Expression of receptor for advanced glycation end-products (RAGE) in thymus from myasthenia patients.

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<0.001) and when the duration of myasthenia was short (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.

[Retroperitoneal leiomyoma: a case report]. / Léiomyome rétro-péritonéal: à propos d'un cas.

Retroperitoneal leiomyoma is a rare benign tumor of the retroperitoneum. We report a clinical case of a 43-year-old patient, who suffered from back pain and weight loss. Imaging revealed a retroperitoneal mass, then the patient had a total excision of the tumor. Histological examination of the surgical specimen concluded to retroperitoneal leiomyoma. The evolution was good without recurrence after 12 months.

Primary omental gastrointestinal stromal tumors.

Gastrointestinal stromal tumors (GISTs) represent the majority of primary non-epithelial neoplasms of the digestive tract, most frequently expressing the KIT protein detected by immunohistochemical staining for the CD117 antigen. Extragastrointestinal stromal tumors (EGISTs), neoplasms with immunohistological features overlapping those of GISTs, are found in the abdomen outside of the gastrointestinal tract with no connection to the gastric or intestinal wall. The present report presents the clinical, macroscopic and immunohistological features of an EGIST arising in the greater omentum of a 63-year-old woman, and discusses the clinical behavior and prognostic factors of such lesions in comparison to their gastrointestinal counterparts.

[Pulmonary alveolar microlithiasis: report of four cases]. / La microlithiase alvéolaire pulmonaire: à propos de quatre cas.

INTRODUCTION: Pulmonary alveolar microlithiasis is a rare disease characterised by the formation and deposition of calcium phosphate microliths in the lung. It is an autosomal recessive disorder, for which mutation in the SLC34A2 gene was recently found to be responsible for the disease. OBSERVATIONS: We report on four cases of pulmonary alveolar microlithiasis. Three patients were asymptomatic. The diagnosis was made after histological confirmation in three patients. The outcome was marked by the death of one patient. CONCLUSION: Pulmonary alveolar microlithiasis is a rare disease. Diagnosis is made with high-resolution computed tomography, which exhibits the calcic character and distribution of the lesions, thus avoiding the need to perform lung biopsy. We suggest that a literature review be performed.

[Duodenal angiodysplasia: an unusual cause of intestinal obstruction]. / Angiodysplasie duodénale: cause inhabituelle d'occlusion intestinale.

Angiodysplasia is a relatively rare lesion that however may be a major source of upper and lower gastrointestinal bleeding. It's mostly related to the aging and degeneration of the blood vessels, as it occurs in older adults. Clinical presentation is variable, ranging from asymptomatic cases over iron deficiency anaemia to acute or recurrent bleeding. We present a case of 60 year-old woman presented with anaemia and intestinal obstruction. Diagnosis can usually be made using endoscopy, sometimes with additional biopsy. Treatment can be symptomatic, including iron supplements and transfusion therapy or causal, including therapeutic endoscopy (laser, electrocautery, heater probe or injection sclerotherapy), therapeutic angiography and surgery.

[Endobronchial schwannoma in a child. A case report]. / Schwannome endobronchique chez l'enfant. A propos d'un cas.

Intrathoracic neurogenic tumors are rare and interest primarily the mediastinum. Their endobronchial localization is exceptional, a fortiori in the child. The authors report a case of endobronchial benign schwannoma in a 11 year-old-child treated by pneumonectomy.

[Biphasic sarcomatoid carcinoma of the thyroid: an exceptional localization of a rare tumor]. / Carcinome sarcomatoïde biphasique de la thyroïde: localisation exceptionnelle d'une tumeur rare.

Thyroid sarcomatoid carcinoma is a rare and aggressive neoplasm composed of a follicular carcinoma which is contiguous or admixed with a pleomorphic spindle cell component. We report the case of a thyroid tumor reputed to have a poor outcome, in a 62-year-old woman. The radical thyroidectomy specimen was totally invaded. Results of immunoperoxidase staining for thyroglobulin and epithelial markers were positive in the areas of follicular carcinoma and negative in the sarcomatous component. The patient died a few days later due to septic shock. The epithelial and mesenchymal components of thyroid carcinosarcoma were both part of the neoplastic parenchyma and evolved from a single common stem cell, in agreement with the hypothesis that the tumors are of monoclonal origin. The definition of this tumor as its histogenesis and prognostic are discussed.

[An unusual localization of a rare tumor: giant-cell tumor of the rib. Case report and review of the literature]. / Une localisation inhabituelle d'une pathologie rare: la tumeur costale à cellules géantes. A propos d'un cas avec revue de la littérature.

INTRODUCTION: Giant-cell tumors of bone are rare primary neoplasms commonly encountered in young adults. Women are slightly more affected than men. CASE REPORT: We report the case a 27 year old woman presenting with a twelve months history of painful and progressively growing thoracic mass in the right anterior chest wall. Physical examination found out a fixed thoracic mass in the right retro-mammary area measuring 8 x 6 centimeters. The overlying skin was normal. Chest roentgenogram demonstrated a large ill defined mass continuing the anterior arc of the fourth right rib. Computed tomography evidenced a well defined pathologic process originating from the fourth right rib without expansion of the surrounding soft tissue. Pulmonary functional tests were normal and other complementary investigations evidenced no abnormalities. Our patient first had a fine needle cytological biopsy that brought strong suspicion of Giant-cell tumor of the rib. She then underwent an "en bloc" resection of the tumor whose histopathologic analysis allowed a definitive diagnosis. The post-surgical follow up during 12 months showed no signs of tumor recurrence. CONCLUSION: Through this observation the authors emphasize not only the rarity of the giant-cell tumors of bone but also its unusual costal localization (few cases reported till date). They focus on the importance of precocious screening and treatment and underline the value of the follow up in order to detect timely any sign of local recurrence or sarcomatous transformation. Finally, they report a current review of the literature.

[Intimal sarcoma of the inferior vena cava]. / Sarcome intimal de la veine cave inférieure.

INTRODUCTION: Primary sarcomas of the great vessels, that is, the aorta, pulmonary artery, and inferior vena cava, are rare. They can be classified according to the location of the sarcoma in the vessel wall and by their gross appearance. Most often they are leiomyosarcomas or fibrosarcomas. CASE: We report here a case of an intimal sarcoma of the inferior vena cava. Histological and immunohistochemical findings confirmed the diagnosis for this 17-year-old girl and distinguished it from leiomyosarcoma and angiosarcoma, both of which have better prognoses. DISCUSSION: Intimal sarcoma of the inferior vena cava is rare and difficult to diagnose before surgery or biopsy. Histologically, it is a poorly differentiated tumor with the worst prognosis among the primary vascular sarcomas. Pathologic findings and immunohistochemical staining are useful for a positive diagnosis.

[Hepatic alveolar hydatidosis in Morocco]. / Hydatidose alvéolaire hépatique au Maroc.

This report describes a case of hepatic alveolar echinococcosis. To our knowledge it is the first documented report of human alveolar echinococcosis in Morocco. Alveolar echinococcosis of the liver is a relatively rare, severe chronic parasitic disease. It is characterized by slow-growing cysts that progressively invade and destroy the liver parenchyma like liver cancer. The discussion presents an update of the clinical, histologic, and therapeutic features of alveolar echinococcosis based on the findings in this case report and a review of the literature.

[An unusual diagnosis of post-traumatic hemothorax]. / Hémothorax post-traumatique révélant un mésothéliome pleural malin.

We report the case of malignant pleural mesothelioma revealed by a post-traumatic hemothorax.

[Intra-abdominal desmoplastic small round cell tumor. A case report]. / Tumeur desmoplastique intra-abdominale à petites cellules rondes. A propos d'un cas.

Intra-abdominal desmoplastic small round cell tumor is an extremely rare and aggressive neoplasm that predominantly occurs in young adult men; it has an uncertain histogenesis, and predominant or exclusive intra-abdominal localisation without visceral origin involvement. This tumor is characterized by its distinct morphology and its multiphenotypic differentiation. We report a case of an intra-abdominal desmoplastic round cell tumor in a 24 years old woman. In the light of this case, the clinical morphological immunohistochemical and molecular of this rare desease are revewed.

[Fibrous tumor of the pleura]. / Tumeur fibreuse localisée de la plèvre.

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.

[Two cases of malignant tumors of the inferior vena cava]. / Deux cas de tumeurs malignes de la veine cave inférieure.

The malignant tumors of the inferior vena cava are rare. Their prognosis is bad. We report two cases of a 17-year-old and 46-year-old woman presenting the one an intimal sarcoma of the inferior vena cava and the other a metastatic of adenocarcinoma whose primary tumor was not identified. The aortic wall was invaded in both patients. The ureter repulsed in first case, was invaded in second case. The treatment consisted on resection of the tumor including the aortic wall with vein closure in both patients, with right nephrectomy in second patient. In the two cases, a prosthetic reconstruction of the arterial integrity was attempted with aortobiiliac bypass. The two patients died after relapse tumorous to the 6th month in first patient and by multisystem organ failure 5th day post-operative in second. Through these two personal cases, we try to point out the difficult problem of diagnosis that put these tumors and their bad prognosis despite an improvement of treatment.

Hydatidose alveolaire hepatique au Maroc

This report describes a case of hep atic alveolar echinococcosis. To our knowledge it is the first documented rep o rt of human alveolar echinococcosis in Morocco. Alveolar echinococcosis of the liver is a relatively rare; severe chronic parasitic disease. It is ch a ra c t e ri zed by slow - growing cysts that progre s s ive ly invade and destroy the liver pare n chyma like liver cancer. The discussion presents an update of the clinical; histologic; and therapeutic features of alveolar echinococcosis based on the findings in this case report and a review of the literature

[Malignant adrenocortical tumour with inferior vena cava invasion]. / Le corticosurrénalome avec envahissement de la veine cave inférieure.

Carcinoma of the adrenal cortex is a rare tumour. The incidence of vena cava involvement may be present in 15 to 20% of patients. The intra caval tumour thrombus can attain the right atrium. Even if some authors consider these lesions as a metastasis, long-term survival can be obtained after radical resection. The surgical tactical depend on the extension of thrombus into the vena cava inferior. The authors report a case with 4 years survival without recurrence after surgical treatment.