Mediastinal Involvement Research Articles

Invasive Mediastinal Mucormycosis with Pulmonary and Cardiac Involvement in an Adult with Chronic Granulomatous Disease: Case Report and Review of the Literature.

Mucormycosis is a very rare cause of mediastinal mass, and has a high risk of mortality.Early recognition and treatment will likely increase the patient's chances of survival.Chronic granulomatous disease (CGD) is associated with an increased risk of fungal infections and should be considered for itraconazole prophylaxis.

Lung carcinoid tumors with Diffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) exhibit pejorative pathological features

IntroductionDiffuse Idiopathic Pulmonary NeuroEndocrine Cell Hyperplasia (DIPNECH) is a rare disease often associated with carcinoid tumors. We aimed at evaluating the impact of DIPNECH on characteristics and prognosis of patients who underwent radical treatment of pulmonary carcinoid tumors. Material and methodsWe reviewed all patients operated on for curative-intent resection of carcinoid tumor in our department from 2001 to 2020. Cases exhibiting both pathological and radiological features of DIPNECH, as assessed by respective thoracic expert physicians, were analyzed separately. Results172 cases of resected carcinoid tumors were identified, including 25 (14.5 %) harboring pathological criteria of DIPNECH and radiologic features like mosaic attenuation (92.0 %), multiple nodules < 5 mm (76.0 %), and mucoid impactions (32 %). In DIPNECH patients, major pulmonary resections were usually performed (92.0 %) and resected tumors were mostly classified as pT1 (92 %). Mean Ki67 staining was 3.7 ± 5.2 %. The early postoperative period was mostly uneventful (96.0 %) and 5-year survival was 92.9 ± 6.9 %. Compared to non-DIPNECH cases, we found that patients were older (mean 65.6 ± 9.3 versus 54.1 ± 17.9, p = 0.002), more frequently female (84.0 % versus 56.5 %, p = 0.009), and exhibiting diabetes mellitus (45.8 % versus 18.5 %, p < 0.001) or hypertension (45.8 % versus 24.1 %, p = 0.039). The rate of atypical carcinoid tumors was significantly higher in DIPNECH patients (40.0 % versus 19.9 %, p = 0.027), as well as rate of mediastinal lymph-nodes involvement (pN2+) (36.0 % versus 4.1 %, p < 0.001). At multivariate analysis, only DIPNECH pattern and atypical histology were independent factors of pN2 invasion which was the only predictor of poorer prognosis on Log-Rank test. ConclusionCarcinoid tumors with proven DIPNECH are associated with negative pathological features and may deserve a dedicated perioperative management.

Selection of Mediastinal Lymphoma Patients for Proton Therapy Within the Proton Collaborative Group Registry: Concordance With the ILROG Guidelines.

As patients with mediastinal lymphoma are typically young with curable disease, advanced radiation techniques such as proton therapy are often considered to minimize subacute and late toxicity. However, it is unclear which mediastinal lymphoma patients are treated with proton therapy. Within a prospective, multi-institutional proton registry, we characterized mediastinal lymphoma patients treated with proton therapy and assessed concordance with consensus recommendations published in 2018 by the International Lymphoma Radiation Oncology Group (ILROG). Eligible patients included those with lymphoma of the mediastinum treated exclusively with proton therapy for whom digital imaging and communications in medicine (DICOM) treatment data were available for review. Given the challenge with reliably visualizing the left mainstem coronary artery, the inferior-most aspect of the left pulmonary artery (PA) was used as a surrogate. Extent of disease was characterized as upper mediastinum (above level of left PA), middle mediastinum (below left PA but at or above level of T8), or low mediastinum (below T8). Between November 2012 and April 2019, 56 patients were treated and met inclusion criteria. Patients treated with proton therapy were young (median, 24 y; range: 12 to 88), with over half being female (55%). Patients were most commonly treated at initial diagnosis (86%) and had Hodgkin lymphoma (79%). Most patients (96%) had mediastinal disease that extended down to the level of the heart: 48% had middle and 48% had low mediastinal involvement. Nearly all patients (96%) met the ILROG consensus recommendations: 95% had lower mediastinal disease, 46% were young females, and 9% were heavily pretreated. Heart (mean) and lung dose (mean, V5, V20) were significantly associated with lowest extent of mediastinal disease. Mediastinal lymphoma patients treated with proton therapy are typically young with lower mediastinal involvement. Within a prospective, multi-institutional proton registry, nearly all treated patients fit the ILROG consensus recommendations regarding which mediastinal lymphoma patients may most benefit from proton therapy.

Stage III N2 non-small cell lung cancer treatment: decision-making among surgeons and radiation oncologists.

BackgroundStage III N2 non-small cell lung cancer (NSCLC) is a very heterogeneous disease associated with a poor prognosis. A number of therapeutic options are available for patients with Stage III N2 NSCLC, including surgery [with neoadjuvant or adjuvant chemotherapy (CTx)/neoadjuvant chemoradiotherapy (CRT)] or CRT potentially followed by adjuvant immunotherapy. We have no clear evidence demonstrating a significant survival benefit for either of these approaches, the selection between treatments is not always straightforward and can come down to physician and patient preference. The very heterogeneous definition of resectability of N2 disease makes the decision-making process even more complex.MethodsWe evaluated the treatment strategies for preoperatively diagnosed stage III cN2 NSCLC among Swiss thoracic surgeons and radiation oncologists. Treatment strategies were converted into decision trees and analysed for consensus and discrepancies. We analysed factors relevant to decision-making within these recommendations.ResultsFor resectable “non-bulky” mediastinal lymph node involvement, there was a trend towards surgery. Numerous participants recommend a surgical approach outside existing guidelines as long as the disease was resectable, even in multilevel N2. With increasing extent of mediastinal nodal disease, multimodal treatment based on radiotherapy was more common.ConclusionsBoth, surgery- or radiotherapy-based treatment regimens are feasible options in the management of Stage III N2 NSCLC. The different opinions reflected in the results of this manuscript reinforce the importance of a multidisciplinary setting and the importance of shared decision-making with the patient.

Spanish Lung Cancer Group SCAT trial: surgical audit to lymph node assessment based on IASLC recommendations

BackgroundThe Spanish Customized Adjuvant Therapy (SCAT) trial assessed the role of individualized adjuvant therapy in clinical N0 incidental pN1 and/or N2 non-small cell lung cancer (NSCLC) completely resected. We assessed surgical topics with an in-depth analysis of quality of lymphadenectomy based on International Association for the Study of Lung Cancer (IASLC) recommendations.MethodsPatients with information about lymphadenectomy available were included (N=451). Prospectively collected data about tumor, type of resection, and postoperative morbidity and quality of lymph node dissection (LND) were retrospectively evaluated. Role of lymph node assessment on survival was analyzed using Kaplan-Meier curves, using regression models to identify prognostic factors.ResultsIn 33.7%, 17.7% and 49.9% of cases, regions 7, 10 and 11 respectively were not assessed. In 21.1% of patients, less than three lymph node regions were biopsied, while in 19.6% of patients less than six lymph nodes were assessed. In 53,4% of patients only one N1 region was evaluated. From patients with positive N2, 8.9% had no N1 regions biopsied. Twenty-nine percent of patients with at least one N2 lymph node resected shown the highest region involved. Thirty-day postoperative mortality was unknown. Five-year overall survival (OS) was 61.7% (95% CI: 55.4–67.4%), 51.5% (95% CI: 39.2–62.4%) and 42.3% (95% CI: 32.1–52.2%) for patients with N1, N2 and N1+N2 disease, respectively (P<0.01). Both number of lymph nodes resected and number of lymph nodes involved by tumor were significantly related to prognosis.ConclusionsIASLC recommendations for surgical resections were not followed in a high proportion of surgical procedures. Hilar and mediastinal lymph node assessment and involvement showed to impact prognosis. Surgical issues such as postoperative mortality could not be evaluated owing to trial design.

Characterization of DLBCL with a PMBL gene expression signature

AbstractPrimary mediastinal large B-cell lymphoma (PMBL) is a type of aggressive B-cell lymphoma that typically affects young adults, characterized by presence of a bulky anterior mediastinal mass. Lymphomas with gene expression features of PMBL have been described in nonmediastinal sites, raising questions about how these tumors should be classified. Here, we investigated whether these nonmediastinal lymphomas are indeed PMBLs or instead represent a distinct group within diffuse large B-cell lymphoma (DLBCL). From a cohort of 325 de novo DLBCL cases, we identified tumors from patients without evidence of anterior mediastinal involvement that expressed a PMBL expression signature (nm-PMBLsig+; n = 16; 5%). A majority of these tumors expressed MAL and CD23, proteins typically observed in bona fide PMBL (bf-PMBL). Evaluation of clinical features of nm-PMBLsig+ cases revealed close associations with DLBCL, and a majority displayed a germinal center B cell–like cell of origin (GCB). In contrast to patients with bf-PMBL, patients with nm-PMBLsig+ presented at an older age and did not show pleural disease, and bone/bone marrow involvement was observed in 3 cases. However, although clinically distinct from bf-PMBL, nm-PMBLsig+ tumors resembled bf-PMBL at the molecular level, with upregulation of immune response, JAK-STAT, and NF-κB signatures. Mutational analysis revealed frequent somatic gene mutations in SOCS1, IL4R, ITPKB, and STAT6, as well as CD83 and BIRC3, with the latter genes significantly more frequently affected than in GCB DLBCL or bf-PMBL. Our data establish nm-PMBLsig+ lymphomas as a group within DLBCL with distinct phenotypic and genetic features. These findings may have implications for gene expression– and mutation-based subtyping of aggressive B-cell lymphomas and related targeted therapies.

Joseph V. Simone: In Memoriam (1935–2021)

![][1] He was a poet. A prodigious writer. A giant in the world of pediatric oncology. Those of us in the field of cancer research, as well as patients around the globe, owe a tremendous debt of gratitude to Joseph “Joe” Simone. A first-generation American who was born amid the

A Validation Study of the Recommended Change in Residual Tumor Descriptors Proposed by the International Association for the Study of Lung Cancer for Patients With pN2 NSCLC

IntroductionThis study aimed to validate the residual tumor (R) descriptors proposed by the International Association for the Study of Lung Cancer (IASLC) for patients with pathologic N2 (pN2) NSCLC. MethodsWe retrospectively reviewed the data of patients with pN2 NSCLC who underwent anatomical resection during the period 2004 to 2018. The R status classified using the Union for International Cancer Control (UICC) criteria was compared with that reassigned using the IASLC criteria. Survival analysis was performed using Cox proportional hazards models to assess the prognostic significance of IASLC R descriptors. ResultsAmong 1039 patients, 91.1%, 8.1%, and 0.8% of the patients respectively received complete resection (R0), R1, and R2 defined using the UICC criteria, whereas 41.6%, 20.4%, and 38% respectively received R0, uncertain resection (R[un]), and R1/2 resection defined using the IASLC criteria. Furthermore, 206 patients (21.8%) were reclassified from having UICC R0 to having IASLC R(un) mainly owing to the highest mediastinal lymph node involvement (89.8%). Owing to extracapsular extension, 309 patients (32.6%) with UICC R0 were reclassified as having IASLC R1/2. Patients with IASLC R(un) had significantly worse and better prognosis than those with IASLC R0 and IASLC R1/2, respectively. In multivariable analysis, the prognostic difference between IASLC R0 and R(un) was similar after adjustment for subdivided N2 descriptors including pN2a1, pN2a2, and pN2b. ConclusionsAlong with subdivided N descriptors, detailed R descriptors proposed on the basis of the IASLC criteria can ensure optimal staging, enabling the most appropriate decision-making on adjuvant therapy for patients with pN2 NSCLC.

Prognostic significance of histopathological factors in survival and recurrence of atypical carcinoid tumours.

Atypical carcinoids are neuroendocrine neoplasms of intermediate degree and low frequency. The aim of this study is to analyse their clinical characteristics and the importance of different histopathological factors in their prognosis. Multicentre cooperative group EMETNE prospectively reviewed 153 patients operated on between 1998 and 2016 with diagnosis of atypical carcinoids. Clinical variables and histopathological features were assessed. Mean age was 54.36 years, similar for both genders. Concerning pathological study, mean tumour size was 31.7 mm. Rosettes were presented in 17% of the cases and tumoural necrosis in 23.3%. The cell proliferation factor Ki-67 index was 10.7%. The 2- and 5-year overall survival rates were 95.8% and 88.9%, respectively. In the univariate study, statistically significant differences in survival were found for each of the categories of T, N and M factors. Mitotic index and quantification of expression of Ki-67 showed influence in overall survival, although without statistical significance. In the multivariate analysis, factors N, M and mitotic index behaved as independent prognostic factors related to survival. Median disease-free interval in the series was 163.35 months. In cases with loco-regional recurrence, 53% had positive hiliar or mediastinal nodal involvement at the time of the surgery. In the univariate analysis, we observed statistically significant differences in disease-free interval in patients with nodal involvement (P = 0.024) and non-anatomical resections (P = 0.04). Histological characteristics showed no statistically significant differences in disease-free interval. Lymph node involvement, the development of distant metastasis and mitotic index, more than Ki-67 determination, were shown as independent prognostic factors related to survival of these patients.

Akut Lenfoblastik Lösemide Flow Sitometri ile İmmünofenotiplemenin Prognoz Üzerine Etkileri

Background: The identification of immunophenotype subgroups is very important for the diagnosis and prognosis of acute lymphoblastic leukemia(ALL). Material and Methods: The study included 105 children with ALL(65 males, 40 females; mean age 5.9±3.8 years) who were treated TR-ALL 2000(modified) BFM treatment protocol. Results: The distributions of EGIL classification were pro-B ALL(n=1), common B ALL(n=46), pre-B ALL(n=40), pre-T ALL(n=8), cortical T ALL(n=6), and mature T ALL(n=4). Leukocyte≥100,000/mm³, lymphadenopathy≥2 cm, mediastinal involvement were commonly identified in T ALL group. T ALL had a poor response to chemotherapy according to 8th-day peripheral circulation blast counts and 15th-day bone marrow aspiration(BMA) blast counts. The recurrence, mortality, and death rate in the induction period of treatment were frequently detected in T ALL group. The variables that had prognostic potential, as indicated by univariate analyses, were leukocyte count, hepatomegaly, splenomegaly, and lymphadenopathy at the time of diagnosis, 8th-day steroid response, 15th-day BMA response, risk group, recurrence, and immunophenotyping. Multivariate Cox regression analysis demonstrated that only the leukocyte count(HR 2.51, p &amp;lt; 0.001) was a predictor of prognosis. Conclusion: Immunophenotyping may be effective in the diagnosis and prognosis of ALL, identification of risk groups, and in risk-based treatment planning. T ALL had a poor prognosis.

The role of 18F-FDG PET/CT metabolic parameters in pediatric lymphoblastic lymphoma

ObjectiveThis retrospective study aimed to evaluate the role of metabolic parameters of 18F-FDG PET/CT in pediatric lymphoblastic lymphoma (LBL). MethodsThirty patients with LBL underwent 42 scans. Metabolic parameters including maximum standardized uptake value (SUVmax), total metabolic tumor volume (TMTV), and total lesion glycolysis (TLG) were measured at baseline PET/CT. Univariate and multivariate analysis for survival were performed to assess their prognostic value. Twelve patients underwent PET/CT after reinduction regime, and the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of PET/CT for predicting relapse were calculated. ResultsPatients with stage Ⅳ had a higher TMTV than those with stage III (p = 0.031). Besides, patients with T-LBL or mediastinal involvement had a high TMTV and TLG (p < 0.05). There was no significant difference in PET/CT metabolic parameters between patients with different outcomes (p > 0.05). Children with a low TMTV (<242.91 cm³) had a better 3-year EFS compared with those with a high TMTV (88.9% vs. 56.3%; p = 0.036). SUVmax and TLG were not predictive of EFS (p = 0.874; p = 0.152). However, none of the metabolic parameters of baseline PET/CT were independent prognostic factors for outcomes of pediatric LBL. PET/CT underwent after reinduction regime present with higher sensitivity (50% vs. 0%) and NPV (90% vs. 83.3%) for predicting relapse than CT alone. ConclusionsMetabolic parameters of baseline PET/CT were not predictive of outcomes in children with LBL. PET/CT done after the reinduction regime had better sensitivity and NPV than CT alone, and a negative scan could be a reliable indicator for sustained remission.

The Outcomes of Multi-Disciplinary Treatment of Esophageal Cancer in Vajira Hospital

Background: Esophageal cancer is one of the most fatal and difficult-to-treat cancer. Multi-modality management is the key to success of improving outcomes, however, which modality is the most proper is difficult to determine. Objective: To evaluate the overall survival (OS) of patients with early or locally-advanced (E/LA) esophageal carcinoma treated in Vajira Hospital. The outcomes of the multi-modality management among patients with E/LA diseases were evaluated. Materials and Methods: The retrospective analyses of esophageal carcinoma patients who attended at Vajira Hospital between January 1, 2012 and December 31, 2016 were performed. Results: There were 86 patients with complete medical records. The median age was 60.5 years (IQR 52 to 66). Sixty-five patients (75.6%) presented with E/LA diseases. Most of the patients had primary site at thoracic part of esophagus (58 patients, 67.4%) and had squamous cell carcinoma histology (84 patients, 97.7%). Tri-modality treatment including neoadjuvant chemoradiation and esophagectomy for clinically fitted patients without evidence of mediastinal involvement and non-regional lymph node metastasis resulted in the best survival outcome [28.56 months (IQR 10.64 to 46.47)]. The OS of patients with E/LA disease was only 9.15 months (IQR 4.49 to 23.02). Male patients, non-cervical site, and non-surgical treatment were associated with the worse OS. Conclusion: The outcomes of patients with esophageal carcinoma treated in a real-world practice is still not impressive. Tri-modality management would be the best paradigm; however, it is suitable for well-selected patients. Keywords: Esophageal cancer, Multi-modality treatment, Real-world practice

Uveitis as an Open Window to Systemic Inflammatory Diseases.

Spondyloarthritis (Spa), Behçet’s disease (BD) and sarcoidosis are major systemic inflammatory diseases worldwide. They are all multisystem pathologies and share a possible ocular involvement, especially uveitis. We hereby describe selected cases who were referred by ophthalmologists to our internal medicine department for unexplained uveitis. Physical examination and/or the use of laboratory and imaging investigations allowed to make a diagnosis of a systemic inflammatory disease in a large proportion of patients. In our tertiary referral center, 75 patients have been diagnosed with Spa (n = 20), BD (n = 9), or sarcoidosis (n = 46) in the last two years. There was a significant delay in the diagnosis of Spa-associated uveitis. Screening strategies using Human Leukocyte Antigen (HLA)-B27 determination and sacroiliac magnetic resonance imaging in patients suffering from chronic low back pain and/or psoriasis helped in the diagnosis. BD’s uveitis affects young people from both sexes and all origins and usually presents with panuveitis and retinal vasculitis. The high proportion of sarcoidosis in our population is explained by the use of chest computed tomography (CT) and 18F-fluorodeoxyglucose positron emission tomography CT that helped to identify smaller hilar or mediastinal involvement and allowed to further investigate those patients, especially in the elderly. Our results confirm how in these sight- and potentially life-threatening diseases a prompt diagnosis is mandatory and benefits from a multidisciplinary approach.

The Application of the ThroLy Risk Assessment Model to Predict Venous Thromboembolism in Patients with Diffuse Large B-Cell Lymphoma.

BackgroundPatients with aggressive lymphomas are at higher risk for venous thromboembolism (VTE). ThroLy is a risk assessment model (RAM) derived to predict the occurrence of VTE in various types of lymphomas. In this study, we assess the clinical application of ThroLy RAM in a unified group of patients with diffuse large B-cell lymphoma (DLBCL).MethodsHospital databases were searched for patients with DLBCL and radiologically-confirmed VTE. Items in the ThroLy RAM, including prior VTE, reduced mobility, obesity, extranodal disease, mediastinal involvement, neutropenia and hemoglobin < 10.0 g/dL, were retrospectively reviewed.ResultsA total of 524 patients, median age 49 (range: 18-90) years were included. Patients had high disease burden; 57.3% with stage III/IV and 34.0% with bulky disease. All were treated on unified guidelines; 63 (12.0%) had primary refractory disease. Venous thromboembolic events were reported in 71 (13.5%) patients. Among 121 patients with high (> 3) ThroLy score, 22.3% developed VTE compared to 8.4% and 12.4% in those with low and intermediate risk scores, respectively (P = .014). Simplifying the ThroLy model into two risk groups; high-risk (score ≥ 3) and low risk (score < 3) can still segregate patients; VTE developed in 44 (17.2%) high-risk patients (n = 256) compared to 27 (10.1%) in the low-risk group (n = 268), P = .038. Neutropenia, a component of the ThroLy, was encountered in only 14 (2.7%) patients.ConclusionsThroLy RAM can identify patients with DLBCL at high risk for VTE. Model can be modified by dividing patients into two, rather than three risk groups, and further simplified by omitting neutropenia.

The echocardiographic exam for the diagnosis of cardiac involvement in non-Hodgkin lymphoma in childhood

Clinical signs and symptoms of cardiac(Cd) involvement in non-Hodgkin lymphoma(NHL), is frequently undetected and diagnosis could be missed unless a routine echocardiographical investigation(echo) might be performed. To reveal the value and utility of echo for diagnose and follow up the Cd involvement in Nnhl n child, other than that determined by the specific treatment. Authors were studied 38 children: 21 males and 17 females, aged between 3month and 17 years with NHL), hospitalised. In all cases were performed: clinical exam, ECG, chest X-ray and echo. Cd involvement was proved in 6 cases (15, 8%); 5 cases (83%) T-cell and 1 case (17%) B-cell lymphoma; clinical signs on onset like astenia, dyspnea, cough, superior vena cava sdr have been assigned to the base disease. ECG: low voltage of QRS complexes and T waves. Chest X-ray: mediastinal involvement (5 pts) and massive right-side pleural effusion (1 case). Eco aspects: pericardial effusion (4) to cardiac tamponade (2); pericardial tumor (1 case). In 2 cases at first eco exam intra Cd masses: case 1 with a tumoral mass filling the right atrium(RA) with implantation base towards superior vena cava- confirmed later in the autopsy; case 2-tumoral mass extending from the free wall of the RA to the septal tricuspid valve leaflet, associated with an massive pericardial effusion. In the case 2 and in all cases with pericardial effusion on onset:at 1 year the echo turned to normality after chemotherapy. Because of high incidence of Cd involvement in NHL in child and antemortem diagnosis is often difficult, echo is necessary in all cases at the first exam, even if Cd involvement signs are missing, in order to prevent the evolution to Cd tamponade or other Cd emergency. Echo is the most important investigation to diagnose and follow-up the Cd involvement in NHL in child.

Acute lymphoblastic leukemia in Indian children at a tertiary care center: A multiparametric study with prognostic implications

Background: Leukemia is a varied group of hematological malignancies due to uncontrolled proliferation of blast cells. Among childhood leukemias, acute lymphoblastic leukemia (ALL) comprise 70%–80% of all childhood leukemias in India. The current study aims to report the various prognostic markers of disease severity. Methodology: Bone marrow and peripheral blood samples from 20 patients of ALL were subjected to cytogenetic and flow cytometric analysis after recording clinical history and laboratory findings. Patients were classified according to immunophenotyping markers.: For risk stratification, patients were divided into two subgroups B ALL and T ALL. The age group of majority of patients was 1–9 years (90%) with 5% each belonging to 9 years. Male: female ratio was 1.1:1. Results: Hepatosplenomegaly, lymphadenopathy, and mediastinal involvement was found in 45%, 40%, and 5% of patients, respectively. Hemoglobin levels 5 g/dl were seen in 50% of patients in each range. White blood cell counts >50,000 were seen in 3 (15%) of patients. Cytogenetic analysis revealed hypodiploid karyotype for majority (64%) of cases, normal karyotype in 28% and hyperdiploidy in rest (7%). Structural aberrations like t (21;4), del (5p), dic (5) were found all in B ALL subgroup. Patients were stratified into high and standard risk groups based on good and prognostic factors. Conclusions: This study reinforces the significance of immunophenotyping cytogenetics, clinical presentation as a prognostic tool, and their significance in risk stratification.

Minimal invasive surgery in locally advanced N2 non-small cell lung cancer.

Locally advanced lung cancer, defined by nodal involvement in upper mediastinal stations (N2) (stage IIIA–N2), includes a wide spectrum of patients with multiple therapeutic alternatives. Such heterogeneity is explained, at least in part, by tumor size and magnitude of mediastinal nodal involvement. In this setting, many variants can influence the prognosis, such as the specific nodal stations compromised, the burden of mediastinal disease, and the presence of skip metastasis. In the surgical field, the advent of minimally invasive techniques, including video-assisted thoracoscopic and robotic surgery, have revolutionized the management of early-stage lung cancer, but implementations of these approaches in the locally advanced setting have been erratic. This review attempts to highlight the most relevant scientific data of the surgical management of locally advanced lung cancer patients, analyzing not only the medical evidence but also the cost-effectiveness and accessibility.

Atypical CT manifestations of pulmonary sarcoidosis

SummarySarcoidosis is a granulomatous disease of unknown etiol-ogy that affects different organs and tissues. Pulmonary, mediastinal, and intrathoracic lymph nodes involvement occur in about 90% of patients with sarcoidosis. Radio-logical manifestations of pulmonary sarcoidosis vary sig-nificantly. This article describes focal and reticulonodu-lar changes, as well as consolidation zones and fibroticchanges. Isolated reticular changes due to thickening of intra-, and interlobular septa occur in approximately 50% of sarcoid patients. However, this CT pattern pre-vails only in 15–20% of patients. As a rule, in 80% of sar-coid patients changes in lung parenchyma are combined with intrathoracic lymphadenopathy. In the majority of cases, these changes are bilateral. In 10–13% of patients, foci 89–12 mm in diameter are detected. These foci have a homogenous structure and a well-defined outline. They locate mainly along costal and interlobar pleura, in interlobular septa, and may resemble metastases. In 2.4–4% of patients, these small foci, typical for sarcoid-osis are absent, while the changes are presented by larger nodular masses (&gt;2 cm in diameter) or by masses with fuzzy outlines. Often sarcoidosis mimicking interstitial pneumonia is manifested by the ground-glass opacity of spot-like shape. Abnormalities are located in the up-per lobes. This sign occurs in 16–83% of sarcoidotic pa-tients, mainly at the onset of the disease. It usually com-bines with focal changes in the lungs and intrathoracic lymphadenopathy. Cavitary forms of sarcoidosis are also described. Conclusion. There are multiple pulmonary sarcoidosis manifestations on CT, and they vary a lot. The former allows suspecting the diagnosis, which is later confirmed clinically, morphologically, or by lab meth-ods. Аtypical radiological forms of lung sarcoidosis often mimic other pulmonary diseases. These forms require in-vasive diagnostic methods including abdominal surgery.

CD30+ Anaplastic Large-Cell Lymphoma in Children: Analysis of 82 Patients Enrolled in Two Consecutive Studies of the French Society of Pediatric Oncology

AbstractThe purpose of this study was (1) to investigate the efficacy of chemotherapy regimens designed by the French Society of Pediatric Oncology for childhood anaplastic large-cell lymphoma (ALCL) and (2) to identify prognostic factors in these children. Eighty-two children with newly diagnosed ALCL were enrolled in two consecutive studies, HM89 and HM91. The diagnosis of ALCL was based on immuno-morphological features and all the cases but 2 were investigated using ALK1 antibody directed to the NPM/ALK protein associated with the 2;5 translocation. Treatment consisted of 2 courses of COPADM (methotrexate, cyclophosphamide, doxorubicin, vincristine, and prednisone) and a maintenance treatment of 5 to 7 months. Seventy-eight patients (95%) achieved a complete remission and 21 relapsed. The probability of survival and event-free survival at 3 years was of 83% (72% to 90%) and 66% (54% to 76%), respectively, with a median follow-up of 49 months. In multivariate analysis, visceral involvement, mediastinal involvement, and lacticodeshydrogenase (LDH) level ≥800 UI/L were shown to be predictive of a higher risk of failure. In conclusion, this type of regimen demonstrated efficacy in childhood ALCL. However, therapeutic results have to be improved for children with adverse prognostic parameters such as visceral or mediastinal involvement or a high LDH level.

European Task Force on Lymphoma project on lymphocyte predominance Hodgkin disease: histologic and immunohistologic analysis of submitted cases reveals 2 types of Hodgkin disease with a nodular growth pattern and abundant lymphocytes

AbstractParaffin blocks and clinical data from 521 patients with lymphocyte predominance Hodgkin disease (LPHD) diagnosed between 1970 and 1994 were collected from 16 European and United States oncological centers to establish the pathologic and clinical characteristics of a large patient cohort, to determine how frequent T-cell–rich large B-cell lymphoma (TCRLBCL) is among LPHD, and to find differential diagnostic criteria distinguishing between the 2 lymphoma categories. For this purpose, conventionally and immunohistologically stained sections were reviewed by a panel of hematopathologists. The diagnosis of LPHD was confirmed in only 219 of the 388 assessable cases (56.5%). This low confirmation rate was due mainly to the presence of a new variant of classical Hodgkin disease (CHD), which resembled, in terms of nodular growth and lymphocyte-richness, nodular LPHD and, in terms of the immunophenotype of the tumor cells, CHD and was designated nodular lymphocyte-rich CHD (NLRCHD). The nodules of LRCHD consisted—as in nodular LPHD—predominantly of B cells but differed from those present in LPHD in that they represented expanded mantle zones with atrophic germinal centers. Clinically, patients with LPHD and NLRCHD showed similar disease characteristics at presentation but differed in the frequency of multiple relapses and prognosis after relapse. Patients with LPHD and NLRCHD clearly differed from patients with CHD with nodular sclerosis or mixed cellularity, as they presented with an earlier disease stage and infrequent mediastinal involvement. As 97% of the LPHD cases showed a complete or partial nodular growth pattern, their differentiation from TCRLBCL was a rare problem in the present series.