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Objectives The receptor for advanced glycation end-products (RAGE) is a membranous immunoglobulin involved in the pathogenesis of numerous autoimmune diseases and tumors. The aim of this study was to investigate the possible involvement of RAGE in the pathogenesis of myasthenia gravis. Material and methods This prospective study included 41 cases of myasthenia gravis treated at our institution between 2010 and 2015. There were 18 men and 23 women, with an average age of 36.44 ± 14.47 years. The majority of patients (24.4%) were classified as IIb, according to MGFA scoring, and 21 of them required corticosteroid and/or immunosuppressive treatment. Assessment of RAGE in thymus specimens was done by immunohistochemistry using RAGE antibody (C-term). RAGE expression was assessed according to various clinical, paraclinical and pathological parameters. Results Histopathological studies found 18 thymomas, 17 hyperplasias and six other types of pathology. Expression of RAGE was negative/weak in 19 cases and moderate/strong in 22 cases. It was more important in thymoma type B2 ( P P = 0.04), and was not significantly related to either myasthenia clinical severity or preoperative treatment. Conclusion Our results suggest that the RAGE pathway is involved in myasthenia gravis pathophysiology, especially at disease onset, and in forms with thymomas. Further studies would be indispensable to explore other aspects of this signaling pathway, especially the potential role of different ligands and soluble forms of RAGE.

Introduction Le seminome spermatocytaire est une tumeur rare, representant moins de 1% des tumeurs malignes du testicule. C’est une tumeur germinale consideree comme une entite a part, avec ses specificites histogenetiques, cliniques, anatomopathologiques et pronostiques. Le diagnostic est histopathologique.

Introduction La neurofibromatose 1 (NF1) ou maladie de von Recklinghausen est une maladie genetique caracterisee par une grande variabilite de son expression clinique. Le diagnostic est le plus souvent clinique. Nous presentons un cas revele par une enorme tumeur maligne des gaines des nerfs peripheriques ou MPNST (malignant peripheral nerve sheath tumor).

Small-cell carcinoma affecting the anal canal is an extremely rare and aggressive tumor. The majority of cases are found with distant metastases at the time of diagnosis. We present a case of a 53-year-old male, who was diagnosed with this rare malignancy. Despite aggressive treatment consisting of radio-chemotherapy, the disease progressed rapidly with metastases occurring 12 months following the initial diagnosis. In summary, final diagnosis requires careful histological and immunohistochemical examination to determine the therapeutic strategy to be followed. Chemotherapy remains the mainstay of treatment for small-cell carcinoma of the anus. Radiotherapy exerts additional activity and remains a prime choice to gain local control. In this report we aim to describe the clinical, pathological features of this rare entity, and to discuss therapeutic options.

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.

RésuméIntroductionLes pseudokystes géants de la surrénale sont exceptionnels. A travers une nouvelle observation, nous en décrivons les aspects épidémiologiques diagnostiques et thérapeutiques.ObservationUn patient de 52ans, sans antécédents notables, rapportait depuis 15jours des douleurs à l'hypochondre gauche, des vomissements bilieux, et une altération de l’état général. L'examen clinique révélait une volumineuse masse de l'hypochondre gauche. L’échographie et la tomodensitométrie abdominales montraient une énorme lésion kystique intersplénorénale, refoulant les organes voisins. Le bilan biologique surrénalien était normal et la sérologie hydatique négative. La laparotomie avait confirmé la nature kystique de la masse. L'analyse histopathologique de la paroi du kyste n'a montré ni épithélium; ni endothélium, mais un infiltrat inflammatoire polymorphe.ConclusionLe pseudokyste géant surrénalien observé était symptômatique et non fonctionnel. Le diagnostic de certitude en a été histopathologique, après exérèse chirurgicale.AbstractIntroductionAdrenal pseudocysts are rare benign lesions. Mostly, these are small lesions discovered incidentally by imaging. The giant forms are rare. Through a new observation, we describe the different epidemiological, clinical, pathological features and treatment of this entity.ObservationA patient of 52 years old, without notable medical history, suffered left flank pain associated with bilious vomiting of 15 days duration. Abdominal examination revealed a voluminous mass at the left hypochondrium. Imaging revealed a huge heterogeneous mass, located between the spleen and kidney, compressing adjacent organs. Given the diagnostic difficulty, a laparotomy was undertaken to excise the mass. Histological examination revealed a giant benign adrenal pseudocyst.CommentGiant adrenal pseudocysts are very rare. Clinical and radiological signs are not specific. Only pathological study can confirm the diagnosis and exclude malignancy. The treatment is based on surgery for large or symptomatic cysts.

Infantile digital fibromatosis or inclusion body fibromatosis is a rare, benign fibroproliferative lesion with recurrent potential that occurs on the digits of infants. A highly characteristic morphologic finding is the presence of paranuclear inclusion within the tumoral cells. We report here a case occurring in an 8-month-old infant with 2 asynchronous lesions of the toes.