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Microvillositary inclusion disease also known as microvillositary atrophy is a rare congenital enteropathy containing a border abnormality in the brushes of enterocytes, manifesting as severe rebellious diarrhea in newborns and infants. It was first described in 1978 by Davidson, et al. The autosomal recessive mode of transmission is suggested because of the frequency of familial cases and inbreeding. Histopathology plays an essential role in establishing the diagnosis. In 2008, a common mutation was identified in most of the patients studied in the MYO5B gene that codes for the Myosin Vb protein, which helped in understanding the etiopathogeny of this pathology poorly described in the literature. The prognosis for this pathology is extremely bleak, requiring total parenteral nutrition for child survival. Intestinal transplantation is for the moment the only long-term solution. Materials and methods: We report the case of an infant aged 6 months, with no perinatal antecedent. There is 1st degree consanguinity, the mother has a history of deaths in younger siblings in undetermined circumstances. Who since the age of 3 days presents profuse liquid diarrhoea with malnutrition, dehydration and enormous abdominal distension? Several diagnoses were suspected before the jejune biopsy was carried out, which led to the diagnosis of a microvilliositary inclusion disease. The aim of our work is to highlight the rarest cause of neonatal rebel diarrhoea and to know how to include it among other differential diagnoses.

Introduction Choristoma is a mass presenting normal histology, but in an abnormal location. Cystic choristoma is rarely reported in the head and neck region. Neonatal cystic masses in the neck suggest usually correspond to a diagnosis of cystic lymphangioma. Case report We report a case of a congenital cystic choristoma of the neck clinically and radiologically mimicking cystic lymphangioma. Discussion Congenital cystic choristoma is an extremely rare lesion, essentially described in neonates, composed of various types of tissues. The diagnosis of congenital cystic choristoma may be suggested on imaging and must be confirmed by histopathological examination. Treatment consists of complete surgical resection.

L’oesophagite a eosinophiles est une pathologie chronique allergique rare, de description recente. Les premiers cas ont ete publies en 1977. Depuis, l’entite est de plus en plus rapportee dans la litterature medicale.

Pleural fibroma, or fibrous tumor of the pleura, is an uncommon entity which is characterized by slow proliferation of undifferentiated, intermediary or mature fibroblasts associated with collagen fibers forming a tumor stroma. We report a case in a 49-Year-old man who developed exercise-induced dyspnea and right chest pain. The thoracic CT scan revealed the presence of a mass in the right lung base composed of heterogeneous encapsulated tIssue. Tumor resection was performed leading to the histological diagnosis of pleural fibroma. Immunohistochemistry tests revealed positive vimetin and CD34, and negative cytokeratin uptake. These immunohistochemistry data contributed to the differential diagnosis with malignant pleural mesothelium. Pleural fibroma is a benign tumor in 80% of the cases. Prognosis is excellent. Local recurrence is exceptional and generally occurs after incomplete resection. Radial surgical treatment determines the prognosis and is required to prevent local recurrence. Other criteria of malignancy are not correlated with the clinical course of this type of tumor.

Le teratome intrarenal est une tumeur extremement rare. Peu de cas ont ete publies dans la litterature medicale. Dans sa localisation renale, le diagnostic de teratome est rarement evoque en preoperatoire. Ses signes cliniques et radiologiques etant peu specifiques, le diagnostic final est souvent confirme par l’etude anatomopathologique. Nous rapportons le cas d’un teratome kystique de localisation intrarenal chez un nourrisson de cinq mois. Une exerese radicale de sa tumeur intrarenale a ete realisee. Devant la suspicion de tumeur de Wilms renale, une chimiotherapie etait administree en preoperatoire. La chirurgie est le traitement de choix du teratome intrarenal. Il n’est en general pas sensible a la chimiotherapie. Cela est du au caractere mature et bien differencie des tissus tumoraux qui le constituent. Pour le chirurgien et le pathologiste pediatres, ce diagnostic doit toujours etre present a l’esprit devant une masse renale kystique pour eviter une chimiotherapie nocive pour ces jeunes patients.

We report on a rare case of botryoid Wilms tumor extending into the duodenum. This uncommon macroscopic form of nephroblastoma consists entirely of a polypoid renal intrapelvic mass. The main differential diagnosis of this unusual tumor is botryoid rhabdomyosarcoma. A 14-month-old boy presented with a painful abdominal mass. Radiology revealed a large heterogeneous mass in the renal calyx, protruding into the ureter. A right radical nephroureterectomy was carried out. The tumor was found to extend into the 2nd portion of the duodenum. The pathologic diagnosis was mixed type nephroblastoma, SIOP 2001 stage III. The patient was given a course of postoperative chemotherapy. No local recurrence or metastatic spread has been detected after 2 years. Only few such cases have been previously reported, some of them extending into the bladder. To our knowledge, botryoid nephroblastoma has not previously been described as extending into the digestive system.

Resume Introduction Les tumeurs primitives des grosses structures vasculaires (aorte, artere pulmonaire et veine cave inferieure) sont tres rares. Une classification anatomopathologique de ces differentes tumeurs a ete etablie en se fondant sur leur siege de developpement et leur aspect macroscopique. Elle distingue le sarcome intimal a point de depart intimal et les tumeurs intramurales prenant naissance de la media ou de l’adventice et correspondant a des leiomyosarcomes ou des fibrosarcomes. Les sarcomes de la veine cave inferieure sont generalement des leiomyosarcomes. Observation Un sarcome intimal de la veine cave inferieure a ete diagnostique chez une jeune patiente âgee de 17 ans. L’etude microscopique, aidee par l’immunohistochimie, a permis ce diagnostic et l’elimination un leiomyosarcome ou d’un angiosarcome, de meilleur pronostic que celui du sarcome intimal. Discussion Le sarcome intimal de la veine cave inferieure est exceptionnel. Son diagnostic pre-operatoire est difficile. Il s’agit d’une tumeur peu differenciee et de tres mauvais pronostic par rapport aux autres tumeurs primitives du systeme vasculaire. L’etude anatomopathologique et surtout immunohistochimique permet de faire le diagnostic positif.

Resume Les auteurs rapportent un nouveau cas de mesotheliome sarcomateux pleural. L’originalite de cette observation reside dans la revelation de la tumeur par un hemothorax post-traumatique.

Endometriosis is a rare entity, related after operation on the uterus or uterine tubes or a laparotomy procedures or other extrapelvic procedures, when seeding of endometrial fragments were shed into the peritoneal cavity. We report the case of a menopaused woman with a subcutaneous incisional scar mass that appeared 22 years after a caesarean section. The diagnosis was made by histological examination.

La fistule omphalo-mesenterique est une anomalie congenitale exceptionnelle en rapport avec la persistance complete du canal omphalo-mesenterique sur tout son trajet. Le diagnostic est pose par les donnees cliniques et la fistulographie sans avoir recours a d’autres examens complementaires. Bien qu’une faible proportion de cette malformation se complique, le traitement chirurgical precoce est necessaire.