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Resume Introduction Les tumeurs carcinoides bronchiques sont des tumeurs neuro-endocrines bien differenciees, rares, a malignite attenuee. Elles sont distinguees en 2 groupes differents : les carcinoides typiques et les carcinoides atypiques. Le but de ce travail est d’etudier les particularites cliniques, chirurgicales et histologiques de ces tumeurs. Methodes Etude retrospective de 115 tumeurs carcinoides bronchiques operees sur une periode de 21 ans (1992–2012). Resultats Il s’agissait de 56 hommes et 59 femmes avec un âge moyen de 43,73 ans. Cent patients avaient un carcinoide typique et les 15 autres, un carcinoide atypique. La majorite des patients etaient symptomatiques (96,52 %). La tomodensitometrie a objective, dans 51 % des cas, une masse proximale, qui etait obstructive avec trouble ventilatoire d’aval dans 80 % des cas. La fibroscopie a objective un bourgeon endo-bronchique dans 83,48 % des cas. Une exerese carcinologique reglee a ete realisee chez 99 patients, et une resection conservatrice dans 16 cas de carcinoide typique. Un envahissement ganglionnaire etait present dans 12,17 % des cas. Au cours du suivi, un patient a presente une recidive locale et 4 autres des metastases a distance. La survie a 5 ans etait plus defavorable pour les carcinoides atypiques avec 45 % versus 95 % pour les carcinoides typiques. Conclusions Malgre une evolution favorable apres traitement chirurgical, les tumeurs carcinoides presentent, quelle que soit leur forme typique ou atypique, un risque de recurrence et/ou de metastase justifiant l’interet d’un diagnostic precoce et d’un suivi prolonge. Les facteurs influencant la survie sont le type histologique, les metastases a distance et l’envahissement des ganglions mediastinaux.

Phyllodes tumor of the breast is a biphasic fibroepithelial neoplasm. 10 to 20% of phyllodes tumor show malignant transformation, often in the form of stroma, which usually shows fibrosarcomatous differentiation and rarely heterologous sarcomatous elements. Liposarcomatous differentiation is not common among phyllodes tumors. The correct diagnosis of heterologous liposarcomatous differentiation in a malignant PT requires identification of the biphasic component of the tumor. We reported a case of malignant phyllodes tumor which initially transformed into liposarcoma, in addition to a very rare intraductal hyperplasia and flat epithelial atypia. The patient was a 75-year-old woman, with a lump in the left breast without axillary lymphadenopathy. She also have a positive family history of breast carcinoma. She underwent surgery and still alive and disease free after one year.

Resume Les tumeurs primitives de la trachee sont tres rares. Chez l’adulte, la majorite d’entre elles sont malignes. Les schwannomes sont des tumeurs benignes, exceptionnelles dans leur localisation tracheobronchique. Nous rapportons le cas d’un patient âge de 37 ans, traite pour asthme depuis 4 ans, hospitalise pour dyspnee d’aggravation progressive resistante au traitement. La tomodensitometrie thoracique et la fibroscopie bronchique mettaient en evidence une image intratracheale se prolabant au niveau de la bronche souche gauche. Une resection chirurgicale a ete indiquee apres echec d’une tentative de desobstruction au laser. Une excision de la base d’implantation de la tumeur a ete realisee, emportant les 2 premiers anneaux de la bronche souche gauche avec retablissement de la continuite. L’analyse histologique de la tumeur affirmait le diagnostic de schwannome sans caractere suspect de malignite. La surveillance ne constatait pas de recidive. Nous concluons a partir de ce cas qu’il faut penser a une masse intratracheale chez les patients presentant une dyspnee ou souffrant d’un asthme refractaire au traitement habituel.

Resume La mucormycose est une infection fongique opportuniste, rare et souvent fatale, causee par des champignons de l’ordre des mucorales appartenant a la famille des zygomycetes. La mucormycose pulmonaire isolee est rare, survenant principalement sur des terrains fragilises et, en particulier, chez les diabetiques non controles. Les filaments envahissent les vaisseaux sanguins causant une necrose ischemique. Nous rapportons l’observation d’une adolescente diabetique âgee de 13 ans qui a presente une mucormycose pulmonaire compliquee d’un thrombus intra-auriculaire gauche. Le diagnostic de mucormycose a ete porte sur l’examen histologique des prelevements biopsiques bronchiques. Un traitement associant l’amphotericine B par voie systemique et la chirurgie a permis une evolution favorable. Cette infection, de diagnostic difficile, doit etre envisagee chez les diabetiques presentant une infection pulmonaire resistante au traitement, afin d’appliquer une therapeutique agressive precoce. Le traitement optimal associe un traitement antifongique systemique prolonge, une resection chirurgicale du foyer pulmonaire et la correction du facteur predisposant sous-jacent.

Resume Introduction Le carcinome sarcomatoide (CS) du poumon est une tumeur maligne rare, constitue d’un groupe heterogene de tumeurs peu differenciees non a petites cellules qui comportent un contingent sarcomateux ou sarcomatoide. La classification de l’OMS (2004) distingue dans ce groupe 5 sous-types histologiques qui representent un continuum de differenciation epitheliale et mesenchymateuse : le carcinome pleomorphe, le carcinome a cellules geantes, le carcinome a cellules fusiformes, le carcinosarcome et le blastome pulmonaire. Le diagnostic est anatomopathologique et necessite un bon echantillonnage de la tumeur. Patients et methodes Etude retrospective de 28 CS du poumon diagnostiques entre 1993 et 2010 avec une analyse des aspects anatomocliniques. Resultats Notre serie etait constituee de 25 hommes et de 3 femmes avec un âge moyen de 62,9 ans (48–75). La symptomatologie etait dominee par des signes respiratoires. L’imagerie montrait une masse pulmonaire, envahissant la paroi et/ou la plevre dans 5 cas. Le diagnostic etait dans tous les cas anatomopathologique. Ces 28 tumeurs se repartissaient en 19 carcinomes pleomorphes (67,8 %), 4 carcinomes a cellules geantes (14,3 %), 1 carcinome a cellules fusiformes (3,6 %) et 4 carcinosarcomes (14,3 %). Le traitement etait chirurgical dans 27 cas. Les traitements associes etaient une chimiotherapie neoadjuvante (3 cas) ou adjuvante (1 cas) et/ou une radiotherapie preoperatoire (1 cas). Sept patients sont decedes. Les autres patients etaient perdus de vue. Conclusion Les CS sont des tumeurs hautement agressives avec un potentiel metastatique important et un haut risque de recidive. Son pronostic est plus sombre que celui des autres cancers non a petites cellules.

Le ganglioneurome est une tumeur neurogene benigne rare qui se developpe a partir des cellules ganglionnaires sympathiques. Elle se localise essentiellement au niveau du mediastin posterieur et dans la region retroperitoneale. Nous rapportons un cas de ganglioneurome se presentant comme une masse kystique sur une tomodensitometrie thoracique, chez une femme de 45 ans. Le diagnostic a ete confirme par l’examen anatomopathologique. A travers cette observation, nous allons decrire cette tumeur rare et nous insisterons sur ses differents aspects anatomo-cliniques et radiologiques ainsi que sur les principaux diagnostics differentiels.

Malignant pleural mesothelioma (MPM) is a challenging diagnosis characterized by the absence of real specific diagnostic markers. Positivity with the galectin-3 antibody was assessed by a cytoplasmic expression in 17 MPM. Fourteen cases expressed the galectin-3 antibody. The three negative cases consisted of epithelioid, biphasic, and sarcomatoid MPM. The 14 positive cases consisted of epithelioid MPM in 12 cases, sarcomatoid MPM in one case, and biphasic MPM in one case. In spite of our inability to prove the real diagnostic value of the galectin-3 antibody, our findings make us wonder about the implication of this antibody in the carcinogenesis of MPM.

Resume Introduction L’amyloidome pulmonaire ou amylose nodulaire est une forme localisee d’amylose pouvant mimer un cancer bronchopulmonaire et pouvant etre observee en association avec une pathologie infectieuse, une maladie de systeme ou un lymphome. Observation Nous decrivons le cas d’une patiente âgee de 36 ans ayant pour antecedent un diabete insulinodependant et une hypothyroidie sous traitement medical. Cette patiente presentait un syndrome de Gougerot-Sjogren et etait hospitalisee pour une symptomatologie respiratoire. L’examen physique etait sans particularite. Le bilan biologique revelait un pic monoclonal d’immunoglobulines. A l’imagerie, des nodules pulmonaires bilateraux associes a des adenopathies mediastinales etaient objectives. Une biopsie pulmonaire chirurgicale a ete effectuee. L’examen histologique montrait un nodule tumoral centre par un abondant materiel eosinophile, acellulaire et craquele cerne par un infiltrat lymphomateux dense, diffus et monomorphe detruisant le parenchyme pulmonaire. Les etudes histochimiques et immuno-histochimiques ont permis de conclure a une association d’une amylose nodulaire pulmonaire et d’un lymphome primitif du MALT, compliquant un syndrome de Gougerot-Sjogren. Conclusion L’association lymphome MALT pulmonaire primitif et depots amyloides localises est rarement observee dans le cadre du syndrome de Gougerot-Sjogren. La pathogenie de cette association demeure discutee et sa prise en charge ainsi que son pronostic demeurent sujets a debat vu la rarete des cas rapportes dans la litterature. Cependant, il apparaitrait que l’amylose localisee associee au lymphome MALT aurait un meilleur pronostic que l’amylose disseminee.

Resume Les tumeurs primitives costales sont des tumeurs rares dominees par les tumeurs malignes. L’hemangiome osseux ne represente que 1 % des tumeurs osseuses. La localisation costale ne represente que 1 % des cas avec uniquement une cinquantaine de cas decrits dans la litterature. But Les auteurs ont pour objectif de rapporter une tumeur costale rare, de decrire ses aspects histologiques et de rapporter les principaux diagnostics differentiels. Observation Les auteurs decrivent le cas d’une patiente âgee de 46 ans qui consultait pour des douleurs thoraciques. Les donnees radiologiques n’ont pas permis d’eliminer une tumeur maligne et le traitement a consiste en une resection de l’arc posterieur de la sixieme cote emportant toute la tumeur. L’examen microscopique a conclu a un hemangiome costal et la patiente n’a pas presente de recidive apres un recul de six ans. Conclusion L’hemangiome costal est une tumeur tres rare d’etiologie controversee. Certains aspects radiologiques sont specifiques comme l’aspect de bulles de savon ou de nids d’abeilles. Cependant, le diagnostic de certitude demeure microscopique. Ces tumeurs sont de bon pronostic et aucun cas de recidive n’a ete decrit apres resection complete.

Resume Introduction L’hamartochondrome endobronchique est une tumeur benigne, derivee du tissu mesenchymateux peribronchique. Il presente des particularites par rapport a la forme parenchymateuse notamment un diagnostic plus difficile, une symptomatologie bruyante et un traitement base souvent sur une exerese conservatrice par voie endoscopique. Parfois, les lesions parenchymateuses sont irreversibles imposant une chirurgie d’exerese radicale. Le but de notre travail est d’etudier les particularites anatomocliniques des hamartochondromes endobronchiques ayant necessite un geste chirurgical. Patients et methodes Etude retrospective de sept hamartochondromes endobronchiques diagnostiques et operes dans notre institution (1995 et 2009). Resultats Les patients etaient constitues de quatre hommes et de trois femmes avec un âge moyen de 53,7 ans (41–68). La symptomatologie etait dominee par des signes d’irritation ou d’obstruction bronchique. La fibroscopie et l’imagerie objectivaient le plus souvent un bourgeon endobronchique dont les biopsies etaient non concluantes. Le traitement chirurgical a consiste en une resection atypique (un cas), une lobectomie (cinq cas) et une pneumonectomie (un cas). La taille tumorale variait entre 0,5 et 3,5 cm. L’examen anatomopathologique avait permis de poser le diagnostic positif. L’evolution etait favorable dans tous les cas. Conclusion Le diagnostic et le traitement de l’hamartochondrome endobronchique sont essentiellement endoscopiques. La prise en charge doit etre rapide afin de limiter les sequelles parenchymateuses irreversibles impliquant un geste chirurgical souvent plus mutilant.

Resume Introduction Le kyste hydatique est une affection parasitaire encore endemique dans plusieurs pays. Le pneumothorax peut etre un signe revelateur de cette pathologie, ce qui pose un probleme diagnostique et therapeutique dans une situation d’urgence. Observation Nous rapportons le cas d’une patiente âgee de 23 ans, enceinte a 22 semaines d’amenorrhee, qui s’est presentee pour dyspnee et douleur thoracique. La radiographie du thorax a objective un pneumothorax droit compressif. Le controle radiologique apres drainage pleural a montre une opacite homogene de tonalite hydrique occupant les deux tiers inferieurs du champ pulmonaire droit. L’echographie thoracique etait en faveur d’un kyste hydatique non complique basal droit. L’exploration peroperatoire a trouve une hydatide de 14 cm libre dans l’espace pleural et une cavite du lobe inferieur droit ou siegeaient deux fistules bronchiques. Le traitement a consiste a une extraction de la vesicule intacte, fermeture des fistules bronchiques et capitonnage de la cavite residuelle. Les suites operatoires ont ete simples. Conclusion Le kyste hydatique pleural primitif heterotopique est une etiologie exceptionnelle de pneumothorax, qu’il faut evoquer dans les pays a forte endemie hydatique. Le traitement est chirurgical apres drainage du pneumothorax.

Summary Purpose of the study Desmoid-type fibromatosis is a fibroblastic proliferation that develops in the deep soft tissues and is characterized by infiltrative growth and a tendency towards local recurrence but does not metastasize. Fibromatosis of the chest wall is rare, representing only 10 to 20% of all deep fibromatoses. Case report We report a six cases series of fibromatosis of the chest wall treated at our institution between 1996 and 2009. There were five men and one woman with a mean age of 42.2. Swelling of the chest wall was the most frequent symptom. Imaging showed an expansive homogeneous mass, invading the rib (four cases) or the sternum (two cases). Surgical resection was performed in all cases. The outcome was favorable (five cases) or marked by local recurrence (one case). Discussion Although it is considered to be a benign lesion, this tumor can be locally very aggressive, and has a high local recurrence rate depending upon the efficacy of surgical resection.

Resume Les fibromatoses de type desmoide sont des tumeurs fibroblastiques des tissus mous profonds, caracterisees par une capacite infiltrante invasive, un taux de recidive locale tres eleve mais sans potentiel metastatique. Les fibromatoses de la paroi thoracique sont rares ne representant que 10 a 20 % de l’ensemble des fibromatoses profondes. Nous rapportons une serie de six cas de fibromatose de la paroi thoracique operes et colliges dans notre institution entre 1996 et 2009. Il s’agissait de cinq hommes et une femme avec un âge moyen de 42,2 ans. Une tumefaction parietale etait le principal motif de consultation. L’imagerie montrait une masse tissulaire homogene expansive, de siege costal (quatre cas) ou presternal (deux cas). Une exerese chirurgicale etait realisee dans tous les cas. L’evolution etait favorable (cinq cas) ou marquee par des recidives locales (un cas). Bien qu’elles soient considerees comme des lesions benignes, les fibromatoses peuvent etre tres agressives localement avec un haut risque de recidive locale qui reste lie a la qualite de l’exerese chirurgicale.

Resume Introduction Les sarcomes primitifs du mediastin sont rares, representant entre 2 a 8 % de l’ensemble des tumeurs mediastinales malignes. Le but de notre travail etait d’etudier les caracteristiques anatomocliniques de ces tumeurs. Patients et methodes Etude retrospective de 15 sarcomes primitifs mediastinaux diagnostiques entre 1993 et 2009 avec une analyse des aspects anatomocliniques. Resultats Il s’agissait de huit hommes et de sept femmes avec un âge moyen de 40 ans (14–73). La symptomatologie etait dominee par des signes respiratoires. L’imagerie montrait une opacite mediastinale qui envahissait les organes adjacents dans neuf cas. Le diagnostic etait dans tous les cas anatomopathologique. Ces 15 sarcomes se repartissaient en 12 tumeurs malignes des gaines des nerfs peripheriques, deux liposarcomes et un angiosarcome. Dix tumeurs etaient traitees chirurgicalement, dont sept par une exerese radicale. Les traitements associes etaient une chimiotherapie neo-adjuvante (un cas) ou adjuvante (un cas), et/ou une radiotherapie postoperatoire (cinq cas). Par ailleurs, une radiotherapie etait realisee d’emblee dans trois tumeurs inoperables. Huit patients sont decedes (53 %). Conclusion La prise en charge des sarcomes mediastinaux est multidisciplinaire. Le pronostic est sombre et depend essentiellement de l’exerese chirurgicale et du grade histologique.

Background The skin is a rare site of metastasis of the internal malignancies. On the contrary, lung cancer is a common primary malignancy of skin metastasis. The latter is either synchronous with the primary lung tumour or can reveal it. Objective To analyse the clinical findings and the outcome of cutaneous metastasis in patients with primary lung cancer. Methods Retrospective study including 12 cases of cutaneous metastasis of lung cancer diagnosed in our institution during 6 years. Results Our patients included three women and nine men with a median age of 59.83 years. The cutaneous metastases were unique in seven cases. They revealed the primary tumour in two cases, were discovered synchronously with the primary tumour in six cases and after the primary tumour in four cases. They appeared in patients presenting multiple metastases in 10 cases. Clinical appearance consisted on a firm nodule, sometimes ulcerated with a diameter ranging from 0.5 to 5.5 cm. The tumour was sited in the thorax, the back, the abdomen; or the upper or lower limbs. Pathological examination of bronchial biopsy revealed an adenocarcinoma in eight cases, a squamous cell carcinoma in two cases and a small cell carcinoma in two cases. In spite of lung cancer therapies, the patients with metastasis to other organs with a median follow-up time of 4 months died. Conclusion Cutaneous metastases of lung cancer are of poor prognosis. They are most of the time associated with an advanced neoplastic process with metastasis to other organs. Median survival after skin involvement does not exceed months.

Wegener's granulomatosis (WG) is the most frequent antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. It affects mainly the upper airways, lungs, and kidneys. Two forms are identified: systemic and limited. We describe three cases of limited WG diagnosed during a 7-year period. Our aim is to report three localized forms of WG and to put emphasis on the necessity of differentiating localized from systemic forms because of their different prognoses and manner of management. Our study contained two men and one woman with a mean age of 43 years. All our patients were symptomatic and presented with nonspecific respiratory signs. The cANCA were positive in all patients. The imaging findings consisted of cavitary masses. The diagnosis was based on surgical lung biopsy in all cases. All patients were put on cyclophosphamide and prednisolone. Only one patient presented with renal complications after a 2-year follow-up period. The two other patients did not present complications after, respectively, 1 month and 1 year of follow-up. These case reports put emphasis on a rare form of WG, the limited form. The low number of patients, due to the rarity of this disease, does not allow us to delineate the characteristics and the differences between this form and the systemic form, but we highlight the necessity of future investigations in order to explore the pathogenesis, therapeutic, and prognosis differences between these two subsets.

Resume Nous rapportons cinq cas de chondrosarcomes (CS) costaux survenus chez quatre femmes et un homme, âges entre 28 et 49 ans. Dans quatre cas, la tumeur etait etendue et infiltrait les structures adjacentes (tissus mous, vertebres thoraciques, mediastin…). Le diagnostic de CS a ete pose sur les pieces d’exereses chirurgicales larges chez trois malades et sur des biopsies de la tumeur dans deux cas. Le CS etait de grade II dans trois cas. Chez les deux autres malades, le CS etait respectivement de grade I et III. Une resection chirurgicale large a ete pratiquee chez trois malades, associee dans un cas a une radiotherapie et chimiotherapie adjuvantes. Dans les trois cas, l’evolution etait favorable apres un recul de un mois a quatre ans. Deux malades sont decedees, dont un apres radio- et chimiotherapie seules, pratiquees d’emblee devant l’importance de l’envahissement tumoral. Le CS costal se caracterise par un haut potentiel d’invasion locoregionale et a distance. Son diagnostic est anatomopathologique. L’exerese chirurgicale large est le seul traitement curatif meme dans les CS de haut grade.

Summary We report five cases of coastal chondrosarcoma (CS) in four women and one man between 28 and 49 years of age. In four cases, the tumor had spread and infiltrated the adjacent structures (soft tissues, thoracic vertebrae, mediastinum, etc.). CS was diagnosed based either on wide surgical resection specimens in three patients, or on tumor biopsies in two cases. The CS was grade I in one patient, grade II in three cases, and grade III in one case. Wide surgical resection which was performed in three patients was associated in one case with adjuvant radiotherapy and chemotherapy. In the three cases, the disease natural history appeared favorable after a follow-up duration ranging from 1 month to 4 years. Two patients died, one after radiotherapy and chemotherapy done immediately after diagnosis as a result of the large size and invasive nature of the tumor. Coastal CS is characterized by a distinct potential for locoregional and distant metastasis. Diagnosis remains anatomopathological. Wide surgical resection is the only demonstrated curative treatment, even for high-grade CS.

Thymic carcinoma is a very rare malignancy. In 1999, a World Health Organization committee published histologic criteria for distinct thymoma entities (labelled as type A, AB, B1, B2, B3 thymomas) and for the heterogeneous group of thymic carcinomas, collectively called type C tumour. Thymic carcinoma differs from thymoma in that it displays cytologically malignant features, extensive local invasion, and a substantial potential for metastasis. It constitutes a heterogeneous group of tumours that display different biological behaviours and prognoses. The majority of thymic carcinomas are either squamous carcinomas or lymphoepithelioma-like carcinomas. This study included three male patients aged 20, 46 and 19years respectively with histologically proven thymic carcinoma diagnosed at the author's institution. All of the patients presented a large mass of the anterior mediastinum. Histological examination of the different tumours revealed three distinct variants of thymic carcinoma, namely: epidermoid carcinoma, clear cell carcinoma and lymphoepithelioma-like carcinoma.

Introduction Les teratomes matures du mediastin sont rares, neanmoins ils representent la plus frequentes des tumeurs germinales mediastinales. Le but de notre travail etait d’etudier les particularites anatomocliniques de ces tumeurs mediastinales benignes. Patients et methodes Nous avons procede a une etude retrospective de 14 teratomes matures benins du mediastin diagnostiques entre janvier 1992 et decembre 2006, avec analyse des aspects cliniques, radiologiques, chirurgicaux et histologiques. Resultats Nos patients etaient constitues de 10 femmes et de 4 hommes avec un âge moyen de 29 ans (extremes de 5 a 56 ans). Le principal signe clinique etait la douleur thoracique. L’imagerie montrait une opacite mediastinale anterieure presentant un aspect heterogene avec des plages de densite variable solide, liquidienne, graisseuse et calcique. Macroscopiquement, toutes les tumeurs etaient kystiques ou a predominance kystique renfermant un materiel volontiers gremuleux. La taille moyenne etait de 9 cm (5 a 12 cm). L’examen histologique trouvait constamment un tissu malpighien associe ou non a des annexes pilo-sebaces. Les autres constituants identifies etaient representes par un epithelium respiratoire (12 cas), des lobules adipeux (12 cas), du muscle lisse (10 cas), du cartilage (8 cas), de l’os (7 cas), un epithelium digestif (7 cas), du tissu pancreatique (5 cas), un epithelium urothelial (3 cas), du tissu nerveux (1 cas) et du tissu prostatique (1 cas). Tous ces teratomes etaient matures, et 7 d’entre eux etaient intra-thymiques. Conclusion Toutes les formes de teratomes peuvent survenir dans le mediastin (mature, immature ou avec contingent malin), mais dans notre serie tous les teratomes etaient matures. Leur aspect histologique est globalement similaire a leurs homologues gonadiques. Neanmoins, le tissu pancreatique frequent dans le mediastin, n’est pas retrouve dans les gonades. Le tissu thyroidien, au contraire, n’a jamais ete identifie dans les teratomes mediastinaux.

Resume La dysplasie fibreuse est une maladie sporadique rare, representant 0,8 % de l’ensemble des tumeurs primitives des os. Cette lesion benigne pseudotumorale se caracterise par la presence dans l’os d’une proliferation de tissu fibreux et de tissu osseux immature depourvu de couronne osteoblastique. Elle peut atteindre un ou plusieurs os ; l’atteinte des cotes est rarement decrite. Nous rapportons une serie de dix cas de dysplasie fibreuse costale operee sur une periode de dix ans allant de 1996 a 2005. Il s’agissait de cinq femmes et cinq hommes avec un âge moyen de 38,4 ans et des extremes allant de 27 a 52 ans. Chez huit patients, une seule cote etait atteinte, les deux autres avaient deux cotes atteintes. La douleur etait le symptome le plus frequent. Les images radiologiques etaient parfois evocatrices de dysplasie fibreuse. Devant l’absence de certitude diagnostique, une resection costale a ete pratiquee dans les dix cas. La confirmation diagnostique etait anatomopathologique. Les suites operatoires etaient simples dans tous les cas, sans recidive avec un recul moyen de 50 mois. La prise en charge de la dysplasie fibreuse necessite simplement une surveillance de la maladie dans la majorite des cas. Neanmoins, dans le cadre d’une maladie unifocale et surtout de localisation costale, la dysplasie fibreuse peut poser des problemes de diagnostic differentiel avec des tumeurs malignes motivant l’exerese chirurgicale.

La malformation adenomatoide kystique congenitale (MAKC) est une anomalie congenitale du developpement pulmonaire, representant environ 25 % des lesions congenitales du poumon. Souvent, une detresse respiratoire survient au cours de la periode neonatale, et dans 80 a 85 % des cas, le diagnostic se fait avant l’âge de 2 ans suite a des infections respiratoires. La MAKC est rarement decouverte a l’âge adulte. Nous rapportons 3 cas de MAKC diagnostiquee a l’âge adulte. Le diagnostic etait base sur des elements cliniques et radiologiques dans un cas. Les deux autres etaient diagnostiques a l’etude histologique. La lesion se localisait dans le poumon droit dans 2 cas et dans le poumon gauche chez un patient. Nos patients ont subi une resection chirurgicale. L’examen histologique confirmait le diagnostic de MAKC de type 1 selon Stocker, sans signe de malignite. L’evolution postoperatoire etait bonne. La decouverte tardive de la MAKC a l’adolescence voire a l’âge adulte doit etre reconnue par les cliniciens et les pathologistes. Le diagnostic clinique fortement oriente par l’approche radiologique est confirme par l’anatomie pathologique dans la mesure ou la sanction therapeutique est chirurgicale dans la majorite des cas.

Fibromatosis are uncommon connective tissue tumours arising from musculo-aponeurotic tissue and characterised by spindle cell fibroblastic and myofibroblastic proliferation. The exact aetiology is unknown but several factors are considered to be positively correlated with their development and growth (genetic and hormonal factors and trauma). Although they are considered histologically benign they behave aggressively locally and relapse repeatedly after surgical excision. Mediastinal localisation is very rare. We describe a case of de novo fibromatosis of the posterior mediastinum in a 61 year old man with no history of thoracotomy or trauma. Although mediastinal fibromatosis is very uncommon physicians should be aware of this disease in order to ensure appropriate surgical treatment.

Chondromyxoid fibroma is a rare benign bone tumor observed in less than 1% of all bone tumors. Pathological diagnosis remains difficult. All bone sites may be involved with a predominance of the metaphysis of long tubular bones, particularly the proximal tibia. The tumor has been rarely reported in ribs. We present of case of chondromyxoid fibroma observed in this unusual location in a 31-year-old woman. Routine chest x-ray and computed tomography revealed an expansive destructive mass with a lobulated contour which arose in the fourth left rib. Local resection was performed and the histological examination showed benign chondromyxoid fibroma. The clinical outcome was favorable without recurrence after 15 months follow-up.

Tubulopapillary tumors of the kidney represent a particular group of the renal tumors. They cover a continuous spectrum of tumors ranging from the adenoma to the renal cells papillary carcinoma. The histological and immunohistochemical similarities, as well as the high recurrence of the association of these two entities suggest a continuity of the same biological process. Although rare, the association between adenoma and papillary carcinoma remains still subject to controversy and plead in favor of a narrow relation between them. We report the case of a multicentric tubulopapillary carcinoma of the kidney associated with multifocal adenomas, discovered by fortuity in a right nephrectomy at a 57-year-old patient operated for an obstructive chronic pyelonephritis.

Glomus tumors of the trachea are extremely rare and generally benign, with very few cases of malignant transformation reported in literature. We present the case of a 74-year-old man explored for cough and dyspnea. Bronchoscopy showed a polypoid mass arising from the posterolateral tracheal wall. Computed tomography demonstrated a mid-tracheal tissular mass obliterating the tracheal lumen. Resection and anastomosis of the trachea were performed. The histological and immunohistochemical findings were consistent with malignant glomus tumor.

Pulmonary mucormycosis is a rare, devastating, opportunistic fungal infection, caused by the ubiquitous filamentous fungi of the Mucorales order of the class of Zygomycetes. This infection occurs principally in some particular conditions, specially in diabetic patients and immunocompromised host, and rarely in cirrhotic patients. The diagnosis of mucormycosis can only be confirmed by pathological and mycological examination of biopsy specimens. We report a case of pulmonary mucormycosis in a 68-year-old woman with underlying liver cirrhosis and diabetes mellitus. Endoscopic and radiologic findings supported the diagnosis of hydatid cyst of the lung. The patient underwent surgical resection and was started on amphotericin B, after pathological examination. Unfortunately, she succumbed to the infection within one month of surgery.

Primary pulmonary leiomyosarcomas are a rare malignant tumor with generally poor prognosis. They pose important problems of differential diagnosis especially with pulmonary metastatic sarcoma. They are subdivided into two categories those originating from pulmonary parenchyma and those originating from pulmonary arteries. The authors describe a case of 54-year-old woman that had her left upper lobe mass checked. The diagnosis was made based on the microscopic examination of the percutaneous trans-thoracic needle biopsy. The general examination did not show any metastatic lesions in other areas. The treatment was based on chemotherapy followed by a surgical resection. The patient did not experience recurrences or complications.

Secretory carcinoma is a very rare and distinctive type of low-grade breast carcinoma, initially termed “juvenile breast cancer”, but it is now known to occur in adults of both sexes. It is the only epithelial tumor of the breast characterized by specific translocation. In adults, it is potentially more aggressive than in children. We report a case of an indolent breast tumor in a 66-years-old woman. The patient presented with a locally advanced mass in the right breast. She had radical mastectomy with axillary node dissection. Grossly, the mass was circumscribed, measured 50 × 35 mm, with a white glistening cut section. Microscopic examination revealed the classical features of secretory carcinoma with microcystic and solid patterns with abundant intra and extracellular PAS-positive secretory material. The tumor cells showed abundant amphophilic and clear cytoplasm with mildly atypical nuclei. Mitotic figures were infrequent. The axillary node dissection was free of tumor. The patient is disease free after a follow-up period of 12 months. Secretory carcinoma is a very rare type of breast carcinoma and is uncommon in adults. Despite their good behavior, the prognostic factors and the therapeutic management are non-consensual. The majority of patients usually remain free of disease after surgical excision.

Resume La tumeur a cellules claires du poumon est une entite rare, d’histogenese incertaine et discutee. Cette tumeur se presente typiquement comme une masse asymptomatique, peripherique et grossierement arrondie du poumon. L’examen histologique montre qu’elle est faite de cellules polygonales claires regulieres a cytoplasme rempli de glycogene, au sein d’un riche reseau de capillaires sinusoides. Certaines caracteristiques immunohistochimiques et ultrastructurales permettent de confirmer le diagnostic. Nous rapportons le cas d’une tumeur a cellules claires primitive du poumon chez une jeune femme âgee de 28 ans, avec une revue de la litterature. L’histogenese encore hypothetique de cette tumeur, son potentiel evolutif, et les differentes difficultes diagnostiques souvent rencontrees sont discutes.

Mucormycosis is a rare, devastating, opportunistic fungal infection, which occurs principally in some particular conditions, specially in non-controlled diabetic patients, notably during keratoacidosis. We report two cases in 62 and 72 year-old diabetic women. In both cases, histologic examination of endobronchial biopsies showed tissue invasion by hyphae with characteristic morphology leading to the diagnosis of pulmonary zygomycosis. This difficult diagnosis must be evoked in diabetic patients with non-responsive lung infections in order to apply early aggressive therapy.

Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. Although considered a benign lesion, it can be very aggressive locally and has a high rate of local recurrence after incomplete surgical excision. Radiologically, the lesion shows no distinctive features, often simulating osteosarcoma, fibrous dysplasia, or fibrosarcoma. Rib involvement by desmoplastic fibroma is extremely rare. We present two cases of desmoplastic fibroma involving this unusual location with lytic costal lesion and chest wall extension. Histological examination after surgical resection revealed that the tumor consisted of spindle cells with small, elongated nuclei in a background of numerous collagen fibers and infiltrating lamellar bone. There was no evidence of nuclear atypia, mitotic activity, or necrosis. We also discuss histological differential diagnosis as well as clinical features and the radiological and pathologic findings of this rare disease. Recognition of this entity is important to ensure proper surgical treatment.

Mucoepidermoid carcinoma of the lung is rare. The microscopic findings distinguish low grade and high grade tumors. Conservative surgical resection is appropriate if possible for low grade tumors. For the high grade tumors, combined wide surgical resection and radiotherapy is recommended. We report 10 cases of mucoepidermoid carcinoma of the lung (5 low grade, 5 high grade) in 8 male and 2 female patients with a mean age of 43.9 years. Only 5 of 10 were smokers. All 10 patients underwent surgery. Operative procedures included 8 lobectomies and 2 pneumonectomies. Two patients received radiation therapy postoperatively. Three patients (2 high grade and 1 low grade) died. The other 7 patients were alive without evidence of recurrence.

Eccrine skin tumors are rare and represent only 0,05% of all cutaneaous neoplasms. They represent a pitfall especially with cutaneous metastases of carcinoma which are more frequent. We report the case of a 60-year-old woman presented with a frontal scalp mass whose histologic and immunohistochemical features concluded initially to a cutaneous metastasis of breast carcinoma. The diagnosis was reviewed because of the absence of a breast lesion. The final diagnosis was primary sweet gland carcinoma. Histologic distinction between cutaneous metastatic breast carcinoma and primary cutaneous adnexal neoplasms can be very challenging or even impossible. This case illustrates this difficulty and puts emphasis on the necessity of keeping in mind the distinctive features between these two entities.

Background: Multiple thoracic hydatidosis is uncommon even in endemic countries. Case Presentation: A 37-year-old Caucasian man came to our observation with a 10-day history of basi-thoracic chest pain and dyspnea. Chest X-ray showed a well limited cardiac opacity associated to an opacity of the upper lobe of the right lung. Chest CT-scan showed a mediastinal cyst evoking a hydatid cyst in the left ventricle associated to a right pleural effusion. The patient underwent surgery that consisted in the drainage of multiple pericardial cysts, cystectomy of a left ventricle cyst and right pleural cystectomy. Microscopic examination showed a pleuro-pulmonary and cardio- pericardial hydatidosis. The patient was treated with albendazole and did not present complications or recurrences after one year of follow up. Conclusion: Multiple thoracic hydatidosis is rare. Cardiac location is most challenging because it is difficult to manage and life threatening. Treatment modalities continue to be based mainly on surgery. Preventive measures are necessary to avoid disease recurrence.

Post-traumatic fibro-osseous lesion is a rare benign osseous lesion usually affecting the ribs and relatively under-recognised. Histologically, it showed a bland fibrous stroma in which resided an anastomosing network of bone trabeculae, having a zonal pattern of maturation from metaplastic woven to mature lamellar bone, with or without an associated xanthomatous component. We report a well-documented case of mixed variant post-traumatic fibro-osseous lesion of the sixth rib, which was initially misdiagnosed as particular form of fibrous dysplasia associated with a xanthoma.

Thoracic actinomycosis is a suppurative infection which can be difficult to diagnose as its presentation may mimic cancer or tuberculosis. We report a new case of thoracic actinomycosis in a 35-year-old man who presented with thoracic symptoms associated to a productive parietal fistula. Imaging exploration revealed an opacity of the right ventroapical segment with parietal infiltration. A bilobectomy and a parietectomy were performed. The anatomopathologic diagnosis actinomycosis was confirmed. The patient was first put on a treatment of azathioprine 1g daily during two weeks, then switched to a combination with Vibramycin 100 mg twice a day during 17 months, The evolution was marked by the persistence of productive fistulae, which were treated surgically, and resistance to the initial treatment leading to a switch to Augmentin 3 g daily during 25 days. The patient experienced clinical improvement with a follow up of 18 months than was lost to follow-up.

Summary Primary bone invasion by xanthoma is rare particularly in normolipidemic patients. In fact, lipid disorders are present in most patients. Xanthomatous lesions were observed in the ninth rib's posterior arch of a 45-year-old man with a history of labour accident. To our knowledge, this is the fourth case reported in the literature. Histologically, the lesion was characterized by a replacement of normal bone and marrow with xanthoma cells. The treatment approach consisted in a total removal of the lesion and the patient has not presented recurrences after a follow up of 10 years.

Summary Neuroblastoma is an embryonal tumour that evolves from the neural crest cell and rarely occurs in adults. Adult neuroblastoma is an uncommon malignancy that often pursues an aggressive clinical course, involves multiple sites, and has a poor prognosis. However, the prognosis of mediastinal neuroblastoma has been reported to be better than other neuroblastomas. We report a case of posterior mediastinal neuroblastoma differentiating type in a 61-year-old woman that was symptomatic and completely resected. Diagnosis was based on pathologic and immunohistochemical studies of the surgical specimen. Two years after the operation and without further treatment postoperatively, the patient showed no evidence of recurrence or metastasis.

Serous cystadenoma of the pancreas is a benign cystic tumor, which radiological diagnosis is easy in its typical microcystic variant. The macrocystic variant is uncommon and raises diagnostic problems with other macrocystic lesions of the pancreas such as pseudocysts and mucinous cystadenomas. We report the case of a young woman with a unilocular macrocystic serous cystadenoma of the pancreas which was identified on pathologic examination of the surgical specimen, after unconclusive abdominal ultrasound and CT-scan. This case describes an unusual clinical presentation of this cystic tumor and emphasizes that the diagnosis of such an entity is still based on pathological examination after cyst removal.

Sarcomatoid carcinoma (SC) of the lung is defined by the World Health Organization as poorly differentiated non-small cell carcinoma that contains a component of sarcoma or sarcoma-like elements. It represents an overall continuum of epithelial and mesenchymal differentiation. Five subtypes are recognized: pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. The diagnosis is pathological and requires a good sampling of the tumor.Twenty-eight cases of primary sarcomatoid carcinoma, diagnosed between 1993 and 2010, were reviewed retrospectively, noting the clinicopathological characteristics.The patient population consisted of 25 males and 3 females with mean age of 62.9 years (48-75 years). The symptomatology was dominated by respiratory symptoms. Imaging features showed a pulmonary mass invading pleura or thoracic wall in 5 cases. The diagnosis was made in all cases on histological examination. These 28 tumors were divided as below: into 19 pleomorphic carcinomas, 4 giant cell carcinomas, 1 spindle cell carcinoma and 4 carcinosarcomas. Twenty-seven tumors were treated surgically. Associated treatments were neoadjuvant (3 cases) or adjuvant chemotherapy (1 case) and preoperative radiotherapy (5 cases). Deaths occurred in 7 patients. Twenty-two patients were lost to follow up.These tumors are frequently symptomatic, are locally advanced, and have higher rates of recurrence. Its prognosis is worse than that of other non-small cell lung cancer.

Diffuse pulmonary lymphangiomatosis is an extremely rare congenital lesion affecting the lymphatic pulmonary system. It is carcterized by a multifocal proliferation and a pulmonary lymphatic vessels dilatation. It is essentially observed in newborns and infants. The diagnosis is based on histological findings.The purpose of this article is to describe the main clinical and pathological features of this rare entity difficult to diagnose.We report a case of a 19-year old girl who presented with a 2-month standing dyspnea. Radiographic findings consisted in a soft tissue infiltration and thickening in the two lobes. Based on histological and immunohistochemical findings, the diagnosis of diffuse pulmonary lymphangiomatosis was retained.Diffuse pulmonary lymphangiomatosis is a difficult diagnosis which can be confused with lymphangioleiomyomatosis. Its worse prognosis could be improved by an early diagnosis enabling the use of interferon alpha 2B at the proper time.

Studies on bronchoalveolar lavage (BAL) fluid samples profile characteristics in sarcoidosis patients hitherto reported in literature give rise to conflicting data. The typical finding is lymphocytic alveolitis with a high CD4/CD8 ratio, although a broad range of values has been found. THE AIM of the study was to evaluate different parameters of BAL fluid in patients with sarcoidosis before and after corticosteroid treatment and to determine the reliability of BAL in evaluating prognosis of sarcoidosis.The study involved 40 patients with clinical and histological evidence of sarcoidosis.Before treatment, BAL fluid showed an increase in total cell count and a T lymphocytosis with an increase in the CD4/CD8 ratio. After treatment, we have noted a significantly increase in macrophage proportion, a significantly decrease in lymphocyte proportion and in CD4/CD8 ratio. A significant higher CD4/CD8 ratio was noted in patients with multiple extrathoracic lesions compared with patients with only intrathoracic involvement and in patients with unfavourable evolution compared with patients with improved evolution.When typical clinical and radiological findings definitely suggest sarcoidosis, BAL results may add a biological support. Our data suggests that a high CD4/CD8 ratio seems to be predictive of poor prognosis with a multiple extrathoracic organ involvement and an unfavourable outcome.