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Résumé La neurofibromatose de type 1 (NF1) est une maladie génétique prédisposant au développement de tumeurs malignes et bénignes. La mutation du gène NF1 entraîne une dérégulation de la voie de signalisation RAS-MAP-kinase aboutissant à un dysfonctionnement du contrôle de la prolifération cellulaire et à la prolifération tumorale. L’épidémiologie des cancers chez les enfants atteints de neurofibromatose est très différente de celle de la population pédiatrique générale, imposant une surveillance spécifique et rapprochée. Le neurofibrome est la tumeur bénigne la plus fréquente. Elle peut être très invalidante selon sa localisation et sa taille. Il n’existe actuellement pas de traitement spécifique pour ces tumeurs. Les tumeurs malignes les plus fréquentes sont les leucémies, les rhabdomyosarcomes, les gliomes et les tumeurs malignes des nerfs périphériques. La prise en charge thérapeutique doit prendre en compte le risque de développement de tumeurs secondaires aux traitements. Nous rapportons le cas d’un enfant ayant développé avant l’âge de 5 ans deux tumeurs. Summary Neurofibromatosis type 1 (NF1) is a frequent autosomal dominant genetic disorder that predisposes to the development of benign and malignant tumors. Mutation of the NF1 gene affects the RAS-MAPK signaling pathway and leads to a dysfunction in cell proliferation and induces tumor development. Epidemiology of cancer in children with NF1 is very different from the general pediatric population, which requires regular and specific monitoring. Neurofibroma is the most frequent benign tumor. It can be very invalidating depending on the size and location of the tumor. Currently, there is no specific treatment for these tumors. The most frequent malignancies in children with NF1 are leukemias, rhabdomyosarcomas, malignant peripheral nerve sheath tumors and gliomas. The treatment of these tumors should consider the risk of second cancers induced by radio- and chemotherapy. We report on the case of a 5-year-old boy with NF1 developing two tumors. [ABSTRACT FROM AUTHOR]

Neurofibromatosis type 1 (NF1) is a frequent autosomal dominant genetic disorder that predisposes to the development of benign and malignant tumors. Mutation of the NF1 gene affects the RAS-MAPK signaling pathway and leads to a dysfunction in cell proliferation and induces tumor development. Epidemiology of cancer in children with NF1 is very different from the general pediatric population, which requires regular and specific monitoring. Neurofibroma is the most frequent benign tumor. It can be very invalidating depending on the size and location of the tumor. Currently, there is no specific treatment for these tumors. The most frequent malignancies in children with NF1 are leukemias, rhabdomyosarcomas, malignant peripheral nerve sheath tumors and gliomas. The treatment of these tumors should consider the risk of second cancers induced by radio- and chemotherapy. We report on the case of a 5-year-old boy with NF1 developing two tumors., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Introduction: Paraneoplastic nephrotic syndrome associated with malignant broncho-pulmonary tumors. Three cases and literature review. Malignant broncho-pulmonary tumours are the principal cause of a paraneoplastic nephrotic syndrome. These tumours are most often small cell or squamous bronchial carcinomas. In 75% of cases the nephrotic syndrome is due to a membranous glomerulonephritis and it usually precedes the discovery of the causative tumour., Cases Report: Three cases of paraneoploastic nephrotic syndrome associated with bronchial carcinoma are described. The first is of a bronchial carcinoid tumour associated with a membrano-proliferative glomerulonephritis. In the second a bronchial adenocarcinoma was revealed by a membranous glomerulonephritis. The last case concerns a squamous carcinoma associated with a nephrotic syndrome in which the histological lesions were not documented on account of progression to rapidly fatal acute renal failure., Conclusion: A revue of the literature describes the associations between paraneoplastic nephrotic syndrome and bronchial carcinoma.

A 58 year-old woman developed an acute renal failure very quickly after ingestion of two 500 mg tablets of ciprofloxacin, without any other identifiable risk factor. Renal biopsy was performed. No sign of acute interstitial nephritis was observed but tubular lesions were found, accompanied by deposits of a brown-yellowish substance identified by infrared microscopy as a ciprofloxacin salt. The outcome was favourable. This observation gives the opportunity to remind the different forms of quinolone-induced renal injury and to discuss the possible ways for preventing renal side-effects related to the quinolone use.

Clear cell carcinoma has been described in numerous anatomic sites. Renal location is the most frequent. The occurrence in the gallbladder is exceptional. We report the case of a 71-Year-old woman who presented with sub-acute angiocrolitis. Computer tomographic scan revealed a polypoid mass close to the neck of the gallbladder; there was no renal lesion. Histological analysis of the gallbladder showed a primitive clear cell carcinoma with a papillary pattern associated with carcinoma in situ. Immunohistochemical study confirmed the primitive character of the tumor, characterized by an expression of KL-1, EMA and ACE and an absence of vimentin, CD 10 and CD15. Clear cell carcinomas of the gallbladder are uncommon neoplasms which could only be diagnosed on clinical, histological and immunohistochemical arguments.

Background: Malignant blue nevus is a very rare tumor. Argyrophilic nucleolar organizer regions (AgNORs) have been reported to be both a diagnostic and prognostic clue in various tumors, especially if standardized using an image analysis systems., Patients and Methods: Seven cases of malignant blue nevus were retrospectively recorded between 1974 and 1999, and their clinical and pathological features described. Using an image analysis system, AgNOR measurements were studied in all cases. These results were compared with those obtained in 10 cases of commun blue nevus, 10 cases of cellular blue nevus and 10 cases of malignant melanoma., Results: The most frequent location of malignant blue nevus was the scalp. Clinically, the tumor generally consisted of a blue nodule, 2.5 cm in diameter. There was no single histopathological criterion for the diagnosis of malignant blue nevus. AgNOR measurement was significantly higher in malignant blue nevus in comparison with commun blue nevus (p<0.0004) or cellular blue nevus (p<0.012), whereas there was no difference between malignant blue naevus and malignant melanoma (p > 0.50)., Discussion: Our results confirm the severe prognosis of malignant blue nevus and highlight the necessity of removing all blue tumours located on the scalp. AgNOR measurement using an image analysis system appears to be a useful tool for the diagnosis of malignant blue nevus, but further studies remain necessary.

A 26-year old female developed acute fulminant amoebic colitis during the post-partum period, successfully managed by subtotal colonic resection without anastomosis. Fulminant transmural amoebic colitis is a rare life-threatening complication of invasive bowel amebiasis. Pregnancy, delivery, diabetes mellitus and immunodeficiency are the main risk factors. At pathologic examination, bowel wall necrosis can be seen with amoebae present in the lumen of capillary vessels. The diagnosis of amoebic colonic perforation is difficult, especially in a non-endemic area. Conservative surgical management is required in non-perforated forms. If perforated, the bowel must be resected, limited to macroscopic lesions.