%) had preoperative reduction in eGFR, whereas 6 patients (27.2%) had preoperative reduction according to the CG (P = .04). The mean values were higher for the CG (103.2 vs. 102 vs. 94.4). Five patients (22.7%) developed perioperative acute renal failure requesting a dialysis treatment; four of these showed a preoperative eGFR to the highest CG (P = .05). At 1 and 6 months after lung transplantation, the CG, MDRD and CKD-EPI eGFR values were, respectively, 86.6, 84.1 and 76.6 mL/min/1.73m2 and 75.8, 72.7, and 72.3 mL/min/1.73m2. CKD-EPI eGFR values are more predictable than the other equations of AKI. CONCLUSIONS: Preoperative assessment of eGFR using the MDRD and CKD-EPI seems to correlate better than the CG to the prediction of acute renal failure, whereas for the chronic form the three equations seem equivalent.

Extracorporeal membrane oxygenation as bridge to lung transplantation.

Lung transplantation (OLT) is a viable option for end-stage pulmonary diseases in selected patients with satisfactory long-term results. However, the paucity of available donors engenders a prolonged stay on the waiting list with progressive decline of lung function. In cases of sudden respiratory failure, admission to an intensive care unit with institution of extracorporeal membrane oxygenation (ECMO) may be an option while a waiting an emergency OLT. In 12 OLT candidates we started ECMO because of acute decline of lung function. Eleven patients had cystic fibrosis and the other subject, histiocytosis X. In 7 patients bilateral OLT was performed after a mean waiting time of 6 days from ECMO institution; 5 patients died on ECMO at a mean time of 11.6 days. After OLT 2 patients required reoperation for hemothorax; renal failure and acute leg ischemia occurred in 2 patients. The mean weaning time from ECMO after OLT was 2.14 days. No patient died in the perioperative period and 1-year survival was 85.7%. ECMO represents a valid option as a bridge to urgent OLT for selected candidates.

Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation.

AIM: Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome. METHODS: We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women. RESULTS: Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO(2) (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P<0.001). Pulmonary vascular resistance was associated with mortality (P=0.03). When both PH and PVR were included in the model, only PH predicted mortality. CONCLUSION: Pulmonary hypertension of mild degree is frequently found in CF patients with advanced lung disease and its presence significantly worsens survival.

Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation.

AIM:Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome. METHODS: We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women. RESULTS:Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO2 (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P<0.001). Pulmonary vascular resistance was associated with mortality (P=0.03). When both PH and PVR were included in the model, only PH predicted mortality. CONCLUSION: Pulmonary hypertension of mild degree is frequently found in CF patients with advanced lung disease and its presence significantly worsens survival.

Adenosquamous carcinoma of the esophagogastric junction. Case report.

Adenosquamous carcinoma is a rare tumor with coexisting elements of infiltrating squamous cell carcinoma and adenocarcinoma. This tumor is reported to arise in different organs but rarely in the oesophagus. In most cases, it shows highly aggressive biological behaviour with high propensity to regional lymph-node metastasis and poor prognosis. We describe the management of a patient with an aggressive adenosquamous carcinoma of the esophagogastric junction.

Lung transplantation for cystic fibrosis after thoracic surgical procedures.

During their life, cystic fibrosis (CF) patients may require thoracic surgical procedures for a number of reasons before undergoing lung transplantation. In the past, this has been considered to be a contraindication to lung transplantation. However, a meticulous surgical technique and careful intraoperative management allows one to perform the transplantation safely. Herein we have reported our experience with CF patients undergoing lung transplantation after previous surgical treatment for pneumothorax or bronchiectasis.

Treatment of complex airway lesions after lung transplantation with self-expandable nitinol stents: early experience.

Airway complications (AC) are considered a serious cause of morbidity after lung transplantation (LT). Mechanical dilatation, laser vaporization, and silicone stent placement usually solve it. However, the use of self-expandable metallic stents (SENS) may be indicated in selected cases. Ten lung transplant recipients with AC were treated with SENS. Six patients underwent LT for cystic fibrosis, 2 for idiopathic pulmonary fibrosis, 1 for bronchiectasis, and 1 for emphysema. All patients received at least 1 treatment attempt with dilatation and silicone stent placement. The indications for SENS placement were the presence of a tortuous airway axis with stenosis and malacia of the right main bronchus in 5 patients; a long stenosis of the main and intermediate right bronchus involving the upper lobe orifice in 3 patients; or malacia that could not be stabilized with silicone stents in 3 cases. In 1 patient the procedure was bilateral. Functional improvement was immediate with a mean forced expiratory volume at 1 second (FEV(1)) gain of 35%. No stent dislocation was observed. Symptoms did not occur again in 5 patients with previous recurrent episodes of pneumonia. One stenosis, which was due to the ingrowth of granulation tissue occurred at 6 months after the procedure, was successfully treated with mechanical dilatation and laser vaporization. The deployment of SENS in a selected group of patients with AC after LT was easy, safe, and effective.

Extracorporeal circulatory support for lung transplantation: institutional experience.

Lung transplantation (LT) represents the only available therapy for selected patients affected by end-stage pulmonary disease. Cardiopulmonary bypass (CPBP) is used, when required, during single and sequential double lung transplantation; however, it increases the risk of bleeding, early graft dysfunction, failure, and other potential side effects. We report our experience with 145 patients who underwent lung transplantations, among whom 34 required intraoperative CPBP. The indications for LT among these 34 patients were cystic fibrosis (n = 22), chronic obstructive pulmonary disease (n = 3), bronchiectasis (n = 2), primary pulmonary hypertension (n = 1), fibrosis (n = 2), pulmonary microlithiasis (n = 1), and retransplantation for obliterative bronchilitis (n = 3). CPBP was planned in 12 cases (group I) and unplanned in 22 (group II). The main reason for planning CPBP was primary and secondary pulmonary hypertension (mean pulmonary artery pressure >or=25 mm Hg). Acute right ventricular failure, hemodynamic instability, arterial desaturation, and increased pulmonary artery pressure were mandatory for unplanned CPBP. Among the 34 CPBP patients, the 30-day mortality rate was 35% (12/34) including 9 (70%) in group II (unplanned CPBP). The leading cause of death was multiorgan failure. The 1-year survival rates were 67% and 36%, and the 3-year survival rates were 47% and 18% for groups I and II, respectively. In conclusion, even if it represents a useful tool in the management of critical events, the use of unscheduled CPBP during LT procedures is associated with an increased postoperative morbidity and mortality.

Surgical treatment of redundancy after retrosternal esophagocoloplasty.

Redundancy is a well-recognized complication of esophageal replacement with colonic interposition, occurring several years after surgery. In a small number of patients, symptoms are disabling and might require reoperation. This article describes the surgical treatment of a 54-year-old male presenting with severe dysphagia, malnutrition and recurrent aspiration pneumonia, progressively developed 30 years after esophageal replacement with retrosternal ileocolonic interposition for caustic strictures.

Sub-lobar lung resection of peripheral T1N0M0 NSCLC does not affect local recurrence rate.

BACKGROUND AND AIMS: The use of sub-lobar resection versus lobectomy for stage I non small cell lung cancer is still controversial. This study was undertaken to compare the results of limited resection in terms of survival and local recurrence rate to lobectomy in patients with peripheral stage I non small cell lung cancer. MATERIAL AND METHODS: During the 8 year period from 1999 to 2007, 152 consecutive patients with stage I non-small cell lung cancer underwent lung resection at our thoracic surgery unit. In 116 cases we performed a standard lobectomy while in the remaining 36 cases we did sub lobar resection through mini-thoracotomy or video-assisted thoracoscopy. The survival, local recurrence rate and the clinical outcome were analyzed and compared. RESULTS: Fifty-one patients were staged as T1 N0 M0, 22 in the sub-lobar resection group (61,1%) and 29 (25%) in the lobectomy group. The remaining were staged as T2 N0 M0. Although the patient population undergone to sub-lobar resection was older, with poorer lung function and more co-morbidities, the Kaplan-Meier survival proportion at 5 year did not differ significantly between the two groups: 64% for lobectomy group vs 66,7% for sub-lobar resection group. Overall local recurrence did approach significance in favour of lobectomy group but analyzing only T1 patients, no differences in terms of survival and local recurrence rate were observed. CONCLUSIONS: The results of this study indicate that in patients with peripheral T1N0M0 non small cell lung cancer the outcome of limited resection is comparable with that of pulmonary lobectomy.

Lung transplantation for cystic fibrosis: ten years of experience.

Lung transplantation represents the only therapeutic option for patients affected by end-stage cystic fibrosis (CF). We performed 76 lung transplantations in 73 patients from 1996-2007. The mean time on the waiting list was 10+/-6 months. The median follow-up after the transplantation was 69.3 months. Twenty-one transplants (27.6%) were performed under cardiopulmonary bypass. Perioperative mortality, excluding retransplants, was 16.4% (12 patients) and the causes of death were sepsis, primary graft failure, and myocardial infarction. The overall survival was 74.5%+/-5%, 62.9%+/-5%, 54.1%+/-6%, and 43.4%+/-6% at 1, 3, 5, and 10 years, respectively. The accurate selection of potential recipients and the correct timing of referral and transplantation are factors that play crucial roles to obtain satisfactory results in term of improvement of quality of life and long-term survival.

Preoperative anaemia does not affect the early postoperative outcome in patients with lung cancer.

AIM: Several prognostic factors like age, gender, histology, stage, type of operation, associated disorders and administration of induction therapy have been evaluated to assess the risk of postoperative complications and outcome in patients with resectable lung cancer. Anemia is a frequent condition in this subset of patients being estimated up to 50%. The aim of this retrospective study was to evaluate the effect of preoperative anemia on early outcome after lung cancer resection. METHODS: One-hundred thirty nine consecutive patients undergoing surgery for non small cell lung cancer were retrospectively considered. The mean age was 64.8+/-11.6 years. No patient received blood transfusions or administration of erythropoetin preoperatively. Overall, we performed 96 lobectomies, 14 pneumonectomies, 2 bilobectomies and 27 atypical resections. A subset of 27 patients (19.4%) (group I) had a preoperative value of Hb less than 12 g/dl (10.4+/-1.9 g/dL). Seven patients of them were stage IA (26%), 9 stage IB (33.3%), 2 stage IIA (7.4%), 6 stage IIB (22.2%), 2 stage IIIA (7.4%) and 1 stage IIIB (3.7%). Age, gender, stage, type of operation, induction chemotherapy, comorbidities were evaluated by univariate analysis comparing patients with and without preoperative anaemia. The two groups were homogenous regarding demographic characteristics. RESULTS: Three patients (11.1%) in group I and 2 (1.8%) in group II required blood transfusions after surgery (P=0.01); 4 of them received pneumonectomy (P<0.0001). The overall morbidity was 17.9% (25/139); the most frequent complication was persistent air leakage, followed by retention of secretions. No statistically significant difference was observed between the 2 groups about early mortality (1 patient-3.7% in group I and 2 patients-1.8% in group II) and postoperative complications (5 patients-18.5% in group I and 20 patients-17.9% in group II). CONCLUSION: Preoperative anaemia is not a risk factor for an increased rate of postoperative complications and should not be considered a contraindication to surgery.

Non-Hodgkin's lymphoma, presenting as an isolated endobronchial mass after bilateral lung transplantation: a case report.

BACKGROUND: Lymphoma is a serious complication following solid organ transplantation. Endobronchial involvement of non-Hodgkin's lymphoma (NHL) is rare usually occurring in the setting of widely disseminated disease. Only a few cases of isolated endobronchial NHL have been reported. They were mostly confined to the main bronchi. METHODS: Herein we have described a case of an obstructive endobronchial NHL lesion in a young patient, which developed 6 years after bilateral lung transplantation for cystic fibrosis. RESULTS: Successful treatment was obtained with endoscopic resection of the lesion followed by chemotherapy. CONCLUSIONS: In rare cases NHL may present as an isolated endobronchial mass in the main bronchi. Because in these cases the prognosis seems to be better, early diagnosis is essential. Rigid bronchoscopy offers the opportunity to safely obtain large amounts of tissue for complete histological diagnosis and to de-obstruct the airway to relieve the symptoms.

Malignancies following lung transplantation.

During the last 2 decades, long-term survival after lung transplantation has significantly improved. However, among the complications related to the continuous administration of immunosuppressive drugs, malignancy plays an important role. We retrospectively revisited our series of patients to report our experience. From January 1991 we performed 134 lung transplantations in 128 recipients (mean age, 33.4 +/- 13.5 years). In all patients the first-line immunosuppressive regimen was based on a calcineurin inhibitor (cyclosporine or tacrolimus), an antimetabolic agent (azathioprine), and steroids. Five patients (4.2%) developed malignancy and the mean time of occurrence after the transplantation was 46.4+/-23 months. The mean age was 41 +/- 16 years (P = not significant [ns]). The tumors were as follows: laryngeal cancer (radiotherapy), colon cancer (surgery plus adjuvant chemotherapy), gastric cancer (surgery plus adjuvant chemotherapy), endobronchial non-Hodgkin lymphoma (NHL) (endoscopic resection plus chemoradiotherapy), and cutaneous and visceral Kaposi's sarcoma (KS) (chemotherapy). All patients have reduced the dose of immunosuppressive drugs; in 1 of them, tacrolimus was changed to rapamycin. Two patients died because of neoplastic dissemination, another 1 due to obliterans bronchiolitis. The 2 patients with NHL and KS are alive at 6 and 9 months, respectively, without signs of recurrence. Malignancies after lung transplantation represent an important problem. A multidisciplinary approach is mandatory to obtain satisfactory results in terms of improved quality of life and long-term survival.

[Surgical treatment of iatrogenic perforations of the distal third of the esophagus. Personal experience]. / Trattamento chirurgico delle perforazioni iatrogene del terzo inferiore dell'esofago. Esperienza personale.

AIM: The esophageal perforations are associated with a high mortality and morbidity when they are not diagnosed and treated quickly. The aim of our study is to analyze the treatment and prognosis of the distal iatrogenic esophageal perforations on the basis of time of onset, concomitant disease and size of perforations. METHODS: The retrospective review was performed on 10 patients treated for distal iatrogenic esophageal perforations at our Institution from 1994 to 2003. The cause of perforations was: pneumatic dilation (7 patients) and esophageal endoprosthesis placing (3 patients). Seven patients presented within 24 h (Group A), and 3 patients presented after 24 h (Group B). In Group A, 4 patients underwent primary repair, 2 patients required esophagectomy and 1 patient was treated conservatively. In Group B, 2 patients were treated conservatively and 1 patient required an esophagectomy. RESULTS: Hospital morbidity was 20% and mortality was 30%. In Group A no patients died. In Group B hospital mortality was 100%. The most common cause of death was multiorgan failure resulting from sepsis. CONCLUSIONS: The prognosis for esophageal perforations is influenced by the time elapsed between diagnosis and treatment. Esophagectomy is indicated for patients with extensive perforation and necrosis of the esophagus when primary repair cannot be carried out. It is indicated also as treatment for the concomitant disease.

[Mechanical cervical esophagogastric anastomosis after esophagectomy for cancer]. / Anastomosi meccanica esofagogastrica dopo esofagectomia per neoplasia.

AIM: Fibrous stenosis of the esophagogastric cervical anastomosis remains a significant complication occurring in up to one third of cases. Trying to reduce the incidence of this complication, we describe our technique of cervical esophago-gastric anastomosis using endoscopic linear stapler which seems to reduce the incidence of fibrous stricture formation after resection of esophageal cancer. METHODS: Between March 2000 and December 2004, 34 patients (20 males and 14 females) underwent esophagectomy using tubulized stomach for reconstruction. Mean age was 57 years. Eight patients with advanced stage (5 T3 and 3 T4) underwent induction chemotherapy. The most of patients was affected by squamous cell carcinoma. In all cases we performed cervical esophagogastric anastomosis using linear endoscopic stapler. The occurrence of postoperative anastomotic leak and development of anastomotic stricture were recorded and analyzed. RESULTS: All patients survived esophagectomy and 30 of them (88%) were available for postoperative follow-up at 6 months. Anastomotic leak developed in 1 case. No patient developed fibrous stenosis that required dilatation therapy. CONCLUSIONS: Complete mechanical esophago-gastric anastomosis, using endoscopic linear stapler is effective and safe, even when a narrow gastric tube is used as esophageal substitute. These technique seems superior to other techniques to reduce the incidence of postoperative anastomotic complications.

Lung cancer after treatment for non-Hodgkin lymphoma.

AIM: Because of the improvement in treatment and survival of patients with lymphoma, late sequelae, including secondary cancers have been extensively studied. Lung cancer is one of the two most common solid tumors after Hodgkin's disease but fewer studies have been published about lung cancer after non-Hodgkin lymphoma (NHL). METHODS: Over the last five years at our Institution we have observed 16 patients, 13 male and 3 female, with a mean age of 61 years, previously treated for NHL and lung cancer. Median latency between NHL and lung cancer was 7 years. In 6 patients (37.5%) the latency period was shorter than 5 years and 3 of them developed lung cancer within 2 years after the end of NHL therapy. RESULTS: Ten patients underwent lung complete resection. Two, 3 and 5 year survival rate was respectively 52.7%, 26.3% and 13%. In contrast, the median survival of non surgical patients was 9 months. Comparison of survival between surgical and non-surgical group demonstrated a statistically significant better survival for surgically treated patients (P<0.04). CONCLUSIONS: Surgery can improve survival in patients with history of NHL and lung cancer. Early diagnosis and treatment is crucial. NHL survivors should undergo careful follow-up and surveillance for secondary malignancy.