Pemphigus foliaceus (PF) is a rare autoimmune skin disease caused by anti-Dsg1 pathogenic autoantibodies. It is considered as a Th2-mediated disease. Likewise, Th17 cells were recently described in the pathogenesis of the disease but their role is still unclear. We aimed to unravel the eventual implication of the IL23/Th17 pathway in the development of PF. A case-control study was conducted on 115 PF patients and 201 healthy controls using PCR-RFLP and AS-PCR methods. SNPs inIL23R,RORγt,IL17A,IL17F,IL17AR,TNFa, andSTAT3genes were genotyped. mRNA expression ofIL23RandRORγtwas evaluated using Q-PCR. The frequency of circulating Th17 cells was analyzed by flow cytometry. Genetic associations betweenIL23R>rs11209026,IL17A>rs3748067,IL17F>rs763780, andTNFa>rs1800629 and the susceptibility to PF were reported. Moreover, we revealed a significant increased frequency of circulating CD4+IL17+cells as well as higher mRNA levels of RORγt and IL23R in PBMCs of patients. However, no significant increase of RORγt and IL23R mRNA expression was observed in lesional skin biopsies. In spite of the little size of specimens, our results provide converging arguments for the contribution of the IL23/Th17 pathway in the pathogenesis of PF.

Introduction Les anticorps anticytoplasme des polynucléaires neutrophiles (ANCA pour antineutrophil cytoplasmic antibodies) sont des auto-anticorps dirigés contre des constituants antigéniques principalement présents dans les granules primaires des polynucléaires neutrophiles (PNN). Ils sont de pertinents marqueurs diagnostiques et pronostiques des vascularites systémiques associées aux ANCA mais ils peuvent être aussi retrouvés dans diverses maladies inflammatoires ou non. L’objectif de notre étude est de déterminer les principales pathologies associées aux ANCA, préciser leurs cibles antigéniques et leurs corrélations cliniques et diagnostiques. Patients et méthodes Notre étude est rétrospective menée au laboratoire d’immunologie CHU Habib-Bourguiba, Sfax, Tunisie sur une période de 4 ans (janvier 2010 à décembre 2013) portant sur 106 patients avec des ANCA positifs et suivis dans différents services du CHU Hédi-Chaker, Sfax. Les ANCA ont été détectés par immunofluorescence indirecte (IFI). La spécificité antigénique MPO ou PR3 a été déterminée par immunodot. Résultats Durant les 4 années d’étude, 1 927 demandes d’ANCA étaient parvenues à notre laboratoire, 106 (5,5 %) se sont révélées positives. Il s’agissait d’échantillons appartenant à 55 femmes (51,9 %) et 51 hommes (48,1 %). L’âge moyen était de 48±17 ans (17–84 ans). Dans notre série, 45 patients (42,5 %) avaient une maladie inflammatoire chronique (MICI) du tube digestif : RCH dans 36 cas, maladie de Crohn dans 6 cas et colite indéterminée dans 3 cas. Seuls 26 patients (24,5 %) avaient une vascularite systémique des petits vaisseaux. La polyangéite microscopique a été retenue dans 18 cas (17 %), la granulomatose avec polyangéite (GPA) dans 5 cas (4,7 %), la granulomatose éosinophilique avec polyangéite (EGPA) dans 2 cas (1,8 %) et le syndrome de Goodpasture ou maladie des anticorps anti-MBG dans 1 cas (0,9 %). Le diagnostic d’une vascularite induite par le benzylthiouracile était retenu dans 2 cas (1,8 %), d’une glomérulonéphrite extracapillaire pauci-immune dans 3 cas (2,8 %) et d’une péri-artérite noueuse dans 3 cas (2,8 %). Les ANCA étaient présents dans diverses autres pathologies : polyarthrite rhumatoïde dans 3 cas (2,8 %), lupus érythémateux systémique dans 3 cas (2,8 %), maladie de Behçet dans 3 cas (2,8 %), cholangite sclérosante dans 1 cas, sclérodermie systémique dans 1 cas, syndrome de Sjörgen dans 1 cas, maladie de Horton dans 1 cas et syndrome myélodysplasique dans 1 cas. Aucune étiologie n’a pu être déterminée chez 10 malades. Les ANCA étaient dirigés contre la MPO dans 56 cas (52,8 %) et la PR3 dans 9 cas (8,5 %). Le reste était des xANCA (37,7 %). Au moment du diagnostic, les signes généraux étaient objectivés chez 50 malades (47,2 %) et les signes digestifs chez 44 patients (41,5 %). L’atteinte pulmonaire était observée dans 37 cas (35 %) et les manifestations rhumatologiques étaient présentes chez 32 patients (30,2 %). Les manifestations cutanées à type de purpura vasculaire étaient notées chez 5 patients (4,7 %). Les manifestations ORL étaient présentes chez 12 malades (11,2 %) : épistaxis (7 cas), sinusite (3 cas), otite (1 cas) et surdité (1 cas). Une neuropathie périphérique était notée dans 5 cas (4,7 %). L’atteinte rénale était présente chez 39 malades (37 %) révélée par une insuffisance rénale dans tous les cas, une protéinurie (33 cas) et une hématurie (29 cas). La spécificité antigénique MPO et xANCA était significativement corrélée à la présence de signes cliniques digestifs (diarrhée et douleur abdominale, p<0,005), à la présence d’une dyspnée (p=0,039 et 0,05), d’une hémoptysie (p=0,005 et 0,022), d’une hypertension artérielle (p=0,005 et 0,02) et une neuropathie (p=0,004 et 0,017). En revanche, la spécificité PR3 est significativement corrélée à la présence d’otite (p=0,001) et de surdité (p=0,001). Le diagnostic de vascularite des petits vaisseaux et de MICI était significativement corrélé à la présence de MPO et xANCA (p<0,005). Aucune corrélation significative n’a été établie entre les différentes spécificités des ANCA et la glomérulonéphrite extracapillaire (p=0,87). Conclusion Les ANCA contribuent à faciliter le diagnostic précoce et parfois urgent de vascularite et de MICI. Les pièges diagnostiques peuvent être évités par une confrontation des résultats aux données cliniques.

Polymorphism in the genes of TH2 cytokines and/or theirs receptors can influence serum cytokine levels in and the switch to the pathologic IgG4 auto-antibodies. In order to underline the role of these genes in the aethiopathogenesis of Pemphigus Foliaceus, we conduct a familial and a case control studies including 80 Tunisian patients, 147 related subjects and 160 matched healthy controls. We investigated, by PCR-RFLP technique, seven nucleotide polymorphisms: rs2243250 in promoter region of IL4 gene, rs47877948, rs3024530 and rs30246223 in the IL4R gene, rs1881457and rs205412 SNPs in IL13 gene and rs535036 in IL13RA2 gene. After Bonferroni adjustment, T allele and the TT genotype of IL4-590 were significantly increased in the PF patients group compared to healthy controls. This association was confirmed by the family study. Interestingly, the serum IL-4 levels were significantly increased in patients with the TT genotype compared to CT or CC genotypes. Interestingly, the IL4/IL13:T-A-C haplotype exhibited a significant effect on PF susceptibility. In addition, a significant gene-gene interaction between the IL4/IL4R (TACA) significantly increases in PF patients as compared to controls. These findings assess the role of the IL4/IL4R axis in the aethiopathogenesis of Tunisian endemic PF by the induction of a high transcriptional activity which could enhance the T-cell balance and inducing immunoglobulin isotype switching.

Type 1 diabetes (T1D) is caused by an immune-mediated destruction of the insulin-producing β-cells. Several studies support the involvement of T cell activation molecules. In order to underline the role of the genes involved in this pathway, we investigated, using the Sequenom MassARRAY platform, polymorphisms of sixteen single-nucleotide polymorphisms (SNPs) belonging to PTPN22, CD28, CTLA-4, and ZAP-70 genes in 76 T1D patients and 162 unrelated healthy controls from Southern Tunisia. We confirmed the association with PTPN22 (rs2476601, Corrected P (Pcorr)=0.002, OR=6.20) and CD28 gene (rs1879877, Pcorr=0.003; OR=4.27 and rs3181096, Pcorr=0.02; OR=1.73). We also identified an association with rs17695937 of ZAP-70 gene (Pcorr=0.02, OR=1.87). Our results suggest a significant effect on T1D susceptibility for A-C-A-G-C and T-C-C-T-A-C haplotypes, of ZAP-70 and CD28 genes, respectively. In addition, (A-G-C) combination of ZAP-70/CD28 gene was significantly increased in T1D patients as compared to controls, suggesting the possible interaction between these genes. These results confirm the involvement of PTPN22 and CD28 genes in the genetic susceptibility to T1D. Interestingly, ZAP-70 seems to contribute to the susceptibility to the disease in our population. However, this finding has to be confirmed in further studies.

BACKGROUND:Reactive oxygen species play a key role in the development of many dermatological disorders.OBJECTIVE:The purpose of this study is to examine the lipid peroxidation, protein oxidation and antioxidative profile in Tunisian pemphigus foliaceus (PF) patients.METHODS:Malondialdehyde (MDA), conjugated dienes (CD), protein thiol levels, catalase (CAT) and superoxide dismutase (SOD) activities were evaluated in skin biopsies of 13 patients compared to biopsies of 7 healthy controls.RESULTS:Oxidative stress was confirmed in these three types of patient biopsies as compared to controls. Thus, MDA, CD levels and catalase CAT and SOD activities were significantly increased in lesional, perilesional and normal biopsies of PF patients than in those of control subjects. Protein oxidative was confirmed by lower levels of protein thiols in lesional, perilesional and normal biopsies than in control's biopsies. Otherwise, in patients, a significant rise of these biomarkers was observed in lesional and perilesional biopsies compared with normal biopsies.CONCLUSION:This study shows that oxidative stress could be involved in the pathogenesis of PF by the spread of skin lesions and/or by the increase in auto-antibodies' reactivity.

Le syndrome des antisynthétases est un syndrome associant une myopathie inflammatoire (polymyosite ou dermatomyosite), une pneumopathie interstitielle diffuse, une polyarthrite, un phénomène de Raynaud et une atteinte cutanée type « mains de mécaniciens » associés à des auto-anticorps (AAC) antiaminoacyl transfert RNA synthétases, le chef de file étant l’anti-Jo1 (anti-histidyl-tRNAsynthétase). À travers ce travail, nous décrirons les particularités cliniques, thérapeutiques, évolutives et les facteurs pronostiques de cette entité au sein des myosites. Il s’agit de quatre femmes, âgées en moyenne de 42ans (28–62ans). L’atteinte pulmonaire était retrouvée dans tous les cas à type de pneumopathie interstitielle objectivée par l’examen tomodensitométrique. Les anticorps anti-Jo1 étaient positifs chez les quatre patientes. La symptomatologie pulmonaire révélatrice de la pneumopathie interstitielle était sous forme de dyspnée (un cas) et sous forme d’un syndrome de détresse respiratoire aiguë (un cas). Chez ces deux femmes, la pneumopathie interstitielle était elle-même révélatrice du syndrome des antisynthétases. Toutes nos patientes ont été traitées par une corticothérapie. Un traitement immunosuppresseur à base de bolus mensuels de cyclophosphamide était indiqué chez deux patientes devant la sévérité de l’atteinte pulmonaire. L’évolution était favorable chez deux patientes, partiellement favorable chez une patiente qui a évolué vers la fibrose pulmonaire. Un décès était déploré chez la quatrième patiente par la survenue de multiples abcès cérébraux.

PURPOSE:The objective of this study was to determine the clinical relevance and the diagnostic significance of positive antinuclear antibodies (ANA) without identified antigenic target by the usual characterization technique. PATIENTS AND METHODS:Retrospective study conducted in the Laboratory of Immunology of Habib Bourguiba Hospital (Sfax, Tunisia) during 18 months. The inclusion criteria were the presence of an ANA titer greater or equal to 1/320 with negative characterization result. ANA screening was performed by indirect immunofluorescence (IIF) on Hep2 cells. Each positive serum was tested by IIF on Crithidia luciliae (anti-native DNA) and by immunodot (anti-nucleosome, anti-histone, anti-Sm, anti-RNP, anti-SSA, anti-SSB, anti-Scl 70, anti-PM-Scl, anti-Jo1, anti-PCNA and anti-ribosomal protein). Sera of systemic lupus erythematosus (SLE), myositis, and scleroderma patients were tested for anti-Ku, anti-PL7, anti-PL12 and anti-Ro-52 using dot myositis. RESULTS:Sera of 90 patients were studied: 18 men and 72 women (average age: 44 years). Drug-induced ANA was found in eight patients. The most frequent clinical symptoms were joint (56.7%), cutaneous (54.4%) and constitutional symptoms (45.6%). The diagnosis of an autoimmune disease was suspected in 49 patients (54.5%) and confirmed in 30 (33.3%) including 20 cases of connective tissue disease: myositis (n=6), scleroderma (n=5), Sjögren's syndrome (n=3), SLE (n=4), rheumatoid arthritis (n=6) and antiphospholipid syndrome (n=4). Other autoimmune diseases were less frequent. The anti-Ku antibody was detected in the majority of patients with connective tissue disease. The diagnosis of non-autoimmune diseases was established in 25.5% of patients. Eighteen patients (20%) had no diagnosis orientation. CONCLUSION:Our study demonstrated the diagnostic value of the presence of ANA even in the absence of known antigenic target, confirmed the role of the IIF as "gold standard" test for ANA screening, and suggested the usefulness of the addition of Ku antigen in the immunodot classic profile.

Crohn's disease (CD), ulcerative colitis (UC), systemic lupus erythematosus (SLE) and autoimmune polyglandular syndromes (APS) are autoimmune diseases (ADs) that may share common susceptibility pathways. We examined ribonucleo-protein, polypyrimidine tract-binding protein (PTB)-binding 2 (RAVER2) loci for these diseases in a cohort of 39 CD cases, 67 UC cases, 93 SLE cases, 60 APS cases and 162 healthy control subjects of Tunisian origin. We genotyped 3 SNPs of RAVER2 (rs2780814, rs1333739 and rs2780889) and evaluated it genetic association with each ADs, using X2-test. For each association, odds ratio (OR) and 95% CI were calculated. We show that rs2780814 is significantly associated with UC (P = 0.00016, P(corr) = 0.00048, OR = 3.66 (1.82; 7.34)). We also observed a trend of possible association to SLE (P = 0.023, P(corr) = 0.69, OR = 2.19 (1.1; 4.36)). None of these RAVER2 SNPs were associated with CD and APS susceptibility. These findings establish RAVER2 as a new UC genetic susceptibility factor and reveal a genetic heterogeneity of the associated polymorphisms and risk alleles between ADs suggesting different immunopathological roles of RAVER2 in these diseases.

The antisynthetase syndrome (ASS) includes inflammatory myopathy (polymyositis or dermatomyositis), interstitial lung disease (ILD), arthritis, Raynaud's phenomenon, and mechanic's hands, associated with antibodies against aminoacyl-tRNA-synthetases, the most well-recognized being the anti-Jo1 antibody (anti-histidyl-tRNAsynthetase). We report four cases of antisynthetase syndrome and review the clinical characteristics and prognosis factors dominated by ILD. We report the cases of four women with a mean age of 42 years (28-62 years). The interstitial lung disease was found in four cases and was objectified by CT-scan in all cases. The pulmonary symptoms were consisted of dyspnea (one case) and respiratory distress (one case). The anti-Jo1 antibodies were present in the four patients. The myopathy was concomitant with pulmonary involvement (two cases), preceded it in 6 months (one case) and in the course of evolution and after 1 month (one case). All patients received corticosteroid treatment. The immunosuppressive treatment was necessary for two patients because of the severity of the pulmonary involvement. The outcome was favorable in two patients, partially favorable in a patient who presented pulmonary fibrosis. However, one patient died after developing brain abscesses.

Objectives. – The aim of our study was to investigate the association of HLA-DRB1 and HLA-DQB1 alleles with autoimmune polyglandular syndromes (APS) type II and III in a southern Tunisian population. Patients and methods. – Sixty-two unrelated patients with APSII (n = 20) and APSIII (n = 42) and 146 healthy controls were genotyped for HLA class II alleles (DRB1*, DQB1*) by PCR-SSP technique. Results. – An increased frequencies of HLA-DQB1*03:02 (P = 0,02; OR = 2.98) in APSII patients, HLA-DRB1*03 (P = 3 10−6; OR = 4.28) and HLADQB1*02:01 (P = 0.04; OR = 1.95) in APSIII patients were found compared to healthy controls. Study of the HLA-DRB1*;DQB1* haplotype −3 frequencies showed a higher occurrence of DRB1*04;DQB1*03:02 and DRB1*03;DQB1*02:01 in APSII patients (P = 4 10 ; OR = 3.31 and P = 0.03; OR = 2.74 respectively) whereas APSIII was only associated with DRB1*03;DQB1*02:01 (P = 7.2 10−8, OR = 4.71). Conclusion. – Our data suggest that the variation in class II HLA alleles and haplotypes could be a genetic factor involved in the susceptibility of APS syndrome. © 2011 Elsevier Masson SAS. All rights reserved.

La tumeur myofibroblastique inflammatoire est une tumeur dont le contexte clinicobiologique et histologique prête souvent à confusion avec un processus d’origine infectieuse. Plusieurs aspects sont encore obscurs notamment les circonstances de survenue. Nous rapportons trois cas cliniques dans un contexte de post-partum (précoce dans deux cas et lointain dans un cas), celle d’une patiente de 28 ans qui présentait à j1 du post-partum un psoïtis gauche dans un contexte fébrile et chez laquelle une tumeur myofibroblastique inflammatoire de siège mésovarien était découverte. L’autre patiente, âgée de 40 ans, présentait à j4 du post-partum un état de choc hémorragique secondaire à la rupture d’un hématome sous capsulaire du foie et chez laquelle la tumeur myofibroblastique inflammatoire se localisait au foie gauche. Le troisième cas concernait une patiente de 32 ans qui avait présenté, cinq mois après son accouchement, une tumeur myofibroblastique inflammatoire pulmonaire ayant été opérée mais ayant récidivé dix ans après la chirurgie. Ces cas cliniques illustrent la survenue possible des tumeurs myofibroblastiques inflammatoires dans le cadre particulier du post-partum et mettent l’accent sur les difficultés de diagnostic dans ce contexte.

Background Pemphigus is a life-threatening autoimmune blistering disease mediated by autoantibodies against adhesion molecule of the skin. Its concurrence with systemic and organ-specific autoimmune disease was described in case reports.Objectives To evaluate the presence of a broad spectrum of organ-specific and non-organ-specific autoantibodies other than anti-desmoglein antibodies in pemphigus patients.Patients and methods Serum samples were obtained from 105 pemphigus foliaceus (PF) patients, 51 pemphigus vulgaris (PV) patients and 50 controls. Both indirect immunofluorescence assay and ELISA were used to assess the presence of autoantibodies related to connective tissue diseases, autoimmune hepatitis, vasculitis, rheumatoid arthritis, coeliac disease, diabetes and thyroiditis.Results Significant difference was observed between the three groups for anti-thyroglobulin antibodies in the pemphigus foliaceus group (18% vs. 4%, P = 0.03). A significantly higher occurrence of IgM anti-cardiolipin (P = 0.03), IgG anti-reticulin (P = 0.01) and IgG anti-gliadin antibodies (P = 0.008) were observed in the PV group. Cases with more than four autoantibodies were frequently positives for both anti-desmoglein 1 and anti-desmoglein 3.Conclusion Autoantibodies other than anti-desmoglein antibodies are not rare in pemphigus patients. Clinical and serological follow-up of pemphigus patients with positive autoantibodies are needed to clarify their impact in disease evolution.

Objectives. - The aim of our study was to investigate the association of HLA-DRB1 and HLA-DQB1 alleles with autoimmune polyglandular syndromes (APS) type II and III in a southern Tunisian population. Patients and methods. - Sixty-two unrelated patients with APSII (n = 20) and APSIII (n = 42) and 146 healthy controls were genotyped for HLA class II alleles (DRB1*, DQB1*) by PCR-SSP technique. Results. An increased frequencies of HLA-DQB1*03:02 (P = 0,02; OR = 2.98) in APSII patients, HLA-DRB1*03 (P=3 10(-6); OR = 4.28) and HLA-DQB1*02:01 (P = 0.04; OR = 1.95) in APSIII patients were found compared to healthy controls. Study of the HLA-DRB1*;DQB1* haplotype frequencies showed a higher occurrence of DRB1*04;DQB1*03:02 and DRB1*03;DQB1*02:01 in APSII patients (P = 4 10(-3); OR = 3.31 and P = 0.03; OR = 2.74 respectively) whereas APSIII was only associated with DRB1*03;DQB1*02:01 (P = 7.2 10(-8), OR = 4.71). Conclusion. - Our data suggest that the variation in class II HLA alleles and haplotypes could be a genetic factor involved in the susceptibility of APS syndrome. (C) 2011 Elsevier Masson SAS. All rights reserved.

The inflammatory myofibroblastic tumour has clinical, biological or histological features sometimes misleading with a septic condition. Presenting symptoms are variable and arising circumstances remain obscure. We report three cases occurring in a postpartum context. The first patient, a 28-year-old female, had left psoitis with a sepsis the first day postpartum in relation with an inflammatory myofibroblastic tumour of the meso-ovary. The second patient, a 40-year-old woman, had a hepatic inflammatory myofibroblastic tumour revealed by a ruptured sub-capsular haematoma of the liver in the forth day postpartum. The third patient, a 32-year-old woman, had a pulmonary inflammatory myofibroblastic tumour, diagnosed 5 months after a delivery and which recurred 10 years after surgical treatment. These cases illustrate the difficulty to diagnose inflammatory myofibroblastic tumour, particularly in postpartum.

BACKGROUND:The salivary gland tumors are rare (less than 3% of all tumors) and poorly known. In fact, they are numerous and histologically difficult to diagnose.AIM:This work aims to point at the different histological types of salivary gland tumors, to draw out the principal epidemiological, clinical, radiological and histological characteristics, and to compare our cases to those of the literature.METHODS:Accordingly, we performed a descriptive type study about 180 cases of salivary gland tumors from the departments of pathology and oto-rhino-laryngology of Habib Thameur hospital during 25 years, extending from April 1979 to December 2004.RESULTS:Benign tumors were predominant (88%), while malignant ones represented 12% of our cases dominated by carcinomas. The sex-ratio was 0.96. Parotid gland location was the most frequent one, and pleomorphic adenoma was the most frequent tumor (62%).CONCLUSION:Histological diversity of salivary tumors results in difficulties for differential diagnosis. These problems can be solved by a precise diagnostic approach and sometimes by an immunohistochemistry study.

les objectifs de ce travail sont de déterminer la prévalence de la maladie coeliaque chez les enfants ayant un diabète de type1 de primo découverte et d'étudier les particularités de la maladie coeliaque chez les enfants diabétiques. nous avons réalisé un étude prospective durant une période de14 ans (1996-2009) au cours de laquelle un dépistage de la maladie coeliaque a été réalisé chez tout enfant présentant un diabète de type 1 lors de la première hospitalisation. Ce dépistage a touché 351 enfants et il était basé sur les AAG (IgG-IgA), AAE et ATG. Les AAG de type IgG étaient positifs chez 27 patients, de type IgA étaient positifs chez 14patients. Les AAE étaient positifs dans7cas et les ATG dans 8 cas. La biopsie jéjunale a été pratiquée chez 10 patients uniquement, quatorze patients ont refusé la biopsie dont 3 avaient des AAG, AAE et ATG positifs et 3 avaient des ATG positifs. Une atrophie villositaire totale a été retrouvée chez 4 patients et une atrophie villositaire partielle avec une augmentation du taux des lymphocytes intra-épithéliaux a été notée dans deux cas, ce qui correspond à 6 cas de maladie coeliaque soit une prévalence de 1,17 %. la prévalence de la maladie coeliaque dans notre étude est probablement sous -estimée si on tient compte des patients qui ont refusé la biopsie jéjunale, par ailleurs on continue à réaliser de façon systématique le dépistage de la maladie coeliaque chez les enfants diabétiques. L'instauration du régime sans gluten même dans les formes asymptomatiques doit être proposé afin d'améliorer la croissance et de réduire le risque de complications à long terme non seulement de la maladie coeliaque, mais peut être également des complications du diabète grâce à une meilleure équilibration.

Pleuropulmonary blastoma is a dysontogenetic neoplasm of childhood, histologically characterized by a primitive variably mixed blastematous and sarcomatous appearance. It presents a pattern of rapid growth and is associated with a poor prognosis. We report the case of a 13-month-old girl, consulting for wheezing, dyspnea and loss of appetite. Chest X-ray revealed a mediastinal deviation with a clear appearance of the right lung. Computed tomography showed a voluminous emphysematous bulla of the right lung and atelectasis of the lower lobe. The final diagnosis was made on the basis of histological features of a cystic tumour, showing blastematous elements associated with sarcomatous compound confirming the diagnosis of pleuropulmonary blastoma type I. Subsequent chemotherapy was performed. Four years after the operation, the child is well with no evidence of disease recurrence or metastasis.